pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLCpulmo-760Research ArticleКЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИCLINICAL GUIDELINESИНТЕРСТИЦИАЛЬНАЯ ПНЕВМОНИЯ С АУТОИММУННЫМИ ПРОЯВЛЕНИЯМИ: СОВМЕСТНЫЙ КОНСЕНСУС ЕВРОПЕЙСКОГО РЕСПИРАТОРНОГО И АМЕРИКАНСКОГО ТОРАКАЛЬНОГО ОБЩЕСТВINTERSTITIAL PNEUMONIAE WITH AUTOIMMUNE MANIFESTATIONS. JOINT CONSENSUS OF THE ERS/ATSРедакционнаястатья201620122016265529538Copyright © Редакционная с., 20162016Редакционная с.Редакционная с.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.pulmonology.ru/pulm/article/view/760

У многих больных идиопатической интерстициальной пневмонией имеются клинические проявления аутоиммунного заболевания, не соответствующие критериям заболевания соединительной ткани (ЗСТ). Для характеристики таких больных предлагаются различные термины и критерии, а отсутствие единого мнения о терминологии и классификации подобных ситуаций не позволяет выполнять проспективные исследования сопоставимых групп больных. Для выработки консенсуса о терминологии и классификации этих случаев Европейским респираторным и Американским торакальным обществами создана Рабочая группа по недифференцированным формам ЗСТ, сопровождающихся интерстициальными заболеваниями легких, специалистами которой предложено ввести термин «интерстициальная пневмония с аутоиммунными проявлениями» (ИПАП) и диагностические критерии, основанные на сочетании 3 групп признаков: клинических (специфические экстраторакальные симптомы), серологических (специфические аутоантитела) и морфологических (специфические проявления при визуализационных, гистологических исследованиях или исследовании физиологии дыхания). По решению экспертов термин «ИПАП» должен применяться к больным интерстициальными пневмониями и симптомами, позволяющими заподозрить ЗСТ, но не подтверждающими его. Введение нового термина позволит в будущем вовлекать в исследования более однородные когорты больных.

Some patients with idiopathic interstitial pneumonias (IIPs) have extrathoracic signs and symptoms suggestive for coexisting autoimmune disease that, however, do not meet criteria for any connective tissue disease (CTD). Currently, there are no unique terms and criteria for these cases. The European Respiratory Society and the American Thoracic Society created the Task Force Group on Undifferentiated Forms of CTD Associated with Interstitial Lung Disease in order to develop consensus on terminology and diagnostic criteria for patients with IIP and clinical features of an autoimmune disorder. The Task Force created the term «interstitial pneumonia with autoimmune features (IPAF)» and diagnostic criteria based on three groups of signs: clinical signs including specific clinical extrathoracic features; serological features including specific autoantibodies and morphological features including imaging, histology and respiratory physiology. Experts supposed that the term IPAF should facilitate identification patients with IIP and specific autoimmune signs but without a definitive CTD. This term should also provide involvement a uniform cohort of patients in clinical studies.

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