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Updated: Aug 4, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Lung and Pleural Immunoglobulin G4-Related Disease: Two Contrasting Case Reports
Mariana Diz-Lopes1, Fernando Nogueira2, José Alberto da Cunha Marques2
1Rheumatology, Centro Hospitalar Universitário de São João, Porto, PRT.
Abstract:
Immunoglobulin G4-related disease (IgG4-RD) is an immunomediated disease that can virtually affect any organ. Despite the pancreas being known as the most frequently involved organ, pulmonary and pleural IgG4-RD is being increasingly reported. The authors present two cases of IgG4-RD diagnosed in the same year, with different presentations and outcomes, in which the lung and pleural involvement were essential for the diagnosis. Recognizing IgG4-RD as a possible cause of chronic pleural effusion and/or thickening and lung abnormalities is important for an early diagnosis and prognosis improvement.
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