Склеродермия системная. Хроническое заболевание системы соединительной ткани и мелких сосудов с распространенным поражением кожи (склероз, фиброз), соединительно-тканной основы внутренних органов. Причина неизвестна. Провоцируется охлаждением, травмой, инфекцией, вакцинацией и др. Имеет значение семейпо-гепетическое предрасположение. Женщины болеют в 3 раза чаще мужчин.

Симптомы и течение. Обычно болезнь начинается с синдрома Рейно (см. раздел 2, гл. 1), болей в суставах, похудания, повышения температуры тела, слабости, утомляемости. Самый характерный признак — поражение кожи. Вначале бывает плотный распространенный отек, в дальнейшем — уплотнение и атрофия кожи, особенно выраженные на лице и конечностях. Возможно появление изъязвлении и гнойничков на копчиках пальцев, длительно незаживающих, очень болезненных. Деформируются ногти, выпадают волосы вплоть до облысения. Болезненное уплотнение, а затем атрофия мышц сопровождается грубыми изменениями сухожилий: они укорачиваются, что приводит к развитию контрактур, нарушающих деятельность различных суставов. В них возникают боли, они деформируются. При рентгенологическом исследовании выявляется разрушение — остеолиз концевых, а в тяжелых случаях и средних фаланг рук, реже ног. В толще подкожной клетчатки могут откладываться глыбки кальция. Заболевание затрагивает и сердечно-сосудистую систему. Возникают боли в области сердца, одышка, различные нарушения сердечного ритма и проводимости. При иммунном воспалении сосудов может возникнуть гангрена, тромбофлебиты с трофическими язвами на стопах, голенях и др. Возможны тяжелые поражения внутренних органов: легких — ппевмофиброз, почек — «склеродермическая почка», диффузный гломерулопефрит (см.). Особенно характерно нарушение прохождения пищи по пищеводу, его расширение, выявляемое при рентгенологическом исследовании. Поражение нервной системы проявляется полиневритами, вегетативной неустойчивостью (нарушение потоотделения, терморегуляции), эмоциональной (раздражительность, плаксивость и мнительность), бессоницей. В редких случаях возникает энцефалит и психоз. Выделяют острое, подострое и хроническое течение заболевания. Изменения в крови неспецифичны. Снижается уровень гемоглобина, может увеличиваться число лейкоцитов, СОЭ.

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Лечение. Применяют большие дозы глюкокортикоидных гормонов (преднизолон), а также Д-пеницилламин, купренил, делагил. Для лечения синдрома Рейно — нифедипин (коринфар, кордафен, форидон). Необходима симптоматическая терапия: лидаза, витамины группы В, сосудорасширяющие, физиотерапия (хвойные, радоновые, сероводородные ванны, грязелечение, парафинолечение и т.д.), лечебная физкультура, массаж.