Association of pulmonary hypertension with outcomes in patients with Systemic sclerosis and other connective tissue disorders: review and meta-analysis PH associated survival in patients with CTDs

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Maka Gegenava
Tea Gegenava


Pulmonary Hypertension, Systemic sclerosis, Systemic Lupus Erythematosis


Background and aim: Pulmonary hypertension (PH) is a frequent complication of connective tissue disorders (CTDs), with a major impact on the prognosis of the disease. The aim of our study was to perform a systemic review and meta-analysis of published literature evaluating survival function in patients with systemic sclerosis (SSc) with and without PH and to compare survival function between patients with SSc, systemic lupus erythematosus (SLE), other CTDs, and conditions associated with PH. Methods: The established protocol of the Cochrane Collaboration Steps and meta-analysis of observational studies in epidemiology recommendations (MOOSE) were used. Results: 7 studies, including 1470 SSc-PH patients and 1368 SSc patients without PH, and 4 studies, including 108 SLE-PH patients and 1288 SLE patients without PH, assessed survival function were selected. Six studies (including 777 SSc, 249 SLE, 90 idiopathic pulmonary arterial hypertension (IPAH) and 29 primary Sjogren’s syndrome patients) comparing survival function in different subgroups of patients with confirmed PH were included. SSc patients with PH showed the worst survival as compared to SSc patients without PH [OR (95% CI) 3.70 (2.42–5.67); p<0.00001]. The same pattern was observed in patients with SLE. SSc patients with PH were characterized by lower survival function compared to other reasons for PH, including SLE [OR (95% CI) 2.76 (1.95–3.91); p<0.00001]. Conclusions: Patients with SSc-PH are characterized by significantly lower survival function as compared to SSc patients without PH. Among the different entities of PH, SSc shows the worst survival, underlining prognostic significance of detection and evaluation of PH according to the currently established approach.

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1. Hurdman J, Condliffe R, Elliot CA, et al. ASPIRE registry: assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre. Eur Respir J. 2012;39(4):945-55.
2. Launay D, Sitbon O, Hachulla E, et al. Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era. Annals of the rheumatic diseases. 2013;72(12):1940-6.
3. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. European heart journal. 2022;43(38):3618-731.
4. 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(6):1383-94.
5. Johnson SR, Gladman DD, Urowitz MB, et al. Pulmonary hypertension in systemic lupus. Lupus. 2004;13(7):506-9.
6. Winslow TM, Ossipov MA, Fazio GP, et al. Five-year follow-up study of the prevalence and progression of pulmonary hypertension in systemic lupus erythematosus. Am Heart J. 1995;129(3):510-5.
7. 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. International journal of cardiology. 2017;236:432-7.
8. Pocock SJ, Collier TJ, Dandreo KJ, et al. Issues in the reporting of epidemiological studies: a survey of recent practice. BMJ (Clinical research ed). 2004;329(7471):883.
9. Stroup DF, Berlin JA, Morton SC, et al. Meta-analysis of observational studies in epidemiology: a proposal for reporting. Meta-analysis Of Observational Studies in Epidemiology (MOOSE) group. Jama. 2000;283(15):2008-12.
10. Higgins JP, Thompson SG. Quantifying heterogeneity in a meta-analysis. Stat Med. 2002;21(11):1539-58.
11. Higgins JP, Thompson SG, Spiegelhalter DJ. A re-evaluation of random-effects meta-analysis. J R Stat Soc Ser A Stat Soc. 2009;172(1):137-59.
12. Hachulla E, Clerson P, Airò P, et al. Value of systolic pulmonary arterial pressure as a prognostic factor of death in the systemic sclerosis EUSTAR population. Rheumatology (Oxford). 2015;54(7):1262-9.
13. Hsu VM, Chung L, Hummers LK, et al. Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis Patients At Risk for Pulmonary Hypertension, in the PHAROS Registry. The Journal of rheumatology. 2019;46(2):176-83.
14. Williams MH, Handler CE, Akram R, et al. Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial hypertension. European heart journal. 2006;27(12):1485-94.
15. Hesselstrand R, Wildt M, Ekmehag B, et al. Survival in patients with pulmonary arterial hypertension associated with systemic sclerosis from a Swedish single centre: prognosis still poor and prediction difficult. Scand J Rheumatol. 2011;40(2):127-32.
16. Pène F, Hissem T, Bérezné A, et al. Outcome of Patients with Systemic Sclerosis in the Intensive Care Unit. The Journal of rheumatology. 2015;42(8):1406-12.
17. Young A, Vummidi D, Visovatti S, et al. Prevalence, Treatment, and Outcomes of Coexistent Pulmonary Hypertension and Interstitial Lung Disease in Systemic Sclerosis. Arthritis Rheumatol. 2019;71(8):1339-49.
18. Noviani M, Saffari SE, Tan JL, et al. Mortality and hospitalization outcomes of interstitial lung disease and pulmonary hypertension in the Singapore systemic sclerosis cohort. Seminars in arthritis and rheumatism. 2020;50(3):473-9.
19. Pope JE, Lee P, Baron M, et al. Prevalence of elevated pulmonary arterial pressures measured by echocardiography in a multicenter study of patients with systemic sclerosis. The Journal of rheumatology. 2005;32(7):1273-8.
20. Mukerjee D, St George D, Coleiro B, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Annals of the rheumatic diseases. 2003;62(11):1088-93.
21. Kolstad KD, Li S, Steen V, et al. Long-Term Outcomes in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry (PHAROS). Chest. 2018;154(4):862-71.
22. Farber HW, Miller DP, Poms AD, et al. Five-Year outcomes of patients enrolled in the REVEAL Registry. Chest. 2015;148(4):1043-54.
23. Condliffe R, Kiely DG, Peacock AJ, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. 2009;179(2):151-7.
24. Chung SM, Lee CK, Lee EY, et al. Clinical aspects of pulmonary hypertension in patients with systemic lupus erythematosus and in patients with idiopathic pulmonary arterial hypertension. Clinical rheumatology. 2006;25(6):866-72.
25. Kuhn KP, Byrne DW, Arbogast PG, et al. Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol. Am J Respir Crit Care Med. 2003;167(4):580-6.
26. Fisher MR, Mathai SC, Champion HC, et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis and rheumatism. 2006;54(9):3043-50.