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 03/2011; 7(1):5. DOI: 10.1186/1710-1492-7-5
Source: PubMed

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.

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    ABSTRACT: Hyper-IgG4 syndrome, or IgG4-related disease, is an emerging disorder, involving one or more organ(s), and characterized by “storiform” fibrosis and inflammatory lesions with a predominance of IgG4+ plasma cells and increased IgG4 serum levels. Since the first report of auto-immune pancreatitis, numerous organ lesions have been reported and have been found to occur in a same patient including: sialadenitis, dacryoadenitis, lymphadenopathy, liver and biliary tract involvement, and renal and retroperitoneal lesions. Renal involvement was first described in 2004 and usually presents as functional and/or morphological abnormalities. In most cases, renal pathological analysis reveals tubulointerstitial nephritis that is rarely associated with glomerular lesions. Retroperitoneal fibrosis is also a typical feature that may be associated with periaortitis or inflammatory abdominal aortic aneurysm. First line treatment is based on corticosteroid therapy. Short-term outcome is usually favorable. However, patients should be carefully monitored for relapses and long-term complications. Although the multiple organ lesions share common clinical, biological, radiological and pathological features, no consensus diagnostic criteria have yet been validated for IgG4-related disease. Ruling out differential diagnoses is thus mandatory. Our literature review provides nephrologists, urologists and pathologists with key elements that will help in the early diagnosis and proper management of this new and emerging disorder.
    Néphrologie & Thérapeutique 12/2012; 8(7):499–507. DOI:10.1016/j.nephro.2012.02.007 · 0.55 Impact Factor
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    ABSTRACT: IgG4-related disease (IgG4-RD) is a recently recognized systemic inflammatory disorder that can affect most organs/tissues such as sarcoidosis. The kidney is a frequently affected organ with tubulointerstitial nephritis (TIN), the representative lesion of IgG4-RD. This review focuses on the latest knowledge of IgG4-related kidney disease (IgG4-RKD). A wide range of renal manifestations of IgG4-RD, that is TIN, membranous glomerulonephritis (MGN) and other glomerular lesions, and pyelitis, are collectively referred to as IgG4-RKD. Clinically, decreased renal function, or characteristic imaging findings such as multiple low-density lesions on contrast-enhanced computed tomography or diffuse thickening of the renal pelvic wall, are typical presenting features. Although a rapid response to corticosteroid therapy is a very important feature of IgG4-TIN, in cases in which renal function is moderately to severely decreased before therapy, only partial recovery of renal function is obtained. TIN with characteristic imaging findings is a typical manifestation of IgG4-RKD in the interstitium, while MGN is a representative manifestation of the glomerular lesions. Although IgG4 is a central feature of IgG4-RD, the recent discovery of IgG4-negative IgG4-RD raises questions about the causative role of the IgG4 molecule in this context.This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License, where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially.
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    ABSTRACT: Abstract Objectives Our study aimed to clarify the immunological characteristics and the mechanism of interstitial fibrosis in IgG4 related kidney disease (IgG4-RKD) by the immunohistological analysis. Methods Immunohistological study was performed in the biopsied renal tissues of 16 IgG4-RKD, 16 Sjögren syndrome (SJS) and 17 idiopathic tubulointerstitial nephritis (ITIN) patients using antibodies against IgG, IgG1, IgG4, CD38, CD3, CXCR3, CCR4, Foxp3, Type I, Type III, Type IV and Type VI collagens and transforming growth factor (TGF)-β1. Results Interstitial lymphoplasmacytic and eosinophilic infiltration and the severity of interstitial fibrosis were greater in IgG4-RKD than SJS and ITIN. The ratio of CXCR3+/CD3+ cells was greater in SJS as compared to that in IgG4-RKD and ITIN. The ratio of CCR4+/CD3+ cells was not different among the three diseases. The ratio of interstitial IgG4+/IgG+ plasma cells, Foxp3+/CD3+ cells and TGF-β1+ cells/total infiltrating cells was higher in IgG4-RKD than SJS and ITIN. There was a positive correlation between the ratio of Foxp3+/CD3+ cells and that of IgG4+ /IgG+ plasma cells in IgG4-RKD. The significant correlation was found between the ratio of Foxp3+/CD3+ cells and that of TGF-β1+ cells/total infiltrating cells in IgG4-RKD. Foxp3+ cells and TGF-β1+ cells were colocalized in the interstitium in IgG4-RKD. The significant correlation between the ratio of TGF-β1+ cells/total infiltrating cells and the severity of fibrosis was noticed in IgG4-RKD. The interstitial distribution of type III collagen and type IV collagen was higher in IgG4-RKD than SJS. Conclusions Our results suggest that Treg cells may play a central role in IgG4 production in the interstitium and TGF-β1 induced by Treg cells may play a pivotal role in the interstitial fibrosis including type III and type IV collagens in IgG4-RKD.
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