pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2022-32-2-199-207pulmo-3851Research ArticleОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯORIGINAL STUDIESХронические фиброзирующие интерстициальные заболевания легких по данным регистра больных, проживающих в Иркутске (Россия)Chronic fibrosing interstitial lung diseases according to the register of Irkutsk (Russia)https://orcid.org/0000-0003-4340-4278НашатыреваМ. С.NashatyrevaM. S.

Нашатырева Мария Сергеевна – аспирант кафедры клинической аллергологии и пульмонологии

Иркутск, мкр Юбилейный, 100

Maria S. Nashatyreva, Postgraduate Student, Department of Clinical Allergology and Pulmonology

mkr Yubilejnyy 100, Irkutsk, 664079, Russia

volga-89@list.ruhttps://orcid.org/0000-0003-2742-3794ТрофименкоИ. Н.TrofimenkoI. N.

Трофименко Ирина Николаевна – доктор медицинских наук, заведующая кафедрой клинической аллергологии и пульмонологии

Иркутск, мкр Юбилейный, 100

Irina N. Trofimenko, Doctor of Medicine, Head of the Department of Clinical Allergology and Pulmonology

mkr Yubilejnyy 100, Irkutsk, 664079, Russia

tin11@mail.ruhttps://orcid.org/0000-0002-5902-6198ЧернякБ. А.ChernyakB. A.

Черняк Борис Анатольевич – доктор медицинских наук, профессор кафедры клинической аллергологии и пульмонологии

Иркутск, мкр Юбилейный, 100

Boris A. Chernyak, Doctor of Medicine, Professor, Department of Clinical Allergology and Pulmonology

mkr Yubilejnyy 100, Irkutsk, 664079, Russia

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 Education – Branch Campus of the Federal State Budgetary Educational Institution of Further Professional Education “Russian Medical Academy of Continuing Professional Education”, Healthcare Ministry of the Russian FederationRussian Federation202202052022322199207Copyright © Нашатырева М.С., Трофименко И.Н., Черняк Б.А., 20222022Нашатырева М.С., Трофименко И.Н., Черняк Б.А.Nashatyreva M.S., Trofimenko I.N., Chernyak B.A.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/3851

Интерстициальные заболевания легких (ИЗЛ) – обширная гетерогенная группа нозологий, различных по многим параметрам, но имеющих близкие патофизиологические механизмы развития воспаления и / или фиброза легких. Идиопатический легочный фиброз (ИЛФ), неспецифическая интерстициальная пневмония (НСИП), хронический гиперчувствительный пневмонит (ХГП), ИЗЛ, связанные с аутоиммунными нарушениями (ассоциированные преимущественно с системной склеродермией (ССД) и ревматоидным артритом (РА)), пневмокониозы и некоторые другие заболевания могут сопровождаться формированием легочного фиброза (ЛФ), течение которого в ряде случаев прогрессирует на фоне проводимой традиционной противовоспалительной терапии. В настоящее время общепринятые критерии диагностики фиброзирующих и прогрессирующих фиброзирующих ИЗЛ отсутствуют, а сведения о частоте этих состояний ограничены.

Целью исследования явилось изучение структуры фиброзирующих ИЗЛ и частоты прогрессирующего фиброзирующего фенотипа (ПФФ) по данным регистра больных ИЗЛ (n = 270), проживающих в Иркутске (Россия).

Материалы и методы

Материалы и методы. На 1-м этапе исследования во все лечебные учреждения Иркутска разосланы информационные письма с рекомендациями о направлении на консультацию всех пациентов с ИЗЛ. На 2-м этапе каждый случай ИЗЛ верифицировался коллегиально. По данным компьютерной томографии высокого разрешения (КТВР) у всех больных (n = 270) проанализированы клинико-анамнестические, функциональные и гистологические данные, на основании которых сформирован регистр пациентов с ИЗЛ. Срок наблюдения составил 1–5 лет.

Результаты

Результаты. По результатам комплексной оценки по данным КТВР признаки ЛФ выявлены у 104 (38,5 %) пациентов. Фиброзирующее течение отмечено у 100 % больных ИЛФ и ИЗЛ, ассоциированных с ССД, у 90,9 % – с ХГП, у 71,4 % – с НСИП, у 60 % – с РА. ПФФ диагностирован у 61 пациента (22,6 % всех случаев ИЗЛ и 58,6 % – фиброзирующих ИЗЛ). ПФФ отмечен у 100 % пациентов с ИЛФ; 5-летний уровень смертности при ИЗЛ с ПФФ составил 55,5 %.

Заключение

Заключение. В случае ПФФ эволюция ИЗЛ утрачивает нозологическую специфичность, приобретая сходство с ИЛФ. Учитывая высокий уровень смертности, с целью своевременного назначения антифибротической терапии необходимо активно и как можно раньше выявлять ПФФ широкого спектра ИЗЛ.

The Interstitial lung diseases (ILD) are a wide heterogeneous group of disorders, different in many parameters, however having similar pathophysiological mechanisms. Idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), chronic hypersensitivity pneumonitis (CHP), ILD associated with autoimmune disorders (predominantly systemic sclerosis (SSc ILD) and rheumatoid arthritis (RA ILD), and other diseases may be accompanied by the formation of pulmonary fibrosis. Currently there are no generally accepted criteria for the fibrosing and progressive fibrosing ILD, and the information on the frequency of these conditions is limited.

The study aims to research the structure of fibrosing ILD and the frequency of the progressive fibrosing phenotype (PFP) according to the register of patients with ILD in Irkutsk, Russia.

Methods

Methods. At the first stage information letters were sent to all medical institutions of Irkutsk with recommendations on all referral patients with ILD for further examination. At the second stage, each ILD case was verified collegially (n = 270). All patients’ data were analysed in terms of clinical, functional, radiological and histological aspects. A register of ILD patients was formed based on this analysis. The follow-up period was 1 to 5 years.

Results

Results. According to the results of a comprehensive assessment, HRCT signs of pulmonary fibrosis were detected by 104 patients (38.5%). fibrosing course was observed in 100% IPF and SSc ILD, in 90.9% of CHP, 71.4% of NSIP and 60% of RA ILD. 61 patients (22.6% of all ILD cases, 58.6% of fibrosing ILD) was diagnosed with PFP. The 5-year mortality for PFP ILD was 55.5%.

Conclusion

Conclusion. In the case of a PFP development, the evolution of ILD loses its nosological specificity, acquiring similarity with IPF. Due to the high mortality rate, it is necessary to actively identify the progressive fibrosing course of a wide range of ILD and to prescribe an antifibrotic therapy in a timely manner.

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