Author:
Volkov A. V.,Martynyuk T. V.
Abstract
Pulmonary arterial hypertension (PAH) is a complex, multidisciplinary problem of modern medicine. The basis for the disease is a microcirculatory lesion in the vessels, which leads to a decrease in their blood flow, to increased pulmonary vascular resistance and, as an outcome, right ventricular failure, and death. PAH may also be associated with systemic connective tissue diseases (SCTDs), in particular with systemic sclerosis (SS), as one of the manifestations of the disease. As well as idiopathic pulmonary hypertension, PAH-SCTDs is an orphan disease: according to the data of the V.A. Nasonova Research Institute of Rheumatology, the incidence of PAH is 5.2% in SS, 0.3% in systemic lupus erythematosus, and 7.4% in mixed connective tissue disease. Low prevalence is responsible for late diagnosis, which is always associated with a poor therapy response and poor prognosis. The purpose of the lecture is to acquaint rheumatologists with the possibilities of diagnosis and treatment of this rare, but prognostic severe manifestation of SCTDs.
Subject
Immunology,Immunology and Allergy,Rheumatology
Reference57 articles.
1. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62 Suppl 25:34-41. doi: 10.1016/j.jacc.2013.10.029
2. Galie N, Hoeper MM, Humbert M, 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 Heart J. 2016;37(1):67-119. doi: 10.1093/eurheartj/ehv317
3. Volkov AV, Martynyuk TV, Yudkina NN, et al. Survival of patients with pulmonary arterial hypertension associated with systemic scleroderma. Terapevticheskiy Arkhiv. 2012;(5):24-8 (In Russ.)
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 Dec;138(6):1383-94. doi: 10.1378/chest.10-0260
5. Avdeev SN, Akchurin RS, Ambat'ello LG, et al; Chazova IE, Martynyuk TV, editors. Legochnaya gipertenziya [Pulmonary hypertension]. Moscow: Praktika; 2015 (In Russ.)
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