Immunoglobulin G4-related disease (IgG4-RD) is increasingly recognized as an autoimmune disease, involvement of multi-organ or system, which is characterized by tissue infiltration of IgG4-positive plasma cells and elevated serum IgG4. We reported a case of 54-year-old man with a medical history of uarthritis, virus B hepatitis, autoimmune pancreatitis, cholecystitis, pulmonary lesion and acute renal failure. The patient was diagnosed with multi-system IgG4-RD based on histological analysis of the renal biopsy revealed IgG4-positive plasmacyte infiltration, elevated serum IgG4(6.95g/L).Corticosteroids(methylprednisolone 250mg/d for 3 days subsequently 40 mg/d) and cyclophosphamide (0.8g iv per month) were used. Not only the IgG4 levels returned to normal but also patient’s renal dysfunction and pulmonary lesions relieved after treatment.
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