Современные представления о гетерогенности поражений почек у больных склеродермией

Системный склероз, или системная склеродермия (ССД), – аутоиммунное заболевание соединительной ткани, основные клинические признаки которого обусловлены распространенными нарушениями микроциркуляции, фиброзом кожи и внутренних органов. По данным морфологических исследований, у 80% пациентов с ССД выявляются изменения почек, в том числе не связанные с ревматическими заболеваниями. В то время как распространенность склеродермического почечного криза в настоящее время оценивается в 2–5%, гораздо чаше отмечается асимптоматическое снижение почечной функции («немая уремия»), обусловленное наличием мультиморбидной и коморбидной патологии. Его частота у пациентов с ССД может достигать 55%. Факт наличия аутоиммунного заболевания соединительной ткани сам по себе может рассматриваться как фактор риска повреждения почек. Пятнадцатилетняя выживаемость больных ССД без поражения почек составляет 72%, при наличии поражения почек – не более 13%. Наличие протеинурии у пациентов с ССД является одним из наиболее значимых независимых факторов риска летальных исходов (относительный риск составляет 3,34), оставляя далеко позади такие канонические факторы риска, как легочная артериальная гипертензия, легочная рестрикция (соотношение объема форсированного выдоха за 1 с и форсированной жизненной емкости легких <80%), дыхательная недостаточность
(III и IV класса по NYHA), а также снижение диффузионной способности легких и высокий кожный счет. Авторы впервые предлагают концепцию существования и патогенеза хронической склеродермической нефропатии, в основе которой лежит феномен сосудистой эндотелиальной дисфункции, формирующийся в различных структурных компонентах нефрона и почки в целом.

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