The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?

Yazici, Ayten; Özdemir Işık, Özlem; Cefle, Ayse

doi:10.55730/1300-0144.5513

The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?

Özlem O. IŞIK, (Kocaeli Üniversitesi, Tıp Fakültesi, İç Hastalıkları Anabilim Dalı, Romatoloji Anabilim Dalı, Kocaeli, Türkiye)

Ayten YAZICI, (Kocaeli Üniversitesi, Tıp Fakültesi, İç Hastalıkları Anabilim Dalı, Romatoloji Anabilim Dalı, Kocaeli, Türkiye)

Ayşe CEFLE (Kocaeli Üniversitesi, Tıp Fakültesi, İç Hastalıkları Anabilim Dalı, Romatoloji Anabilim Dalı, Kocaeli, Türkiye)

Turkish Journal of Medical Sciences

1 0

Yıl: 2022 Cilt: 52 Sayı: 5 Sayfa Aralığı: 1704 - 1712 Metin Dili: İngilizce DOI: 10.55730/1300-0144.5513 İndeks Tarihi: 27-12-2022

The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?

Öz:

Background/aim: Sjögren’s syndrome is a systemic, autoimmune disease and can affect many organs and systems. In this study, we aimed to evaluate the respiratory manifestations, and the association between interstitial lung disease (ILD) and disease activity in primary SS (pSS) patients. Materials and methods: The study design was retrospective cross-sectional, and 151 patients followed up with a diagnosis of pSS between 2004 and 2019 were included in the study. Demographic and clinical data, laboratory results, chest radiographs and thorax computed tomography (CT) results were obtained from patient files and hospital imaging system. Thorax CT was requested from all patients with respiratory complaints and abnormalities in physical examination and pulmonary function test. Disease activity was calculated with EULAR pSS disease activity index (ESSDAI) and clinical European League Against Rheumatism SS Disease Activity Index (clin-ESSDAI). Results: In our study, 97% of pSS patients were female, and the mean age was 55.9 ± 12 years, disease onset age was 45.5 ± 12.1 years, disease duration was 10.7 (1–38) years. According to CT findings of 120 patients, 35% had nodules, and 13.3% had ILD (62.5% nonspecific interstitial pneumonia, 25% lymphocytic interstitial pneumonia, 12.5% usual interstitial pneumonia). Bronchiectasis, emphysema, sequelae fibrotic changes, and pleural thickening was found in 3.3%, 5.8%, 15.8%, and 1.7% of patients, respectively. It was observed that there was a significant relationship between the presence of ILD and persistent cough, mediastinal LAP, low DLCO, high ESSDAI and clin-ESSDAI scores reflecting disease activity. Conclusion: The most common pulmonary manifestation in our patients was ILD. ILD was observed more frequently in patients with moderate and severe disease activity. Some of the ILD patients were diagnosed while they were asymptomatic. Even if they are asymptomatic, it is important to follow up the patients with physical examination, spirometry, DLCO and thorax CT.

Anahtar Kelime: Sjögren’s syndrome lung interstitial lung disease

Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık

1. Robert I Fox. Sjögren’s syndrome. Lancet 2005; 366: 321-331. doi: 10.1016/S0140-6736(05)66990-5
2. Hatron PY, Tillie-Leblond I, Launay D, Hachulla E, Fauchais AL et al. Pulmonary manifestations of Sjögren’s syndrome. Presse Medicale 2011; 40: e49-64. doi: 10.1016/j. lpm.2010.11.002
3. Egashira R, Kondo T, Hirai T, Kamochi N, Yakushiji M. et al. CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation. Radiographics 2013; 33: 1933–1949. doi.org/10.1148/rg.337125107
4. Stojan G, Baer AN, Danoff SK. Pulmonary manifestations of Sjögren’s syndrome. Current Allergy Asthma Reports 2013; 13: 354–360. doi: 10.1007/s11882-013-0357-9
5. Ma D, Lu H, Qu Y, Wang S, Ying Y et al. Primary Sjögren’ s syndrome accompanied by pleural effusion: a case report and literature review. International Journal of Clinical and Experimental Pathology 2015; 8 (11): 15322–15327
6. Shahane A. Pulmonary hypertension in rheumatic diseases: epidemiology and pathogenesis. Rheumatol International 2013; 33 (7):1655–1667. doi: 10.1007/s00296-012-2659-y
7. Shiboski CH, Shiboski SC, Seror R, Criswell AL, Labetoulle M et al. American College of Rheumatology/European League Against Rheumatism Classification Criteria for primary Sjogren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatology 2017; 69 (1): 35–45. doi: 10.1002/ art.39859
8. Seror R, Ravaud P, Bowman SJ, Baron G, Tzioufas A et al. EULAR Sjogren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjogrenꞌs syndrome. Annals of the Rheumatic Diseases 2010; 69 (6): 1103–1109. doi: 10.1136/ard.2009.110619
9. Seror R, Meiners P, Baron G, Bootsma H, Bowman JS et al. EULAR Sjögren Task Force. Annals of the Rheumatic Diseases 2016; 75 (11): 1945-1950. doi: 10.1136/ annrheumdis-2015-208504
10. Ito I, Nagai S, Kitaichi M, Nicholson A.G, Johkoh T 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- 638. doi: 10.1164/rccm.200403-417OC
11. Nannini C, Jebakumar AJ, Crowson CS, Ryu JH, Matteson EL. Primary sjogren’s syndrome 1976-2005 and associated interstitial lung disease: a population-based study of incidence and mortality. British Medical Journal Open. 2013; 3 (11): e003569. doi: 10.1136/bmjopen-2013-003569
12. Papiris SA, Maniati M, Constantopoulos SH, Roussos C, Moutsopoulos H et al. Lung involvement in primary Sjögren’s syndrome is mainly related to the small airway disease. Annals of the Rheumatic Diseases 1999; 58 (1): 61–64. doi: 10.1136/ard.58.1.61
13. Flament T, Bigot A, Chaigne B, Henique H, Diot E et al. Pulmonary manifestations of Sjögren’s syndrome. European Respiratory Review 2016; 25 (140): 110–123. doi: 10.1183/16000617.0011-2016
14. Wislez M, Cadranel J, Antoine M, Milleron B, Bazot M et al. Lymphoma of pulmonary mucosa-associated lymphoid tissue: CT scan findings and pathological correlations. European Respiratory Journal 1999; 14 (2): 423-429. doi: 10.1034/j.1399- 3003.1999.14b30.x
15. J. D. Gillmore, A. Wechalekar, J. Bird, Cavenagh J, Hawkins S et al. Guidelines on the diagnosis and investigation of AL amyloidosis. British Journal of Hematology 2015; 168 (2): 207- 218. doi: 10.1111/bjh.13156
16. Liang Y, Yang Z, Qin B,Zhong R. Primary Sjögren’s syndrome and malignancy risk: a systematic review and meta-analysis. Annals of the Rheumatic Diseases 2014; 73 (6): 1151-1156. doi: 10.1136/annrheumdis-2013-203305
17. Ramos-Casals M, Brito-Zerón P, Seror R, Bootsma H, Bowman JS et al. Characterization of systemic disease in primary Sjögren’s syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements. Rheumatology 2015; 54 (12): 2230–2238. doi: 10.1093/ rheumatology/kev200
18. Roca F, Dominique S, Schmidt J, Smail A, Duhaut P. et al. Interstitial lung disease in primary Sjogren’s syndrome. Autoimmun Review 2017; 16 (1): 48–54. doi: 10.1016/j. autrev.2016.09.017
19. Gao H, Zhang XW, He J, Zhang J, An Y. et al. Prevalence, risk factors, and prognosis of interstitial lung disease in a large cohort of Chinese primary Sjogren syndrome patients: a case- control study. Medicine (Baltimore) 2018; 97 (24): e11003. doi: 10.1097/MD.0000000000011003
20. Wang Y, Hou Z, Qiu M, Ye Q. Risk factors for primary Sjogren syndrome-associated interstitial lung disease. Journal of Thoracic Disease 2018; 10 (4): 2108–2117. doi: 10.21037/ jtd.2018.03.120
21. Yazisiz V, Arslan G, Ozbudak IH, Turker S, Erbasan F et al. Lung involvement in patients with primary Sjögren’s syndrome: what are the predictors? Rheumatol International 2010; 30 (10): 1317–1324. doi: 10.1007/s00296-009-1152-8
22. Soto-Cardenas M-J, Perez-De-Lis M, Bove A, Navarro C, Brito- Zeron P et al. Bronchiectasis in primary Sjogren’s syndrome: prevalence and clinical significance. Clinical and Experimental Rheumatology 2010; 28 (5): 647–653
23. Ito I, Nagai S, Kitaichi M, Nicholson GA, Johkoh T 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): 632–638. doi: 10.1164/rccm.200403-417OC
24. Papiris SA, Kalomenidis I, Malagari K, Kapotsis EG, Harhalakis N et al. Extranodal marginal zone B-cell lymphoma of the lung in Sjögren’s syndrome patients: reappraisal of clinical, radiological, and pathology findings. Respiratory Medicine 2007; 101 (1): 84-92. doi: 10.1016/j.rmed.2006.04.005
25. Shi JH, Liu HR, Xu WB, Feng R, Zhang Z et al. Pulmonary manifestations of Sjogren’s syndrome. Respiration 2009; 78 (4): 377–386. doi: 10.1159/000214841
26. Ciancio N, Pavone M, Torrisi SE, Vancheri A, Sambataro D et Al. Contribution of Pulmonary Function Tests (Pfts) To The Diagnosis And Follow Up Of Connective Tissue Diseases. Multidisciplinary Respiratory Medicine 2019; 14: 17. doi: 10.1186/s40248-019-0179-2
27. Palm O, Garen T, Berge Enger T, Jensen LJ, Lund M 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) 2013; 52 (1): 173-179. doi: 10.1093/rheumatology/kes311
28. Shen T-C, Wu B-R, Chen H-J, Lin CL, Wei CC et al. Risk of chronic obstructive pulmonary disease in female adults with primary Sjögren syndrome. Medicine (Baltimore) 2016; 95 (10): e3066. doi: 10.1097/MD.0000000000003066
29. Sato T, Matsubara O, Tanaka Y, Kasuga T. Association of Sjogren’s syndrome with pulmonary hypertension: report of two cases and review of the literatüre. Human Pathology 1993; 24 (2): 199-205. doi: 10.1016/0046-8177(93)90301-v
30. Nakagawa N, Osanai S, Ide H, Nishigaki Y, Takahashi S et al. Severe pulmonary hypertension associated with primary Sjogren’s syndrome. Internal Medicine 2003; 42 (12): 1248- 1252. doi: 10.2169/internalmedicine.42.1248
31. Kobak S, Kalkan S, Kirilmaz B, Orman M, Ercan E. Pulmonary Arterial Hypertension in Patients with Primary Sjögren’s Syndrome. Autoimmune Diseases 2014; 2014: 710401. doi: 10.1155/2014/710401

APA	Özdemir Işık Ö, Yazici A, Cefle A (2022). The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?. , 1704 - 1712. 10.55730/1300-0144.5513
Chicago	Özdemir Işık Özlem,Yazici Ayten,Cefle Ayse The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?. (2022): 1704 - 1712. 10.55730/1300-0144.5513
MLA	Özdemir Işık Özlem,Yazici Ayten,Cefle Ayse The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?. , 2022, ss.1704 - 1712. 10.55730/1300-0144.5513
AMA	Özdemir Işık Ö,Yazici A,Cefle A The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?. . 2022; 1704 - 1712. 10.55730/1300-0144.5513
Vancouver	Özdemir Işık Ö,Yazici A,Cefle A The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?. . 2022; 1704 - 1712. 10.55730/1300-0144.5513
IEEE	Özdemir Işık Ö,Yazici A,Cefle A "The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?." , ss.1704 - 1712, 2022. 10.55730/1300-0144.5513
ISNAD	Özdemir Işık, Özlem vd. "The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?". (2022), 1704-1712. https://doi.org/10.55730/1300-0144.5513

APA	Özdemir Işık Ö, Yazici A, Cefle A (2022). The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?. Turkish Journal of Medical Sciences, 52(5), 1704 - 1712. 10.55730/1300-0144.5513
Chicago	Özdemir Işık Özlem,Yazici Ayten,Cefle Ayse The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?. Turkish Journal of Medical Sciences 52, no.5 (2022): 1704 - 1712. 10.55730/1300-0144.5513
MLA	Özdemir Işık Özlem,Yazici Ayten,Cefle Ayse The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?. Turkish Journal of Medical Sciences, vol.52, no.5, 2022, ss.1704 - 1712. 10.55730/1300-0144.5513
AMA	Özdemir Işık Ö,Yazici A,Cefle A The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?. Turkish Journal of Medical Sciences. 2022; 52(5): 1704 - 1712. 10.55730/1300-0144.5513
Vancouver	Özdemir Işık Ö,Yazici A,Cefle A The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?. Turkish Journal of Medical Sciences. 2022; 52(5): 1704 - 1712. 10.55730/1300-0144.5513
IEEE	Özdemir Işık Ö,Yazici A,Cefle A "The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?." Turkish Journal of Medical Sciences, 52, ss.1704 - 1712, 2022. 10.55730/1300-0144.5513
ISNAD	Özdemir Işık, Özlem vd. "The respiratory manifestations in patients with primary Sjögren’s syndrome: is interstitial lung disease related to disease activity?". Turkish Journal of Medical Sciences 52/5 (2022), 1704-1712. https://doi.org/10.55730/1300-0144.5513