Connective tissue disease related interstitial lung disease: a single center experience

Year 2023, Volume: 9 Issue: 6 - November 2023, 1360 - 1367, 04.11.2023

Tahsin Gezgin , Nurhan Atilla , Gözde Yıldırım Çetin , Burcu Akkök , Fulsen Bozkuş , Betül Kızıldağ , Ali Çulpan , Hasan Kahraman

https://doi.org/10.18621/eurj.1162806

Abstract

Objectives: We aimed to determine the clinical features of the patients followed by our hospital with the diagnosis of connective tissue disease related interstitial lung disease (CTD-ILD).

Methods: The study included 113 patients who were followed up with the diagnosis of CTD-ILD, admitted to Kahramanmaraş Sütçü İmam University, Faculty of Medicine, Rheumatology Department between January 2019 and December 2020. Demographic characteristics, laboratory data and high-resolution computed tomography (HRCT) patterns of the patients were recorded retrospectively.

Results: Of 113 patients diagnosed with CTD-ILD; 90 were female, 23 were male. When the distribution of connective tissue disease (CTD) evaluated; 50 were rheumatoid arthritis, 41 were systemic sclerosis, 8 were sjogren's syndrome, 4 were systemic lupus erythematosus, 7 were overlap syndrome and 3 were undifferentiated connective tissue disease. There was no statistically significant difference between laboratory parameters. In the HRCT evaluation, 86 patients had nonspecific interstitial pneumonia (NSIP), 25 patients had usual interstitial pneumonia (UIP), and 2 patients had lymphocytic interstitial pneumonia (LIP) pattern. The most common pattern, including rheumatoid arthritis, was NSIP.

Conclusions: In our study, as inconsistent with the literature the most common pattern in RA patients was found to be NSIP.

Keywords

Interstitial lung disease, connective tissue disease, radiological pattern

References

• 1. Solomon JJ, Fischer A. Connective tissue disease-associated interstitial lung disease: a focused review. J Intensive Care Med 2015;30:392-400.
• 2. Crestani B. The respiratory system in connective tissue disorders. Allergy 2005;60:715-73.
• 3. Antoniou KM, Margaritopoulos F, Economidio F, Siafakas NM. Pivotal clinical dilemmas in collogen vascular diseases associated with interstitial lung involvement. Eur Respir J 2009;33:882-96.
• 4. Travis WD. ATS/ERS. International multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2002;165:277-304.
• 5. American Thoracic Society, European Respiratory Society, American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 2002;165:277-304.
• 6..Shou AY, Salvatore MM. CT predictors of outcomes in patients with connective tissue disease and progressive lung fibrosis. Clin Imaging 2023;94:42-9.
• 7. Yoo H, Hino T, Han J, Franks TJ, Im Y, Hatabu H, et al. Connective tissue disease-related interstitial lung disease (CTD-ILD) and interstitial lung abnormality (ILA): evolving concept of CT findings, pathology and management. Eur J Radiol Open 2020;16;8:100311.
• 8. Zhang Y, Li H, Wu N, Dong X, Zheng Y. Retrospective study of the clinical characteristics and risk factors of rheumatoid arthritis-associated interstitial lung disease. Clin Rheumatol 2017;36:817-23.
• 9. Jayasinghe P, Wickramasinghe N, Krishnakumar P, Sadikeen A, Fernando A. Characteristics of connective tissue diseases associated interstitial lung diseases-a single centre study in Sri Lanka. Ceylon Med J 2021;66:38-43.
• 10. Li L, Liu R, Zhang Y, Zhou J, Li Y, Xu Y, et al. A retrospective study on the predictive implications of clinical characteristics and therapeutic management in patients with rheumatoid arthritis-associated interstitial lung disease. Clin Rheumatol 2020;39:1457-70.
• 11. Hyldgaard C. A cohort study of Danish patients with interstitial lung diseases: burden, severity, treatment and survival. Dan Med J 2015;62:B5069.
• 12. Musellim B, Okumus G, Uzaslan E, Akgün M, Cetinkaya E, Turan O, et al. Turkish interstitial lung diseases group epidemiology and distribution of interstitial lung diseases in Turkey. Clin Respir J 2014;8:55-62.
• 13. Keng Chen, Adrian S, Stephen S. Epidemiology and pathogenesis of scleroderma. Aust J Dermatol 2003;44:1-9.
• 14. Bryson T, Sundaram B, Khanna D, Kazerooni EA. Connective tissue disease-associated interstitial pneumonia and idiopathic interstitial pneumonia: similarity and difference. Semin Ultrasound CT MR 2014;35:29-38.
• 15. Weyand CM, Schmidt D, Wagner U. The influence of sex on the phenotype of rheumatoid arthritis. Arthritis Rheum 1998;41:817-22.
• 16. Kelly CA, Saravanan V, Nisar M. British Rheumatoid Interstitial Lung (BRILL) Network. Rheumatoid arthritis related interstitial lung disease: associations, prognostic factors and physiological and radiological characteristics-a large multicentre UK study. Rheumatology (Oxford) 2014;53:1676-82.
• 17. Zhang Y, Li H, Wu N, Dong X, Zheng Y. Retrospective study of the clinical characteristics and risk factors of rheumatoid arthritis-associated interstitial lung disease. Clin Rheumatol 2017;36: 817-23.
• 18. Jayasinghe P, Wickramasinghe N, Krishnakumar P, Sadikeen A, Fernando A. Characteristics of connective tissue diseases associated interstitial lung diseases – a single centre study in Sri Lanka. Ceylon Med J 2021;66:38-43.
• 19. Zamora-Legoff JA, Krause ML, Crowson CS, Ryu JH, Eric L. Matteson EL. Patterns of interstitial lung disease and mortality in rheumatoid arthritis, Rheumatology 2017;56:344-50.
• 20. Gao H, Sun Y, Zhang XY, Xie L, Zhang XW, Zhong YC, et al. Characteristics and mortality in primary Sjögren syndrome-related interstitial lung disease. Medicine (Baltimore) 2021;100:e26777.
• 21. Danchenko N, Satia JA, Anthony MS. Epidemiology of systemic lupus erythematosus: a comparison of Worldwide Disease Burden. Lupus 2006;15:308-18.
• 22. Kilcher G, Hummel N, Didden EM, Egger M, Reichenbach S. Rheumatoid arthritis patients treated in trial and real-world settings: comparison of randomized trials with registries. Rheumatology 2018;57:354-69.
• 23. Sokka T, Pincus T. Erythrocyte sedimentation rate, C-reactive protein, or rheumatoid factor are normal at presentation in 35%–45% of patients with rheumatoid arthritis seen between 1980 and 2004: analyses from Finland and the United States. J Rheumatol 2009;36:1387-90.
• 24. Yang JA, Lee JS, Park JK, Lee EB, Song YW, Lee EY. Clinical characteristics associated with occurrence and poor prognosis of interstitial lung disease in rheumatoid arthritis. Korean J Intern Med 2019;34:434-41.
• 25. Koduri G, Norton S, Young A. Interstitial lung disease has a poor prognosis in rheumatoid arthritis: results from an inception cohort study. Rheumatology 2010;49:1483-9.
• 26. Enocsson H, Gullstrand B, Eloranta ML, Wetterö J, Leonard D, Rönnblom L, et al. C-reactive protein levels in systemic lupus erythematosus are modulated by the interferon gene signature and CRP gene polymorphism. Front Immunol 2021;11:622326.
• 27. Ihn H, Sato S, Fujimoto M, Igarashi A, Yazawa N, Kubo M, et al. Characterization of autoantibodies to endothelial cells in systemic sclerosis (SSc): association with pulmonary fibrosis. Clin Exp Immunol 2000;119:203-9.
• 28. Muangchan C, Harding S, Khimdas S, Bonner A, Baron M, Pope J.; Canadian scleroderma research group. Association of C-reactive protein with high disease activity in systemic sclerosis: Results from the Canadian Scleroderma Research Group. Arthritis Care Res (Hoboken) 2012;64:1405-14.
• 29. Muangchant C, Pope JE. The significance of interleukin-6 and C-reactive protein in systemic sclerosis: a systematic literature review. Clin Exp Rheumatol 2013;31:122-34.
• 30. Madegedara D, Rathnayake S, Bandara A. Analysis of clinical profile, aetiology, classification and outcome of interstitial lung diseases at a single center of Sri Lanka – a descriptive study. Int J Res Granthaalayah 2020;8:1-14.
• 31. Fischer A, West SG, Swigris JJ, Brown KK, Bois RM. Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest 2010;138:251-6.
• 32. Richman NC, Yazdany J, Graf J, Chernitskiy V, Imboden JB. Extraarticular manifestations of rheumatoid arthritis in a multiethnic cohort of predominantly Hispanic and Asian patients. Medicine 2013;92:92-7.
• 33. Nakamura Y, Suda T, Kaida Y, Kono Y, Hozumi H, Hashimoto D, et al. Rheumatoid lung disease: prognostic analysis of 54 biopsy-proven cases. Respir Med 2012;106:1164-9.
• 34. Bouros D, Wells AU, Nicholson AG. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 2002;165:1581-6.
• 35. Brady D, Berkowitz EA, Sharma A, Ackman JB, Bernheim A, Chung M, et al. CT morphologic characteristics and variant patterns of interstitial pulmonary fibrosis in systemic lupus erythematosus. Radiol Cardiothorac Imaging 2021;3:e200625.
• 36. Matsuyama N, Ashizawa K, Okimoto T, Kadota J, Amano H, Hayashi K. Pulmonary lesions associated with Sjögren's syndrome: radiographic and CT findings. Br J Radiol 2003;76:880-4.
• 37. Benad M, Koschel D, Herrmann K, Wiefel K, Kleymann A, Aringer M. Effects of cyclophosphamide and rituximab in patients with connective tissue diseases with severe interstitial lung disease. Clin Exp Rheumatol 2022;40,483-8.