ArticleSarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG2017
Pirfenidone for primary Sjögren's syndrome-related fibrotic interstitial pneumonia.
Article in Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed, 4 citations in OpenAlex.
- Clinical significance of radiological usual interstitial pneumonia pattern in primary Sjögren syndrome-associated interstitial lung disease.Frontiers in immunology · 2026Article
- Interstitial Lung Disease in Primary Sjögren's Syndrome: Risk factors for occurrence and radiographic progression.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2024Article
- Primary-Sjögren's-Syndrome-Related Interstitial Lung Disease: A Clinical Review Discussing Current Controversies.Journal of clinical medicine · 2023Review
- Antifibrotic drugs in connective tissue disease-related interstitial lung disease (CTD-ILD): from mechanistic insights to therapeutic applications.Drugs in context · 2021Review
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Primary Sjögren's syndrome-related interstitial pneumonia (pSS-IP) is occasionally progressive even with immunosuppressive therapy and new treatment options are warranted. A 42-year-old woman was diagnosed with pSS-IP with pathological usual interstitial pneumonia pattern. Although her forced vital capacity (FVC) had temporarily improved after prednisolone therapy, it deteriorated during the prednisolone tapering off period. Therefore, pirfenidone was added in conjunction with prednisolone. During the 12-month treatment period, her FVC was almost stable. A 62-year-old man, a current heavy smoker, was diagnosed with pSS-IP. Chest computed tomography showed bilateral honeycombing with ground-glass attenuation on a background of emphysema. He gradually developed dyspnea on exertion and his FVC deteriorated. Hence, pirfenidone was administered as a monotherapy. Six months later, his FVC and exercise tolerance had significantly improved. Pirfenidone might have a role for the treatment of pSS-IP.
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Registered trials
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