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Updated: Mar 20, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Immunoglobulin G4-related lung disease: Case report and literature review]
Clémence Delteil1, Anne-Laure Chesnais1, Anne-Marie Tasei1
1Service de pathologie, hôpital Henri-Duffaut, centre hospitalier d'Avignon, 84000 Avignon, France.
Abstract:
We report the case of a 61-year-old man with a pulmonary nodule discovered while staging the metastasis of a squamous cell carcinoma. No primary cancer was found. Histological examination of the resected specimen (lingula) was in favor of IgG4-related inflammatory pseudotumor. Histological criteria were described in 2012, combining a dense lymphoplasmacitic infiltrate rich in IgG4-positive plasma cell, storiform fibrosis and obliterative phlebitis. IgG4/IgG plama cell ratio>40 % is mandatory for histological diagnosis of IgG4-related disease. This is a rare and often underdiagnosed disease. Diagnostic criteria are now defined and consensual, combining clinical signs, biology and histology.
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