{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,2]],"date-time":"2026-11-02T17:31:53Z","timestamp":1793640713963,"version":"4.5.1"},"reference-count":27,"publisher":null,"issue":"4","license":[{"start":{"date-parts":[[2025,4,30]],"date-time":"2025-04-30T00:00:00Z","timestamp":1745971200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/cardio.elpub.ru\/jour\/about\/editorialPolicies#openAccessPolicy"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Kardiologiia"],"abstract":"<jats:p>\n                    <jats:italic>Aim<\/jats:italic>\n                    \u00a0 \u00a0Analysis of survival and the impact of etiology, adverse prognosis factors, and therapy on the survival of patients with pulmonary arterial hypertension associated with immune-mediated inflammatory rheumatic diseases (PAH-IIRD).\n                    <jats:italic>Material and methods<\/jats:italic>\n                    \u00a0 \u00a0The study included 95 patients: 76 with systemic scleroderma (SSc), 9 with mixed connective tissue disease (MCTD), 8 with systemic lupus erythematosus (SLE), one with rheumatoid arthritis, and one with Sjogren's disease with diagnosed PAH. All patients were prescribed PAH-specific therapy and followed up for at least 5 years during this treatment. The endpoint of the study was all-cause death.\n                    <jats:italic>Results \u00a0<\/jats:italic>\n                    \u00a0During the 5-year follow-up period, 37 patients with PAH-SSc and 4 with PAH-MCTD (43%) died. There were no fatal outcomes in PAH-SLE. One-, two-, three-, and five-year survival rates in the overall group of patients were 91%, 80%, 73%, and 57%, respectively. In patients with PAH-SSc, one-, two-, three-, and five-year survival rates were worse than in PAH-MCTD (88%, 76%, 68%, 51% and 100%, 89%, 89%, 56%, respectively). The factors associated with a fatal outcome included age, gender, functional class, 6-minute walk test distance, right atrial pressure, cardiac output, pulmonary vascular resistance, and biomarker (uric acid and N-terminal pro-brain natriuretic peptide) concentrations. The use of macitentan and\/or riociguat, as monotherapy or in combination with another PAH-specific drug, significantly reduced the 5-year risk of fatal outcome (OR 0.38 [0.16; 0.89], p=0.027).\n                    <jats:italic>Conclusion \u00a0<\/jats:italic>\n                    \u00a0The survival of patients with PAH-IIRD remains low. Further studies aimed at finding new pathogenetic targets are needed; the use of modern PAH-specific drugs (macitentan and\/or riociguat) modifies the course of the disease, increasing the survival.\n                  <\/jats:p>\n                  <jats:p>\u00a0<\/jats:p>","DOI":"10.18087\/cardio.2025.4.n2919","type":"journal-article","created":{"date-parts":[[2025,5,7]],"date-time":"2025-05-07T07:41:36Z","timestamp":1746603696000},"page":"23-30","source":"Crossref","is-referenced-by-count":3,"title":["Long-Term Prospective Observation Study of Patients with Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases"],"prefix":"10.68900","volume":"65","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1784-3699","authenticated-orcid":true,"given":"A. V.","family":"Volkov","sequence":"first","affiliation":[{"name":"Nasonova Research Institute of Rheumatology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N. N.","family":"Yudkina","sequence":"additional","affiliation":[{"name":"Nasonova Research Institute of Rheumatology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E. L.","family":"Nasonov","sequence":"additional","affiliation":[{"name":"Nasonova Research Institute of Rheumatology"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":null,"published-online":{"date-parts":[[2025,4,30]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","unstructured":"Volkov A.V., Martynyuk T.V. Pulmonary arterial hypertension in systemic connective tissue diseases: the current state of the problem. Rheumatology Science and Practice. 2018;56(4):474\u201385. DOI: 10.14412\/1995-4484-2018-474-485","DOI":"10.14412\/1995-4484-2018-474-485"},{"key":"ref2","doi-asserted-by":"crossref","unstructured":"Mathai SC. Pulmonary Hypertension Associated with Connective Tissue Disease. Rheumatic Disease Clinics of North America. 2024;50(3):359\u201379. DOI: 10.1016\/j.rdc.2024.03.005","DOI":"10.1016\/j.rdc.2024.03.005"},{"key":"ref3","doi-asserted-by":"crossref","unstructured":"Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. European Respiratory Journal. 2019;53(1):1801913. DOI: 10.1183\/13993003.01913-2018","DOI":"10.1183\/13993003.01913-2018"},{"key":"ref4","doi-asserted-by":"crossref","unstructured":"Rubio-Rivas M, Homs NA, Cuartero D, Corbella X. The prevalence and incidence rate of pulmonary arterial hypertension in systemic sclerosis: Systematic review and meta-analysis. Autoimmunity Reviews. 2021;20(1):102713. DOI: 10.1016\/j.autrev.2020.102713","DOI":"10.1016\/j.autrev.2020.102713"},{"key":"ref5","doi-asserted-by":"crossref","unstructured":"Koh E, Lee P, Gladman DD, Abu-Shakra M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Rheumatology. 1996;35(10):989\u201393. DOI: 10.1093\/rheumatology\/35.10.989","DOI":"10.1093\/rheumatology\/35.10.989"},{"key":"ref6","unstructured":"Volkov A.V., Martynyuk T.V., Yudkina N.N., Danilov N.M., Glukhova S.I., Guseva N.G. et al. Survival of patients with pulmonary arterial hypertension, associated with systemic sclerosis. Therapeutic Archive. 2012;84(5):24\u20138."},{"key":"ref7","unstructured":"Volkov A.V., Mach E.S., Guseva N.G. Endothelial dysfunction in systemic sclerosis - clinicopathogenetic correlations. Therapeutic Archive. 2008;80(10):68\u201371."},{"key":"ref8","doi-asserted-by":"crossref","unstructured":"Correale M, Chiriv\u00ec F, Bevere EML, Tricarico L, D\u2019Alto M, Badagliacca R et al. Endothelial Function in Pulmonary Arterial Hypertension: From Bench to Bedside. Journal of Clinical Medicine. 2024;13(8):2444. DOI: 10.3390\/jcm13082444","DOI":"10.3390\/jcm13082444"},{"key":"ref9","doi-asserted-by":"crossref","unstructured":"Denton CP, Humbert M, Rubin L, Black CM. Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Annals of the Rheumatic Diseases. 2006;65(10):1336\u201340. DOI: 10.1136\/ard.2005.048967","DOI":"10.1136\/ard.2005.048967"},{"key":"ref10","unstructured":"Badesch DB, Hill NS, Burgess G, Rubin LJ, Barst RJ, Gali\u00e8 N et al. Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. The Journal of Rheumatology. 2007;34(12):2417\u2013 22. PMID: 17985403"},{"key":"ref11","doi-asserted-by":"crossref","unstructured":"Gali\u00e8 N, Olschewski H, Oudiz RJ, Torres F, Frost A, Ghofrani HA et al. Ambrisentan for the Treatment of Pulmonary Arterial Hypertension: Results of the Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy (ARIES) Study 1 and 2. Circulation. 2008;117(23):3010\u20139. DOI: 10.1161\/CIRCULATIONAHA.107.742510","DOI":"10.1161\/CIRCULATIONAHA.107.742510"},{"key":"ref12","doi-asserted-by":"crossref","unstructured":"Gali\u00e8 N, Denton CP, Dardi F, Manes A, Mazzanti G, Li B et al. Tadalafil in idiopathic or heritable pulmonary arterial hypertension (PAH) compared to PAH associated with connective tissue disease. International Journal of Cardiology. 2017;235:67\u201372. DOI: 10.1016\/j.ijcard.2017.02.094","DOI":"10.1016\/j.ijcard.2017.02.094"},{"key":"ref13","doi-asserted-by":"crossref","unstructured":"Humbert M, Coghlan JG, Ghofrani H-A, Grimminger F, He J-G, Riemekasten G et al. Riociguat for the treatment of pulmonary arterial hypertension associated with connective tissue disease: results from PATENT-1 and PATENT-2. Annals of the Rheumatic Diseases. 2017;76(2):422\u20136. DOI: 10.1136\/annrheumdis-2015-209087","DOI":"10.1136\/annrheumdis-2015-209087"},{"key":"ref14","doi-asserted-by":"crossref","unstructured":"Gaine S, Chin K, Coghlan G, Channick R, Di Scala L, Gali\u00e8 N et al. Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension. European Respiratory Journal. 2017;50(2):1602493. DOI: 10.1183\/13993003.02493-2016","DOI":"10.1183\/13993003.02493-2016"},{"key":"ref15","doi-asserted-by":"crossref","unstructured":"Pulido T, Adzerikho I, Channick RN, Delcroix M, Gali\u00e8 N, Ghofrani H-A et al. Macitentan and Morbidity and Mortality in Pulmonary Arterial Hypertension. New England Journal of Medicine. 2013;369(9):809\u201318. DOI: 10.1056\/NEJMoa1213917","DOI":"10.1056\/NEJMoa1213917"},{"key":"ref16","doi-asserted-by":"crossref","unstructured":"Lef\u00e8vre G, Dauchet L, Hachulla E, Montani D, Sobanski V, Lambert M et al. Survival and Prognostic Factors in Systemic Sclerosis\u2013 Associated Pulmonary Hypertension: A Systematic Review and Meta\u2010 Analysis. Arthritis & Rheumatism. 2013;65(9):2412\u201323. DOI: 10.1002\/art.38029","DOI":"10.1002\/art.38029"},{"key":"ref17","doi-asserted-by":"crossref","unstructured":"Khanna D, Zhao C, Saggar R, Mathai SC, Chung L, Coghlan JG et al. Long\u2010Term Outcomes in Patients With Connective Tissue Disease\u2013 Associated Pulmonary Arterial Hypertension in the Modern Treatment Era: Meta\u2010Analyses of Randomized, Controlled Trials and Observational Registries. Arthritis & Rheumatology. 2021;73(5):837\u201347. DOI: 10.1002\/art.41669","DOI":"10.1002\/art.41669"},{"key":"ref18","doi-asserted-by":"crossref","unstructured":"Qian J, Wang Y, Huang C, Yang X, Zhao J, Wang Q et al. Survival and prognostic factors of systemic lupus erythematosus-associated pulmonary arterial hypertension: A PRISMA-compliant systematic review and meta-analysis. Autoimmunity Reviews. 2016;15(3):250\u20137. DOI: 10.1016\/j.autrev.2015.11.012","DOI":"10.1016\/j.autrev.2015.11.012"},{"key":"ref19","doi-asserted-by":"crossref","unstructured":"Hassan AB, Hozayen RF, Mustafa ZS, Lundberg IE, Jahrami HA. The prevalence of pulmonary arterial hypertension in patients with mixed connective tissue disease: a systematic review and meta-analysis. Clinical and Experimental Rheumatology. 2023;41(11):2301\u201311. DOI: 10.55563\/clinexprheumatol\/srma43","DOI":"10.55563\/clinexprheumatol\/srma43"},{"key":"ref20","doi-asserted-by":"crossref","unstructured":"Distler O, Ofner C, Huscher D, Jordan S, Ulrich S, St\u00e4hler G et al. Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis. Rheumatology. 2024;63(4):1139\u201346. DOI: 10.1093\/rheumatology\/kead360","DOI":"10.1093\/rheumatology\/kead360"},{"key":"ref21","unstructured":"Chazova I.E., Avdeev S.N., Arutyunov G.P., Volkov A.V., Nakonechnikov S.N., Privalova E.V. Diagnosis and treatment of pulmonary hypertension. Russian recommendations. Cardiovascular therapy and prevention. 6(6 S2):503\u201322. https:\/\/scardio.ru\/content\/images\/recommendation\/lg.pdf"},{"key":"ref22","unstructured":"Chazova I.E., Avdeev S.N., Tsareva N.A., Volkov A.V., Martynyuk T.V., Nakonechnikov S.N. Clinical practice guidelines for the diagnosis and treatment of pulmonary hypertension. Therapeutic archive. 2014;86(9):4\u201323."},{"key":"ref23","doi-asserted-by":"crossref","unstructured":"Hachulla E, Jais X, Cinquetti G, Clerson P, Rottat L, Launay D et al. Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus. Chest. 2018;153(1):143\u201351. DOI: 10.1016\/j.chest.2017.08.014","DOI":"10.1016\/j.chest.2017.08.014"},{"key":"ref24","doi-asserted-by":"crossref","unstructured":"Coghlan JG, Gali\u00e8 N, Barber\u00e0 JA, Frost AE, Ghofrani H-A, Hoeper MM 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. Annals of the Rheumatic Diseases. 2017;76(7):1219\u201327. DOI: 10.1136\/annrheumdis-2016-210236","DOI":"10.1136\/annrheumdis-2016-210236"},{"key":"ref25","doi-asserted-by":"crossref","unstructured":"Hassan HJ, Naranjo M, Ayoub N, Housten T, Hsu S, Balasubramanian A et al. Improved Survival for Patients with Systemic Sclerosis\u2013 associated Pulmonary Arterial Hypertension: The Johns Hopkins Registry. American Journal of Respiratory and Critical Care Medicine. 2023;207(3):312\u201322. DOI: 10.1164\/rccm.202204-0731OC","DOI":"10.1164\/rccm.202204-0731OC"},{"key":"ref26","doi-asserted-by":"crossref","unstructured":"Erdogan M, Esatoglu SN, Kilickiran Avci B, Hatemi G. Treatment of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review and meta-analysis. Internal and Emergency Medicine. 2024;19(3):731\u201343. DOI: 10.1007\/s11739-024-03539-1","DOI":"10.1007\/s11739-024-03539-1"},{"key":"ref27","doi-asserted-by":"crossref","unstructured":"Lammi MR, Mathai SC, Saketkoo LA, Domsic RT, Bojanowski C, Furst DE et al. Association Between Initial Oral Therapy and Outcomes in Systemic Sclerosis\u2013Related Pulmonary Arterial Hypertension. Arthritis & Rheumatology. 2016;68(3):740\u20138. 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