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1Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India
2Department of Biostatistics, Christian Medical College, Vellore, India
3Department of Pulmonary Medicine, Christian Medical College, Vellore, India
4Department of Radiology, Christian Medical College, Vellore, India
P Manikuppam
, S Padiyar, B Yadav, A Nair, M Mane, J Mathew
Objectives: To describe the characteristics of primary Sjögren’s syndrome (pSS) patients with interstitial lung disease (ILD) and to assess treatment response. Methods: All patients of pSS from 2010 to 2019 were retrospectively identified. Lung function tests, high resolution computed tomography (HRCT) findings, and treatment outcomes were analysed. Results: Out of 550 patients with pSS, ILD was detected in 33 patients (frequency of 6 %). The mean(±SD) age at the diagnosis of pSS was 50 (± 9.3) years. 28/33(84.8%) were females. ILD onset preceded pSS diagnosis in 2 (6%) patients, simultaneously diagnosed in 21 (63.6%) patients and developed after pSS onset in 10 (30.3%) patients. 5 patients (15.15 %) were asymptomatic for ILD. Non-specific interstitial pneumonia (NSIP) accounted for the most frequent ILD subtype, in 15 patients (45.5%). Mycophenolate mofetil (MMF) was the most frequently used steroid sparing agent, in 25 patients (75.7%). 7 patients were lost to follow up. Response was seen in 22 patients, whereas 3 patients were non responders. There was one mortality due to lower respiratory tract infection-related sepsis. Presence of sicca symptoms [91.5% vs 8.7% (p<0.001)], NSIP pattern of ILD [90% vs 10% (p = 0.002)], and absence of Raynaud’s phenomenon [91.7% vs 8.3% (p<0.001)] were significantly associated with responder status when compared to non-responders. Conclusion: ILD in primary Sjögren’s syndrome is not an uncommon entity, and immunosuppression with steroids along with steroid-sparing agents led to good clinical outcomes of ILD in a majority of the patients in our cohort.
Cite this article as: Manikuppam P, Padiyar S, Yadav B, Nair AA, Mane M, Mathew J. Clinical Characteristics and Outcomes of Interstitial Lung Disease in Primary Sjögren's Syndrome: A Retrospective Cohort Study. Mediterr J Rheumatol 2024;35(1);108-14.
Article Submitted: 26 Mar 2023; Revised Form: 15 Sep 2023; Article Accepted: 10 Oct 2023; Available Online: 16 Jan 2024
This work is licensed under a Creative Commons Attribution 4.0 International License.
©2024 The Author(s).
