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Open Access 01-10-2013 | Original Article

Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature

Authors: Xiaoqin Yang, Jack Mardekian, Kafi N. Sanders, Marko A. Mychaskiw, Joseph Thomas III

Published in: Clinical Rheumatology | Issue 10/2013

Abstract

Pulmonary arterial hypertension (PAH) is a progressive and life-threatening disease. Understanding of PAH prevalence remains limited, but PAH has been reported as a frequent complication in connective tissue diseases. This study estimated prevalence of PAH in patients with connective tissue diseases and prevalence of idiopathic PAH using a systematic review of the literature. We searched PubMed through May 19, 2012 for all studies on prevalence of PAH in patients with connective tissue diseases or prevalence of idiopathic PAH. To be included, studies had to be in English, have humans as subjects, and determine prevalence within a time interval of up to 2 years. Studies only investigating pediatric patients were excluded. Pooled prevalence estimates were calculated. Twenty studies were identified in the review. Seventeen of the 20 studies reported prevalence of PAH in connective tissue diseases and three reported prevalence of idiopathic PAH. The pooled prevalence estimate of idiopathic PAH was 12 cases per million population (95 % CI 5 cases per million to 22 cases per million) with estimates ranging from 5.9 cases per million population to 25 cases per million population. The pooled prevalence estimate of PAH in patients with connective tissue diseases was 13 % (95 % CI, 9.18 % to 18.16 %) with reported estimates ranging from 2.8 % to 32 %. Prevalence of PAH in patients with connective tissue diseases was substantially higher than that of idiopathic PAH based on pooled prevalence estimates. Comparisons of PAH prevalence in persons with connective tissue disease and idiopathic PAH using a large observational study would be helpful in better assessing relative prevalence.

McLaughlin VV, ACCF/AHA et al (2009) Expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol 53(17):1573–1619PubMedCrossRef

Gaine SP, Rubin LJ (1998) Primary pulmonary hypertension. Lancet 352(9129):719–725PubMedCrossRef

Baughman RP, Carbone, RG, Bottino G (2009) Pulmonary arterial hypertension and interstitial lung diseases: a clinical guide. R.G.C. Robert P. Baughman, Giovanni Bottino (ed) Humana Press: New York, NY

Kimberly (2001) Primer on the rheumatic diseases. J.H. Klippel (ed) Arthritis Foundation: Atlanta, GA. p. 325–8

Trell E, Lindstrom C (1971) Pulmonary hypertension in systemic sclerosis. Ann Rheum Dis 30(4):390–400PubMedCrossRef

Perez HD, Kramer N (1981) Pulmonary hypertension in systemic lupus erythematosus: report of four cases and review of the literature. Semin Arthritis Rheum 11(1):177–181PubMedCrossRef

Sullivan WD et al (1984) A prospective evaluation emphasizing pulmonary involvement in patients with mixed connective tissue disease. Med (Baltimore) 63(2):92–107CrossRef

Wiener-Kronish JP et al (1981) Severe pulmonary involvement in mixed connective tissue disease. Am Rev Respir Dis 124(4):499–503PubMed

Galie N et al (2005) Pulmonary arterial hypertension associated to connective tissue diseases. Lupus 14(9):713–717PubMedCrossRef

10.

Peacock AJ et al (2007) An epidemiological study of pulmonary arterial hypertension. Eur Respir J 30(1):104–109PubMedCrossRef

11.

Humbert M et al (2006) Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 173(9):1023–1030PubMedCrossRef

12.

Tueller C et al (2008) Epidemiology of pulmonary hypertension: new data from the Swiss registry. Swiss Med Wkly 138(25–26):379–384PubMed

13.

Higgins JPT, Thompson SG (2002) Quantifying heterogeneity in a meta-analysis. Stat Med 21:1539-1558

14.

DerSimonian R, Laird N (1986) Meta-analysis in clinical trials. Control Clin Trials 7(3):177–188PubMedCrossRef

15.

McGoon M et al (2004) Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 126(1 Suppl):14S–34SPubMedCrossRef

16.

Denton CP et al (1997) Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary hypertension in systemic sclerosis. Br J Rheumatol 36(2):239–243PubMedCrossRef

17.

Humbert M et al (2011) Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum 63(11):3522–3530PubMedCrossRef

18.

Meune C et al (2011) Prediction of pulmonary hypertension related to systemic sclerosis by an index based on simple clinical observations. Arthritis Rheum 63(9):2790–2796PubMedCrossRef

19.

Hachulla E et al (2005) Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 52(12):3792–3800PubMedCrossRef

20.

Hachulla E et al (2009) Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinerAIR-Sclerodermie study. Rheumatology (Oxford) 48(3):304–308CrossRef

21.

Avouac J et al (2010) Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J Rheumatol 37(11):2290–2298PubMedCrossRef

22.

Vonk MC et al (2009) Systemic sclerosis and its pulmonary complications in The Netherlands: an epidemiological study. Ann Rheum Dis 68(6):961–965PubMedCrossRef

23.

Phung S et al (2009) Prevalence of pulmonary arterial hypertension in an Australian scleroderma population: screening allows for earlier diagnosis. Int Med J 39(10):682–691CrossRef

24.

de Azevedo AB et al (2005) Prevalence of pulmonary hypertension in systemic sclerosis. Clin Exp Rheumatol 23(4):447–454PubMed

25.

Coral-Alvarado P et al (2008) Risk factors associated with pulmonary arterial hypertension in Colombian patients with systemic sclerosis: review of the literature. J Rheumatol 35(2):244–250PubMed

26.

Kumar U et al (2008) Prevalence and predictors of pulmonary artery hypertension in systemic sclerosis. J Assoc Phys India 56:413–417

27.

Pope JE et al (2005) Prevalence of elevated pulmonary arterial pressures measured by echocardiography in a multicenter study of patients with systemic sclerosis. J Rheumatol 32(7):1273–1278PubMed

28.

Gindzienska-Sieskiewicz E et al (2005) The occurrence of pulmonary hypertension in patients with systemic sclerosis hospitalized in the Department of Rheumatology and Internal Diseases Medical University of Bialystok in years 2003–2004. Rocz Akad Med Bialymst 50(Suppl 1):297–300PubMed

29.

Chang B et al (2003) Scleroderma patients with combined pulmonary hypertension and interstitial lung disease. J Rheumatol 30(11):2398–2405PubMed

30.

Yamane K et al (2000) Clinical and laboratory features of scleroderma patients with pulmonary hypertension. Rheumatology (Oxford) 39(11):1269–1271CrossRef

31.

Pan TL, Thumboo J, Boey ML (2000) Primary and secondary pulmonary hypertension in systemic lupus erythematosus. Lupus 9(5):338–342PubMedCrossRef

32.

Prabu A et al (2009) Prevalence and risk factors for pulmonary arterial hypertension in patients with lupus. Rheumatology (Oxford) 48(12):1506–1511CrossRef

33.

Dawson JK et al (2000) Raised pulmonary artery pressures measured with Doppler echocardiography in rheumatoid arthritis patients. Rheumatology (Oxford) 39(12):1320–1325CrossRef

34.

Keser G et al (2004) Pulmonary hypertension in rheumatoid arthritis. Scand J Rheumatol 33(4):244–245PubMedCrossRef

35.

Wigley FM et al (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 & Rheumatism 52(7):2125–2132CrossRef

36.

Gavaghan DJ, Moore RA, McQuay HJ (2000) An evaluation of homogeneity tests in meta-analyses in pain using simulations of individual patient data. Pain 85(3):415–424PubMedCrossRef

37.

Hatano S, Strasser T, WH Organization (1975) Primary pulmonary hypertension: report on a WHO meeting: World Health Organization, Geneva

38.

Badesch DB et al (2009) Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 54(1 Suppl):S55–S66PubMedCrossRef

39.

Kovacs G et al (2009) Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J 34(4):888–894PubMedCrossRef

40.

Galie N et al (2004) Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J 25(24):2243–2278PubMedCrossRef

41.

Condliffe R et al (2009) Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 179(2):151–157PubMedCrossRef

42.

Chung L et al (2010) Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest 138(6):1383–1394PubMedCrossRef

Title: Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature
Authors: Xiaoqin Yang
Jack Mardekian
Kafi N. Sanders
Marko A. Mychaskiw
Joseph Thomas III
Publication date: 01-10-2013
Publisher: Springer London
Published in: Clinical Rheumatology / Issue 10/2013
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI: https://doi.org/10.1007/s10067-013-2307-2

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