Interstitial Lung Disease in Primary Sjögren’s Syndrome: Risk factors for occurrence and radiographic progression Interstitial lung disease associated with primary Sjögren's syndrome

Main Article Content

Hatice Ecem Konak
Ebru Atalar
Habibe Hezer
Rezan Koçak Ulucaköy
Esra Kayacan Erdoğan
Hakan Babaoğlu
Berkan Armağan
Kevser Orhan
İsmail Doğan
Yüksel Maraş
Ahmet Omma
Ayşegül Karalezli
Şükran Erten
Orhan Küçükşahin
Serdar Can Güven

Keywords

Sjögren’s disease, interstitial lung disease, progression

Abstract

Objectives: The aim of this study is to investigate the characteristics of Primary Sjögren's syndrome (pSS)- interstitial lung disease (ILD) patients and compare them to those of pSS patients without ILD in the tertiary pSS-ILD cohort to evaluate potential risk factors for ILD occurrence and disease progression. Methods: Patients followed up who met the 2016 American College of Rheumatology-European League Against Rheumatism classification criteria for pSS were retrospectively analyzed. The patients were grouped as those with ILD and those without ILD according to medical records. High-resolution computed tomography (HRCT)/ thorax CT (TCT) results of all ILD patients were evaluated. Data on demographics, comorbidities, clinical characteristics and laboratory findings were collected. Results: A total of 378 pSS patients, including 60 with ILD and 318 without ILD were detected to have at least one obtainable HRCT/TCT and were included in the study. In the cohort of pSS patients with at least one HRCT or TCT, the frequency of ILD was 15.8%. In the ILD group, the most common HRCT pattern was NSIP, and the most common findings were ground glass opacities, traction bronchiectasis, and honeycombing. Logistic regression analysis showed that male gender (OR:2.90), being diagnosed with pSS over the age of 50(OR:4,24), smoking history (OR:2.38), elevated LDH(OR:3.27), elevated ESR(OR:2.51) and lymphopenia (OR:5.12) were related with development of ILD while being diagnosed with ILD after the age of 60 (OR:8.5) was related with radiographic progression. Conclusion: The study results provided a large spectrum view for pSS-ILD and pointed out several risk factors for ILD occurrence and radiographic progression.

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References

1. Mariette X, Criswell LA. Primary Sjögren's Syndrome. The New England journal of medicine. 2018;378(10):931-9.
2. Parambil JG, Myers JL, Lindell RM, Matteson EL, Ryu JH. Interstitial lung disease in primary Sjögren syndrome. Chest. 2006;130(5):1489-95.
3. Berardicurti O, Marino A, Genovali I, et al. Interstitial Lung Disease and Pulmonary Damage in Primary Sjögren's Syndrome: A Systematic Review and Meta-Analysis. Journal of clinical medicine. 2023;12(7).
4. Ito I, Nagai S, Kitaichi M, et al. Pulmonary manifestations of primary Sjogren's syndrome: a clinical, radiologic, and pathologic study. American journal of respiratory and critical care medicine. 2005;171(6):632-8.
5. Enomoto Y, Takemura T, Hagiwara E, et al. Prognostic factors in interstitial lung disease associated with primary Sjögren's syndrome: a retrospective analysis of 33 pathologically-proven cases. PLoS One. 2013;8(9):e73774.
6. Palm O, Garen T, Berge Enger T, et al. Clinical pulmonary involvement in primary Sjogren's syndrome: prevalence, quality of life and mortality--a retrospective study based on registry data. Rheumatology (Oxford, England). 2013;52(1):173-9.
7. Luppi F, Sebastiani M, Silva M, et al. Interstitial lung disease in Sjögren's syndrome: a clinical review. Clinical and experimental rheumatology. 2020;38 Suppl 126(4):291-300.
8. 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. American journal of respiratory and critical care medicine. 2002;165(2):277-304.
9. Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. American journal of respiratory and critical care medicine. 2008;177(11):1248-54.
10. Louza GF, Nobre LF, Mançano AD, et al. Lymphocytic interstitial pneumonia: computed tomography findings in 36 patients. Radiologia brasileira. 2020;53(5):287-92.
11. Lin DF, Yan SM, Zhao Y, et al. Clinical and prognostic characteristics of 573 cases of primary Sjögren's syndrome. Chinese medical journal. 2010;123(22):3252-7.
12. He C, Chen Z, Liu S, Chen H, Zhang F. Prevalence and risk factors of interstitial lung disease in patients with primary Sjögren's syndrome: A systematic review and meta-analysis. International journal of rheumatic diseases. 2020;23(8):1009-18.
13. Roca F, Dominique S, Schmidt J, et al. Interstitial lung disease in primary Sjögren's syndrome. Autoimmunity reviews. 2017;16(1):48-54.
14. Kakugawa T, Sakamoto N, Ishimoto H, et al. Lymphocytic focus score is positively related to airway and interstitial lung diseases in primary Sjögren's syndrome. Respiratory medicine. 2018;137:95-102.
15. Wang Y, Hou Z, Qiu M, Ye Q. Risk factors for primary Sjögren syndrome-associated interstitial lung disease. Journal of thoracic disease. 2018;10(4):2108-17.
16. Kampolis CF, Fragkioudaki S, Mavragani CP, Zormpala A, Samakovli A, Moutsopoulos HM. Prevalence and spectrum of symptomatic pulmonary involvement in primary Sjögren's syndrome. Clinical and experimental rheumatology. 2018;36 Suppl 112(3):94-101.
17. Manfredi A, Sebastiani M, Cerri S, et al. Prevalence and characterization of non-sicca onset primary Sjögren syndrome with interstitial lung involvement. Clinical rheumatology. 2017;36(6):1261-8.
18. Strevens Bolmgren V, Olsson P, Wollmer P, Hesselstrand R, Mandl T. Respiratory symptoms are poor predictors of concomitant chronic obstructive pulmonary disease in patients with primary Sjögren's syndrome. Rheumatol Int. 2017;37(5):813-8.
19. Ramos-Casals M, Brito-Zerón P, Seror R, et al. Characterization of systemic disease in primary Sjögren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements. Rheumatology (Oxford, England). 2015;54(12):2230-8.
20. Uffmann M, Kiener HP, Bankier AA, Baldt MM, Zontsich T, Herold CJ. Lung manifestation in asymptomatic patients with primary Sjögren syndrome: assessment with high resolution CT and pulmonary function tests. Journal of thoracic imaging. 2001;16(4):282-9.
21. Franquet T, Giménez A, Monill JM, Díaz C, Geli C. Primary Sjögren's syndrome and associated lung disease: CT findings in 50 patients. AJR American journal of roentgenology. 1997;169(3):655-8.
22. Gao H, Zou YD, Zhang XW, et al. Interstitial lung disease in non-sicca onset primary Sjögren's syndrome: a large-scale case-control study. International journal of rheumatic diseases. 2018;21(7):1423-9.
23. Reina D, Roig Vilaseca D, Torrente-Segarra V, et al. Sjögren's syndrome-associated interstitial lung disease: A multicenter study. Reumatologia clinica. 2016;12(4):201-5.
24. Kamiya Y, Fujisawa T, Kono M, et al. Prognostic factors for primary Sjögren's syndrome-associated interstitial lung diseases. Respiratory medicine. 2019;159:105811.
25. Ahuja J, Arora D, Kanne JP, Henry TS, Godwin JD. Imaging of Pulmonary Manifestations of Connective Tissue Diseases. Radiologic clinics of North America. 2016;54(6):1015-31.
26. Dong X, Zhou J, Guo X, et al. A retrospective analysis of distinguishing features of chest HRCT and clinical manifestation in primary Sjögren's syndrome-related interstitial lung disease in a Chinese population. Clinical rheumatology. 2018;37(11):2981-8.
27. Lin W, Xin Z, Zhang J, et al. Interstitial lung disease in Primary Sjögren's syndrome. BMC pulmonary medicine. 2022;22(1):73.
28. Dong X, Gao YL, Lu Y, Zheng Y. Characteristics of primary Sjögren's syndrome related lymphocytic interstitial pneumonia. Clinical rheumatology. 2021;40(2):601-12.
29. He SH, He YJ, Guo KJ, Liang X, Li SS, Li TF. Risk factors for progression of interstitial lung disease in Sjögren's syndrome: a single-centered, retrospective study. Clinical rheumatology. 2022;41(4):1153-61.
30. Xu Y, Zhou J, Dong X, Guo X, Lu Y, Zheng Y. Risk factors for progression and prognosis of primary Sjögren's syndrome-associated interstitial lung disease in a Chinese population. International journal of rheumatic diseases. 2020;23(12):1734-40.
31. Enomoto Y, Nakamura Y, Colby TV, Inui N, Suda T. Pirfenidone for primary Sjögren's syndrome-related fibrotic interstitial pneumonia. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG. 2017;34(1):91-6.
32. Ramos-Casals M, Brito-Zerón P, Bombardieri S, et al. EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies. Ann Rheum Dis. 2020;79(1):3-18.
33. Amlani B, Elsayed G, Barvalia U, et al. Treatment of primary sjögren's syndrome-related interstitial lung disease: a retrospective cohort study. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG. 2020;37(2):136-47.