pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2019-29-5-604-611pulmo-1230Research ArticleОБЗОРЫREVIEWПоражения легких при системных заболеваниях соединительной тканиLung lesions in connective tissue diseasesТрофименкоИ. Н.TrofimenkoI. N.

Трофименко Ирина Николаевна – доктор медицинских наук, доцент кафедры клинической аллергологии  и пульмонологии.

664079,  Иркутск,  мкрн Юбилейный,  100.

тел.: (9148) 77-80-96

Irina N. Trofimenko - Doctor  of Medicine,  Associate Professor,  Department of Clinical  Allergology and  Pulmonology,  Irkutsk  State  Medical  Academy  of Postgraduate  Training  – a branch  of Russian Federal  Academy of Continued Medical  Education, Healthcare Ministry of Russia.

mkrn Yubileynyy 100, Irkutsk, 664079.

tin11@mail.ruЧернякБ. А.ChernyakB. A.

Черняк Борис Анатольевич – доктор медицинских наук, профессор, заведующий кафедрой  клинической аллергологии  и пульмонологии.

664079,  Иркутск,  мкрн Юбилейный,  100.

тел.: (9025) 68-78-70

Boris A. Chernyak - Doctor  of Medicine,  Professor,  Head  of Department of Clinical Allergology and Pulmonology,  Irkutsk State Medical  Academy of Post-graduate  Training  – a branch  of Russian  Federal  Academy  of Continued Medical  Education, Healthcare Ministry  of Russia.

mkrn Yubileynyy 100, Irkutsk, 664079.

ba.chernyak@gmail.comИркутская государственная медицинская академия последипломного образования – филиал Федерального государственного бюджетного образовательного учреждения дополнительного профессионального образования «Российская медицинская академия непрерывного профессионального образования» Министерства здравоохранения Российской ФедерацииРоссияIrkutsk State Medical Academy of Postgraduate Training – a branch of Russian Federal Academy of Continued Medical Education, Healthcare Ministry of RussiaRussian Federation201925122019295604611Copyright © Трофименко И.Н., Черняк Б.А., 20192019Трофименко И.Н., Черняк Б.А.Trofimenko I.N., Chernyak B.A.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.pulmonology.ru/pulm/article/view/1230

Частота и варианты  поражения органов дыхания при системных  заболеваниях  соединительной ткани (СЗСТ) существенно  различаются, оказывая  значительное влияние  на их клинические особенности и прогноз.  Легочные  поражения встречаются  у 20–95  % больных СЗСТ,  при этом состояния могут различаться – от бессимптомных до жизнеугрожающих, вплоть до летального исхода. Нередко осложнения, обусловленные интерстициальным заболеванием легких (ИЗЛ), на фоне СЗСТ  становятся  ведущими  в определении прогноза у больных.  Интерстициальные пневмонии являются  самым  частым вариантом  поражения респираторной системы  при СЗСТ.  Среди всех ИЗЛ на долю СЗСТ-ассоциированных приходится от 15 до 25 % случаев. Чаще поражение  органов дыхания формируется в период развернутой  клинической картины  СЗСТ.  Однако  в некоторых  случаях СЗСТ  могут дебютировать  как ИЗЛ,  предшествуя  системным проявлениям болезни,  что существенно  затрудняет раннюю нозологическую диагностику.  В таких случаях физикальное обследование пульмонологом должно включать поиск возможных  экстрапульмональных проявлений СЗСТ.  Кроме того, неотъемлемой частью диагностического алгоритма  является  специфическое лабораторное  обследование  – определение уровня  аутоантител,  которое  при сопоставлении  с клинической картиной обеспечивает  высокую степень вероятности диагноза СЗСТ.

Frequency and clinical manifestations of respiratory disease in systemic connective tissue diseases (CTD) differ significantly including clinical course and outcomes. Pulmonary  abnormalities  are seen in 20% to 95% of patients with CTD ranging from subtle to life-threatening disease and could be fatal. Commonly, CTD-related interstitial lung disease (ILD)  is crucial for prognosis. Interstitial  pneumonias  are the most frequent variant of respiratory disease in patients with CTD.  CTD-related ILDs comprise 15% to 25% of all ILDs. Usually, respiratory disease develops in symptomatic CTD,  but in some cases, ILD can be the first appearance  of CTD which precedes systemic symptoms that could significantly impede early diagnosis. Therefore,  diagnostic workup in ILD should consider extrapulmonary  manifestations  of CTD.  Moreover,  an intrinsic part of diagnostic algorithm should be specific laboratory assessment including measurement  of autoantibodies.  Combination of specific antibodies and clinical features provides higher probability of CTD diagnosis.

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The authors declare that there are no conflicts of interest present.