免疫球蛋白G4相关性疾病多脏器受累一例并文献复习

摘要：To analyze one case of multi-organ involvement of immunoglobulin G4-related disease and to review the related literatures.Through investigating the clinical data of one case of multi-organ involvement of immunoglobulin G4 (IgG4)-related disease,many related references were retrospected.IgG4-related disease and multi-organ involvement are used as the key words to search on Pubmed and Wanfang Database.According to the clinical manifestations,including serology,imaging,histopathology,responses to steroid therapy,a 65-year-old female patient was diagnosed as IgG4-related disease.The patient exhibited salivary gland swelling,lacrimal glands,and orbital soft tissues.And multiple organs such as pancreas,liver,bile duct,retroperitoneum,the lymph node,lung,paranasal sinuses,kidney and ureter were affected in the patient.Glucocorticoids are the mainstay of therapy and the recommended dose is 0.5-1.0 mg · kg-1 · d-1.Immunosuppressive agents need to be used in relapsing patients or in the patients with multi-organ involvement and serious complications.IgG4-related disease usually affects many systems and involves multiple organs,which is always misdiagnosed to other diseases.Both the correct diagnosis with multidisciplinary fields and the early treatment with glucocorticoids are very important.