Clinical characteristics of IgG4-related disease: analysis of 29 cases

Objective To analyze the clinical characteristics of IgG4-related disease (IgG4-RD) and to explore the clinical significance of serum IgG4 elevation in auxiliary diagnosis of IgG4-RD. Methods Clinical data of the patients diagnosed with IgG4-RD in our hospital from January 2010 to November 2020 were collected, and their clinical manifestations, involved organs, results of laboratory examinations, histopathology and treatment were retrospectively analyzed. Results ① Among the 29 cases eventually enrolled, there were 18 males and 11 females, with a ratio of male to female of 1.64; ② Submandibular gland enlargement and multiple lymph node adenopathy were the most common symptoms. And multiple organs such as the lung, liver, lacrimal gland/eyelid, pleura, pericardium, pancreas, skin, biliary tract, parotid gland, kidney and breast were also afflicted; ③ Their serum IgG4 level ranged from 57.3 to 2 000.0 mg/dL. About 93.1% of them had elevated IgG4 level, and 53.8% got IgG elevation; ④ Ten cases were treated with glucocorticoid and/or immunosuppressant, and they got shrunk lesions, dropped IgG4 level and good prognosis. Conclusion IgG4-RD is more common in middle-aged and elderly men, involving multiple organs, with various initial symptoms and clinical manifestations. Serum IgG4 is an important indicator for the diagnosis of IgG4-RD, but vasculitis, tumor diseases, infectious diseases, Castleman disease and other connective tissue diseases should be carefully excluded