Immunoglobulin G4-related disease preceded by lung involvement: A case report

• Published in: Medicine (Baltimore) Vol. 96; no. 24; p. e7086
• Main Authors: Abo, Miki, Takato, Hazuki, Watanabe, Satoshi, Kase, Kazumasa, Sakai, Tamami, Koba, Hayato, Hara, Johsuke, Sone, Takashi, Kimura, Hideharu, Kasahara, Kazuo
• Format: Journal Article
• Language: English
• Published: United States Wolters Kluwer Health 01.06.2017
• Subjects: Aged; Autoimmune Diseases - blood; Autoimmune Diseases - diagnosis; Autoimmune Diseases - drug therapy; Autoimmune Diseases - pathology; Clinical Case Report; Diagnosis, Differential; Humans; Immunoglobulin G - blood; Lung - diagnostic imaging; Lung - drug effects; Lung - pathology; Lung Diseases - diagnosis; Lung Diseases - drug therapy; Lung Diseases - immunology; Lung Diseases - pathology; Male
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000007086

Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition involving various organs and vessels including the pancreas, bile duct, salivary glands, periorbital tissues, kidneys, lungs, lymph nodes, meninges, and aorta. Recently, some cases of IgG4-RD have been reported, in which only pulmonary lesions were present. It is not known whether IgG4-RD can be diagnosed on the basis of pulmonary lesions only, because increases in serum IgG4 levels and infiltration of IgG4-positive plasma cells into the lung tissue also occur in other inflammatory conditions. A case of IgG-RD that was followed-up for 7 years after onset is described. Initially, only pulmonary lesions were present; however, other lesions in the submandibular glands, pancreas, periarterial region, and other areas occurred over time, with a gradual increase in serum IgG4 levels. Histopathology results from the patient's submandibular gland confirmed the diagnosis of IgG4-RD. Following diagnosis, the patient was treated with corticosteroids immediately, and his symptoms disappeared rapidly. Because other diseases, including malignancies, mimic IgG4-RD in clinical and histopathological features, an absolute diagnosis is necessary to avoid missing the presence of underlying diseases. This case more provides insight into the clinical pathology of IgG4-RD.