IgG4 disease is an immune-mediated disorder characterized by fibroinflammatory masses that can infiltrate multiple organ systems. Renal involvement is considered one of the key features of this disease. Report 02 cases of IgG4 disease with renal manifestations revealing the disease such as extramembranous glomerulonephritis and tubulointerstitial nephritis rich in polytypic IgG4+ plasma cells. Associated renal parenchymal lesions concomitant with multiple organ involvement should help improve early recognition of IgG4-RKD disease (IgG4-RKD). In our case series, prednisone was the treatment of choice; the use of rituximab in extramembranous glomerulonephritis had resulted in remission of the disease.
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