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4/2018 vol. 31
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REVIEW
Systemic Sclerosis - Associated Pulmonary Hypertension
Iraklis Tsangaris 1
,
 
Frantzeska Frantzeskaki 1
 
 
 
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2nd Department of Critical Care Medicine and Pulmonary Hypertension Clinic, ATTIKON University Hospital, National and Kapodistrian University of Athens, Chaidari, Greece
 
 
Corresponding author
Iraklis Tsangaris   

2nd Department of Critical Care Medicine and Pulmonary Hypertension Clinic, ATTIKON University Hospital, National and Kapodistrian University of Athens 1 Rimini Str, Chaidari 12462, Greece
 
 
Pneumon 2018;31(4):221-228
 
Article (PDF)
References (47)
 
KEYWORDS
pulmonary hypertension
 scleroderm
 
ABSTRACT
Lung disease is a major complication of systemic sclerosis associated with high morbidity and mortality rates. Pulmonary hypertension and lung fibrosis are the prevalent manifestations of lung disease, usually with overlapping characteristics. A major challenge in the management of the disease, is to identify the phenotypes of patients who might benefit from specific therapeutic modalities. The aim of the present review is to describe the clinical and epidemiological characteristics, classification, risk assessment and management of systemic sclerosis associated pulmonary hypertension.
 
REFERENCES (47)
1.
Johnson SR, Granton JT. Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus. European Respiratory Review: an Official Journal of the European Respiratory Society 2011;20:277-86.
Google Scholar
 
 
2.
Varga J, Whitfield ML. Transforming growth factor-beta in systemic sclerosis (scleroderma). Frontiers in bioscience (Scholar edition) 2009;1:226-35.
Google Scholar
 
 
3.
Denton CP, Wells AU, Coghlan JG. Major lung complications of systemic sclerosis. Nature Reviews Rheumatology 2018;14:511- 27.
Google Scholar
 
 
4.
Sobanski V, Launay D, Hachulla E, Humbert M. Current Approaches to the Treatment of Systemic-Sclerosis-Associated Pulmonary Arterial Hypertension (SSc-PAH). Current Rheumatology Reports 2016;18:10.
Google Scholar
 
 
5.
Galie N, Humbert M, Vachiery JL, 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). The European Respiratory Journal 2015;46:903-75.
Google Scholar
 
 
6.
Sundaram SM, Chung L. An Update on Systemic SclerosisAssociated Pulmonary Arterial Hypertension: a Review of the Current Literature. Current Rheumatology Reports 2018;20:10.
Google Scholar
 
 
7.
Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. Journal of the American College of Cardiology 2013;62(25 Suppl):D34-41.
Google Scholar
 
 
8.
Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Annals of the Rheumatic Diseases 2007;66:940-4.
Google Scholar
 
 
9.
Hao Y, Hudson M, Baron M, et al. Early mortality in a multinational systemic sclerosis inception cohort. Arthritis and Rheumatology 2017;69:1067-77.
Google Scholar
 
 
10.
Chung L, Farber HW, Benza R, et al. Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry. Chest 2014;146:1494-504.
Google Scholar
 
 
11.
Ramjug S, Hussain N, Hurdman J, et al. Idiopathic and Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: A Comparison of Demographic, Hemodynamic, and MRI Characteristics and Outcomes. Chest 2017;152:92-102.
Google Scholar
 
 
12.
Hsu VM, Chung L, Hummers LK, et al. Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study. Seminars in Arthritis and Rheumatism 2014;44:55-62.
Google Scholar
 
 
13.
Nihtyanova SI, Schreiber BE, Ong VH, et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis and Rheumatology (Hoboken, NJ) 2014;66:1625-35.
Google Scholar
 
 
14.
Hachulla E, Launay D, Mouthon L, et al. Is pulmonary arterial hypertension really a late complication of systemic sclerosis? Chest 2009;136:1211-9.
Google Scholar
 
 
15.
Shah AA, Wigley FM, Hummers LK. Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertension. The Journal of Rheumatology 2010;37:98-104.
Google Scholar
 
 
16.
Allanore Y, Borderie D, Avouac J, et al. High N-terminal pro-brain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis. Arthritis and Rheumatism 2008;58:284-91.
Google Scholar
 
 
17.
Kampolis C, Plastiras S, Vlachoyiannopoulos P, Moyssakis I, Tzelepis G. The presence of anti-centromere antibodies may predict progression of estimated pulmonary arterial systolic pressure in systemic sclerosis. Scandinavian Journal of Rheumatology 2008;37:278-83.
Google Scholar
 
 
18.
Thakkar V, Stevens WM, Prior D, et al. N-terminal pro-brain natriuretic peptide in a novel screening algorithm for pulmonary arterial hypertension in systemic sclerosis: a case-control study. Arthritis Research and Therapy 2012;14:R143.
Google Scholar
 
 
19.
Coghlan JG, Denton CP, Grunig E, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Annals of the Rheumatic Diseases 2014; 73:1340-9.
Google Scholar
 
 
20.
Guillen-Del Castillo A, Callejas-Moraga EL, Garcia G, et al. High sensitivity and negative predictive value of the DETECT algorithm for an early diagnosis of pulmonary arterial hypertension in systemic sclerosis: application in a single center. Arthritis Research and Therapy 2017;19:135.
Google Scholar
 
 
21.
Hemnes AR, Humbert M. Pathobiology of pulmonary arterial hypertension: understanding the roads less travelled. European respiratory review: an Official Journal of the European Respiratory Society 2017;26(146):pii:170093.
Google Scholar
 
 
22.
Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. American Journal of Respiratory and Critical Care Medicine 2008; 177:1248-54.
Google Scholar
 
 
23.
Lammi MR, Saketkoo LA, Gordon JK, Steen VD. Changes in hemodynamic classification over time are common in systemic sclerosis-associated pulmonary hypertension: insights from the PHAROS cohort. Pulmonary Circulation 2018;8(2):2045893218757404.
Google Scholar
 
 
24.
Ciurzynski M, Bienias P, Lichodziejewska B, et al. Assessment of left and right ventricular diastolic function in patients with systemic sclerosis. Kardiologia Polska 2008;66:269-76, discussion 77-8.
Google Scholar
 
 
25.
Lazar JM, Flores AR, Grandis DJ, Orie JE, Schulman DS. Effects of chronic right ventricular pressure overload on left ventricular diastolic function. The American Journal of Cardiology 1993;72:1179-82.
Google Scholar
 
 
26.
Launay D, Montani D, Hassoun PM, et al. Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis. PloS one 2018;13:e0197112.
Google Scholar
 
 
27.
Mihai C, Antic M, Dobrota R, et al. Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort. Annals of the Rheumatic Diseases 2018;77:128-32.
Google Scholar
 
 
28.
Bae S, Saggar R, Bolster MB, et al. Baseline characteristics and follow-up in patients with normal haemodynamics versus borderline mean pulmonary arterial pressure in systemic sclerosis: results from the PHAROS registry. Annals of the Rheumatic Diseases 2012;71:1335-42.
Google Scholar
 
 
29.
Coghlan JG, Wolf M, Distler O, et al. Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis. The European Respiratory Journal 2018;51(4):pii:17001197.
Google Scholar
 
 
30.
Boucly A, Weatherald J, Humbert M, Sitbon O. Risk assessment in pulmonary arterial hypertension. European Respiratory Journal 2018; 51(3):1800279.
Google Scholar
 
 
31.
Weatherald JC, Chaouat A, Cottin V, et al. Risk Assessment in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension. A27 you got another thing coming: Diagnosis and prognostication in pulmonary hypertension. p. A1178-A.
Google Scholar
 
 
32.
Weatherald J, Boucly A, Launay D, et al. Haemodynamics and serial risk assessment in systemic-sclerosis associated pulmonary arterial hypertension. European Respiratory Journal 2018; 52(4): pii 1800678. doi: 10.1183/13993003.00678-2018.
CrossRef
 
Google Scholar
 
 
33.
Hyer M, Ramakrishnan V, Lammi MR, Steen V, Argula RG. Can the PHAROS Predict the Future? - Identifying High Risk Phenotypes Among Patients with Systemic Sclerosis Associated Pulmonary Arterial Hypertension (SSc-PAH). A27 you got another thing coming: Diagnosis and prognostication in pulmonary hypertension. p. A1177-A.
Google Scholar
 
 
34.
Schoenfeld SR, Castelino FV. Evaluation and management approaches for scleroderma lung disease. Therapeutic Advances in Respiratory Disease 2017;11:327-40.
Google Scholar
 
 
35.
Hoyles RK, Ellis RW, Wellsbury J, et al. 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 and Rheumatism 2006;54:3962-70.
Google Scholar
 
 
36.
Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, doubleblind, parallel group trial. The Lancet Respiratory medicine. 2016;4:708-19.
Google Scholar
 
 
37.
Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. The New England Journal of Medicine 2014;370:2071-82.
Google Scholar
 
 
38.
Daoussis D, Liossis SN, Tsamandas AC, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford, England) 2010;49:271-80.
Google Scholar
 
 
39.
Badesch DB, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to thescleroderma spectrum of disease. A randomized, controlled trial. Annals of Internal Medicine 2000;132:425-34.
Google Scholar
 
 
40.
Sitbon O, Channick R, Chin KM, et al. Selexipag for the Treatment of Pulmonary Arterial Hypertension. New England Journal of Medicine 2015;373:2522-33.
Google Scholar
 
 
41.
Coghlan JG, Galie N, Barbera JA, et al. Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTDPAH): subgroup analysis from the AMBITION trial. Annals of the Rheumatic Diseases 2017;76:1219-27.
Google Scholar
 
 
42.
Lammi MR, Mathai SC, Saketkoo LA, et al. Association between initial oral therapy and outcomes in systemic sclerosis-related pulmonary arterial hypertension. Arthritis and Rheumatology (Hoboken, NJ). 2016;68:740-8.
Google Scholar
 
 
43.
Preston IR, Roberts KE, Miller DP, et al. Effect of warfarin treatment on survival of patients with pulmonary arterial hypertension (PAH) in the registry to evaluate early and long-term PAH disease management (REVEAL). Circulation 2015;132:2403-11.
Google Scholar
 
 
44.
Seeger W, Adir Y, Barbera JA, et al. Pulmonary hypertension in chronic lung diseases. Journal of the American College of Cardiology 2013;62(25 Suppl):D109-16.
Google Scholar
 
 
45.
Trammell AW, Pugh ME, Newman JH, Hemnes AR, Robbins IM. Use of pulmonary arterial hypertension-approved therapy in the treatment of non-group 1 pulmonary hypertension at US referral centers. Pulmonary Circulation 2015;5:356-63.
Google Scholar
 
 
46.
Crespo MM, Bermudez CA, Dew MA, et al. Lung transplant in patients with scleroderma compared with pulmonary fibrosis. short- and long-term outcomes. Annals of the American Thoracic Society 2016;13:784-92.
Google Scholar
 
 
47.
Miele CH, Schwab K, Saggar R, et al. Lung Transplant outcomes in systemic sclerosis with significant esophageal dysfunction. A Comprehensive Single-Center Experience 2016;13:793-802.
Google Scholar
 
 
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