Abstract
We describe a 72-year-old man, who had been suffered from Henoch-Schönlein purpura (HSP) several times, presented with hematoproteinuria with granular cast, and general lymphadenopathy. The immunological examination of the serum showed polyclonal hypergammagloburinemia with high value of IgG4. The renal biopsy revealed interstitial inflammatory cell infiltration, including infiltration of lymphocytes and plasma cells, and segmental glomerulonephritis. Direct immunofluorescence microscopy revealed apparent positive staining with anti-human IgA, and anti-human IgG in glomeruli, anti-human IgG4 antibody staining showed many positive plasma cells in the interstitium. The patient was diagnosed with HSP nephritis that was complicated by IgG4-related nephropathy. As a result of the treatment with 30mg prednisolone, the swelling of the LNs decreased, but the patient continued to have persistent hematoproteinuria.
Highlights
A novel clinicopathological entity of IgG4-related autoimmune disease characterized by extensive IgG4-positive plasma cell infiltration of organs together with CD4- or CD8-positive T lymphocytes is proposed [1]
Renal involvement in this entity was suggested, and three patterns of renal involvement have been described: 1) extraparenchymal involvement such as hydronephrosis associated with retroperitoneal lesions; 2) diffuse tubulointerstitial nephritis (TIN); and 3) renal lesions composed of focal lymphoplasmacytic infiltration of the renal interstitium [2]
In this report we describe a rare case diagnosed with Henoch-Schönlein purpura (HSP) nephritis that was complicated by IgG4-related nephropathy
Summary
A novel clinicopathological entity of IgG4-related autoimmune disease characterized by extensive IgG4-positive plasma cell infiltration of organs together with CD4- or CD8-positive T lymphocytes is proposed [1]. In this report we describe a rare case diagnosed with HSP nephritis that was complicated by IgG4-related nephropathy. In 2006, the patient was diagnosed with Henoch-Schönlein purpura (HSP) on the basis of histological examination of skin biopsy samples, which showed leukocytoclastic vasculitis. The serum level of immunoglobulin G (IgG) was abnormally high, but IgA and IgM were within normal limits (4,359 mg/dL, 242 mg/dL, and 64 mg/dL, respectively).
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