Cases reported "Glomerulonephritis, IGA"

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1/15. A case of aortitis syndrome and IgA nephropathy: possible role of human leukocyte antigens in both diseases.

    A 51-year-old woman, who had both aortitis syndrome (takayasu arteritis) and IgA nephropathy, presented with hypertension, fever, a high erythrocyte sedimentation rate, high c-reactive protein and serum IgG levels, proteinuria, and renal dysfunction. Renal arteriography showed stenosis and poststenotic dilatation at the origin of the right renal artery, as well as tortuosity of the left renal artery branches and marked atrophy of the left kidney. Renal biopsy showed IgA nephropathy with deposits of IgA, C3, and fibrinogen in the glomeruli and arteriolosclerosis. The present patient had human leukocyte antigen (HLA)-B 52, which is reported to be related to the aortitis syndrome, as well as HLA-DR 4, which is possibly related to IgA nephropathy, suggesting that HLA status may be involved in the pathogenesis of both diseases.
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2/15. IgA nephropathy in patients with congenital C9 deficiency.

    The clinical, histologic, and immunopathological findings of three young Japanese males with congenital C9 deficiency and primary IgA nephropathy are reported. The C9 deficiency was discovered either through mass complement screening, or when low hemolytic activity for CH50 and normal C3 levels were detected in plasma. hematuria and proteinuria were detected at the age of 8 or 9 years as a result of annual urinary screening tests for school children. Renal biopsy showed focal and segmental mesangial proliferation with small epithelial crescents in one patient, and mild, diffuse mesangial proliferation in two. IgA and C3 were deposited predominantly in the mesangial area, and staining for C9 was negative in these patients. Electron microscopy revealed electron dense deposits predominantly in mesangial and paramesangial zones. Immunohistochemical staining in renal biopsy tissues from two patients showed mesangial staining for C5, C8, and S-protein, but staining for C5b-9 neoantigen was completely negative. These results show that the formation of C5b-9 complex is not essential for the induction of human IgA nephropathy, and also for the proliferation of mesangial and even parietal epithelial cells.
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3/15. IgA nephropathy: acute renal failure, acute tubular necrosis, and features of postinfectious acute glomerulonephritis.

    From 1976 to 1987 on our Nephrological Unit, 57 patients with IgA nephropathy (IgAN) proven by renal biopsies were found. Three of those presented with acute tubular necrosis (ATN) and glomerulitis, without extrarenal predisposing cause in two; and showed, as prominent manifestation, a severe acute renal failure syndrome (ARFS), needing dialytic treatment. All three had hematuria, which was macroscopic in two and microscopic in one. Thus the prevalence of the association of glomerulitis and ATN was about 5.2%. There was complete recovery of renal functions in all three patients, but the usual symptomatology of IgAN. Two patients presented polymorphonuclear neutrophils infiltration of glomerular capillaries and in one of them, electron-dense deposits on the epithelial side of glomerular basement membrane ("humps") were observed, as well as those identified in the mesangial area. The glomerular polymorphonuclear neutrophils infiltration and endothelial cells proliferation (cases 1 and 3), the presence of "humps" (case 1), high antistreptolysin O (ASO) titers (cases 1 and 2), and low serum complement levels (case 1), suggest the possibility that antigens able to cause postinfectious glomerulonephritis (streptococcal or not) could induce in some individuals, by another immunopathogenetic route, mixed histopathological and clinical features of IgAN and postinfectious glomerulonephritis.
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4/15. IgA nephritis in HIV-positive patients: a new HIV-associated nephropathy?

    Four HIV-positive patients were shown to have IgA-associated nephritis on biopsy, including one with anaphylactoid purpura. Three were homosexuals, while the fourth acquired the infection from his mother. All had hematuria, a variable degree of proteinuria and renal disease with a benign course. Serologic studies showed elevated levels of IgA as well as IgA immune complexes and rheumatoid factor. IgA antibodies to multiple hiv antigens were detected by Western blot. Pathologic studies showed tubuloreticular inclusions in endothelial cells and nuclear bodies in interstitial cells in all cases. hiv antigens were not detected in kidney biopsies by monoclonal antibodies nor was HIV viral genome demonstrated by in situ hybridization. The possibility that this represents a unique type of IgA-associated HIV nephropathy is discussed.
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5/15. glomerulonephritis associated with hepatitis B surface antigenemia. Report of a case with features of both membranous and IgA nephropathy.

    A 40-year-old man with hepatitis B surface (HBs) antigenemia developed the nephrotic syndrome. Renal biopsy revealed a glomerulonephritis with features of both membranous glomerulonephropathy and IgA nephropathy. Histologically some glomeruli showed mesangial expansion and hypercellularity only, while others contained sclerotic segments. Direct immunofluorescence demonstrated granular IgG-bearing deposits along the peripheral glomerular capillaries and IgA-containing ones in the mesangium. HBs antigen was detected by indirect immunofluorescence both along the glomerular capillary walls and within the mesangium. Granular epimembranous and mesangial deposits were observed by electron microscopy. A few mesangial deposits consisted of spherical particles, 35-100 nm in diameter. Although 3 cases of mixed membranous and IgA nephropathy have been previously reported, this appears to be the first one to be associated with HBs antigenemia.
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6/15. siblings with IgA nephropathy and diffuse proliferative glomerulonephritis (PGN) associated with identical HL-A antigens.

    siblings with IgA nephropathy and diffuse proliferative glomerulonephritis without mesangial IgA deposits (PGN) who had identical HL-A antigens are described. A 21-year-old woman suffered from IgA nephropathy and her 27-year-old sister showed diffuse proliferative glomerulonephritis (PGN) distinct from IgA nephropathy. These siblings had identical HL-A alloantigens which were A2, Aw24; Bw16, Bw35; Cw4, Cw7; DR4. Microhematuria and increased serum IgA levels were shown in their father and other sister but renal biopsies were not performed. The serotypes for the HL-A antigens of their father and other sister were A2, Aw24; Bw27, Bw35; Cw4; DR4 and A2; Bw16; Cw7; Dr2, respectively. It is demonstrated that IgA nephropathy and PGN share a common HL-A phenotype including DR4. It is postulated that the presence of DR4 antigens is not directly related to the occurrence of IgA nephropathy.
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7/15. IgA nephropathy and membranous nephropathy associated with hepatitis B surface antigenemia.

    This is the first report of glomerular disease in a 13-year-old boy who was a carrier of hepatitis b virus both mesangial IgA and subepithelial IgG deposits in the glomeruli at the same time. The clinical findings resembled those of IgA nephropathy. On electron microscopy, electron-dense deposits were identified not only in the mesangium but also on the epithelial side of the glomerular basement membrane. Immunofluorescence and immunoperoxidase staining demonstrated hepatitis B surface antigen and hepatitis B core antigen in the glomeruli but hepatitis B e antigen was not detected. Our findings suggest hepatitis b virus antigens have a pathogenetic role in the simultaneous development of these two glomerulopathies.
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8/15. Striking elevation of serum IgA, IgA-containing immune complexes, and IgA rheumatoid factor in clinically silent dermatitis herpetiformis.

    We describe a patient with dermatitis herpetiformis with immunoglobulin a (IgA)-containing circulating immune complexes and IgA rheumatoid factor who presented with acute renal insufficiency; a renal biopsy specimen showed IgA nephropathy. The renal function and proteinuria spontaneously returned to normal despite markedly elevated levels of IgA-containing circulating immune complexes, IgA rheumatoid factor, and IgA antibodies to some environmental antigens. IgA1 was the predominant subclass. IgA2-containing immune complexes and IgM rheumatoid factor were not detected. Cultures of peripheral blood mononuclear cells produced increased quantities of IgA and IgA rheumatoid factor spontaneously and after pokeweed mitogen stimulation. These data indicate that renal function can improve and remain normal despite the presence of increased levels of IgA-containing circulating immune complexes and IgA rheumatoid factor.
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9/15. IgA nephropathy occurring in the context of previous acute glomerulonephritis.

    A 37-year-old female presented with acute onset of glomerulonephritis 10 days following an upper respiratory infection. serum complement components were depressed and proteinuria exceeded 3.0 g daily. Renal biopsy revealed granular staining of IgG and C3 along the basement membrane as well as small amounts of IgA in a linear pattern. Gradual resolution of symptoms was followed by recrudescent proteinuria 2 years later. Renal biopsy at this time revealed large deposits of IgA in a mesangial staining pattern consistent with a diagnosis of IgA nephropathy. Possible mechanisms for this unusual morphologic transformation include enhanced mesangial permeability as well as mesangial sequestration of an exogenous antigen.
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10/15. IgA nephropathy associated with enteric fever.

    3 cases of enteric fever (2 paratyphoid and 1 typhoid) associated with IgA nephropathy were reported. salmonella Vi antigen was demonstrated in the glomeruli. The clinical syndrome disappeared after enteric fever was treated. Possible pathogenesis was discussed relating this intestinal infection to IgA nephropathy.
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