A case of IgG4-related tubulointerstitial nephritis concurrent with Henoch-Schönlein purpura nephritis

Division of Nephrology and Rheumatology, Department of Internal Medicine, Faculty of Medicine, Fukuoka University, Nanakuma7-45-1, Johnan-ku, Fukuoka city, 814-0180, Japan. .
Allergy Asthma and Clinical Immunology (Impact Factor: 2.03). 03/2011; 7(1):5. DOI: 10.1186/1710-1492-7-5
Source: PubMed


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.

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Available from: Katsuhisa Miyake
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    • "Symptoms of asthma, involvement of the paranasal sinuses, lungs, kidneys and peripheral eosinophilia are common to both conditions. Publications have described cases of IgG4-related disease with concomitant cutaneous leukocytoclastic vasculitis[20], Henoch-Schönlein purpura[21], or allergic vasculitis with hypocomplementaemia[22]. A noteworthy feature is that some patients with AAV have elevated serum IgG4 concentrations and IgG4+ cell infiltration of affected tissues. "
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    ABSTRACT: We report a middle-aged Japanese man who had a past history of malignant lymphoma with tubulointerstitial nephritis (TIN) presenting a high serum immunoglobulin G4 (IgG4) concentration and bilateral kidney enlargement and swelling of many lymph nodes. Although lymph node biopsy was not evident of a recurrence of lymphoma, kidney biopsy showed prominent infiltration of IgG4-positive plasma cells in a tubulointerstitial lesion but not in glomeruli. We made a diagnosis of IgG4-related TIN and lymphadenopathy; administration of oral prednisolone improved his physical and laboratory parameters. This is the first report of a case of IgG4-related TIN and lymphadenopathy after therapy for malignant lymphoma.
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