[Clinical analysis of IgG4-related disease]

To analyze the clinical features, diagnosis and treatment in patients with immunoglobulin 4 (IgG4)-related diseases.
 Methods: The clinical data (including general situation, clinical manifestations, laboratory examination, imaging examination, pathological examination, treatment and follow-up) in 21 patients, who were diagnosed with IgG4-related diseases in Second Xiangya Hospital of Central South University from June 2014 to February 2018, were retrospectively analyzed.
 Results: Among the 21 patients, including men 16 (76.2%) and women 5 (23.8%), the age was 37.00-78.00 (59.19±12.93) years old. Multi-organ involvement was discovered in 10 patients (47.6%), among which the lung (42.9%), pancreas (38.1%), kidney (33.3%), bile duct (19.0%) and liver (14.3%) were the main organs involved. The serum IgG4 levels in 19 patients (90.5%) were increased [8.63 (1.13-36.50) g/L]. The condition of 16 patients (84.2%) was improved after glucocorticoid therapy.
 Conclusion: IgG4-related diseases are mostly found in middle-aged and elderly men. Multiple organs or tissues of the body can be involved. Affected organs are mostly seen in lung, pancreas, kidney and bile duct, with diverse clinical manifestations. Glucocorticoid and immunosuppressive therapy are effective.