Clinical and hemodynamic characteristics and possibilities for therapy in patients with severe (functional class IV) pulmonary arterial hypertension associated with diffuse connective tissue diseases

Ev, Nikolaeva; Yudkina, N.; Курмуков, И. А.; Ds, Novikova; Ei, Markelova; Av, Volkov

doi:10.17116/terarkh201587524-32

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классификация легочной гипертензии (ницца1

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2018

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Cited by 2 publications

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“…Учитывая, что ССД, ассоциированная с ЛАГ, имеет маломанифестную, стертую клиническую симптоматику, диагноз как ССД, так и ЛАГ устанавливается поздно, что является причиной старта ЛАГ-специфической терапии в III, а нередко и в IV ФК [14].…”

Section: Discussionunclassified

Up-Front Triple Combination Therapy for Pulmonary Arterial Hypertension Associated With Systemic Sclerosis: A Case Report

Волков¹,

Юдкина²,

Николаева³

et al. 2018

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Section: Discussionunclassified

Up-Front Triple Combination Therapy for Pulmonary Arterial Hypertension Associated With Systemic Sclerosis: A Case Report

Волков¹,

Юдкина²,

Николаева³

et al. 2018

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“…Добутамин, как и другие инотропные препараты, у больных с ЛГ рекомендован при IV ФК и в терминальной стадии заболевания в качестве инотропной поддержки [2,30,31].…”

Section: классификация легочной гипертензии (ниццаunclassified

Pulmonary Arterial Hypertension in Systemic Connective Tissue Diseases: The Current State of the Problem

Волков¹,

Мартынюк²

2018

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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.

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Clinical and hemodynamic characteristics and possibilities for therapy in patients with severe (functional class IV) pulmonary arterial hypertension associated with diffuse connective tissue diseases

Cited by 2 publications

References 21 publications

Up-Front Triple Combination Therapy for Pulmonary Arterial Hypertension Associated With Systemic Sclerosis: A Case Report

Up-Front Triple Combination Therapy for Pulmonary Arterial Hypertension Associated With Systemic Sclerosis: A Case Report

Pulmonary Arterial Hypertension in Systemic Connective Tissue Diseases: The Current State of the Problem

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