Increased risk of mortality in systemic sclerosis-associated pulmonary hypertension: a systemic review and meta-analysis
Tóm tắt
Pulmonary hypertension (PH) is a frequent complication of systemic sclerosis (SSc) and is currently one of the primary causes of death in patients with this disease. We conducted a systematic review and meta-analysis to assess the association between PH and mortality in patients with SSc to verify trends in mortality in patients with SSc-associated PH.
We searched the PubMed and Embase databases for published studies on SSc-associated PH from inception to May 2021. All cohort studies in which mortality and/or survival for SSc-associated PH were reported were included in the analysis. The outcome parameters were pooled and analyzed using a random-effects model via generic inverse-variance weighting in conventional and cumulative meta-analysis.
The literature search identified 1161 citations, and the full texts of 54 studies were examined. Sixteen articles, with a total of 7857 patients with SSc and 1140 patients with SSc-associated PH, were included in the meta-analysis. Patients with SSc-associated PH had a higher pooled risk of mortality than patients with SSc without PH (risk ratio = 3.12; 95% confidence interval: [2.44, 3.98]).
This meta-analysis revealed a higher mortality in patients with SSc-associated PH. PH was a significant predictor of death in patients with SSc. Thus, early diagnosis and treatment of PH are important in patients with SSc.
Từ khóa
Tài liệu tham khảo
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;62(11):1088–93. https://doi.org/10.1136/ard.62.11.1088.
Herzog EL, Mathur A, Tager AM, Feghali-Bostwick C, Schneider F, Varga J. Review: interstitial lung disease associated with systemic sclerosis and idiopathic pulmonary fibrosis: how similar and distinct? Arthritis Rheumatol (Hoboken, NJ). 2014;66(8):1967–78. https://doi.org/10.1002/art.38702.
Silman AJ. Scleroderma–demographics and survival. J Rheumatol Suppl. 1997;48:58–61.
Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis. 2007;66(7):940–4. https://doi.org/10.1136/ard.2006.066068.
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. Rev Esp Cardiol (Engl Ed). 2016;69(2):177. https://doi.org/10.1016/j.rec.2016.01.002.
Launay D, Sobanski V, Hachulla E, Humbert M. Pulmonary hypertension in systemic sclerosis: different phenotypes. Eur Respir Rev: Off J Eur Respir Soc. 2017;26(145):170056. https://doi.org/10.1183/16000617.0056-2017.
Chung L, Farber HW, Benza R, Miller DP, Parsons L, Hassoun PM, et al. Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry. Chest. 2014;146(6):1494–504. https://doi.org/10.1378/chest.13-3014.
Trad S, Amoura Z, Beigelman C, Haroche J, Costedoat N, le Boutin TH, et al. Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis, independent of interstitial lung disease. Arthritis Rheum. 2006;54(1):184–91. https://doi.org/10.1002/art.21538.
Hachulla E, Carpentier P, Gressin V, Diot E, Allanore Y, Sibilia J, et al. Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study. Rheumatology (Oxford). 2009;48(3):304–8. https://doi.org/10.1093/rheumatology/ken488.
Hashimoto A, Tejima S, Tono T, Suzuki M, Tanaka S, Matsui T, et al. Predictors of survival and causes of death in Japanese patients with systemic sclerosis. J Rheumatol. 2011;38(9):1931–9. https://doi.org/10.3899/jrheum.100298.
Hoffmann-Vold AM, Molberg O, Midtvedt O, Garen T, Gran JT. Survival and causes of death in an unselected and complete cohort of Norwegian patients with systemic sclerosis. J Rheumatol. 2013;40(7):1127–33. https://doi.org/10.3899/jrheum.121390.
Hu S, Hou Y, Wang Q, Li M, Xu D, Zeng X. Prognostic profile of systemic sclerosis: analysis of the clinical EUSTAR cohort in China. Arthritis Res Therapy. 2018;20(1):1–9. https://doi.org/10.1186/s13075-018-1735-4.
Hsu VM, Chung L, Hummers LK, Shah A, Simms R, Bolster M, et al. Risk factors for mortality and cardiopulmonary hospitalization in systemic sclerosis patients at risk for pulmonary hypertension, in the PHAROS Registry. J Rheumatol. 2019;46(2):176–83. https://doi.org/10.3899/jrheum.180018.
Krikeerati T, Pussadhamma B, Mahakkanukrauh A, Suwannaroj S, Nanagara R, Foocharoen C. Associated factors of early-onset pulmonary hypertension and clinical difference between early- and late-onset pulmonary hypertension in Thai systemic sclerosis. Mod Rheumatol. 2020. https://doi.org/10.1080/14397595.2020.1823067.
Noviani M, Saffari SE, Tan JL, Yip JWL, Teng GG, Law WG, et al. Mortality and hospitalization outcomes of interstitial lung disease and pulmonary hypertension in the Singapore systemic sclerosis cohort. Semin Arthritis Rheum. 2020;50(3):473–9. https://doi.org/10.1016/j.semarthrit.2019.11.005.
Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, 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(10):1809–15. https://doi.org/10.1136/ard.2009.114264.
Moher D, Liberati A, Tetzlaff J, Altman DG. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. PLoS Med. 2009;6(7): e1000097. https://doi.org/10.1371/journal.pmed.1000097.
Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum. 1980;23(5):581–90. https://doi.org/10.1002/art.1780230510.
LeRoy EC, Medsger TA Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol. 2001;28(7):1573–6.
Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913. https://doi.org/10.1183/13993003.01913-2018.
Wells G, Shea B, O’Connell J. The Newcastle-Ottawa Scale (NOS) for assessing the quality of nonrandomised studies in meta-analyses. Ottawa: Ottawa Health Research Institute; 2014. p. 7.
Higgins JP, Thompson SG, Deeks JJ, Altman DG. Measuring inconsistency in meta-analyses. BMJ. 2003;327(7414):557–60. https://doi.org/10.1136/bmj.327.7414.557.
Chung MP, Dontsi M, Postlethwaite D, Kesh S, Simard JF, Fiorentino D, et al. Increased mortality in Asians with systemic sclerosis in Northern California. ACR Open Rheumatol. 2020;2(4):197–206. https://doi.org/10.1002/acr2.11126.
Rubio-Rivas M, Corbella X, Pestaña-Fernández M, Tolosa-Vilella C, Guillen-Del Castillo A, Colunga-Argüelles D, et al. First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study. Clin Rheumatol. 2018;37(4):999–1009. https://doi.org/10.1007/s10067-017-3936-7.
Panopoulos S, Bournia VK, Konstantonis G, Fragiadaki K, Sfikakis PP, Tektonidou MG. Predictors of morbidity and mortality in early systemic sclerosis: Long-term follow-up data from a single-centre inception cohort. Autoimmun Rev. 2018;17(8):816–20. https://doi.org/10.1016/j.autrev.2018.02.008.
Moon KW, Lee SS, Lee YJ, Jun JB, Yoo SJ, Ju JH, et al. Clinical and laboratory characteristics and mortality in korean patients with systemic sclerosis: a nationwide multicenter retrospective cohort study. J Rheumatol. 2018;45(9):1281–8. https://doi.org/10.3899/jrheum.171443.
Hu S, Hou Y, Wang Q, Li M, Xu D, Zeng X. Prognostic profile of systemic sclerosis: analysis of the clinical EUSTAR cohort in China. Arthritis Res Ther. 2018;20(1):235. https://doi.org/10.1186/s13075-018-1735-4.
Simeón-Aznar CP, Fonollosa-Plá V, Tolosa-Vilella C, Espinosa-Garriga G, Campillo-Grau M, Ramos-Casals M, et al. Registry of the Spanish network for systemic sclerosis: survival, prognostic factors, and causes of death. Medicine. 2015;94(43): e1728. https://doi.org/10.1097/md.0000000000001728.
Hissaria P, Lester S, Hakendorf P, Woodman R, Patterson K, Hill C, et al. Survival in scleroderma: results from the population-based South Australian Register. Intern Med J. 2011;41(5):381–90. https://doi.org/10.1111/j.1445-5994.2010.02281.x.
Hesselstrand R, Wildt M, Ekmehag B, Wuttge DM, Scheja A. 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. https://doi.org/10.3109/03009742.2010.508751.
Joven BE, Almodovar R, Carmona L, Carreira PE. Survival, causes of death, and risk factors associated with mortality in Spanish systemic sclerosis patients: results from a single university hospital. Semin Arthritis Rheum. 2010;39(4):285–93. https://doi.org/10.1016/j.semarthrit.2009.06.002.
Al-Dhaher FF, Pope JE, Ouimet JM. Determinants of morbidity and mortality of systemic sclerosis in Canada. Semin Arthritis Rheum. 2010;39(4):269–77. https://doi.org/10.1016/j.semarthrit.2008.06.002.
Avouac J, Airò P, Meune C, Beretta L, Dieude P, Caramaschi P, et al. Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J Rheumatol. 2010;37(11):2290–8. https://doi.org/10.3899/jrheum.100245.
Foocharoen C, Nanagara R, Kiatchoosakun S, Suwannaroj S, Mahakkanukrauh A. Prognostic factors of mortality and 2-year survival analysis of systemic sclerosis with pulmonary arterial hypertension in Thailand. Int J Rheum Dis. 2011;14(3):282–9. https://doi.org/10.1111/j.1756-185X.2011.01625.x.
Humbert M, Yaici A, de Groote P, Montani D, Sitbon O, Launay D, et al. Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum. 2011;63(11):3522–30. https://doi.org/10.1002/art.30541.
Lefèvre G, Dauchet L, Hachulla E, Montani D, Sobanski V, Lambert M, et al. Survival and prognostic factors in systemic sclerosis-associated pulmonary hypertension: a systematic review and meta-analysis. Arthritis Rheum. 2013;65(9):2412–23. https://doi.org/10.1002/art.38029.
Komócsi A, Vorobcsuk A, Faludi R, Pintér T, Lenkey Z, Költo G, et al. The impact of cardiopulmonary manifestations on the mortality of SSC: a systematic review and meta-analysis of observational studies. Rheumatology (United Kingdom). 2012;51(6):1027–36. https://doi.org/10.1093/rheumatology/ker357.
Kanemoto N. Natural history of pulmonary hemodynamics in primary pulmonary hypertension. Am Heart J. 1987;114(2):407–13. https://doi.org/10.1016/0002-8703(87)90510-2.
Brun H, Holmström H, Thaulow E. Sudden death during a change in treatment for pulmonary hypertension. Cardiol Young. 2005;15(2):223–5. https://doi.org/10.1017/s1047951105000442.
Kawato H, Hitosugi M, Kido M, Yufu T, Nagai T, Tokudome S. An autopsy case of sudden death in a boy with primary pulmonary hypertension: a case report. Med Sci Law. 2005;45(4):361–3. https://doi.org/10.1258/rsmmsl.45.4.361.
Pope JE, Lee P, Baron M, Dunne J, Smith D, Docherty PS, et al. Prevalence of elevated pulmonary arterial pressures measured by echocardiography in a multicenter study of patients with systemic sclerosis. J Rheumatol. 2005;32(7):1273–8.
Fishman AP. Clinical classification of pulmonary hypertension. Clin Chest Med. 2001;22(3):385–91. https://doi.org/10.1016/s0272-5231(05)70278-1.
Barnett CF, Alvarez P, Park MH. Pulmonary arterial hypertension: diagnosis and treatment. Cardiol Clin. 2016;34(3):375–89. https://doi.org/10.1016/j.ccl.2016.04.006.
Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173(9):1023–30. https://doi.org/10.1164/rccm.200510-1668OC.
Kovacs G, Olschewski H. Potential role of exercise echocardiography and right heart catheterization in the detection of early pulmonary vascular disease in patients with systemic sclerosis. J Scleroderma Relat Disord. 2019. https://doi.org/10.1177/2397198319849805.
Denton CP, Cailes JB, Phillips GD, Wells AU, Black CM, Bois RM. Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary hypertension in systemic sclerosis. Br J Rheumatol. 1997;36(2):239–43. https://doi.org/10.1093/rheumatology/36.2.239.
Giordano N, Corallo C, Chirico C, Brazzi A, Marinetti A, Fioravanti A, et al. Pulmonary arterial hypertension in systemic sclerosis: diagnosis and treatment according to the European Society of Cardiology and European Respiratory Society 2015 guidelines. J Scleroderma Relat Disord. 2019;4(1):35–42. https://doi.org/10.1177/2397198318808998.
Mukerjee D, St George D, Knight C, Davar J, Wells AU, Du Bois RM, et al. Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology (Oxford). 2004;43(4):461–6. https://doi.org/10.1093/rheumatology/keh067.
Coghlan JG, Denton CP, Grünig E, Bonderman D, Distler O, Khanna D, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73(7):1340–9. https://doi.org/10.1136/annrheumdis-2013-203301.
Mathai SC, Hassoun PM. Pulmonary arterial hypertension in connective tissue diseases. Heart Fail Clin. 2012;8(3):413–25. https://doi.org/10.1016/j.hfc.2012.04.001.
Rossi D, Zanatta E, Marson P, Sciascia S, Polito P, Roccatello D, et al. How I treat patients with systemic sclerosis in clinical practice. Autoimmun Rev. 2017;16(10):1024–8. https://doi.org/10.1016/j.autrev.2017.07.018.
Attanasio U, Cuomo A, Pirozzi F, Loffredo S, Abete P, Petretta M, et al. Pulmonary hypertension phenotypes in systemic sclerosis: the right diagnosis for the right treatment. Int J Mol Sci. 2020;21(12):4430. https://doi.org/10.3390/ijms21124430.
Barberà JA, Roger N, Roca J, Rovira I, Higenbottam TW, Rodriguez-Roisin R. Worsening of pulmonary gas exchange with nitric oxide inhalation in chronic obstructive pulmonary disease. Lancet. 1996;347(8999):436–40. https://doi.org/10.1016/s0140-6736(96)90011-2.
Morrell NW, Higham MA, Phillips PG, Shakur BH, Robinson PJ, Beddoes RJ. Pilot study of losartan for pulmonary hypertension in chronic obstructive pulmonary disease. Respir Res. 2005;6(1):88. https://doi.org/10.1186/1465-9921-6-88.
Madani MM, Auger WR, Pretorius V, Sakakibara N, Kerr KM, Kim NH, et al. Pulmonary endarterectomy: recent changes in a single institution’s experience of more than 2,700 patients. Ann Thorac Surg. 2012;94(1):97–103. https://doi.org/10.1016/j.athoracsur.2012.04.004 (discussion).
Simeón-Aznar CP, Fonollosa-Plá V, Tolosa-Vilella C, Espinosa-Garriga G, Ramos-Casals M, Campillo-Grau M, et al. Registry of the Spanish network for systemic sclerosis: clinical pattern according to cutaneous subsets and immunological status. Semin Arthritis Rheum. 2012;41(6):789–800. https://doi.org/10.1016/j.semarthrit.2011.10.004.