Advertisement
Review Article| Volume 19, ISSUE 1, P45-54, January 2023

Pulmonary Arterial Hypertension in Connective Tissue Diseases Beyond Systemic Sclerosis

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Heart Failure Clinics
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Simonneau G.
        • Montani D.
        • Celermajer D.S.
        • et al.
        Haemodynamic definitions and updated clinical classification of pulmonary hypertension.
        Eur Respir J. 2019; 53https://doi.org/10.1183/13993003.01913-2018
        • Humbert M.
        • Guignabert C.
        • Bonnet S.
        • et al.
        Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives.
        Eur Respir J. 2019; 53https://doi.org/10.1183/13993003.01887-2018
        • Mathai S.C.
        • Hassoun P.M.
        Pulmonary arterial hypertension in connective tissue diseases.
        Heart Fail Clin. 2012; 8: 413-425
        • McLaughlin V.V.
        • Archer S.L.
        • Badesch D.B.
        • et al.
        ACCF/AHA 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. 2009; 53: 1573-1619
        • Frost A.
        • Badesch D.
        • Gibbs J.S.R.
        • et al.
        Diagnosis of pulmonary hypertension.
        Eur Respir J. 2019; 53https://doi.org/10.1183/13993003.01904-2018
        • Sitbon O.
        • Gomberg-Maitland M.
        • Granton J.
        • et al.
        Clinical trial design and new therapies for pulmonary arterial hypertension.
        Eur Respir J. 2019; 53https://doi.org/10.1183/13993003.01908-2018
        • Fayed H.
        • Coghlan J.G.
        Pulmonary Hypertension Associated with Connective Tissue Disease.
        Semin Respir Crit Care Med. 2019; 40: 173-183
        • McGoon M.D.
        • Miller D.P.
        REVEAL: a contemporary US pulmonary arterial hypertension registry.
        Eur Respir Rev. 2012; 21: 8-18
        • Zhao J.
        • Wang Q.
        • Liu Y.
        • et al.
        Clinical characteristics and survival of pulmonary arterial hypertension associated with three major connective tissue diseases: A cohort study in China.
        Int J Cardiol. 2017; 236: 432-437
        • Humbert M.
        • Sitbon O.
        • Chaouat A.
        • et al.
        Pulmonary arterial hypertension in France: results from a national registry.
        Am J Respir Crit Care Med. 2006; 173: 1023-1030
        • Ranque B.
        • Mouthon L.
        Geoepidemiology of systemic sclerosis.
        Autoimmun Rev Mar. 2010; 9: A311-A318
        • Jansa P.
        • Jarkovsky J.
        • Al-Hiti H.
        • et al.
        Epidemiology and long-term survival of pulmonary arterial hypertension in the Czech Republic: a retrospective analysis of a nationwide registry.
        BMC Pulm Med. 2014; 14: 45https://doi.org/10.1186/1471-2466-14-45
        • Coghlan J.G.
        • Handler C.
        Connective tissue associated pulmonary arterial hypertension.
        Lupus. 2006; 15: 138-142
        • Mukerjee D.
        • St George D.
        • Coleiro B.
        • et al.
        Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach.
        Ann Rheum Dis. 2003; 62: 1088-1093
        • Avouac J.
        • Airo P.
        • Meune C.
        • et al.
        Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies.
        J Rheumatol. 2010; 37: 2290-2298
        • Hachulla E.
        • Gressin V.
        • Guillevin L.
        • et al.
        Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study.
        Arthritis Rheum. 2005; 52: 3792-3800
        • Sullivan W.D.
        • Hurst D.J.
        • Harmon C.E.
        • et al.
        A prospective evaluation emphasizing pulmonary involvement in patients with mixed connective tissue disease.
        Medicine (Baltimore). 1984; 63: 92-107
        • Burdt M.A.
        • Hoffman R.W.
        • Deutscher S.L.
        • et al.
        Long-term outcome in mixed connective tissue disease: longitudinal clinical and serologic findings.
        Arthritis Rheum. 1999; 42: 899-909
        • Alpert M.A.
        • Goldberg S.H.
        • Singsen B.H.
        • et al.
        Cardiovascular manifestations of mixed connective tissue disease in adults.
        Circulation. 1983; 68: 1182-1193
        • Gunnarsson R.
        • Andreassen A.K.
        • Molberg O.
        • et al.
        Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort: results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature.
        Rheumatology (Oxford). 2013; 52: 1208-1213
        • Arnaud L.
        • Agard C.
        • Haroche J.
        • et al.
        [Pulmonary arterial hypertension in systemic lupus erythematosus].
        Rev Med Interne. 2011; 32 (Hypertension arterielle pulmonaire associee au lupus systemique): 689-697
        • Ruiz-Irastorza G.
        • Garmendia M.
        • Villar I.
        • et al.
        Pulmonary hypertension in systemic lupus erythematosus: prevalence, predictors and diagnostic strategy.
        Autoimmun Rev. 2013; 12: 410-415
        • Chung L.
        • Liu J.
        • Parsons L.
        • et al.
        Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype.
        Chest. 2010; 138: 1383-1394
        • Shirai Y.
        • Yasuoka H.
        • Okano Y.
        • et al.
        Clinical characteristics and survival of Japanese patients with connective tissue disease and pulmonary arterial hypertension: a single-centre cohort.
        Rheumatology (Oxford). 2012; 51: 1846-1854
        • Chung W.J.
        • Park Y.B.
        • Jeon C.H.
        • et al.
        Baseline Characteristics of the Korean Registry of Pulmonary Arterial Hypertension.
        J Korean Med Sci. 2015; 30: 1429-1438
        • Song S.
        • Lee S.E.
        • Oh S.K.
        • et al.
        Demographics, treatment trends, and survival rate in incident pulmonary artery hypertension in Korea: A nationwide study based on the health insurance review and assessment service database.
        PLoS One. 2018; 13: e0209148
        • Chen H.A.
        • Hsu T.C.
        • Yang S.C.
        • et al.
        Incidence and survival impact of pulmonary arterial hypertension among patients with systemic lupus erythematosus: a nationwide cohort study.
        Arthritis Res Ther. 2019; 21: 82
        • Myasoedova E.
        • Davis 3rd, J.M.
        • Crowson C.S.
        • et al.
        Epidemiology of rheumatoid arthritis: rheumatoid arthritis and mortality.
        Curr Rheumatol Rep. 2010; 12: 379-385
        • Cross M.
        • Smith E.
        • Hoy D.
        • et al.
        The global burden of rheumatoid arthritis: estimates from the global burden of disease 2010 study.
        Ann Rheum Dis. 2014; 73: 1316-1322
        • Jeon C.H.
        • Chai J.Y.
        • Seo Y.I.
        • et al.
        Pulmonary hypertension associated with rheumatic diseases: baseline characteristics from the Korean registry.
        Int J Rheum Dis. 2012; 15: e80-e89
        • Montani D.
        • Henry J.
        • O'Connell C.
        • et al.
        Association between Rheumatoid Arthritis and Pulmonary Hypertension: Data from the French Pulmonary Hypertension Registry.
        Respiration. 2018; 95: 244-250
        • Condliffe R.
        • Kiely D.G.
        • Peacock A.J.
        • et al.
        Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era.
        Am J Respir Crit Care Med. 2009; 179: 151-157
        • Launay D.
        • Hachulla E.
        • Hatron P.Y.
        • et al.
        Pulmonary arterial hypertension: a rare complication of primary Sjogren syndrome: report of 9 new cases and review of the literature.
        Medicine (Baltimore). 2007; 86: 299-315
        • Hao Y.J.
        • Jiang X.
        • Zhou W.
        • et al.
        Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients.
        Eur Respir J. 2014; 44: 963-972
        • Taniguchi Y.
        • Horino T.
        • Kato T.
        • et al.
        Acute pulmonary arterial hypertension associated with anti-synthetase syndrome.
        Scand J Rheumatol. 2010; 39: 179-180
        • Grateau G.
        • Roux M.E.
        • Franck N.
        • et al.
        Pulmonary hypertension in a case of dermatomyositis.
        J Rheumatol. 1993; 20: 1452-1453
        • Bunch T.W.
        • Tancredi R.G.
        • Lie J.T.
        Pulmonary hypertension in polymyositis.
        Chest. 1981; 79: 105-107
        • Sanges S.
        • Yelnik C.M.
        • Sitbon O.
        • et al.
        Pulmonary arterial hypertension in idiopathic inflammatory myopathies: Data from the French pulmonary hypertension registry and review of the literature. Medicine (Baltimore).
        Sep. 2016; 95: e4911
        • Hachulla E.
        • Jais X.
        • Cinquetti G.
        • et al.
        Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus: Results From the French Pulmonary Hypertension Registry.
        Chest. 2018; 153: 143-151
        • Humbert M.
        • Yaici A.
        • de Groote P.
        • et al.
        Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival.
        Arthritis Rheum. 2011; 63: 3522-3530
        • Lefevre G.
        • Dauchet L.
        • Hachulla E.
        • et al.
        Survival and prognostic factors in systemic sclerosis-associated pulmonary hypertension: a systematic review and meta-analysis.
        Arthritis Rheum. 2013; 65: 2412-2423
        • Launay D.
        • Sitbon O.
        • Hachulla E.
        • et al.
        Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era.
        Ann Rheum Dis. 2013; 72: 1940-1946
        • Kawut S.M.
        • Taichman D.B.
        • Archer-Chicko C.L.
        • et al.
        Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis.
        Chest. 2003; 123: 344-350
        • Tyndall A.J.
        • Bannert B.
        • Vonk M.
        • et al.
        Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database.
        Ann Rheum Dis. 2010; 69: 1809-1815
        • Coghlan J.G.
        • Denton C.P.
        • Grunig E.
        • et al.
        Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study.
        Ann Rheum Dis. 2014; 73: 1340-1349
        • Vandecasteele E.
        • Drieghe B.
        • Melsens K.
        • et al.
        Screening for pulmonary arterial hypertension in an unselected prospective systemic sclerosis cohort.
        Eur Respir J. 2017; 49https://doi.org/10.1183/13993003.02275-2016
        • Galie N.
        • Humbert M.
        • Vachiery J.L.
        • 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): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).
        Eur Respir J. 2015; 46: 903-975
        • Kato M.
        • Atsumi T.
        Pulmonary arterial hypertension associated with connective tissue diseases: A review focusing on distinctive clinical aspects.
        Eur J Clin Invest. 2018; 48https://doi.org/10.1111/eci.12876
        • Prabu A.
        • Patel K.
        • Yee C.S.
        • et al.
        Prevalence and risk factors for pulmonary arterial hypertension in patients with lupus.
        Rheumatology (Oxford). 2009; 48: 1506-1511
        • Perez-Penate G.M.
        • Rua-Figueroa I.
        • Julia-Serda G.
        • et al.
        Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: Prevalence and Predictors.
        J Rheumatol. 2016; 43: 323-329
        • Zhang N.
        • Li M.
        • Qian J.
        • et al.
        Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR-PAH study: Baseline characteristics and risk factors.
        Int J Rheum Dis. 2019; 22: 921-928
        • Huang D.
        • Cheng Y.Y.
        • Chan P.H.
        • et al.
        Rationale and design of the screening of pulmonary hypertension in systemic lupus erythematosus (SOPHIE) study.
        ERJ Open Res. 2018; 4https://doi.org/10.1183/23120541.00135-2017
        • Coghlan J.G.
        • Galie N.
        • Barbera J.A.
        • et al.
        Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial.
        Ann Rheum Dis. 2017; 76: 1219-1227
        • Gaine S.
        • Chin K.
        • Coghlan G.
        • et al.
        Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension.
        Eur Respir J. 2017; 50https://doi.org/10.1183/13993003.02493-2016
        • Avouac J.
        • Wipff J.
        • Kahan A.
        • et al.
        Effects of oral treatments on exercise capacity in systemic sclerosis related pulmonary arterial hypertension: a meta-analysis of randomised controlled trials.
        Ann Rheum Dis. 2008; 67: 808-814
        • Noel Z.R.
        • Kido K.
        • Macaulay T.E.
        Selexipag for the treatment of pulmonary arterial hypertension.
        Am J Health Syst Pharm. 2017; 74: 1135-1141
        • Ghofrani H.A.
        • Galie N.
        • Grimminger F.
        • et al.
        Riociguat for the treatment of pulmonary arterial hypertension.
        N Engl J Med. 2013; 369: 330-340
        • Rubin L.J.
        • Galie N.
        • Grimminger F.
        • et al.
        Riociguat for the treatment of pulmonary arterial hypertension: a long-term extension study (PATENT-2).
        Eur Respir J. 2015; 45: 1303-1313
        • Galie N.
        • Barbera J.A.
        • Frost A.E.
        • et al.
        Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension.
        N Engl J Med. 2015; 373: 834-844
        • Jais X.
        • Launay D.
        • Yaici A.
        • et al.
        Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases.
        Arthritis Rheum. 2008; 58: 521-531