Abstract
Pulmonary hypertension (PH) associated with scleroderma systematica (SDS) is a menacing manifestation of this systemic disease of connective tissue, in which a rapid progression results in very poor outcomes. In SDS, PH is more frequently observed with the prolonged disease, circumscribed skin lesion, develops after a long benign course, and is one of the common causes of death. The early stage of PH can be identified by instrumental and not always accessible studies. The stage of clinical manifestations, which is frequently manifested only by dyspnea, requires a differential diagnosis from a wide range of conditions both caused by and concurrent with SDS. The need for differential diagnosis stems from the varying course and prognosis of the disease, as well as treatment policy.
Highlights
Pulmonary hypertension (PH) associated with scleroderma systematica (SDS) is a menacing manifestation of this systemic disease of connective tissue, in which a rapid progression results in very poor outcomes
In SDS, PH is more frequently observed with the prolonged disease, circumscribed skin lesion, develops after a long benign course, and is one of the common causes of death
The need for differential diagnosis stems from the varying course and prognosis of the disease, as well as treatment policy
Summary
Ассоциированная с системной склеродермией (ССД), — грозное проявление этого заболевания соединительной ткани, при котором быстрое прогрессирование приводит к крайне неутешительным исходам. The stage of clinical manifestations, which is frequently manifested only by dyspnea, requires a differential diagnosis from a wide range of conditions both caused by and concurrent with SDS. ЛАГ, ассоциированная с системной склеродермией (ССД), широко известна как грозное проявление этого заболевания соединительной ткани, быстрое прогрессирование которого приводит к крайне неутешительным исходам. ЛАГ при ССД чаще возникает на фоне большой длительности заболевания, лимитированного кожного синдрома и наличия антицентромерных антител, однако последние исследования внесли корректировку в общеизвестные закономерности. Диагностика ЛГ при ССД, как и при других состояниях, основана на комплексе клинических и инструментальных исследований, среди которых золотым стандартом является определение давления в легочной артерии при ее ка-
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