Цель. Изучить демографические, клинико-серологические и морфологические признаки интерстициальной пневмонии с аутоиммунными признаками (ИПАП), сравнить выживаемость при ИПАП и интерстициальных заболеваниях легких при системных заболеваниях соединительной ткани (СЗСТ-ИЗЛ), а также выявить предикторы смертности и развития СЗСТ в группе ИПАП. Материалы и методы. Группа ИПАП включала 48 пациентов (75,0% женщин, медиана возраста 57,5 года), СЗСТ-ИЗЛ – 49 пациентов (79,6% женщин, медиана возраста 60,0 года). Проводились анализ демографических, клинико-лабораторных и инструментальных данных, а также сравнение выживаемости с использованием метода Каплана–Мейера и логарифмического рангового теста в группах ИПАП и СЗСТ-ИЗЛ. В группе ИПАП исследовались предикторы смертности и развития СЗСТ с использованием многофакторного регрессионного анализа. Результаты. Длительность наблюдения за пациентами достоверно не отличалась в группах ИПАП и СЗСТ-ИЗЛ (40,0 и 37,0 мес соответственно). Клинические критерии в группе ИПАП отмечались у 25 (52,1%) пациентов, серологические – у 44 (91,7%), морфологические – у 44 (91,7%). Смертность в группе ИПАП оказалась достоверно выше по сравнению с группой СЗСТ-ИЗЛ (29,2 и 6,1% соответственно; p=0,023). Независимыми предикторами смертности в группе ИПАП стали наличие сахарного диабета, компьютерно-томографического паттерна обычной интерстициальной пневмонии и исходное снижение форсированной жизненной емкости легких. В течение периода наблюдения развитие СЗСТ отмечалось у 4 (8,3%) пациентов с ИПАП. Независимым предиктором развития СЗСТ стало только исходное повышение уровня С-реактивного белка. Заключение. ИПАП характеризуется менее благоприятным прогнозом жизни по сравнению с СЗСТ-ИЗЛ и относительно низкой частотой развития СЗСТ.
Aim. To study demographic, clinical, serological and morphological features of interstitial pneumonia with autoimmune features (IPAF), compare survival in IPAF and interstitial lung disease in connective tissue diseases (CTD-ILD), and identify predictors of mortality and transformation to CTD in the IPAF group. Materials and methods. The IPAF group included 48 patients (75.0% women, median age 57.5 years), CTD-ILD – 49 patients (79.6% women, median age 60.0 years). The analysis of demographic, clinical, laboratory and instrumental data was performed, as well as comparison of survival with the Kaplan–Meier method and the log-rank test in the IPAF and CTD-ILD groups. In the IPAF group, predictors of mortality and the development of CTD were studied with multivariate regression analysis. Results. Duration of observation period did not differ significantly in the IPAF and CTD-ILD groups (40.0 and 37.0 months, respectively). Clinical criteria of IPAF were observed in 25 (52.1%) patients, serological – in 44 (91.7%), morphological – in 44 (91.7%). Mortality in the IPAF group was significantly higher than in the CTD-ILD group (29.2 and 6.1%, respectively; p=0.023). The presence of diabetes mellitus, CT-pattern of usual interstitial pneumonia, and an initial low forced vital capacity value were independent predictors of mortality in the IPAF group. During the observation period, the development of CTD was noted in 4 (8.3%) patients with IPAF. The independent predictor of the CTD development was the increased C-reactive protein level. Conclusion. IPAF is characterized by a lower survival rate compared to CTD-ILD, and a relatively low risk of CTD transformation.
1. Fischer A, Antoniou KM, Brown KK, et al. ERS/ATS Task Force on Undifferentiated Forms of CTD-ILD. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46(4):976-87. DOI:10.1183/13993003.00150-2015
2. Mackintosh JA, Wells AU, Cottin V, et al. Interstitial pneumonia with autoimmune features: challenges and controversies. Eur Respir Rev. 2021;30(162):210177. DOI:10.1183/16000617.0177-2021
3. Joerns EK, Adams TN, Sparks JA, et al. Interstitial Pneumonia with Autoimmune Features: What the Rheumatologist Needs to Know. Curr Rheumatol Rep. 2022;24(6):213-26. DOI:10.1007/s11926-022-01072-8
4. Chartrand S, Swigris JJ, Stanchev L, et al. Clinical features and natural history of interstitial pneumonia with autoimmune features: A single center experience. Respir Med. 2016;119:150-4. DOI:10.1016/j.rmed.2016.09.002
5. Kim HC, Lee JH, Chae EJ, et al. Long-term clinical course and outcome of interstitial pneumonia with autoimmune features. Respirology. 2020;25(6):636-43. DOI:10.1111/resp.13665
6. Sebastiani M, Cassone G, De Pasquale L, et al. Interstitial pneumonia with autoimmune features: A single center prospective follow-up study. Autoimmun Rev. 2020;19(2):102451. DOI:10.1016/j.autrev.2019.102451
7. Karampeli M, Thomas K, Flouda S, et al. Interstitial Pneumonia with Autoimmune Features (IPAF): A Single-Centre, Prospective Study. Mediterr J Rheumatol. 2020;31(3):330-6. DOI:10.31138/mjr.31.3.330
8. Finckh A, Alpizar-Rodriguez D, Roux-Lombard P. Value of Biomarkers in the Prevention of Rheumatoid Arthritis. Clin Pharmacol Ther. 2017;102(4):585-7. DOI:10.1002/cpt.727
9. Oldham JM, Adegunsoye A, Valenzi E, et al. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 2016;47(6):1767-75. DOI:10.1183/13993003.01565-2015
10. Sambataro G, Sambataro D, Torrisi SE, et al. Clinical, serological and radiological features of a prospective cohort of Interstitial Pneumonia with Autoimmune Features (IPAF) patients. Respir Med. 2019;150:154-60. DOI:10.1016/j.rmed.2019.03.011
11. Ito Y, Arita M, Kumagai S, et al. Serological and morphological prognostic factors in patients with interstitial pneumonia with autoimmune features. BMC Pulm Med. 2017;17(1):111. DOI:10.1186/s12890-017-0453-z
12. Аверьянов А.В., Лесняк В.Н. От идиопатического фиброзирующего альвеолита к идиопатическому легочному фиброзу. Ч.2. Клиническая практика. 2016;7(4):58-65 [Averyanov AV, Lesnyak VN. From idiopathic fibrosing alveolitis to idiopatic lungs fibrosis. Clinpractice. 2016;7(4):58-65 (in Russian)]. DOI:10.17816/clinpract7458-65
13. Ahmad K, Barba T, Gamondes D, et al. Interstitial pneumonia with autoimmune features: Clinical, radiologic, and histological characteristics and outcome in a series of 57 patients. Respir Med. 2017;123:56-62. DOI:10.1016/j.rmed.2016.10.017
14. Авдеев С.Н. Идиопатический легочный фиброз. Пульмонология. 2015;25(5):600-12 [Avdeev SN. Idiopathic pulmonary fibrosis. Pulmonology. 2015;25(5):600-12 (in Russian)]. DOI:10.18093/0869-0189-2015-25-5-600-612
15. Suzuki A, Kondoh Y, Fischer A. Recent advances in connective tissue disease related interstitial lung disease. Expert Rev Respir Med. 2017;11(7):591-603. DOI:10.1080/17476348.2017.1335600
16. Tashkin DP, Elashoff R, Clements PJ, et al. Scleroderma Lung Study Research Group. Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med. 2007;176(10):1026-34. DOI:10.1164/rccm.200702-326OC
17. Tashkin DP, Roth MD, Clements PJ, et al. Sclerodema Lung Study II Investigators. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4(9):708-19. DOI:10.1016/S2213-2600(16)30152-7
18. Fujisawa T. Management of Myositis-Associated Interstitial Lung Disease. Medicina (Kaunas). 2021;57(4):347. DOI:10.3390/medicina57040347
19. Romero-Bueno F, Diaz Del Campo P, Trallero-Araguás E, et al. Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease. Semin Arthritis Rheum. 2020;50(4):776-90. DOI:10.1016/j.semarthrit.2020.03.007
20. Hazarika K, Sahoo RR, Mohindra N, et al. Clinical, radiologic and serologic profile of patients with interstitial pneumonia with autoimmune features: a cross-sectional study. Rheumatol Int. 2022;42(8):1431-41. DOI:10.1007/s00296-021-04883-7
21. Li Y, Zheng Z, Han Q, et al. IPAF should receive early treatment for sharing similar clinical characteristics as CTD-ILD: a report from 273 Chinese patients. Clin Rheumatol. 2020;39(12):3817-23. DOI:10.1007/s10067-020-05149-6
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1. Fischer A, Antoniou KM, Brown KK, et al. ERS/ATS Task Force on Undifferentiated Forms of CTD-ILD. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46(4):976-87. DOI:10.1183/13993003.00150-2015
2. Mackintosh JA, Wells AU, Cottin V, et al. Interstitial pneumonia with autoimmune features: challenges and controversies. Eur Respir Rev. 2021;30(162):210177. DOI:10.1183/16000617.0177-2021
3. Joerns EK, Adams TN, Sparks JA, et al. Interstitial Pneumonia with Autoimmune Features: What the Rheumatologist Needs to Know. Curr Rheumatol Rep. 2022;24(6):213-26. DOI:10.1007/s11926-022-01072-8
4. Chartrand S, Swigris JJ, Stanchev L, et al. Clinical features and natural history of interstitial pneumonia with autoimmune features: A single center experience. Respir Med. 2016;119:150-4. DOI:10.1016/j.rmed.2016.09.002
5. Kim HC, Lee JH, Chae EJ, et al. Long-term clinical course and outcome of interstitial pneumonia with autoimmune features. Respirology. 2020;25(6):636-43. DOI:10.1111/resp.13665
6. Sebastiani M, Cassone G, De Pasquale L, et al. Interstitial pneumonia with autoimmune features: A single center prospective follow-up study. Autoimmun Rev. 2020;19(2):102451. DOI:10.1016/j.autrev.2019.102451
7. Karampeli M, Thomas K, Flouda S, et al. Interstitial Pneumonia with Autoimmune Features (IPAF): A Single-Centre, Prospective Study. Mediterr J Rheumatol. 2020;31(3):330-6. DOI:10.31138/mjr.31.3.330
8. Finckh A, Alpizar-Rodriguez D, Roux-Lombard P. Value of Biomarkers in the Prevention of Rheumatoid Arthritis. Clin Pharmacol Ther. 2017;102(4):585-7. DOI:10.1002/cpt.727
9. Oldham JM, Adegunsoye A, Valenzi E, et al. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 2016;47(6):1767-75. DOI:10.1183/13993003.01565-2015
10. Sambataro G, Sambataro D, Torrisi SE, et al. Clinical, serological and radiological features of a prospective cohort of Interstitial Pneumonia with Autoimmune Features (IPAF) patients. Respir Med. 2019;150:154-60. DOI:10.1016/j.rmed.2019.03.011
11. Ito Y, Arita M, Kumagai S, et al. Serological and morphological prognostic factors in patients with interstitial pneumonia with autoimmune features. BMC Pulm Med. 2017;17(1):111. DOI:10.1186/s12890-017-0453-z
12. Averyanov AV, Lesnyak VN. From idiopathic fibrosing alveolitis to idiopatic lungs fibrosis. Clinpractice. 2016;7(4):58-65 (in Russian). DOI:10.17816/clinpract7458-65
13. Ahmad K, Barba T, Gamondes D, et al. Interstitial pneumonia with autoimmune features: Clinical, radiologic, and histological characteristics and outcome in a series of 57 patients. Respir Med. 2017;123:56-62. DOI:10.1016/j.rmed.2016.10.017
14. Авдеев С.Н. Идиопатический легочный фиброз. Пульмонология. 2015;25(5):600-12 [Avdeev SN. Idiopathic pulmonary fibrosis. Pulmonology. 2015;25(5):600-12 (in Russian)]. DOI:10.18093/0869-0189-2015-25-5-600-612
15. Suzuki A, Kondoh Y, Fischer A. Recent advances in connective tissue disease related interstitial lung disease. Expert Rev Respir Med. 2017;11(7):591-603. DOI:10.1080/17476348.2017.1335600
16. Tashkin DP, Elashoff R, Clements PJ, et al. Scleroderma Lung Study Research Group. Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med. 2007;176(10):1026-34. DOI:10.1164/rccm.200702-326OC
17. Tashkin DP, Roth MD, Clements PJ, et al. Sclerodema Lung Study II Investigators. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4(9):708-19. DOI:10.1016/S2213-2600(16)30152-7
18. Fujisawa T. Management of Myositis-Associated Interstitial Lung Disease. Medicina (Kaunas). 2021;57(4):347. DOI:10.3390/medicina57040347
19. Romero-Bueno F, Diaz Del Campo P, Trallero-Araguás E, et al. Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease. Semin Arthritis Rheum. 2020;50(4):776-90. DOI:10.1016/j.semarthrit.2020.03.007
20. Hazarika K, Sahoo RR, Mohindra N, et al. Clinical, radiologic and serologic profile of patients with interstitial pneumonia with autoimmune features: a cross-sectional study. Rheumatol Int. 2022;42(8):1431-41. DOI:10.1007/s00296-021-04883-7
21. Li Y, Zheng Z, Han Q, et al. IPAF should receive early treatment for sharing similar clinical characteristics as CTD-ILD: a report from 273 Chinese patients. Clin Rheumatol. 2020;39(12):3817-23. DOI:10.1007/s10067-020-05149-6
1 ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия;
2 ФГБОУ ВО «Московский государственный университет им. М.В. Ломоносова», Москва, Россия
*akullar.ru@mail.ru
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Larisa A. Akulkina*1, Anastasia A. Shchepalina1, Alexey S. Moiseev1,2, Mikhail Yu. Brovko1, Victoria I. Sholomova1, Sergey V. Moiseev1,2
1 Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia;
2 Lomonosov Moscow State University, Moscow, Russia
*akullar.ru@mail.ru