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BMC Pulmonary Medicine
Published in: BMC Pulmonary Medicine 1/2020

01-12-2020 | Multiple Myeloma | Case report

Severe but reversible pulmonary hypertension in scleromyxedema and multiple myeloma: a case report

Authors: Mazen Kreidy, Ali Al-Hilli, Ralph Yachoui, Jeffrey Resnick

Published in: BMC Pulmonary Medicine | Issue 1/2020

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Abstract

Background

Scleromyxedema is a progressive, systemic connective tissue disorder characterized by fibro-mucous skin lesions and increased serum monoclonal immunoglobulin levels. Pulmonary involvement occurs in a subset of patients, though the overall prevalence of pulmonary lesions in scleromyxedema is unknown. Since pulmonary hypertension presumably occurs in these patients due to disease progression and development of additional conditions, treatment of the underlying plasma cell dyscrasia and connective tissue disorder may improve pulmonary hypertension symptoms.

Case presentation

An elderly patient with scleromyxedema developed pulmonary hypertension refractory to vasodilator and diuretic therapy and subsequently multiple myeloma that responded to a combination therapy of bortezomib, cyclophosphamide, and dexamethasone treatment.

Conclusions

Treatment of the underlying disease(s) that contributed to pulmonary hypertension development with anti-neoplastic agents like bortezomib may improve cardiopulmonary symptoms secondary to reducing abnormal blood cell counts and paraprotein levels.
Literature
1.
McCarthy JT, Osserman E, Lombardo PC, Takatsuki K. An abnormal serum globulin in lichen myxedematosus. Arch Dermatol. 1964;89:446–50.PubMedCrossRef
2.
Gabriel SE, Perry HO, Oleson GB, Bowles CA. Scleromyxedema: a scleroderma-like disorder with systemic manifestations. Medicine (Baltimore). 1988;67(1):58–65.CrossRef
3.
4.
Cokonis Georgakis CD, Falasca G, Georgakis A, Heymann WR. Scleromyxedema. Clin Dermatol 2006;24(6):493–7.
5.
Blum M, Wigley FM, Hummers LK. Scleromyxedema: a case series highlighting long-term outcomes of treatment with intravenous immunoglobulin (IVIG). Medicine (Baltimore). 2008;87(1):10–20.CrossRef
6.
Rongioletti F, Merlo G, Cinotti E, Fausti V, Cozzani E, Cribier B, et al. Scleromyxedema: a multicenter study of characteristics, comorbidities, course, and therapy in 30 patients. J Am Acad Dermatol. 2013;69(1):66–72.PubMedCrossRef
7.
Rongioletti F, Merlo G, Carli C, Cribier B, Metze D, Calonje E, et al. Histopathologic characteristics of scleromyxedema: a study of a series of 34 cases. J Am Acad Dermatol. 2016;74(6):1194–200.PubMedCrossRef
8.
9.
Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L. Cutaneous manifestations of scleroderma and scleroderma-like disorders: a comprehensive review. Clin Rev Allergy Immunol. 2017;53(3):306–36.PubMedCrossRef
10.
Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, et al. European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, part 2: Scleromyxedema, scleredema and nephrogenic s ystemic fibrosis. J Eur Acad Dermatol Venereol. 2017;31(9):1581–94.PubMedCrossRef
11.
Rongioletti F. Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease. Semin Cutan Med Surg. 2006;25:100.PubMedCrossRef
12.
Alberts AS, Schulz EJ, Falkson G, Simson IW, Coccia-Portugal MA. Normalization of skin appearance in a patient with scleromyxoedema after intensive chemotherapy for Hodgkin's disease. Dermatol. 1989;178(4):221–4.CrossRef
13.
Giménez Garcia R, Garcia SG, Suarez Vilela D, Moro Sanchez MJ. Scleromyxedema associated with non-Hodgkin lymphoma. Int J Dermatol. 1989;28(10):670–1.PubMedCrossRef
14.
Godby A, Bergstresser PR, Chaker B, Pandya AG. Fatal scleromyxedema: report of a case and review of the literature. J Am Acad Dermatol. 1998;38(2 Pt 2):289–94.PubMedCrossRef
15.
Loggini B, Pingitore R, Avvenente A, Giuliano G, Barachini P. Lichen myxedematosus with systemic involvement: clinical and autopsy findings. J Am Acad Dermatol. 2001;45(4):606–8.PubMedCrossRef
16.
De Simone C, Castriota M, Carbone A, Marini Bettolo P, Pieroni M, Rongioletti F. Cardiomyopathy in scleromyxedema: Report of a fatal case. Eur J Dermatol. 2010;20(6):852–3.PubMed
17.
Fett NM, Toporcer MB, Dalmau J, Shinohara MM, Vogl DT. Scleromyxedema and dermato–neuro syndrome in a patient with multiple myeloma effectively treated with dexamethasone and bortezomib. Am J Hematol. 2011;86(10):893–6.PubMedCrossRef
18.
Sala AC, Cunha PR, Pinto CA, Alves CA, Paiva IB, Araujo AP. Scleromyxedema: clinical diagnosis and autopsy findings. An Bras Dermatol. 2016;91(5 suppl 1):48–50.PubMedPubMedCentralCrossRef
19.
Muldrow ML, Bailin PL. Scleromyxedema associated with IgG lambda multiple myeloma. Cleve Clin Q. 1983 Summer;50(2):189–95.PubMedCrossRef
20.
Yeung CK, Loong F, Kwong YL. Scleromyxoedema due to a plasma cell neoplasm: rapid remission with bortezomib, thalidomide and dexamethasone. Br J Haematol. 2012;157(4):411.PubMedCrossRef
21.
Bos R, de Waal EG, Kuiper H, Hazenberg BP, Vellenga E. Thalidomide and dexamethasone followed by autologous stem cell transplantation for scleromyxoedema. Rheumatol (Oxf). 2011;50(10):1925–6.CrossRef
22.
Cañueto J, Labrador J, Román C, Santos-Briz A, Contreras T, et al. The combination of bortezomib and dexamethasone is an efficient therapy for relapsed/refractory scleromyxedema: A rare disease with new clinical insights. Eur J Haematol. 2012;88(5):450–4.PubMedCrossRef
23.
Migkou M, Gkotzamanidou M, Terpos E, Dimopoulos MA, Kastritis E. Response to bortezomib of a patient with scleromyxedema refractory to other therapies. Leuk Res. 2011;35(11):e209–11.PubMedCrossRef
24.
Yaqub S, Moder KG, Lacy MQ. Severe, reversible pulmonary hypertension in a patient with monoclonal gammopathy and features of dermatomyositis. Mayo Clin Proc. 2004;79(5):687–9.PubMedCrossRef
25.
Feyereisn WL, Fenstad ER, McCully RB, Lacy MQ. Severe reversible pulmonary hypertension in smoldering multiple myeloma: two cases and review of the literature. Pulm Circ. 2015;5(1):211–6.PubMedPubMedCentralCrossRef
26.
Rather PA, Hussain M, Bagdadi F. Localized cutaneous mucinosis associated with multiple myeloma: A rare presentation. Indian J Dermatol. 2014;59(4):422.PubMedPubMedCentralCrossRef
27.
Truhan AP, Roenigk HH Jr. Lichen myxedematosus. An unusual case with rapid progression and possible internal involvement. Int J Dermatol. 1987;26(2):91–5.PubMedCrossRef
28.
Kuldeep CM, Mittal AK, Gupta LK, Paliwal VK, Sharma P, Garg A. Successful treatment of scleromyxedema with dexamethasone cyclophosphamide pulse therapy. Indian J Dermatol Venereol Leprol. 2005;71(1):44–5.PubMedCrossRef
29.
Feasel AM, Donato ML, Duvic M. Complete remission of scleromyxedema following autologous stem cell transplantation. Arch Dermatol. 2001;137:1071.PubMed
30.
Ataergin S, Arpaci F, Demiriz M, Ozet A. Transient efficacy of double high-dose chemotherapy and autologous peripheral stem cell transplantation, immunoglobulin, thalidomide, and bortezomib in the treatment of scleromyxedema. Am J Clin Dermatol. 2008;9(4):271–3.PubMedCrossRef
31.
Rayson D, Lust JA, Duncan A, Su WP. Scleromyxedema: A complete response to prednisone. Mayo Clin Proc. 1999 May;74(5):481–4.PubMedCrossRef
32.
33.
Lin YC, Wang HC, Shen JL. Scleromyxedema: an experience using treatment with systemic corticosteroid and review of the published work. J Dermatol. 2006 Mar;33(3):207–10.PubMedCrossRef
34.
Kreuter A, Altmeyer P. High-dose dexamethasone in scleromyxedema: report of 2 additional cases. J Am Acad Dermatol. 2005 Oct;53(4):739–40.PubMedCrossRef
35.
Horn KB, Horn MA, Swan J, Singhal S, Guitart J. A complete and durable clinical response to high-dose dexamethasone in a patient with scleromyxedema. J Am Acad Dermatol. 2004 Aug;51(2 Suppl):S120–3.PubMedCrossRef
36.
Lacy MQ, Hogan WJ, Gertz MA, Dispenzieri A, Rajkumar SV, Hayman S, et al. Successful treatment of scleromyxedema with autologous peripheral blood stem cell transplantation. Arch Dermatol. 2005 Oct;141(10):1277–82.PubMedCrossRef
37.
MacFarlane AW, Davenport A, Verbov JL, Goldsmith HJ. Scleromyxoedema—successful treatment with plasma exchange and immunosuppression. Br J Dermatol. 1987;117:653–7.PubMedCrossRef
38.
Keong CH, Asaka Y, Fukuro S, Miyamoto C, Katsumata M, Iino Y, et al. Successful treatment of scleromyxedema with plasmapheresis and immunosuppression. J Am Acad Dermatol. 1990 May;22(5 Pt 1):842–4.PubMedCrossRef
39.
Hill TG, Crawford JN, Rogers CC. Successful management of lichen myxedematosus. Rep Case Arch Dermatol. 1976 Jan;112(1):67–9.CrossRef
40.
Morris-Jones R, Staughton RC, Walker M, Sheridan DJ, Rajappan K, Leonard J, et al. Lichen myxoedematosus with associated cardiac abnormalities. Br J Dermatol. 2001;144(3):594–6.PubMedCrossRef
41.
Bidier M, Zschoche C, Gholam P, Enk AH, Hadaschik EN. Scleromyxoedema: clinical follow-up after successful treatment with high-dose immunoglobulins reveals different long-term outcomes. Acta Derm Venereol. 2012;92(4):408–9.PubMedCrossRef
42.
Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. The joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Respir J. 2015;46(4):1855–6.CrossRef
43.
Marvin KS, Spellberg RD. Pulmonary hypertension secondary to thrombocytosis in a patient with myeloid metaplasia. Chest. 1993;103(2):642–4.PubMedCrossRef
44.
45.
Rossoff LJ, Genovese J, Coleman M, Dantzker DR. Primary pulmonary hypertension in a patient with CD8/T-cell large granulocyte leukemia: amelioration by cladribine therapy. Chest. 1997;112(2):551–3.PubMedCrossRef
46.
García-Manero G, Schuster SJ, Patrick H, Martinez J. Pulmonary hypertension in patients with myelofibrosis secondary to myeloproliferative diseases. Am J Hematol. 1999;60(2):130–5.PubMedCrossRef
47.
Dingli D, Utz JP, Krowka MJ, Oberg AL, Tefferi A. Unexplained pulmonary hypertension in chronic myeloproliferative disorders. Chest. 2001;120(3):801–8.PubMedCrossRef
48.
Steensma DP, Hook CC, Stafford SL, Tefferi A. Low-dose, single-fraction, whole-lung radiotherapy for pulmonary hypertension associated with myelofibrosis with myeloid metaplasia. Br J Haematol. 2002;118(3):813–6.PubMedCrossRef
49.
Popat U, Frost A, Liu E, May R, Bag R, Reddy V, et al. New onset of myelofibrosis in association with pulmonary arterial hypertension. Ann Intern Med. 2005;143(6):466–7.PubMedCrossRef
50.
Rostagno C, Ciolli S. Transient severe symptomatic pulmonary hypertension as onset symptom in multiple myeloma. Ann Hematol. 2006;85(9):627–8.PubMedCrossRef
51.
Cortelezzi A, Gritti G, Del Papa N, Pasquini MC, Calori R, Gianelli U, et al. Pulmonary arterial hypertension in primary myelofibrosis is common and associated with an altered angiogenic status. Leukemia. 2008;22(3):646–9.PubMedCrossRef
52.
Willems E, Canivet JL, Ghaye B, de Leval L, Radermecker M, et al. Pulmonary veno-occlusive disease in myeloproliferative disorder. Eur Respir J. 2009;33(1):213–6.PubMedCrossRef
53.
Adir Y, Elia D, Harari S. Pulmonary hypertension in patients with chronic myeloproliferative disorders. Eur Respir Rev. 2015;24(137):400–10.PubMedCrossRef
54.
55.
Roach EC, Park MM, Tang WH, Thomas JD, Asosingh K, Kalaycio M, et al. Impaired right ventricular-pulmonary vascular function in myeloproliferative neoplasms. J Heart Lung Transplant. 2015;34(3):390–4.PubMedCrossRef
56.
Mattar MM, Morad MA, El Husseiny NM, Ali NH, El Demerdash DM. Correlation between JAK2 allele burden and pulmonary arterial hypertension and hematological parameters in Philadelphia negative JAK2 positive myeloproliferative neoplasms. Ann Hematol. 2016;95(10):1611–6.PubMedCrossRef
57.
Montani D, Achouh L, Marcelin AG, Viard JP, Hermine O, Canioni D, et al. Reversibility of pulmonary arterial hypertension in HIV/HHV8-associated Castleman's disease. Eur Respir J. 2005 Nov;26(5):969–72.PubMedCrossRef
58.
Taytawat P. Reversible pulmonary hypertension and isolated right heart failure associated with multiple myeloma. Chest. 2013;144(4_MeetingAbstracts):155A.CrossRef
59.
Tamura S, Koyama A, Shiotani C, Kurihara T, Nishikawa A, Okamoto Y, et al. Successful bortezomib/dexamethasone induction therapy with lenalidomide in an elderly patient with primary plasma cell leukemia complicated by renal failure and pulmonary hypertension. Intern Med. 2014;53(11):1171–5.PubMedCrossRef
60.
Warsame R, Kohut IE, Dispenzieri A. Successful use of cyclophosphamide, bortezomib, and dexamethasone to treat a case of relapsed POEMS. Eur J Haematol. 2012;88(6):549–50.PubMedCrossRef
61.
Adam Z, Krejčí M, Pour L, Feit J, Büchler T, Hájek R. IgA pemphigus associated with monoclonal gammopathy completely resolved after achievement of complete remission of multiple myeloma with bortezomib, cyclophosphamide and dexamethasone regimen. Wien Klin Wochenschr. 2010;122(9–10):311–4.PubMedCrossRef
62.
Chinen K, Fujioka Y. Severe pulmonary hypertension caused by smoldering plasma cell myeloma: an autopsy case of POEMS syndrome. Case Rep Med. 2012:836893.
63.
Fox A, Farr G, Grinnan D. Improvement in pulmonary arterial hypertension associated with Castleman's disease after treatment with ambrisentan and rituximab. Chest. 2015 Oct;148(4 Supp):994A.CrossRef
64.
Miyakoshi S, Kami M, Yuji K, Matsumura T, Takatoku M, Sasaki M, et al. Severe pulmonary complications in Japanese patients after bortezomib treatment for refractory multiple myeloma. Blood. 2006;107(9):3492–4.PubMedCrossRef
65.
Boyer JE, Batra RB, Ascensao JL, Schechter GP. Severe pulmonary complication after bortezomib treatment for multiple myeloma. Blood. 2006;108(3):1113.PubMedCrossRef
66.
Gotoh A, Ohyashiki K, Oshimi K, Usui N, Hotta T, et al. Lung injury associated with bortezomib therapy in relapsed/refractory multiple myeloma in Japan: a questionnaire-based report from the "lung injury by Bortezomib" joint committee of the Japanese Society of Hematology and the Japanese Society of Clinical Hematology. Int J Hematol. 2006;84(5):406–12.PubMedCrossRef
67.
Ohri A, Arena FP. Severe pulmonary complications in African-American patient after bortezomib therapy. Am J Ther. 2006;13(6):553–5.PubMedCrossRef
68.
Chew E, Filshie R, Wei A. Development of fatal bortezomib induced acute lung injury despite concurrent therapy with high-dose dexamethasone. Leuk Lymphoma. 2007;48(1):212–3.PubMedCrossRef
69.
Duek A, Feldberg E, Haran M, Berrebi A. Pulmonary fibrosis in a myeloma patient on bortezomib treatment. A new severe adverse effect of a new drug. Am J Hematol. 2007;82(6):502–3.PubMedCrossRef
70.
Pitini V, Arrigo C, Altavilla G, Naro C. Severe pulmonary complications after bortezomib treatment for multiple myeloma: an unrecognized pulmonary vasculitis? Leuk Res. 2007;31(7):1027–8.PubMedCrossRef
71.
Zappasodi P, Dore R, Castagnola C, Astori C, Varettoni M, et al. Rapid response to high-dose steroids of severe bortezomib-related pulmonary complication in multiple myeloma. J Clin Oncol. 2007;25(22):3380–1.PubMedCrossRef
72.
Lafaras C, Mandala E, Verrou E, Platogiannis D, Barbetakis N, et al. Non-thromboembolic pulmonary hypertension in multiple myeloma, after thalidomide treatment: a pilot study. Ann Oncol. 2008;19(10):1765–9.PubMedCrossRef
73.
Ghose A, Tariq Z, Taj A, Chaudhary R. Acute dyspnea from treatment of AL amyloidosis with bortezomib. Am J Ther. 2011;18(4):e123–5.PubMedCrossRef
74.
Montani D, Bergot E, Günther S, Savale L, Bergeron A, Bourdin A, et al. Pulmonary arterial hypertension in patients treated by dasatinib. Circ. 2012;125(17):2128–37.CrossRef
75.
Akosman C, Ordu C, Eroglu E, Oyan B. Development of acute pulmonary hypertension after bortezomib treatment in a patient with multiple myeloma: a case report and the review of the literature. Am J Ther. 2015;22(3):e88–92.PubMedCrossRef
76.
Sugita Y, Ohwada C, Nagao Y, Kawajiri C, Shimizu R, Togasaki E, et al. Early-onset severe diffuse alveolar hemorrhage after bortezomib administration suggestive of pulmonary involvement of myeloma cells. J Clin Exp Hematop. 2015;55(3):163–8.PubMedCrossRef
77.
Ranchoux B, Günther S, Quarck R, Chaumais MC, Dorfmüller P, Antigny F, et al. Chemotherapy-induced pulmonary hypertension: role of alkylating agents. Am J Pathol. 2015;185(2):356–71.PubMedCrossRef
78.
Dumitrescu D, Seck C, ten Freyhaus H, Gerhardt F, Erdmann E, Rosenkranz S. Fully reversible pulmonary arterial hypertension associated with dasatinib treatment for chronic myeloid leukaemia. Eur Respir J. 2011 Jul;38(1):218–20.PubMedCrossRef
79.
Meka S, Otoupalova E, Patel V, Cichewiz A, Dalal B. Rituximab: Friend or foe for PAH? 2016 Oct;150(4 Supp):1215A.
80.
Montrone D, Correale M, Franzese MG, Ieva R, Di Biase M, Capalbo SF. Pulmonary arterial hypertension in a chronic lymphocytic leukemia patient in treatment with rituximab. J Cardiovasc Med (Hagerstown). 2015 Jan;16(Suppl 1):S65.CrossRef
81.
Spiekerkoetter E, Sung YK, Sudheendra D, Bill M, Aldred MA, van de Veerdonk MC, et al. Low-dose FK506 (Tacrolimus) in end-stage pulmonary arterial hypertension. Am J Respir Crit Care Med. 2015 Jul 15;192(2):254–7.PubMedPubMedCentralCrossRef
82.
Spiekerkoetter E, Tian X, Cai J, Hopper RK, Sudheendra D, Li CG, et al. FK506 activates BMPR2, rescues endothelial dysfunction, and reverses pulmonary hypertension. J Clin Invest. 2013 Aug;123(8):3600–13.PubMedPubMedCentralCrossRef
83.
Schermuly RT, Dony E, Ghofrani HA, Pullamsetti S, Savai R, Roth M, et al. Reversal of experimental pulmonary hypertension by PDGF inhibition. J Clin Invest. 2005 Oct;115(10):2811–21.PubMedPubMedCentralCrossRef
84.
Kamata Y, Nara H, Sato H, Masuyama JI, Minota S, Yoshio T. Effect of steroid pulse therapy on mixed connective tissue disease with pulmonary arterial hypertension. Ann Rheum Dis. 2005 Aug;64(8):1236–7.PubMedPubMedCentralCrossRef
85.
Harbaum L, Hennigs JK, Baumann HJ, Bokemeyer C, Olschewski H, Klose H. Complete resolution of idiopathic pulmonary arterial hypertension following chemotherapy. Eur Respir J. 2014 May;43(5):1513–5.PubMedCrossRef
86.
Ferri C, Emdin M, Storino FA, Giuggioli D, Longombardo G, Greco F, et al. Isolated pulmonary hypertension in diffuse cutaneous systemic sclerosis successfully treated with long-term plasma exchange. Scand J Rheumatol. 2000;29(3):198–200.PubMedCrossRef
87.
Meloche J, Renard S, Provencher S, Bonnet S. Anti-inflammatory and immunosuppressive agents in PAH. Handb Exp Pharmacol. 2013;218:437–76.PubMedCrossRef
88.
Ogawa A, Nakamura K, Mizoguchi H, Fujii N, Fujio H, Kusano KF, et al. Prednisolone ameliorates idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med. 2011 Jan 1;183(1):139–40.PubMedCrossRef
89.
Bellotto F, Chiavacci P, Laveder F, Angelini A, Thiene G, Marcolongo R. Effective immunosuppressive therapy in a patient with primary pulmonary hypertension. Thorax. 1999 Apr;54(4):372–4.PubMedPubMedCentralCrossRef
90.
Aggarwal M. Grady RM1. Glucocorticoids for treating paediatric pulmonary hypertension: a novel use for a common medication. Cardiol Young. 2017 Sep;27(7):1410–2.PubMedCrossRef
91.
Haritha J, Syed H, Nandan A, Grinnan D. Report shows rituximab may help treat MCTD-associated PAH. Rheumatologist. 2018 April 26.
92.
Hennigan S, Channick RN, Silverman GJ. Rituximab treatment of pulmonary arterial hypertension associated with systemic lupus erythematosus: a case report. Lupus. 2008 Aug;17(8):754–6.PubMedCrossRef
93.
Padilla-Ibarra J, Sanchez-Ortiz A, Sandoval-Castro C, Ramos-Remus C. Rituximab treatment for pulmonary arterial hypertension in adult-onset Still's disease. Clin Exp Rheumatol. 2013 Jul-Aug;31(4):657–8.PubMed
94.
Dandel M, Wallukat G, Englert A, Hetzer R. Immunoadsorption therapy for dilated cardiomyopathy and pulmonary arterial hypertension. Atheroscler Suppl. 2013 Jan;14(1):203–11.PubMedCrossRef
95.
Nagel C, Ewert R, Egenlauf B, Lehmkuhl HB, Rosenkranz S, Benjamin N, et al. Safety and efficacy of immunoadsorption as an add-on to medical treatment in patients with severe idiopathic pulmonary arterial hypertension. Respir. 2017;94(3):263–71.CrossRef
96.
Wang W, Wang YL, Chen XY, Li YT, Hao W, Jin YP, et al. Dexamethasone attenuates development of monocrotaline-induced pulmonary arterial hypertension. Mol Biol Rep. 2011 Jun;38(5):3277–84.PubMedCrossRef
97.
Price LC, Montani D, Tcherakian C, Dorfmüller P, Souza R, Gambaryan N, et al. Dexamethasone reverses monocrotaline-induced pulmonary arterial hypertension in rats. Eur Respir J. 2011 Apr;37(4):813–22.PubMedCrossRef
98.
Kim SY, Lee JH, Huh JW, Kim HJ, Park MK, et al. Bortezomib alleviates experimental pulmonary arterial hypertension. Am J Respir Cell Mol Biol. 2012;47(5):698–708.PubMedCrossRef
99.
Wang YY, Luan Y, Zhang X, Lin M, Zhang ZH, et al. Proteasome inhibitor PS-341 attenuates flow-induced pulmonary arterial hypertension. Clin Exp Med. 2014;14(3):321–9.PubMedCrossRef
100.
Zhang X, Wang ZS, Luan Y, Lin M, Zhu XB, Ma Y, et al. The effect of PS-341 on pulmonary vascular remodeling in high blood flow-induced pulmonary hypertension. Int J Mol Med. 2014;33(1):105–10.PubMedCrossRef
101.
Zhang J, Lu W, Chen Y, Jiang Q, Yang K1, Li M, et al. Bortezomib alleviates experimental pulmonary hypertension by regulating intracellular calcium homeostasis in PASMCs. Am J Physiol Cell Physiol 2016;311(3):C482–C497.PubMedPubMedCentralCrossRef
102.
Zhu Y, Wu Y, Shi W, Wang J, Yan X, Wang Q, et al. Inhibition of ubiquitin proteasome function prevents monocrotaline-induced pulmonary arterial remodeling. Life Sci. 2017;173:36–42.PubMedCrossRef
103.
Ibrahim YF, Wong CM, Pavlickova L, Liu L, Trasar L, et al. Mechanism of the susceptibility of remodeled pulmonary vessels to drug-induced cell killing. J Am Heart Assoc. 2014;3(1):e000520.PubMedPubMedCentralCrossRef
104.
Rongioletti F, Cattarini G, Sottofattori E, Rebora A. Granulomatous reaction after intradermal injections of hyaluronic acid gel. Arch Dermatol. 2003 Jun;139(6):815–6.PubMedCrossRef
105.
Price LC, Wort SJ, Perros F, Dorfmüller P, Huertas A, Montani D, et al. Inflammation in pulmonary arterial hypertension. Chest. 2012 Jan;141(1):210–21.PubMedCrossRef
106.
Yuan JX, Rubin LJ. Pathogenesis of pulmonary arterial hypertension: the need for multiple hits. Circ. 2005 Feb 8;111(5):534–8.CrossRef
107.
Boucherat O, Vitry G, Trinh I, Paulin R, Provencher S, Bonnet S. The cancer theory of pulmonary arterial hypertension. Pulm Circ. 2017 Apr-Jun;7(2):285–99.PubMedPubMedCentralCrossRef
108.
Tamby MC, Chanseaud Y, Humbert M, Fermanian J, Guilpain P, Garcia-de-la-Peña-Lefebvre P, et al. Anti-endothelial cell antibodies in idiopathic and systemic sclerosis associated pulmonary arterial hypertension. Thorax. 2005 Sep;60(9):765–72.PubMedPubMedCentralCrossRef
109.
Tamby MC, Humbert M, Guilpain P, Servettaz A, Dupin N, Christner JJ, et al. Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension. Eur Respir J. 2006 Oct;28(4):799–807.PubMedCrossRef
110.
Guignabert C, Tu L, Le Hiress M, Ricard N, Sattler C, Seferian A, et al. Pathogenesis of pulmonary arterial hypertension: lessons from cancer. Eur Respir Rev. 2013 Dec;22(130):543–51.PubMedCrossRef
111.
Stacher E, Graham BB, Hunt JM, Gandjeva A, Groshong SD, McLaughlin VV, et al. Modern age pathology of pulmonary arterial hypertension. Am J Respir Crit Care Med. 2012 Aug 1;186(3):261–72.PubMedPubMedCentralCrossRef
112.
Kawamura N, Tsutsui H, Fukuyama K, Hayashidani S, Koike G, Egashira K, et al. Severe pulmonary hypertension in a patient with systemic lupus erythematosus and minimal lupus activity. Intern Med. 2002 Feb;41(2):109–12.PubMedCrossRef
113.
Mukerjee D, St George D, Coleiro B, Knight C, Denton CP, Davar J, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003 Nov;62(11):1088–93.PubMedPubMedCentralCrossRef
114.
Bordley JE, Carey RA, et al. Preliminary observations on the effect of adrenocorticotropic hormone in allergic diseases. Bull Johns Hopkins Hosp. 1949 Nov;85(5):396–8.PubMed
115.
116.
117.
Richardson PG, Weller E, Lonial S, Jakubowiak AJ, Jagannath S, Raje NS, et al. Lenalidomide, bortezomib, and dexamethasone combination therapy in patients with newly diagnosed multiple myeloma. Blood. 2010 Aug 5;116(5):679–86.PubMedPubMedCentralCrossRef
118.
Lub S, Maes K, Menu E, De Bruyne E, Vanderkerken K, Van Valckenborgh E. Novel strategies to target the ubiquitin proteasome system in multiple myeloma. Oncotarget. 2016;7(6):6521–37.PubMedCrossRef
Metadata
Title
Severe but reversible pulmonary hypertension in scleromyxedema and multiple myeloma: a case report
Authors
Mazen Kreidy
Ali Al-Hilli
Ralph Yachoui
Jeffrey Resnick
Publication date
01-12-2020
Publisher
BioMed Central
Published in
BMC Pulmonary Medicine / Issue 1/2020
Electronic ISSN: 1471-2466
DOI
https://doi.org/10.1186/s12890-019-1020-6

Other articles of this Issue 1/2020

BMC Pulmonary Medicine 1/2020 Go to the issue

Correction

Correction to: Assessment of stigma in patients with cystic fibrosis

Research article

Development of chronic lung impairment in Mozambican TB patients and associated risks

Research article

Smoking is associated with pneumonia development in lung cancer patients

Research article

Determinants of empirical antipseudomonal antibiotic prescription for adults with pneumonia in the emergency department

Research article

Effect of Dexmedetomidine on duration of mechanical ventilation in septic patients: a systematic review and meta-analysis

Study protocol

A phase Ib/IIa, randomised, double-blind, multicentre trial to assess the safety and efficacy of expanded Cx611 allogeneic adipose-derived stem cells (eASCs) for the treatment of patients with community-acquired bacterial pneumonia admitted to the intensive care unit
Obesity Clinical Trial Summary

At a glance: The STEP trials

Read more

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

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Pediatric Cardiology Video

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

Cardiopulmonary Comorbidity Video

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Valvular Heart Disease Video

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Heart Failure Video

Watch this official video from ACC.24. Dr. Biykem Bozkurt discuss last year's major advances in heart failure and cardiomyopathies.

ACC 2024 Congress Coverage

Heart Failure Video

Year in Review: Heart failure and cardiomyopathies

Watch now

Watch this official video from ACC.24. Dr. Biykem Bozkurt discuss last year's major advances in heart failure and cardiomyopathies.

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

16-04-2024 Type 2 Diabetes News

Incentivised to exercise

11-04-2024 Lifestyle Modifications News

New intervention for STEMI-related cardiogenic shock

10-04-2024 Myocardial Infarction News

» View more highlights on the ACC 2024 congress hub page

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