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Treatment and follow-up of patients with Sjögren's disease associated interstitial lung disease: a case series
P.K. Panagopoulos1, L.G. Chatzis2, K. Malagari3, A.V. Goules4, A.G. Tzioufas5
- Department of Pathophysiology, Joint Academic Rheumatology Program, School of Medicine, National and Kapodistrian University of Athens, Laiko General Hospital, Athens; and Department of Rheumatology, Naval Hospital of Athens, Greece.
- Department of Pathophysiology, Joint Academic Rheumatology Program, School of Medicine, National and Kapodistrian University of Athens, Laiko General Hospital, Athens; Research Institute for Systemic Autoimmune Diseases, Athens; and Laboratory of Immunobiology, Center for Clinical, Experimental Surgery and Translational Research, Biomedical Research Foundation of the Academy of Athens, Greece.
- 2nd Department of Radiology, School of Medicine, National and Kapodistrian University of Athens, Attikon Hospital, Athens, Greece.
- Department of Pathophysiology, Joint Academic Rheumatology Program, School of Medicine, National and Kapodistrian University of Athens, Laiko General Hospital, Athens; and Research Institute for Systemic Autoimmune Diseases, Athens, Greece.
- Department of Pathophysiology, Joint Academic Rheumatology Program, School of Medicine, National and Kapodistrian University of Athens, Laiko General Hospital, Athens; Research Institute for Systemic Autoimmune Diseases, Athens; and Laboratory of Immunobiology, Center for Clinical, Experimental Surgery and Translational Research, Biomedical Research Foundation of the Academy of Athens, Greece. agtzi@med.uoa.gr
CER19429
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Received: 19/10/2025
Accepted : 17/11/2025
In Press: 05/12/2025
Abstract
OBJECTIVES:
To investigate the effect of treatment in a series of patients with Sjögren’s disease (SjD) associated interstitial lung disease (ILD).
METHODS:
Twenty-four primary SjD-ILD patients, followed-up from October 2022 to June 2025 were included in the study. Based on clinical judgment, 12 received treatment for ILD, while 12 did not, following a “watch-and-wait” policy. Participants were evaluated in 2 time points with an interval of 24±6 months. ILD was diagnosed by HRCT according to Fleischner Society definitions, performed at baseline due to respiratory symptoms and/or abnormal pulmonary function tests. Spirometry and diffusing capacity for carbon monoxide (DLCO) were performed at both visits. Progression of ILD was defined as absolute decline of predicted forced vital capacity (FVC) ≥5%.
RESULTS:
Treatment regimens of 12 SjD-ILD patients who received treatment included rituximab, mycophenolate mofetil, azathioprine and tocilizumab. Treated group displayed higher extent of ILD on HRCT at baseline visit (median: 20% vs. 10%, p=0.006), more frequently findings of small airways disease on HRCT (58% vs. 8%, p=0.027) and tended to present lower FVC (mean: 81.3% vs. 96.7%, p=0.086) compared to untreated. FVC and DLCO remained stable between baseline and follow-up visit in both groups. However, change in DLCO between the two visits was worse in treated than untreated patients (mean: -11.2% vs. 4.8%, p=0.003). The number of SjD-ILD patients presenting progression of ILD did not differ between the two groups (25% vs. 33%, p=0.999).
CONCLUSIONS:
SjD-ILD clinical course is variable, with the most aggressive form to be controlled by immunosuppressive treatment.
