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We aimed to show the differentiation of the degree and distribution on Fluorine-18 fluorodeoxyglucosepositron emission tomography (FDG-PET) between patients with immunoglobulin G4-related disease (IgG4-RD) and sarcoidosis, though both diseases frequently show bilateral hilar lymphadenopathy (BHL). The clinical records were retrospectively reviewed in 25 patients with IgG4-RD with BHL and 15 patients with sarcoidosis (stage I–II) diagnosed at Shinshu University Hospital. All patients underwent FDG-PET at Aizawa Hospital from January 2004 to December 2015. The FDG accumulation pattern and maximum standardized uptake value (SUVmax) of the hilar lymph nodes were compared between the two groups. The IgG4-RD group (21 men; median age 69 years) showed a significant male predominance and older age compared with the sarcoidosis group (3 men, median age 55.4 years). The IgG4-RD group showed a significantly higher incidence of FDG accumulation in the lachrymal gland, submandibular gland, pancreas, prostate and periurethral and periarterial regions compared with the sarcoidosis group. In contrast, the sarcoidosis group showed a significantly higher incidence of FDG accumulation in the supraclavicular and abdominal lymph nodes, muscle and soft tissues compared with the IgG4-RD group. Furthermore, the SUVmax of the hilar lymph nodes was significantly higher in the sarcoidosis group (median 7.20) than in the IgG4-RD group (median 4.20, p=0.002). In conclusion, significant differences were observed in the FDG accumulation patterns and SUVmax values of the hilar lymph nodes between IgG4-RD with BHL and sarcoidosis, although both diseases develop through the lymphatic routes of the lungs and are frequently associated with BHL., This study was supported by the Research Program of Intractable Disease, the Ministry of Health, Labor, and Welfare of Japan (No. H29-Nanchi-Ippan-058), and the Japan Society for the Promotion of Science (No. 15K09169).

A 66-year-old man was diagnosed with autoimmune pancreatitis in February 2009 and started 40 mg of oral prednisolone followed by a maintenance dose of 5 mg daily. The patient developed a cough in October 2010 and visited our division. He had a high serum concentration of immunoglobulin (Ig) G4 and his chest computed tomography showed airway stenosis without bilateral hilar lymphadenopathy (BHL). The bronchial biopsy specimens revealed lymphoplasmacytic infiltrations with IgG4-positive/IgG-positive plasma cells of more than 50%. Thus, we diagnosed the airway lesion with IgG4-related airway involvement. This is the first report of a patient with IgG4-related airway involvement without BHL., Article, INTERNAL MEDICINE. 50(24):3023-3026 (2011)