1/132. biopsy-proven resolution of immune complex-mediated crescentic glomerulonephritis with mycophenolate mofetil therapy in an allograft.We report biopsy-proven resolution of immune-complex-mediated crescentic glomerulonephritis (ICMCGn) using mycophenolate mofetil (MMF). Therapy with steroids and cyclophosphamide failed twice in a 39-year-old white man who developed ICMCGn in his native kidneys, and subsequently in a human lymphocyte antigen-identical renal allograft. When he developed ICMCGn in a second, now cadaver, allograft, he was treated with steroids and MMF instead. His serum creatinine (Cr) improved from 4.4 mg/dL to 2.1 mg/dL. A biopsy 21 months later showed him to be free of glomerular disease. MMF is known to be an effective immunosuppressant. In our patient, ICMCGn, a notoriously difficult entity to treat effectively, seemingly resolved with MMF therapy. We suggest that MMF may be effective in the treatment of immunologically mediated pre-end-stage renal disease (ESRD). It should be considered in any posttransplantation setting where the original cause of organ failure is known to be immunologically mediated and likely to recur.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/132. Fibrillary glomerulonephritis in siblings.Fibrillary/immunotactoid glomerulopathy is characterized by organized glomerular deposition of extracellular, nonbranching, immunoglobulin-derived microfibrils, which is not associated with systemic diseases such as amyloidosis, cryoglobulinemia, or monoclonal gammopathy. This is an uncommon condition with an obscure etiology and accounts for approximately 1% of primary glomerular diseases in white populations. We report the first case of familial fibrillary/immunotactoid glomerulopathy affecting a brother and a sister in a Chinese family. Both patients presented with heavy proteinuria, which improved transiently on treatment with prednisolone and cyclophosphamide. Human lymphocyte antigen typing for the siblings showed no haplotype association. Despite the generally poor renal prognosis reported in the literature, with 50% of patients reaching end-stage renal failure within 2 to 4 years, both patients had relative preservation of renal function (creatinine clearance from 79 to 76 mL/min/1.73 m2 after 2 years in one patient and from 111 to 99 mL/min/1.73 m2 after 3 years in the other). Our observations show that fibrillary/immunotactoid glomerulopathy can present as a familial condition. Compared with sporadic cases, patients with familial fibrillary/immunotactoid glomerulopathy may have a more favorable renal prognosis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/132. Antineutrophil cytoplasmic antibodies (ANCA) and systemic vasculitis: update of assays, immunopathogenesis, controversies, and report of a novel de novo ANCA-associated vasculitis after kidney transplantation.OBJECTIVES: To characterize antineutrophil cytoplasmic antibodies (ANCA), their major autoantigens, disease associations, and pathophysiology in systemic vasculitides. To describe a patient with a novel de novo ANCA-associated vasculitis after kidney transplantation. methods: We reviewed and compiled the literature on ANCA-related topics and systemic vasculitis. Laboratory and clinical data from a cadaveric kidney transplant patient who developed necrotizing vasculitis involving glomerular capillaries, with crescent formation associated with P-ANCA and myeloperoxidase, were analyzed. RESULTS: Large-scale multi-center testing of patient and normal sera by the European ANCA Assay Standardization Project using immunofluorescence assays and enzyme immunoassays indicate the assays have good sensitivity and specificity, and diagnostic utility for ANCA-associated vasculitis. A few investigations covering basic and clinical research with ANCA remain controversial: whether endothelial cells do or do not express a 29-kd neutral serine protease termed proteinase-3 (PR-3), the target of ANCA in most individuals with Wegener's granulomatosis, and whether anti-myeloperoxidase (MPO) ANCAs recognize a restricted number of epitopes on MPO. This issue has relevance for using monoclonal antibodies to treat patients with vasculitis who have adverse effects from immunosuppressive drugs. The two allelic forms of FcgammaRIIa (H131/R131) and the two of FcgammaRIIlb (NA1/NA2) are discussed as possible inheritable genetic elements for vasculitic disorders and for signaling responses. Stimulatory and costimulatory molecules, and cytokine profiles of T lymphocytes are characterized to show that these cells are actively involved in the ANCA-associated vasculitides. The patient described had a de novo ANCA associated small vessel vasculitis which developed after renal transplantation. CONCLUSIONS: There have been significant advances in the development of sensitive and specific ANCA assays. The immunopathogenetic mechanism of ANCA involves the constitutive FcgammaRs, ligands, and signaling responses to activate cytokine-primed neutrophils. This may lead to the generation of reactive oxygen intermediates, degranulation, and secretion of intracellular granule contents, and ultimately inflammation and vasculitis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/132. Transformation of hepatitis b virus-related membranous glomerulonephritis to crescentic form.We report a case of hepatitis b virus-(HBV) related membranous glomerulonephritis which progressed to crescentic transformation after withdrawal of immunosuppressive treatment. Immunosuppressive therapy probably enhanced HBV replication, and its withdrawal led to a return of immune competence resulting in progression of the glomerulonephritis. Prior screening of all patients for hepatitis B surface antigen, before using immunosuppressive therapy may prevent this complication. The usage of immunosuppressive therapy as a first-line choice in HBV-related glomerulonephritis may result in harmful complications.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/132. Endocapillary proliferative glomerulonephritis in a patient with parvovirus B19 infection.A 45-year-old woman developed acute nephritic syndrome after erythema infectiosum. Laboratory data on admission showed decreased serum C3, C4, and CH50 levels and the presence of both immunoglobulin (Ig) M and IgG antibodies to human parvovirus B19 (HPV). A renal biopsy showed diffuse endocapillary proliferative glomerulonephritis. Immunofluorescence microscopy indicated 2 granular staining for IgG, IgM, and C3 over the mesangial area and along glomerular capillary walls. HPV antigen was also detected in glomeruli by immunohistochemistry. Electron microscopy showed electron-dense deposits in the subendothelial space and the paramesangial area. These findings suggest that immune complex-type glomerulonephritis is caused by glomerular deposition of HPV antigen-antibody complexes in some patients with HPV infection.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
6/132. recurrence of acute poststreptococcal glomerulonephritis.recurrence of acute poststreptococcal glomerulonephritis (APSGN) is a rare phenomenon. We present an 8-year-old boy with a second episode of APSGN 12 months following a complete clinical recovery from his initial attack. Renal histology, obtained from renal biopsies of the patient during the second attack, showed diffuse endocapillary proliferation, granular deposition of C3, IgG, IgA, and fibrinogen along capillary walls, and subepithelial electron-dense deposits. A new streptococcal cytoplasmic antigen (nephritis-associated plasmin receptor protein, NAP1r), which was recently identified as the pathogenic antigen in APSGN, was detected in the glomeruli of an early kidney biopsy specimen from the patient during the second attack of APSGN, using fluorescein isothiocyanate-labeled rabbit anti-NAP1r. However, antibodies against NAP1r, examined by Western blotting, were not present in sera from the patient. These results suggest that recurrence of APSGN in some patients may be caused by an absence of a natural immune response to NAP1r.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
7/132. Longitudinal study of circulating immune complexes in a patient with staphylococcus albus-induced shunt nephritis.The direct measurement and partial characterization of circulating immune complexes has been performed in a longitudinal study of a patient with staphylococcus albus-induced shunt nephritis. The high levels of immune complexes were associated with cryoglobulinaemia and hypocomplementaemia. The activation of complement was found to be via the classical pathway, but the functioning of the alternative pathway may have been impaired in vivo due to very low levels of C3. The host response to the infection was also characterized by the production of a marked macroglobulinaemia, high titres of rheumatoid factor and a typical acute phase increase in the c-reactive protein level. Immune complex levels were persistently elevated many months after the removal of the focus of the infection. A possible explanation for this surprising finding may lie in the nature of the antigens in the immune complexes. It was found that the immune complexes contained both antibodies to and antigens from Staphlococcus albus. In particular, glycerol teichoic acid and staphylococcal nuclease were identified as components of the immune complexes present during the acute phase. glycerol teichoic acid was also identified in the immune complexes found later although other staphylococcus albus antigens as yet unidentified were also present and persisted in the circulation for several months.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
8/132. glomerulonephritis after recombinant hepatitis B vaccine.Renal complications after vaccination with hepatitis B recombinant vaccine are rare; very few cases are reported without a histopathologic definition. We describe a case of glomerulonephritis in a 12-year-old girl, beginning after the inoculation of hepatitis B vaccine. The immunohistochemical examination of her renal biopsy, with the use of monoclonal antibodies, shows the presence of hepatitis B surface antigen in renal tissue. After 2 years chronic glomerulonephritis persisted and was treated with angiotensin-converting enzyme inhibitors. After 3 years of follow-up, the renal disease was in remission, and treatment was stopped.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/132. Acute glomerulonephritis complicating australia antigen-negative viral hepatitis.A case of acute glomerulonephritis complicating australia antigen-negative hepatitis in a 17-year-old man is described. The literature relating the onset of glomerulonephritis to virus infection is general, and to that of hepatitis in particular, is reviewed.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
10/132. Rapidly progressive, pauci-immune diffuse crescentic glomerulonephritis in an infant.A Japanese male infant presented with nephrotic syndrome at 41 days. His renal function progressively deteriorated, and he died at 4 months of the age. An open renal biopsy revealed diffuse crescentic glomerulonephritis (CrGN) without immune complex deposition, which is not characteristic of the congenital nephrotic syndrome (CNS). Examination for nephrin antigen using rabbit anti-nephrin extra- and intracellular site antibodies was positive. These clinical observations suggest that the patient had a unique histological variant of CNS. This is the first report of rapidly progressive, pauci-immune diffuse CrGN in infancy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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