IgG4-Related Kidney Disease

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IgG4-Related Kidney Disease

Clinical Features of IgG4-RKD


IgG4-RKD shows a striking male predominance (73–87%), and the average patient age is about 65 years. Systemic symptoms are relatively mild, and the condition usually becomes clinically apparent when renal dysfunction and/or renal radiographic abnormalities occur, during systemic examination for extrarenal IgG4-RD or by chance. Most patients with IgG4-RKD have IgG4-related extrarenal lesions, the salivary glands, lacrimal glands, lymph nodes, and pancreas being frequently affected. Edema may be evident in patients with IgG4-RKD accompanied by glomerular lesions or in patients with hydronephrosis due to retroperitoneal fibrosis.

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