Skip to main content
Top
Published in: Clinical Reviews in Allergy & Immunology 2/2011

01-04-2011

Scleroderma Lung Disease

Authors: Jérôme Le Pavec, David Launay, Stephen C. Mathai, Paul M. Hassoun, Marc Humbert

Published in: Clinical Reviews in Allergy & Immunology | Issue 2/2011

Login to get access

Abstract

Pulmonary involvement is second in frequency only to esophageal involvement as a visceral complication of systemic sclerosis (SSc) and has surpassed renal involvement as the most common cause of death. Interstitial lung disease and pulmonary vascular disease, particularly pulmonary arterial hypertension, are the most commonly encountered types of lung involvement. Chronic aspiration, airway disease, neuromuscular weakness, extrinsic pulmonary restrictive pathology, pleural effusions, pneumothorax, and lung cancer cause clinically significant disease and occur commonly enough to be routinely considered in the assessment of the SSc patient with respiratory symptoms. Affected patients have a significantly worse prognosis than patients with SSc who are free of pulmonary involvement.
Literature
1.
go back to reference Jimenez SA, Derk CT (2004) Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis. Ann Intern Med 140:37–50PubMed Jimenez SA, Derk CT (2004) Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis. Ann Intern Med 140:37–50PubMed
2.
go back to reference Tan FK (2003) Systemic sclerosis: the susceptible host (genetics and environment). Rheum Dis Clin North Am 29:211–237PubMed Tan FK (2003) Systemic sclerosis: the susceptible host (genetics and environment). Rheum Dis Clin North Am 29:211–237PubMed
3.
go back to reference LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr, Rowell N, Wollheim F (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15:202–205PubMed LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr, Rowell N, Wollheim F (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15:202–205PubMed
4.
go back to reference Silver RM (1996) Scleroderma. Clinical problems. The lungs. Rheum Dis Clin North Am 22:825–840PubMed Silver RM (1996) Scleroderma. Clinical problems. The lungs. Rheum Dis Clin North Am 22:825–840PubMed
5.
go back to reference Steen VD, Medsger TA (2007) Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 66:940–944PubMed Steen VD, Medsger TA (2007) Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 66:940–944PubMed
6.
go back to reference Diot E, Boissinot E, Asquier E, Guilmot JL, Lemarie E, Valat C, Diot P (1998) Relationship between abnormalities on high-resolution ct and pulmonary function in systemic sclerosis. Chest 114:1623–1629PubMed Diot E, Boissinot E, Asquier E, Guilmot JL, Lemarie E, Valat C, Diot P (1998) Relationship between abnormalities on high-resolution ct and pulmonary function in systemic sclerosis. Chest 114:1623–1629PubMed
7.
go back to reference Wells AU, Rubens MB, du Bois RM, Hansell DM (1993) Serial CT in fibrosing alveolitis: prognostic significance of the initial pattern. AJR Am J Roentgenol 161:1159–1165PubMed Wells AU, Rubens MB, du Bois RM, Hansell DM (1993) Serial CT in fibrosing alveolitis: prognostic significance of the initial pattern. AJR Am J Roentgenol 161:1159–1165PubMed
8.
go back to reference Ostojic P, Damjanov N (2006) Different clinical features in patients with limited and diffuse cutaneous systemic sclerosis. Clin Rheumatol 25:453–457PubMed Ostojic P, Damjanov N (2006) Different clinical features in patients with limited and diffuse cutaneous systemic sclerosis. Clin Rheumatol 25:453–457PubMed
9.
go back to reference Toya SP, Tzelepis GE (2009) The many faces of scleroderma sine scleroderma: a literature review focusing on cardiopulmonary complications. Rheumatol Int 29:861–868PubMed Toya SP, Tzelepis GE (2009) The many faces of scleroderma sine scleroderma: a literature review focusing on cardiopulmonary complications. Rheumatol Int 29:861–868PubMed
10.
go back to reference Morelli S, Barbieri C, Sgreccia A, Ferrante L, Pittoni V, Conti F, Gualdi G, Polettini E, Carlesimo OA, Calvieri S (1997) Relationship between cutaneous and pulmonary involvement in systemic sclerosis. J Rheumatol 24:81–85PubMed Morelli S, Barbieri C, Sgreccia A, Ferrante L, Pittoni V, Conti F, Gualdi G, Polettini E, Carlesimo OA, Calvieri S (1997) Relationship between cutaneous and pulmonary involvement in systemic sclerosis. J Rheumatol 24:81–85PubMed
11.
go back to reference McNearney TA, Reveille JD, Fischbach M, Friedman AW, Lisse JR, Goel N, Tan FK, Zhou X, Ahn C, Feghali-Bostwick CA et al (2007) Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Rheum 57:318–326PubMed McNearney TA, Reveille JD, Fischbach M, Friedman AW, Lisse JR, Goel N, Tan FK, Zhou X, Ahn C, Feghali-Bostwick CA et al (2007) Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Rheum 57:318–326PubMed
12.
go back to reference Kane GC, Varga J, Conant EF, Spirn PW, Jimenez S, Fish JE (1996) Lung involvement in systemic sclerosis (scleroderma): relation to classification based on extent of skin involvement or autoantibody status. Respir Med 90:223–230PubMed Kane GC, Varga J, Conant EF, Spirn PW, Jimenez S, Fish JE (1996) Lung involvement in systemic sclerosis (scleroderma): relation to classification based on extent of skin involvement or autoantibody status. Respir Med 90:223–230PubMed
13.
go back to reference Alton E (1988) Turner-Warwick M. Lung involvement in scleroderma. Black, CM, Jayson MIV, Wiley, Chichester Alton E (1988) Turner-Warwick M. Lung involvement in scleroderma. Black, CM, Jayson MIV, Wiley, Chichester
14.
go back to reference Greidinger EL, Flaherty KT, White B, Rosen A, Wigley FM, Wise RA (1998) African-American race and antibodies to topoisomerase I are associated with increased severity of scleroderma lung disease. Chest 114:801–807PubMed Greidinger EL, Flaherty KT, White B, Rosen A, Wigley FM, Wise RA (1998) African-American race and antibodies to topoisomerase I are associated with increased severity of scleroderma lung disease. Chest 114:801–807PubMed
15.
go back to reference Okano Y, Steen VD, Medsger TA Jr (1992) Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis. Arthritis Rheum 35:95–100PubMed Okano Y, Steen VD, Medsger TA Jr (1992) Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis. Arthritis Rheum 35:95–100PubMed
16.
go back to reference Sacks DG, Okano Y, Steen VD, Curtiss E, Shapiro LS, Medsger TA Jr (1996) Isolated pulmonary hypertension in systemic sclerosis with diffuse cutaneous involvement: association with serum anti-U3rnp antibody. J Rheumatol 23:639–642PubMed Sacks DG, Okano Y, Steen VD, Curtiss E, Shapiro LS, Medsger TA Jr (1996) Isolated pulmonary hypertension in systemic sclerosis with diffuse cutaneous involvement: association with serum anti-U3rnp antibody. J Rheumatol 23:639–642PubMed
17.
go back to reference Steen VD (2005) Autoantibodies in systemic sclerosis. Semin Arthritis Rheum 35:35–42PubMed Steen VD (2005) Autoantibodies in systemic sclerosis. Semin Arthritis Rheum 35:35–42PubMed
18.
go back to reference Steen VD, Powell DL, Medsger TA Jr (1988) Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum 31:196–203PubMed Steen VD, Powell DL, Medsger TA Jr (1988) Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum 31:196–203PubMed
19.
go back to reference Wallace DJ, Lin HC, Shen GQ, Peter JB (1994) Antibodies to histone (h2a–h2b)–DNA complexes in the absence of antibodies to double-stranded DNA or to (h2a–h2b) complexes are more sensitive and specific for scleroderma-related disorders than for lupus. Arthritis Rheum 37:1795–1797PubMed Wallace DJ, Lin HC, Shen GQ, Peter JB (1994) Antibodies to histone (h2a–h2b)–DNA complexes in the absence of antibodies to double-stranded DNA or to (h2a–h2b) complexes are more sensitive and specific for scleroderma-related disorders than for lupus. Arthritis Rheum 37:1795–1797PubMed
20.
go back to reference Fischer A, Swigris JJ, Groshong SD, Cool CD, Sahin H, Lynch DA, Curran-Everett D, Gillis JZ, Meehan RT, Brown KK (2008) Clinically significant interstitial lung disease in limited scleroderma: histopathology, clinical features, and survival. Chest 134:601–605PubMed Fischer A, Swigris JJ, Groshong SD, Cool CD, Sahin H, Lynch DA, Curran-Everett D, Gillis JZ, Meehan RT, Brown KK (2008) Clinically significant interstitial lung disease in limited scleroderma: histopathology, clinical features, and survival. Chest 134:601–605PubMed
21.
go back to reference Wells AU, Hansell DM, Rubens MB, King AD, Cramer D, Black CM, du Bois RM (1997) Fibrosing alveolitis in systemic sclerosis: indices of lung function in relation to extent of disease on computed tomography. Arthritis Rheum 40:1229–1236PubMed Wells AU, Hansell DM, Rubens MB, King AD, Cramer D, Black CM, du Bois RM (1997) Fibrosing alveolitis in systemic sclerosis: indices of lung function in relation to extent of disease on computed tomography. Arthritis Rheum 40:1229–1236PubMed
22.
go back to reference Bouros D, Wells AU, Nicholson AG, Colby TV, Polychronopoulos V, Pantelidis P, Haslam PL, Vassilakis DA, Black CM, du Bois RM (2002) Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 165:1581–1586PubMed Bouros D, Wells AU, Nicholson AG, Colby TV, Polychronopoulos V, Pantelidis P, Haslam PL, Vassilakis DA, Black CM, du Bois RM (2002) Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 165:1581–1586PubMed
23.
go back to reference Kim EA, Lee KS, Johkoh T, Kim TS, Suh GY, Kwon OJ, Han J (2002) Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. Radiographics 22 Spec No:S151–S165 Kim EA, Lee KS, Johkoh T, Kim TS, Suh GY, Kwon OJ, Han J (2002) Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. Radiographics 22 Spec No:S151–S165
24.
go back to reference Launay D, Remy-Jardin M, Michon-Pasturel U, Mastora I, Hachulla E, Lambert M, Delannoy V, Queyrel V, Duhamel A, Matran R et al (2006) High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis. J Rheumatol 33:1789–1801PubMed Launay D, Remy-Jardin M, Michon-Pasturel U, Mastora I, Hachulla E, Lambert M, Delannoy V, Queyrel V, Duhamel A, Matran R et al (2006) High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis. J Rheumatol 33:1789–1801PubMed
25.
go back to reference Goldin JG, Lynch DA, Strollo DC, Suh RD, Schraufnagel DE, Clements PJ, Elashoff RM, Furst DE, Vasunilashorn S, McNitt-Gray MF et al (2008) High-resolution ct scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest 134:358–367PubMed Goldin JG, Lynch DA, Strollo DC, Suh RD, Schraufnagel DE, Clements PJ, Elashoff RM, Furst DE, Vasunilashorn S, McNitt-Gray MF et al (2008) High-resolution ct scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest 134:358–367PubMed
26.
go back to reference Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM, Corte TJ, Sander CR, Ratoff J, Devaraj A et al (2008) Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med 177:1248–1254PubMed Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM, Corte TJ, Sander CR, Ratoff J, Devaraj A et al (2008) Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med 177:1248–1254PubMed
27.
go back to reference Fujita J, Yoshinouchi T, Ohtsuki Y, Tokuda M, Yang Y, Yamadori I, Bandoh S, Ishida T, Takahara J, Ueda R (2001) Non-specific interstitial pneumonia as pulmonary involvement of systemic sclerosis. Ann Rheum Dis 60:281–283PubMed Fujita J, Yoshinouchi T, Ohtsuki Y, Tokuda M, Yang Y, Yamadori I, Bandoh S, Ishida T, Takahara J, Ueda R (2001) Non-specific interstitial pneumonia as pulmonary involvement of systemic sclerosis. Ann Rheum Dis 60:281–283PubMed
28.
go back to reference Nakamura Y, Chida K, Suda T, Hayakawa H, Iwata M, Imokawa S, Tsuchiya T, Ida M, Gemma H, Yasuda K et al (2003) Nonspecific interstitial pneumonia in collagen vascular diseases: comparison of the clinical characteristics and prognostic significance with usual interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 20:235–241PubMed Nakamura Y, Chida K, Suda T, Hayakawa H, Iwata M, Imokawa S, Tsuchiya T, Ida M, Gemma H, Yasuda K et al (2003) Nonspecific interstitial pneumonia in collagen vascular diseases: comparison of the clinical characteristics and prognostic significance with usual interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 20:235–241PubMed
29.
go back to reference Tansey D, Wells AU, Colby TV, Ip S, Nikolakoupolou A, du Bois RM, Hansell DM, Nicholson AG (2004) Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology 44:585–596PubMed Tansey D, Wells AU, Colby TV, Ip S, Nikolakoupolou A, du Bois RM, Hansell DM, Nicholson AG (2004) Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology 44:585–596PubMed
30.
go back to reference al-Sabbagh MR, Steen VD, Zee BC, Nalesnik M, Trostle DC, Bedetti CD, Medsger TA Jr (1989) Pulmonary arterial histology and morphometry in systemic sclerosis: a case-control autopsy study. J Rheumatol 16:1038–1042PubMed al-Sabbagh MR, Steen VD, Zee BC, Nalesnik M, Trostle DC, Bedetti CD, Medsger TA Jr (1989) Pulmonary arterial histology and morphometry in systemic sclerosis: a case-control autopsy study. J Rheumatol 16:1038–1042PubMed
31.
go back to reference Wells AU, Hansell DM, Rubens MB, Cullinan P, Black CM, du Bois RM (1993) The predictive value of appearances on thin-section computed tomography in fibrosing alveolitis. Am Rev Respir Dis 148:1076–1082PubMed Wells AU, Hansell DM, Rubens MB, Cullinan P, Black CM, du Bois RM (1993) The predictive value of appearances on thin-section computed tomography in fibrosing alveolitis. Am Rev Respir Dis 148:1076–1082PubMed
32.
go back to reference Muller NL, Miller RR (1990) Computed tomography of chronic diffuse infiltrative lung disease. Part 1. Am Rev Respir Dis 142:1206–1215PubMed Muller NL, Miller RR (1990) Computed tomography of chronic diffuse infiltrative lung disease. Part 1. Am Rev Respir Dis 142:1206–1215PubMed
33.
go back to reference Wells AU, Hansell DM, Rubens MB, Cullinan P, Haslam PL, Black CM, Du Bois RM (1994) Fibrosing alveolitis in systemic sclerosis. Bronchoalveolar lavage findings relation to computed tomographic appearance. Am J Respir Crit Care Med 150:462–468PubMed Wells AU, Hansell DM, Rubens MB, Cullinan P, Haslam PL, Black CM, Du Bois RM (1994) Fibrosing alveolitis in systemic sclerosis. Bronchoalveolar lavage findings relation to computed tomographic appearance. Am J Respir Crit Care Med 150:462–468PubMed
34.
go back to reference Harrison NK, Glanville AR, Strickland B, Haslam PL, Corrin B, Addis BJ, Lawrence R, Millar AB, Black CM, Turner-Warwick M (1989) Pulmonary involvement in systemic sclerosis: the detection of early changes by thin section CT scan, bronchoalveolar lavage and 99mtc-dtpa clearance. Respir Med 83:403–414PubMed Harrison NK, Glanville AR, Strickland B, Haslam PL, Corrin B, Addis BJ, Lawrence R, Millar AB, Black CM, Turner-Warwick M (1989) Pulmonary involvement in systemic sclerosis: the detection of early changes by thin section CT scan, bronchoalveolar lavage and 99mtc-dtpa clearance. Respir Med 83:403–414PubMed
35.
go back to reference Bronchoalveolar lavage constituents in healthy individuals, idiopathic pulmonary fibrosis, and selected comparison groups (1990) The BAL cooperative group steering committee. Am Rev Respir Dis 141:S169–S202 Bronchoalveolar lavage constituents in healthy individuals, idiopathic pulmonary fibrosis, and selected comparison groups (1990) The BAL cooperative group steering committee. Am Rev Respir Dis 141:S169–S202
36.
go back to reference Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, Arriola E, Silver R, Strange C, Bolster M et al (2006) Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 354:2655–2666PubMed Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, Arriola E, Silver R, Strange C, Bolster M et al (2006) Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 354:2655–2666PubMed
37.
go back to reference Al-Dhaher FF, Pope JE, Ouimet JM (2008) Determinants of morbidity and mortality of systemic sclerosis in canada. Semin Arthritis Rheum Al-Dhaher FF, Pope JE, Ouimet JM (2008) Determinants of morbidity and mortality of systemic sclerosis in canada. Semin Arthritis Rheum
38.
go back to reference Altman RD, Medsger TA Jr, Bloch DA, Michel BA (1991) Predictors of survival in systemic sclerosis (scleroderma). Arthritis Rheum 34:403–413PubMed Altman RD, Medsger TA Jr, Bloch DA, Michel BA (1991) Predictors of survival in systemic sclerosis (scleroderma). Arthritis Rheum 34:403–413PubMed
39.
go back to reference Highland KB, Silver RM (2005) New developments in scleroderma interstitial lung disease. Curr Opin Rheumatol 17:737–745PubMed Highland KB, Silver RM (2005) New developments in scleroderma interstitial lung disease. Curr Opin Rheumatol 17:737–745PubMed
40.
go back to reference Steen VD, Owens GR, Medsger TA Jr (1994) Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 37:1283–1289PubMed Steen VD, Owens GR, Medsger TA Jr (1994) Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 37:1283–1289PubMed
41.
go back to reference Steen VD, Medsger TA Jr (2000) Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 43:2437–2444PubMed Steen VD, Medsger TA Jr (2000) Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 43:2437–2444PubMed
42.
go back to reference Strange C, Bolster MB, Roth MD, Silver RM, Theodore A, Goldin J, Clements P, Chung J, Elashoff RM, Suh R et al (2008) Bronchoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease. Am J Respir Crit Care Med 177:91–98PubMed Strange C, Bolster MB, Roth MD, Silver RM, Theodore A, Goldin J, Clements P, Chung J, Elashoff RM, Suh R et al (2008) Bronchoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease. Am J Respir Crit Care Med 177:91–98PubMed
43.
go back to reference Akesson A, Scheja A, Lundin A, Wollheim FA (1994) Improved pulmonary function in systemic sclerosis after treatment with cyclophosphamide. Arthritis Rheum 37:729–735PubMed Akesson A, Scheja A, Lundin A, Wollheim FA (1994) Improved pulmonary function in systemic sclerosis after treatment with cyclophosphamide. Arthritis Rheum 37:729–735PubMed
44.
go back to reference White B, Moore WC, Wigley FM, Xiao HQ, Wise RA (2000) Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis. Ann Intern Med 132:947–954PubMed White B, Moore WC, Wigley FM, Xiao HQ, Wise RA (2000) Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis. Ann Intern Med 132:947–954PubMed
45.
go back to reference Airo P, Danieli E, Parrinello G, Antonioli CM, Cavazzana I, Toniati P, Franceschini F, Cattaneo R (2004) Intravenous cyclophosphamide therapy for systemic sclerosis. A single-center experience and review of the literature with pooled analysis of lung function test results. Clin Exp Rheumatol 22:573–578PubMed Airo P, Danieli E, Parrinello G, Antonioli CM, Cavazzana I, Toniati P, Franceschini F, Cattaneo R (2004) Intravenous cyclophosphamide therapy for systemic sclerosis. A single-center experience and review of the literature with pooled analysis of lung function test results. Clin Exp Rheumatol 22:573–578PubMed
46.
go back to reference Griffiths B, Miles S, Moss H, Robertson R, Veale D, Emery P (2002) Systemic sclerosis and interstitial lung disease: a pilot study using pulse intravenous methylprednisolone and cyclophosphamide to assess the effect on high resolution computed tomography scan and lung function. J Rheumatol 29:2371–2378PubMed Griffiths B, Miles S, Moss H, Robertson R, Veale D, Emery P (2002) Systemic sclerosis and interstitial lung disease: a pilot study using pulse intravenous methylprednisolone and cyclophosphamide to assess the effect on high resolution computed tomography scan and lung function. J Rheumatol 29:2371–2378PubMed
47.
go back to reference Pakas I, Ioannidis JP, Malagari K, Skopouli FN, Moutsopoulos HM, Vlachoyiannopoulos PG (2002) Cyclophosphamide with low or high dose prednisolone for systemic sclerosis lung disease. J Rheumatol 29:298–304PubMed Pakas I, Ioannidis JP, Malagari K, Skopouli FN, Moutsopoulos HM, Vlachoyiannopoulos PG (2002) Cyclophosphamide with low or high dose prednisolone for systemic sclerosis lung disease. J Rheumatol 29:298–304PubMed
48.
go back to reference Silver RM, Warrick JH, Kinsella MB, Staudt LS, Baumann MH, Strange C (1993) Cyclophosphamide and low-dose prednisone therapy in patients with systemic sclerosis (scleroderma) with interstitial lung disease. J Rheumatol 20:838–844PubMed Silver RM, Warrick JH, Kinsella MB, Staudt LS, Baumann MH, Strange C (1993) Cyclophosphamide and low-dose prednisone therapy in patients with systemic sclerosis (scleroderma) with interstitial lung disease. J Rheumatol 20:838–844PubMed
49.
go back to reference Steen VD, Lanz JK Jr, Conte C, Owens GR, Medsger TA Jr (1994) Therapy for severe interstitial lung disease in systemic sclerosis. A retrospective study. Arthritis Rheum 37:1290–1296PubMed Steen VD, Lanz JK Jr, Conte C, Owens GR, Medsger TA Jr (1994) Therapy for severe interstitial lung disease in systemic sclerosis. A retrospective study. Arthritis Rheum 37:1290–1296PubMed
50.
go back to reference Apras S, Ertenli I, Ozbalkan Z, Kiraz S, Ozturk MA, Haznedaroglu IC, Cobankara V, Pay S, Calguneri M (2003) Effects of oral cyclophosphamide and prednisolone therapy on the endothelial functions and clinical findings in patients with early diffuse systemic sclerosis. Arthritis Rheum 48:2256–2261PubMed Apras S, Ertenli I, Ozbalkan Z, Kiraz S, Ozturk MA, Haznedaroglu IC, Cobankara V, Pay S, Calguneri M (2003) Effects of oral cyclophosphamide and prednisolone therapy on the endothelial functions and clinical findings in patients with early diffuse systemic sclerosis. Arthritis Rheum 48:2256–2261PubMed
51.
go back to reference Davas EM, Peppas C, Maragou M, Alvanou E, Hondros D, Dantis PC (1999) Intravenous cyclophosphamide pulse therapy for the treatment of lung disease associated with scleroderma. Clin Rheumatol 18:455–461PubMed Davas EM, Peppas C, Maragou M, Alvanou E, Hondros D, Dantis PC (1999) Intravenous cyclophosphamide pulse therapy for the treatment of lung disease associated with scleroderma. Clin Rheumatol 18:455–461PubMed
52.
go back to reference Martinez FJ, McCune WJ (2006) Cyclophosphamide for scleroderma lung disease. N Engl J Med 354:2707–2709PubMed Martinez FJ, McCune WJ (2006) Cyclophosphamide for scleroderma lung disease. N Engl J Med 354:2707–2709PubMed
53.
go back to reference Tashkin DP, Elashoff R, Clements PJ, Roth MD, Furst DE, Silver RM, Goldin J, Arriola E, Strange C, Bolster MB et al (2007) Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med 176:1026–1034PubMed Tashkin DP, Elashoff R, Clements PJ, Roth MD, Furst DE, Silver RM, Goldin J, Arriola E, Strange C, Bolster MB et al (2007) Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med 176:1026–1034PubMed
54.
go back to reference Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS, Roberts C, Desai S, Herrick AL, McHugh NJ et al (2006) A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum 54:3962–3970PubMed Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS, Roberts C, Desai S, Herrick AL, McHugh NJ et al (2006) A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum 54:3962–3970PubMed
55.
go back to reference Nannini C, West CP, Erwin PJ, Matteson EL (2008) Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies. Arthritis Res Ther 10:R124PubMed Nannini C, West CP, Erwin PJ, Matteson EL (2008) Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies. Arthritis Res Ther 10:R124PubMed
56.
go back to reference Berezne A, Ranque B, Valeyre D, Brauner M, Allanore Y, Launay D, Le Guern V, Kahn JE, Couderc LJ, Constans J et al (2008) Therapeutic strategy combining intravenous cyclophosphamide followed by oral azathioprine to treat worsening interstitial lung disease associated with systemic sclerosis: a retrospective multicenter open-label study. J Rheumatol 35:1064–1072PubMed Berezne A, Ranque B, Valeyre D, Brauner M, Allanore Y, Launay D, Le Guern V, Kahn JE, Couderc LJ, Constans J et al (2008) Therapeutic strategy combining intravenous cyclophosphamide followed by oral azathioprine to treat worsening interstitial lung disease associated with systemic sclerosis: a retrospective multicenter open-label study. J Rheumatol 35:1064–1072PubMed
57.
go back to reference Nadashkevich O, Davis P, Fritzler M, Kovalenko W (2006) A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis. Clin Rheumatol 25:205–212PubMed Nadashkevich O, Davis P, Fritzler M, Kovalenko W (2006) A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis. Clin Rheumatol 25:205–212PubMed
58.
go back to reference Stratton RJ, Wilson H, Black CM (2001) Pilot study of anti-thymocyte globulin plus mycophenolate mofetil in recent-onset diffuse scleroderma. Rheumatology (Oxford) 40:84–88 Stratton RJ, Wilson H, Black CM (2001) Pilot study of anti-thymocyte globulin plus mycophenolate mofetil in recent-onset diffuse scleroderma. Rheumatology (Oxford) 40:84–88
59.
go back to reference Liossis SN, Bounas A, Andonopoulos AP (2006) Mycophenolate mofetil as first-line treatment improves clinically evident early scleroderma lung disease. Rheumatology (Oxford) 45:1005–1008 Liossis SN, Bounas A, Andonopoulos AP (2006) Mycophenolate mofetil as first-line treatment improves clinically evident early scleroderma lung disease. Rheumatology (Oxford) 45:1005–1008
60.
go back to reference Swigris JJ, Olson AL, Fischer A, Lynch DA, Cosgrove GP, Frankel SK, Meehan RT, Brown KK (2006) Mycophenolate mofetil is safe, well tolerated, and preserves lung function in patients with connective tissue disease-related interstitial lung disease. Chest 130:30–36PubMed Swigris JJ, Olson AL, Fischer A, Lynch DA, Cosgrove GP, Frankel SK, Meehan RT, Brown KK (2006) Mycophenolate mofetil is safe, well tolerated, and preserves lung function in patients with connective tissue disease-related interstitial lung disease. Chest 130:30–36PubMed
61.
go back to reference Zamora AC, Wolters PJ, Collard HR, Connolly MK, Elicker BM, Webb WR, King TE Jr, Golden JA (2008) Use of mycophenolate mofetil to treat scleroderma-associated interstitial lung disease. Respir Med 102:150–155PubMed Zamora AC, Wolters PJ, Collard HR, Connolly MK, Elicker BM, Webb WR, King TE Jr, Golden JA (2008) Use of mycophenolate mofetil to treat scleroderma-associated interstitial lung disease. Respir Med 102:150–155PubMed
62.
go back to reference Gerbino AJ, Goss CH, Molitor JA (2008) Effect of mycophenolate mofetil on pulmonary function in scleroderma-associated interstitial lung disease. Chest 133:455–460PubMed Gerbino AJ, Goss CH, Molitor JA (2008) Effect of mycophenolate mofetil on pulmonary function in scleroderma-associated interstitial lung disease. Chest 133:455–460PubMed
63.
go back to reference Vonk MC, Marjanovic Z, van den Hoogen FH, Zohar S, Schattenberg AV, Fibbe WE, Larghero J, Gluckman E, Preijers FW, van Dijk AP et al (2008) Long-term follow-up results after autologous haematopoietic stem cell transplantation for severe systemic sclerosis. Ann Rheum Dis 67:98–104PubMed Vonk MC, Marjanovic Z, van den Hoogen FH, Zohar S, Schattenberg AV, Fibbe WE, Larghero J, Gluckman E, Preijers FW, van Dijk AP et al (2008) Long-term follow-up results after autologous haematopoietic stem cell transplantation for severe systemic sclerosis. Ann Rheum Dis 67:98–104PubMed
64.
go back to reference Bonner JC (2004) Regulation of PDGF and its receptors in fibrotic diseases. Cytokine Growth Factor Rev 15:255–273PubMed Bonner JC (2004) Regulation of PDGF and its receptors in fibrotic diseases. Cytokine Growth Factor Rev 15:255–273PubMed
65.
go back to reference Rosenbloom J, Jimenez SA (2008) Molecular ablation of transforming growth factor beta signaling pathways by tyrosine kinase inhibition: the coming of a promising new era in the treatment of tissue fibrosis. Arthritis Rheum 58:2219–2224PubMed Rosenbloom J, Jimenez SA (2008) Molecular ablation of transforming growth factor beta signaling pathways by tyrosine kinase inhibition: the coming of a promising new era in the treatment of tissue fibrosis. Arthritis Rheum 58:2219–2224PubMed
66.
go back to reference Rosas V, Conte JV, Yang SC, Gaine SP, Borja M, Wigley FM, White B, Orens JB (2000) Lung transplantation and systemic sclerosis. Ann Transplant 5:38–43PubMed Rosas V, Conte JV, Yang SC, Gaine SP, Borja M, Wigley FM, White B, Orens JB (2000) Lung transplantation and systemic sclerosis. Ann Transplant 5:38–43PubMed
67.
go back to reference Massad MG, Powell CR, Kpodonu J, Tshibaka C, Hanhan Z, Snow NJ, Geha AS (2005) Outcomes of lung transplantation in patients with scleroderma. World J Surg 29:1510–1515PubMed Massad MG, Powell CR, Kpodonu J, Tshibaka C, Hanhan Z, Snow NJ, Geha AS (2005) Outcomes of lung transplantation in patients with scleroderma. World J Surg 29:1510–1515PubMed
68.
go back to reference Rubin LJ (1997) Primary pulmonary hypertension. N Engl J Med 336:111–117PubMed Rubin LJ (1997) Primary pulmonary hypertension. N Engl J Med 336:111–117PubMed
69.
go back to reference Chang B, Wigley FM, White B, Wise RA (2003) Scleroderma patients with combined pulmonary hypertension and interstitial lung disease. J Rheumatol 30:2398–2405PubMed Chang B, Wigley FM, White B, Wise RA (2003) Scleroderma patients with combined pulmonary hypertension and interstitial lung disease. J Rheumatol 30:2398–2405PubMed
70.
go back to reference MacGregor AJ, Canavan R, Knight C, Denton CP, Davar J, Coghlan J, Black CM (2001) Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology (Oxford) 40:453–459 MacGregor AJ, Canavan R, Knight C, Denton CP, Davar J, Coghlan J, Black CM (2001) Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology (Oxford) 40:453–459
71.
go back to reference Murata I, Takenaka K, Yoshinoya S, Kikuchi K, Kiuchi T, Tanigawa T, Ito K (1997) Clinical evaluation of pulmonary hypertension in systemic sclerosis and related disorders. A Doppler echocardiographic study of 135 Japanese patients. Chest 111:36–43PubMed Murata I, Takenaka K, Yoshinoya S, Kikuchi K, Kiuchi T, Tanigawa T, Ito K (1997) Clinical evaluation of pulmonary hypertension in systemic sclerosis and related disorders. A Doppler echocardiographic study of 135 Japanese patients. Chest 111:36–43PubMed
72.
go back to reference Wigley FM, Lima JA, Mayes M, McLain D, Chapin JL, Ward-Able C (2005) The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of community-based rheumatologists (the uncover study). Arthritis Rheum 52:2125–2132PubMed Wigley FM, Lima JA, Mayes M, McLain D, Chapin JL, Ward-Able C (2005) The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of community-based rheumatologists (the uncover study). Arthritis Rheum 52:2125–2132PubMed
73.
go back to reference Pope JE, Lee P, Baron M, Dunne J, Smith D, Docherty PS, Bookman A, Abu-Hakima M (2005) Prevalence of elevated pulmonary arterial pressures measured by echocardiography in a multicenter study of patients with systemic sclerosis. J Rheumatol 32:1273–1278PubMed Pope JE, Lee P, Baron M, Dunne J, Smith D, Docherty PS, Bookman A, Abu-Hakima M (2005) Prevalence of elevated pulmonary arterial pressures measured by echocardiography in a multicenter study of patients with systemic sclerosis. J Rheumatol 32:1273–1278PubMed
74.
go back to reference Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J, Kahan A, Cabane J, Frances C, Launay D et al (2005) Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 52:3792–3800PubMed Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J, Kahan A, Cabane J, Frances C, Launay D et al (2005) Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 52:3792–3800PubMed
75.
go back to reference Mukerjee D, St George D, Coleiro B, Knight C, Denton CP, Davar J, Black CM, Coghlan JG (2003) Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 62:1088–1093PubMed Mukerjee D, St George D, Coleiro B, Knight C, Denton CP, Davar J, Black CM, Coghlan JG (2003) Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 62:1088–1093PubMed
76.
go back to reference Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, Yaici A, Weitzenblum E, Cordier JF, Chabot F et al (2006) Pulmonary arterial hypertension in france: results from a national registry. Am J Respir Crit Care Med 173:1023–1030PubMed Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, Yaici A, Weitzenblum E, Cordier JF, Chabot F et al (2006) Pulmonary arterial hypertension in france: results from a national registry. Am J Respir Crit Care Med 173:1023–1030PubMed
77.
go back to reference Hachulla E, de Groote P, Gressin V, Sibilia J, Diot E, Carpentier P, Mouthon L, Hatron PY, Jego P, Allanore Y et al (2009) The 3-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France (itinérair-sclérodermie study). Arthritis Rheum 60:1831–1839PubMed Hachulla E, de Groote P, Gressin V, Sibilia J, Diot E, Carpentier P, Mouthon L, Hatron PY, Jego P, Allanore Y et al (2009) The 3-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France (itinérair-sclérodermie study). Arthritis Rheum 60:1831–1839PubMed
78.
go back to reference Steen V, Medsger TA Jr (2003) Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 48:516–522PubMed Steen V, Medsger TA Jr (2003) Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 48:516–522PubMed
79.
go back to reference Steen V (2003) Predictors of end stage lung disease in systemic sclerosis. Ann Rheum Dis 62:97–99PubMed Steen V (2003) Predictors of end stage lung disease in systemic sclerosis. Ann Rheum Dis 62:97–99PubMed
80.
go back to reference Chang B, Schachna L, White B, Wigley FM, Wise RA (2006) Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma. J Rheumatol 33:269–274PubMed Chang B, Schachna L, White B, Wigley FM, Wise RA (2006) Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma. J Rheumatol 33:269–274PubMed
81.
go back to reference Cox SR, Walker JG, Coleman M, Rischmueller M, Proudman S, Smith MD, Ahern MJ, Roberts-Thomson PJ (2005) Isolated pulmonary hypertension in scleroderma. Intern Med J 35:28–33PubMed Cox SR, Walker JG, Coleman M, Rischmueller M, Proudman S, Smith MD, Ahern MJ, Roberts-Thomson PJ (2005) Isolated pulmonary hypertension in scleroderma. Intern Med J 35:28–33PubMed
82.
go back to reference Hachulla E, Coghlan JG (2004) A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism. Ann Rheum Dis 63:1009–1014PubMed Hachulla E, Coghlan JG (2004) A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism. Ann Rheum Dis 63:1009–1014PubMed
83.
go back to reference Scorza R, Caronni M, Bazzi S, Nador F, Beretta L, Antonioli R, Origgi L, Ponti A, Marchini M, Vanoli M (2002) Post-menopause is the main risk factor for developing isolated pulmonary hypertension in systemic sclerosis. Ann N Y Acad Sci 966:238–246PubMed Scorza R, Caronni M, Bazzi S, Nador F, Beretta L, Antonioli R, Origgi L, Ponti A, Marchini M, Vanoli M (2002) Post-menopause is the main risk factor for developing isolated pulmonary hypertension in systemic sclerosis. Ann N Y Acad Sci 966:238–246PubMed
84.
go back to reference Plastiras SC, Karadimitrakis SP, Kampolis C, Moutsopoulos HM, Tzelepis GE (2007) Determinants of pulmonary arterial hypertension in scleroderma. Semin Arthritis Rheum 36:392–396PubMed Plastiras SC, Karadimitrakis SP, Kampolis C, Moutsopoulos HM, Tzelepis GE (2007) Determinants of pulmonary arterial hypertension in scleroderma. Semin Arthritis Rheum 36:392–396PubMed
85.
go back to reference Ong YY, Nikoloutsopoulos T, Bond CP, Smith MD, Ahern MJ, Roberts-Thomson PJ (1998) Decreased nailfold capillary density in limited scleroderma with pulmonary hypertension. Asian Pac J Allergy Immunol 16:81–86PubMed Ong YY, Nikoloutsopoulos T, Bond CP, Smith MD, Ahern MJ, Roberts-Thomson PJ (1998) Decreased nailfold capillary density in limited scleroderma with pulmonary hypertension. Asian Pac J Allergy Immunol 16:81–86PubMed
86.
go back to reference Lee CY, Chang SM, Hsiao SH, Tseng JC, Lin SK, Liu CP (2007) Right heart function and scleroderma: insights from tricuspid annular plane systolic excursion. Echocardiography 24:118–125PubMed Lee CY, Chang SM, Hsiao SH, Tseng JC, Lin SK, Liu CP (2007) Right heart function and scleroderma: insights from tricuspid annular plane systolic excursion. Echocardiography 24:118–125PubMed
87.
go back to reference Hsiao SH, Lee CY, Chang SM, Lin SK, Liu CP (2006) Right heart function in scleroderma: insights from myocardial Doppler tissue imaging. J Am Soc Echocardiogr 19:507–514PubMed Hsiao SH, Lee CY, Chang SM, Lin SK, Liu CP (2006) Right heart function in scleroderma: insights from myocardial Doppler tissue imaging. J Am Soc Echocardiogr 19:507–514PubMed
88.
go back to reference Meune C, Avouac J, Wahbi K, Cabanes L, Wipff J, Mouthon L, Guillevin L, Kahan A, Allanore Y (2008) Cardiac involvement in systemic sclerosis assessed by tissue-doppler echocardiography during routine care: a controlled study of 100 consecutive patients. Arthritis Rheum 58:1803–1809PubMed Meune C, Avouac J, Wahbi K, Cabanes L, Wipff J, Mouthon L, Guillevin L, Kahan A, Allanore Y (2008) Cardiac involvement in systemic sclerosis assessed by tissue-doppler echocardiography during routine care: a controlled study of 100 consecutive patients. Arthritis Rheum 58:1803–1809PubMed
89.
go back to reference Williams MH, Handler CE, Akram R, Smith CJ, Das C, Smee J, Nair D, Denton CP, Black CM, Coghlan JG (2006) Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial hypertension. Eur Heart J 27:1485–1494PubMed Williams MH, Handler CE, Akram R, Smith CJ, Das C, Smee J, Nair D, Denton CP, Black CM, Coghlan JG (2006) Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial hypertension. Eur Heart J 27:1485–1494PubMed
90.
go back to reference Williams MH, Das C, Handler CE, Akram MR, Davar J, Denton CP, Smith CJ, Black CM, Coghlan JG (2006) Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart 92:926–932PubMed Williams MH, Das C, Handler CE, Akram MR, Davar J, Denton CP, Smith CJ, Black CM, Coghlan JG (2006) Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart 92:926–932PubMed
91.
go back to reference Koh ET, Lee P, Gladman DD, Abu-Shakra M (1996) Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 35:989–993PubMed Koh ET, Lee P, Gladman DD, Abu-Shakra M (1996) Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 35:989–993PubMed
92.
go back to reference Mayes MD (2003) Scleroderma epidemiology. Rheum Dis Clin North Am 29:239–254PubMed Mayes MD (2003) Scleroderma epidemiology. Rheum Dis Clin North Am 29:239–254PubMed
93.
go back to reference Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T, Hummers L, Krishnan JA, Wigley F, Hassoun PM (2006) Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 54:3043–3050PubMed Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T, Hummers L, Krishnan JA, Wigley F, Hassoun PM (2006) Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 54:3043–3050PubMed
94.
go back to reference Girgis RE, Mathai SC, Krishnan JA, Wigley FM, Hassoun PM (2005) Long-term outcome of bosentan treatment in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with the scleroderma spectrum of diseases. J Heart Lung Transplant 24:1626–1631PubMed Girgis RE, Mathai SC, Krishnan JA, Wigley FM, Hassoun PM (2005) Long-term outcome of bosentan treatment in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with the scleroderma spectrum of diseases. J Heart Lung Transplant 24:1626–1631PubMed
95.
go back to reference Kawut SM, Taichman DB, Archer-Chicko CL, Palevsky HI, Kimmel SE (2003) Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 123:344–350PubMed Kawut SM, Taichman DB, Archer-Chicko CL, Palevsky HI, Kimmel SE (2003) Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 123:344–350PubMed
96.
go back to reference Kuhn KP, Byrne DW, Arbogast PG, Doyle TP, Loyd JE, Robbins IM (2003) Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol. Am J Respir Crit Care Med 167:580–586PubMed Kuhn KP, Byrne DW, Arbogast PG, Doyle TP, Loyd JE, Robbins IM (2003) Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol. Am J Respir Crit Care Med 167:580–586PubMed
97.
go back to reference Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, Das C, Elliot CA, Johnson M, DeSoyza J et al (2009) Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 179:151–157PubMed Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, Das C, Elliot CA, Johnson M, DeSoyza J et al (2009) Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 179:151–157PubMed
98.
go back to reference Hachulla E, Carpentier P, Gressin V, Diot E, Allanore Y, Sibilia J, Launay D, Mouthon L, Jego P, Cabane J et al (2009) Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French itinérair-sclérodermie study. Rheumatology (Oxford) 48:304–308 Hachulla E, Carpentier P, Gressin V, Diot E, Allanore Y, Sibilia J, Launay D, Mouthon L, Jego P, Cabane J et al (2009) Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French itinérair-sclérodermie study. Rheumatology (Oxford) 48:304–308
99.
go back to reference Forfia PR, Fisher MR, Mathai SC, Housten-Harris T, Hemnes AR, Borlaug BA, Chamera E, Corretti MC, Champion HC, Abraham TP et al (2006) Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 174:1034–1041PubMed Forfia PR, Fisher MR, Mathai SC, Housten-Harris T, Hemnes AR, Borlaug BA, Chamera E, Corretti MC, Champion HC, Abraham TP et al (2006) Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 174:1034–1041PubMed
100.
go back to reference Forfia PR, Mathai SC, Fisher MR, Housten-Harris T, Hemnes AR, Champion HC, Girgis RE, Hassoun PM (2008) Hyponatremia predicts right heart failure and poor survival in pulmonary arterial hypertension. Am J Respir Crit Care Med 177:1364–1369PubMed Forfia PR, Mathai SC, Fisher MR, Housten-Harris T, Hemnes AR, Champion HC, Girgis RE, Hassoun PM (2008) Hyponatremia predicts right heart failure and poor survival in pulmonary arterial hypertension. Am J Respir Crit Care Med 177:1364–1369PubMed
101.
go back to reference D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Kernis JT et al (1991) Survival in patients with primary pulmonary hypertension. Results from a national prospective study. Ann Intern Med 115:343–349PubMed D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Kernis JT et al (1991) Survival in patients with primary pulmonary hypertension. Results from a national prospective study. Ann Intern Med 115:343–349PubMed
102.
go back to reference Budhiraja R, Tuder RM, Hassoun PM (2004) Endothelial dysfunction in pulmonary hypertension. Circulation 109:159–165PubMed Budhiraja R, Tuder RM, Hassoun PM (2004) Endothelial dysfunction in pulmonary hypertension. Circulation 109:159–165PubMed
103.
go back to reference Sitbon O, Humbert M, Jais X, Ioos V, Hamid AM, Provencher S, Garcia G, Parent F, Herve P, Simonneau G (2005) Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 111:3105–3111PubMed Sitbon O, Humbert M, Jais X, Ioos V, Hamid AM, Provencher S, Garcia G, Parent F, Herve P, Simonneau G (2005) Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 111:3105–3111PubMed
104.
go back to reference Dorfmuller P, Perros F, Balabanian K, Humbert M (2003) Inflammation in pulmonary arterial hypertension. Eur Respir J 22:358–363PubMed Dorfmuller P, Perros F, Balabanian K, Humbert M (2003) Inflammation in pulmonary arterial hypertension. Eur Respir J 22:358–363PubMed
105.
go back to reference Sanchez O, Sitbon O, Jais X, Simonneau G, Humbert M (2006) Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest 130:182–189PubMed Sanchez O, Sitbon O, Jais X, Simonneau G, Humbert M (2006) Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest 130:182–189PubMed
106.
go back to reference Jais X, Launay D, Yaici A, Le Pavec J, Tcherakian C, Sitbon O, Simonneau G, Humbert M (2008) Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases. Arthritis Rheum 58:521–531PubMed Jais X, Launay D, Yaici A, Le Pavec J, Tcherakian C, Sitbon O, Simonneau G, Humbert M (2008) Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases. Arthritis Rheum 58:521–531PubMed
107.
go back to reference Vane JR, Anggard EE, Botting RM (1990) Regulatory functions of the vascular endothelium. N Engl J Med 323:27–36PubMed Vane JR, Anggard EE, Botting RM (1990) Regulatory functions of the vascular endothelium. N Engl J Med 323:27–36PubMed
108.
go back to reference Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, Groves BM, Tapson VF, Bourge RC, Brundage BH et al (1996) A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The primary pulmonary hypertension study group. N Engl J Med 334:296–302PubMed Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, Groves BM, Tapson VF, Bourge RC, Brundage BH et al (1996) A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The primary pulmonary hypertension study group. N Engl J Med 334:296–302PubMed
109.
go back to reference McLaughlin VV, Genthner DE, Panella MM, Rich S (1998) Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med 338:273–277PubMed McLaughlin VV, Genthner DE, Panella MM, Rich S (1998) Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med 338:273–277PubMed
110.
go back to reference Rubin LJ, Mendoza J, Hood M, McGoon M, Barst R, Williams WB, Diehl JH, Crow J, Long W (1990) Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med 112:485–491PubMed Rubin LJ, Mendoza J, Hood M, McGoon M, Barst R, Williams WB, Diehl JH, Crow J, Long W (1990) Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med 112:485–491PubMed
111.
go back to reference McLaughlin VV, Shillington A, Rich S (2002) Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 106:1477–1482PubMed McLaughlin VV, Shillington A, Rich S (2002) Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 106:1477–1482PubMed
112.
go back to reference Sitbon O, Humbert M, Nunes H, Parent F, Garcia G, Herve P, Rainisio M, Simonneau G (2002) Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 40:780–788PubMed Sitbon O, Humbert M, Nunes H, Parent F, Garcia G, Herve P, Rainisio M, Simonneau G (2002) Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 40:780–788PubMed
113.
go back to reference Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM, Rich S, Barst RJ, Barrett PS, Kral KM et al (2000) Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 132:425–434PubMed Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM, Rich S, Barst RJ, Barrett PS, Kral KM et al (2000) Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 132:425–434PubMed
114.
go back to reference Farber HW, Graven KK, Kokolski G, Korn JH (1999) Pulmonary edema during acute infusion of epoprostenol in a patient with pulmonary hypertension and limited scleroderma. J Rheumatol 26:1195–1196PubMed Farber HW, Graven KK, Kokolski G, Korn JH (1999) Pulmonary edema during acute infusion of epoprostenol in a patient with pulmonary hypertension and limited scleroderma. J Rheumatol 26:1195–1196PubMed
115.
go back to reference Palmer SM, Robinson LJ, Wang A, Gossage JR, Bashore T, Tapson VF (1998) Massive pulmonary edema and death after prostacyclin infusion in a patient with pulmonary veno-occlusive disease. Chest 113:237–240PubMed Palmer SM, Robinson LJ, Wang A, Gossage JR, Bashore T, Tapson VF (1998) Massive pulmonary edema and death after prostacyclin infusion in a patient with pulmonary veno-occlusive disease. Chest 113:237–240PubMed
116.
go back to reference Dorfmuller P, Humbert M, Perros F, Sanchez O, Simonneau G, Muller KM, Capron F (2007) Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases. Hum Pathol 38:893–902PubMed Dorfmuller P, Humbert M, Perros F, Sanchez O, Simonneau G, Muller KM, Capron F (2007) Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases. Hum Pathol 38:893–902PubMed
117.
go back to reference Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A, Pulido T, Frost A, Roux S, Leconte I et al (2002) Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 346:896–903PubMed Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A, Pulido T, Frost A, Roux S, Leconte I et al (2002) Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 346:896–903PubMed
118.
go back to reference Channick RN, Simonneau G, Sitbon O, Robbins IM, Frost A, Tapson VF, Badesch DB, Roux S, Rainisio M, Bodin F et al (2001) Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 358:1119–1123PubMed Channick RN, Simonneau G, Sitbon O, Robbins IM, Frost A, Tapson VF, Badesch DB, Roux S, Rainisio M, Bodin F et al (2001) Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 358:1119–1123PubMed
119.
go back to reference Hachulla AL, Launay D, Gaxotte V, de Groote P, Lamblin N, Devos P, Hatron PY, Beregi JP, Hachulla E (2008) Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients. Ann Rheum Dis Hachulla AL, Launay D, Gaxotte V, de Groote P, Lamblin N, Devos P, Hatron PY, Beregi JP, Hachulla E (2008) Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients. Ann Rheum Dis
120.
go back to reference Denton CP, Humbert M, Rubin L, Black CM (2006) Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis 65:1336–1340PubMed Denton CP, Humbert M, Rubin L, Black CM (2006) Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis 65:1336–1340PubMed
121.
go back to reference Badesch DB, Hill NS, Burgess G, Rubin LJ, Barst RJ, Galie N, Simonneau G (2007) Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol 34:2417–2422PubMed Badesch DB, Hill NS, Burgess G, Rubin LJ, Barst RJ, Galie N, Simonneau G (2007) Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol 34:2417–2422PubMed
122.
go back to reference Simonneau G, Rubin LJ, Galie N, Barst RJ, Fleming TR, Frost AE, Engel PJ, Kramer MR, Burgess G, Collings L et al (2008) Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 149:521–530PubMed Simonneau G, Rubin LJ, Galie N, Barst RJ, Fleming TR, Frost AE, Engel PJ, Kramer MR, Burgess G, Collings L et al (2008) Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 149:521–530PubMed
123.
go back to reference Mathai SC, Girgis RE, Fisher MR, Champion HC, Housten-Harris T, Zaiman A, Hassoun PM (2007) Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J 29:469–475PubMed Mathai SC, Girgis RE, Fisher MR, Champion HC, Housten-Harris T, Zaiman A, Hassoun PM (2007) Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J 29:469–475PubMed
124.
go back to reference Schachna L, Wigley FM, Chang B, White B, Wise RA, Gelber AC (2003) Age and risk of pulmonary arterial hypertension in scleroderma. Chest 124:2098–2104PubMed Schachna L, Wigley FM, Chang B, White B, Wise RA, Gelber AC (2003) Age and risk of pulmonary arterial hypertension in scleroderma. Chest 124:2098–2104PubMed
125.
go back to reference Mathai SC, Hummers L, Champion HC, Wigley F, Zaiman A, Hassoun PM, Girgis R (2009) Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: impact of interstitial lung disease. Arthritis Rheum 60:569–577PubMed Mathai SC, Hummers L, Champion HC, Wigley F, Zaiman A, Hassoun PM, Girgis R (2009) Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: impact of interstitial lung disease. Arthritis Rheum 60:569–577PubMed
126.
go back to reference Johnson DA, Drane WE, Curran J, Cattau EL Jr, Ciarleglio C, Khan A, Cotelingam J, Benjamin SB (1989) Pulmonary disease in progressive systemic sclerosis. A complication of gastroesophageal reflux and occult aspiration? Arch Intern Med 149:589–593PubMed Johnson DA, Drane WE, Curran J, Cattau EL Jr, Ciarleglio C, Khan A, Cotelingam J, Benjamin SB (1989) Pulmonary disease in progressive systemic sclerosis. A complication of gastroesophageal reflux and occult aspiration? Arch Intern Med 149:589–593PubMed
127.
go back to reference Marie I, Dominique S, Levesque H, Ducrotte P, Denis P, Hellot MF, Courtois H (2001) Esophageal involvement and pulmonary manifestations in systemic sclerosis. Arthritis Rheum 45:346–354PubMed Marie I, Dominique S, Levesque H, Ducrotte P, Denis P, Hellot MF, Courtois H (2001) Esophageal involvement and pulmonary manifestations in systemic sclerosis. Arthritis Rheum 45:346–354PubMed
128.
go back to reference Lock G, Pfeifer M, Straub RH, Zeuner M, Lang B, Scholmerich J, Holstege A (1998) Association of esophageal dysfunction and pulmonary function impairment in systemic sclerosis. Am J Gastroenterol 93:341–345PubMed Lock G, Pfeifer M, Straub RH, Zeuner M, Lang B, Scholmerich J, Holstege A (1998) Association of esophageal dysfunction and pulmonary function impairment in systemic sclerosis. Am J Gastroenterol 93:341–345PubMed
129.
go back to reference Ing AJ (2001) Interstitial lung disease and gastroesophageal reflux. Am J Med 111(Suppl 8A):41S–44SPubMed Ing AJ (2001) Interstitial lung disease and gastroesophageal reflux. Am J Med 111(Suppl 8A):41S–44SPubMed
130.
go back to reference Troshinsky MB, Kane GC, Varga J, Cater JR, Fish JE, Jimenez SA, Castell DO (1994) Pulmonary function and gastroesophageal reflux in systemic sclerosis. Ann Intern Med 121:6–10PubMed Troshinsky MB, Kane GC, Varga J, Cater JR, Fish JE, Jimenez SA, Castell DO (1994) Pulmonary function and gastroesophageal reflux in systemic sclerosis. Ann Intern Med 121:6–10PubMed
131.
go back to reference Daniels CE, Jett JR (2005) Does interstitial lung disease predispose to lung cancer? Curr Opin Pulm Med 11:431–437PubMed Daniels CE, Jett JR (2005) Does interstitial lung disease predispose to lung cancer? Curr Opin Pulm Med 11:431–437PubMed
132.
go back to reference Hazleman B (1985) Incidence of neoplasms in patients with rheumatoid arthritis exposed to different treatment regimens. Am J Med 78:39–43PubMed Hazleman B (1985) Incidence of neoplasms in patients with rheumatoid arthritis exposed to different treatment regimens. Am J Med 78:39–43PubMed
133.
go back to reference Hill CL, Nguyen AM, Roder D, Roberts-Thomson P (2003) Risk of cancer in patients with scleroderma: a population based cohort study. Ann Rheum Dis 62:728–731PubMed Hill CL, Nguyen AM, Roder D, Roberts-Thomson P (2003) Risk of cancer in patients with scleroderma: a population based cohort study. Ann Rheum Dis 62:728–731PubMed
134.
go back to reference Rosenthal AK, McLaughlin JK, Linet MS, Persson I (1993) Scleroderma and malignancy: an epidemiological study. Ann Rheum Dis 52:531–533PubMed Rosenthal AK, McLaughlin JK, Linet MS, Persson I (1993) Scleroderma and malignancy: an epidemiological study. Ann Rheum Dis 52:531–533PubMed
135.
go back to reference Yang Y, Fujita J, Tokuda M, Bandoh S, Ishida T (2001) Lung cancer associated with several connective tissue diseases: with a review of literature. Rheumatol Int 21:106–111PubMed Yang Y, Fujita J, Tokuda M, Bandoh S, Ishida T (2001) Lung cancer associated with several connective tissue diseases: with a review of literature. Rheumatol Int 21:106–111PubMed
136.
go back to reference Roumm AD, Medsger TA Jr (1985) Cancer and systemic sclerosis. An epidemiologic study. Arthritis Rheum 28:1336–1340PubMed Roumm AD, Medsger TA Jr (1985) Cancer and systemic sclerosis. An epidemiologic study. Arthritis Rheum 28:1336–1340PubMed
137.
go back to reference Chatterjee S, Dombi GW, Severson RK, Mayes MD (2005) Risk of malignancy in scleroderma: a population-based cohort study. Arthritis Rheum 52:2415–2424PubMed Chatterjee S, Dombi GW, Severson RK, Mayes MD (2005) Risk of malignancy in scleroderma: a population-based cohort study. Arthritis Rheum 52:2415–2424PubMed
138.
go back to reference Highland KB, Garin MC, Brown KK (2007) The spectrum of scleroderma lung disease. Semin Respir Crit Care Med 28:418–429PubMed Highland KB, Garin MC, Brown KK (2007) The spectrum of scleroderma lung disease. Semin Respir Crit Care Med 28:418–429PubMed
139.
go back to reference Clements PJ, Furst DE, Campion DS, Bohan A, Harris R, Levy J, Paulus HE (1978) Muscle disease in progressive systemic sclerosis: diagnostic and therapeutic considerations. Arthritis Rheum 21:62–71PubMed Clements PJ, Furst DE, Campion DS, Bohan A, Harris R, Levy J, Paulus HE (1978) Muscle disease in progressive systemic sclerosis: diagnostic and therapeutic considerations. Arthritis Rheum 21:62–71PubMed
140.
go back to reference Olsen NJ, King LE Jr, Park JH (1996) Muscle abnormalities in scleroderma. Rheum Dis Clin North Am 22:783–796PubMed Olsen NJ, King LE Jr, Park JH (1996) Muscle abnormalities in scleroderma. Rheum Dis Clin North Am 22:783–796PubMed
141.
go back to reference Abramson MJ, Barnett AJ, Littlejohn GO, Smith MM, Hall S (1991) Lung function abnormalities and decline of spirometry in scleroderma: an overrated danger? Postgrad Med J 67:632–637PubMed Abramson MJ, Barnett AJ, Littlejohn GO, Smith MM, Hall S (1991) Lung function abnormalities and decline of spirometry in scleroderma: an overrated danger? Postgrad Med J 67:632–637PubMed
142.
go back to reference Vitali C, Viegi G, Tassoni S, Tavoni A, Paoletti P, Bibolotti E, Ferri C, Bombardieri S (1986) Lung function abnormalities in different connective tissue diseases. Clin Rheumatol 5:181–188PubMed Vitali C, Viegi G, Tassoni S, Tavoni A, Paoletti P, Bibolotti E, Ferri C, Bombardieri S (1986) Lung function abnormalities in different connective tissue diseases. Clin Rheumatol 5:181–188PubMed
143.
go back to reference Blom-Bulow B, Jonson B, Brauer K (1985) Lung function in progressive systemic sclerosis is dominated by poorly compliant lungs and stiff airways. Eur J Respir Dis 66:1–8PubMed Blom-Bulow B, Jonson B, Brauer K (1985) Lung function in progressive systemic sclerosis is dominated by poorly compliant lungs and stiff airways. Eur J Respir Dis 66:1–8PubMed
144.
go back to reference Owens GR, Fino GJ, Herbert DL, Steen VD, Medsger TA Jr, Pennock BE, Cottrell JJ, Rodnan GP, Rogers RM (1983) Pulmonary function in progressive systemic sclerosis. Comparison of crest syndrome variant with diffuse scleroderma. Chest 84:546–550PubMed Owens GR, Fino GJ, Herbert DL, Steen VD, Medsger TA Jr, Pennock BE, Cottrell JJ, Rodnan GP, Rogers RM (1983) Pulmonary function in progressive systemic sclerosis. Comparison of crest syndrome variant with diffuse scleroderma. Chest 84:546–550PubMed
145.
go back to reference Bjerke RD, Tashkin DP, Clements PJ, Chopra SK, Gong H Jr, Bein M (1979) Small airways in progressive systemic sclerosis (PSS). Am J Med 66:201–209PubMed Bjerke RD, Tashkin DP, Clements PJ, Chopra SK, Gong H Jr, Bein M (1979) Small airways in progressive systemic sclerosis (PSS). Am J Med 66:201–209PubMed
146.
go back to reference Boehler A, Vogt P, Speich R, Weder W, Russi EW (1996) Bronchiolitis obliterans in a patient with localized scleroderma treated with d-penicillamine. Eur Respir J 9:1317–1319PubMed Boehler A, Vogt P, Speich R, Weder W, Russi EW (1996) Bronchiolitis obliterans in a patient with localized scleroderma treated with d-penicillamine. Eur Respir J 9:1317–1319PubMed
147.
go back to reference Thompson AE, Pope JE (1998) A study of the frequency of pericardial and pleural effusions in scleroderma. Br J Rheumatol 37:1320–1323PubMed Thompson AE, Pope JE (1998) A study of the frequency of pericardial and pleural effusions in scleroderma. Br J Rheumatol 37:1320–1323PubMed
148.
go back to reference Taormina VJ, Miller WT, Gefter WB, Epstein DM (1981) Progressive systemic sclerosis subgroups: variable pulmonary features. AJR Am J Roentgenol 137:277–285PubMed Taormina VJ, Miller WT, Gefter WB, Epstein DM (1981) Progressive systemic sclerosis subgroups: variable pulmonary features. AJR Am J Roentgenol 137:277–285PubMed
149.
go back to reference Zeuner M, Muller-Ladner U, Mohr VD, Lang B (1996) Spontaneous pneumothorax in a patient with systemic sclerosis. Clin Rheumatol 15:211–213PubMed Zeuner M, Muller-Ladner U, Mohr VD, Lang B (1996) Spontaneous pneumothorax in a patient with systemic sclerosis. Clin Rheumatol 15:211–213PubMed
150.
go back to reference Suresh K, Radha R, Ananthasubramaniam G, Chandrasekaran AN (1994) An unusual presentation of progressive systemic sclerosis. J Assoc Physicians India 42:163–164PubMed Suresh K, Radha R, Ananthasubramaniam G, Chandrasekaran AN (1994) An unusual presentation of progressive systemic sclerosis. J Assoc Physicians India 42:163–164PubMed
151.
go back to reference Ng SC, Tan WC (1990) Bilateral spontaneous pneumothorax in systemic sclerosis—report of two cases. J Rheumatol 17:689–691PubMed Ng SC, Tan WC (1990) Bilateral spontaneous pneumothorax in systemic sclerosis—report of two cases. J Rheumatol 17:689–691PubMed
152.
go back to reference Lang B, Ortlieb H, Meske S, Hauke G, Peter HH (1989) Progressive systemic sclerosis presenting with spontaneous pneumothorax. J Rheumatol 16:254–256PubMed Lang B, Ortlieb H, Meske S, Hauke G, Peter HH (1989) Progressive systemic sclerosis presenting with spontaneous pneumothorax. J Rheumatol 16:254–256PubMed
Metadata
Title
Scleroderma Lung Disease
Authors
Jérôme Le Pavec
David Launay
Stephen C. Mathai
Paul M. Hassoun
Marc Humbert
Publication date
01-04-2011
Publisher
Humana Press Inc
Published in
Clinical Reviews in Allergy & Immunology / Issue 2/2011
Print ISSN: 1080-0549
Electronic ISSN: 1559-0267
DOI
https://doi.org/10.1007/s12016-009-8194-2

Other articles of this Issue 2/2011

Clinical Reviews in Allergy & Immunology 2/2011 Go to the issue
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine