Патогенетические особенности легочной гипертензии при системной склеродермии

Системные заболевания соединительной ткани могут быть связаны с различными формами легочной гипертензии (ЛГ). Распространенность ЛГ варьируется среди различных системных заболеваний соединительной ткани, при этом системная склеродермия (ССД) имеет самый высокий показатель и составляет от 8 до 12 %. при ССД ЛГ является одним из тяжелых и жизнеугрожающих проявлений, что обусловливает необходимость изучения данной проблемы. патогенез ЛГ при ССД различен и зависит от преимущественного поражения органов. ЛГ может быть представлена легочной артериальной гипертензией (ЛАГ) (1-я группа) при изолированном поражении легочных сосудов или при более редких состояниях, таких как веноокклюзионная болезнь, портопульмональная гипертензия, или при лекарственной токсичности. при поражениях левых камер сердца, таких как фиброз миокарда, диастолическая дисфункция левого желудочка или патология клапанов, развивается венозная ЛГ (2-я группа). при интерстициальных заболеваниях легких, таких как неспецифическая и обычная интерстициальная пневмония, может возникать ЛГ, связанная с поражением легких (3-я группа). пациенты с ССД подвержены развитию тромбозов и тромбоэмболии легочной артерии в связи с образованием антифосфолипидных антител, что может привести к хронической тромбоэмболической ЛГ (4-я группа). Характерным для ССД является сочетание у одного пациента нескольких причин формирования ЛГ. Многообразие патогенетических вариантов ЛГ при ССД требует индивидуального и комплексного диагностического подхода к каждому пациенту. Именно от варианта и патогенеза ЛГ зависят дальнейшая тактика ведения больного и определение показаний к назначению ЛАГ-специфической терапии.

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