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• W4311583191 abstract "Microscopically, the central and peripheral lung parenchyma revealed a patchy fibrous proliferation with an extensive fibrosis pattern and lymphohistiocytic infiltration, especially peripheral with bronchiolization and scattered fibroblastic foci (C, partial lymphocytic infiltration [arrow]), CD4+ and CD8+ lymphocytic infiltrates (D-1 and D-2), and increased level of tenascin as extracellular marker of fibrosis (3) (D-3), compatible with the diagnosis of ILD in SD. The histologic pattern corresponded to the fibrotic subtype in nonspecific interstitial pneumonia (fibrotic pattern) with partial inflammatory superimposition, although aspects of a typical UIP pattern (bronchiolization, fibroblastic foci, peripheral predominance) were also evident. The present case demonstrated the relevance of a rheumatological (interdisciplinary) evaluation even at the initial diagnosis of IPF. Castelino et al showed a switch from IPF to ILD in connective tissue disease (CTD) in up to 28% of the patients after an interdisciplinary evaluation (4). Moreover, the change of diagnosis was associated with a modification of the therapy in 80% of the patients with CTD-ILD (4). Even pulmonary asymptomatic patients with CTD can show ILD, making structured screening essential (5, 6). That is why all patients with CTD should receive pulmonary function testing including the quantification of diffusing capacity for carbon monoxide (DLCO) at initial diagnosis. In case of reduced DLCO (<80%) or other risk factors, HRCT should be performed to verify CTD-ILD (5, 6). Immunologic bronchoalveolar lavage or cryobiopsy may be considered useful to verify differential diagnosis of CTD-ILD (6). ILD in SD can include active inflammatory changes in addition to fibrotic components. As inflammatory changes can be amenable to immunosuppressive treatment, the importance of early recognition of ILD in SD should be emphasized (7). For a few years antifibrotic drugs (eg, nintedanib) were used for treatment of the fibrotic component (8). Currently, no guideline is available for the treatment of SD-ILD, although reviews recommend immunosuppressive therapy (eg, cyclophosphamide and mycophenolate mofetil) (9). In systemic sclerosis, a reduced progression rate of ILD was observed in the combined immunosuppressive and antifibrotic treatment (10). In this context, the early combination of antifibrotic and immunosuppressive drugs should be discussed and will be given a higher priority in the future (9). In summary, this case highlighted the need of a structured rheumatological screening in IPF regarding inflammatory rheumatic diseases. The treatment strategy of SD-ILD should include immunosuppressive and antifibrotic drugs to address the inflammatory as well as fibrotic component. Open Access funding enabled and organized by Projekt DEAL. Disclosure Form Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article." @default.
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• W4311583191 date "2022-12-15" @default.
• W4311583191 modified "2023-09-26" @default.
• W4311583191 title "Clinical Images: Severe interstitial lung disease in Sjögren disease — What happens in the lungs? Inflammation or fibrosis?" @default.
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• W4311583191 doi "https://doi.org/10.1002/acr2.11516" @default.
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