Одностороннее поражение легких при системной склеродермии (описание случая)

Системная склеродермия (ССД) – клинически гетерогенное заболевание, которое характеризуется облитерирующей микроангиопатией, аутоиммунной активацией и фиброзом кожи и внутренних органов. Интерстициальный фиброз легких (ИФЛ) является характерным висцеральным поражением при ССД и считается одной из основных причин инвалидизации и смертности больных. Диагноз ИФЛ, ассоциированного с ССД, устанавливается на основе совокупности симптомов, данных физикального обследования, изменений функции внешнего дыхания и компьютерной томографии высокого разрешения. Фиброз легких включен в классификационные критерии Американской коллегии ревматологов (ACR) 1980 г. и в совместные диагностические критерии ACR и Европейской антиревматической лиги 2013 г. Согласно данному в этих критериях определению, фиброз легких при ССД описывается как двусторонние изменения, наиболее выраженные в базальных отделах легких, которые не являются признаком первичного заболевания легких. В представленном случае описывается наблюдение больной ССД с полным спектром характерных признаков фиброза легких, которые носили односторонний характер. Это случай является первым описанием одностороннего ИФЛ при ССД и указывает на необходимость исключения заболеваний соединительной ткани, в первую очередь ССД, при выявлении подобных изменений в легких в сочетании с внелегочными проявлениями.

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