Antibodies, Antineutrophil Cytoplasmic ; immunology ; Glomerulonephritis ; immunology ; Humans

Association of HLA antigens with certain diseases provide insights into genetically determined susceptibility to disease. Although nephrotic syndrome is one of the commonest diseases, it is poorly understood. A group of 57 patients suffering from a minimal lesion nephrotic syndrome (33 patients) and mesangioproliferative glomerulonephritis (24 patients) was studied for immunologic markers. The incidence of HLA-A w 24 is significantly greater in the minimal lesion nephrotic syndrome patients than in controls (18.7% in patients, 0% in controls, p < 0.01). This report fails to show a high incidence of specific HLA antigen in mesangioproliferative glomerulonephritis patients. We believe that the high incidence of HLA-Aw 24 in minimal lesion nephrotic syndrome is indicative of a congenital predisposition to nephrotic syndrome.
Glomerulonephritis/immunology ; HLA Antigens/analysis* ; Human ; Nephrosis, Lipoid/immunology*

Percutaneous renal biopsy was performed on a 34 year old male patient with mild proteinuria and microhematuria. Histopathologic examination showed a focal mesangiopathic glomerulonephritis, simulating a "minimal change" disease pattern by light microscope. Granular deposits of IgA, C3, IgG, IgM, and fibrinogen were present in the glomerular mesangial area by immunofluorescent technique. A special prevalence of IgA was found. The intensity of immunofluorescent staining was correlated with the mesangial proliferative reaction by light microscopy. Electron microscopy showed electron dense granular deposits in the mesangial areas. The glomerulonephritis in this patient was related with the IgA antibody associated mesangial immune complex deposit disease mediated by the classic complement pathway. This glomerulonephritis is known to have a good prognosis. The antigenic nature, the reason of predominant immune deposits in the mesangium, and the mechanism of a special prevalence of IgA and IgM immunoglobulin classes are discussed, and special attention to the value of immunofluorescent study of renal diseases, with a review of the literature, is given.
Adult ; Case Report ; Glomerulonephritis/immunology* ; Glomerulonephritis/pathology ; Human ; Immunoglobulin A/analysis* ; Immunoglobulin G/analysis* ; Kidney/ultrastructure ; Male

T cell immunity and phagocytic activity were studied in the blood of patients with IgA nephropathy in order to clarify their roles in the pathogenesis of IgA nephropathy. The percentages of total T lymphocytes, helper T cell and suppressor T cells were significantly reduced in patients. A significantly elevated helper T cell/suppressor T cell ratio in patients showed a predominant reduction in suppressor T cells. There was a significant relationship between histologic findings and helper T cell/suppressor T cell ratio in patients. Natural Killer (NK) cell activity was significantly reduced but the lymphocyte response after phytohemagglutinin (PHA) stimulation was not in patients. ConA-induced suppressor cell activity was not depressed despite of a decrease in suppressor T cells in patients. Phagocytic activity of polymorphonuclear leucocytes (PMNs) ingesting yeasts was significantly reduced in patients. Also an inverse correlation was found between serum IgA levels and phagocytic activity of PMN. It is concluded that suppressor T cell defects, depressed phagocytic activity and impaired NK cell activity may play a role in the pathogenesis of IgA nephropathy.
B-Lymphocytes/immunology ; Glomerulonephritis, IGA/*immunology/pathology ; Humans ; Killer Cells, Natural/immunology ; Neutrophils/immunology ; *Phagocytosis ; T-Lymphocytes/*immunology ; T-Lymphocytes, Regulatory/immunology

In order to determine the extent to which specific forms of glomerulonephritis (GN) contribute to the pool of crescentic GN, renal tissues from 17 crescentic GN patients were examined with special attention to glomerular and interstitial neutrophil infiltration. Renal tissues from five normal kidneys served as normal controls. Renal biopsy tissues from five patients with postinfectious GN in which crescent formation was not observed were also examined as disease controls. The patients were put into both three groups according to immunofluorescence findings and two groups according to the active or inactive phase of the crescents: group 1 with anti-glomerular basement membrane crescentic GN, one case; group 2 with immune complex crescentic GN, ten cases; and group 3 with pauci-immune crescentic GN, six cases. Four of the nine individuals tested were positive for anti-neutrophil cytoplasmic antibody (44.4%). Glomerular and interstitial neutrophil infiltrations were prominent in both the active and inactive phase groups, compared to normal controls (p<.05). Glomerular neutrophil infiltration was significantly prominent in the active phase group, compared to the inactive phase group (p<.001). In both the active and inactive phase groups, interstitial neutrophil infiltration was prominent, compared to disease control groups (p<.05). These results support the concept of the participation of periglomerular leukocytes in the renal tissue damage of crescentic GN, although the role of neutrophils was not examined.
Adult ; Aged ; Female ; Follow-Up Studies ; Glomerulonephritis/pathology* ; Glomerulonephritis/immunology ; Glomerulonephritis/classification ; Human ; Kidney Glomerulus/pathology* ; Kidney Glomerulus/immunology ; Male ; Middle Age ; Nephritis, Interstitial/pathology* ; Nephritis, Interstitial/immunology ; Neutrophils/physiology*

OBJECTIVETo study the clinicopathologic features of membranous nephropathy coexisting with IgA nephropathy.

METHODSThe renal biopsies performed in Peking University First Hospital during the period from January, 1998 to April, 2006 were retrospectively reviewed. The clinicopathologic features of 11 cases of membranous nephropathy coexisting with IgA nephropathy were studied. Electron microscopy with immunogold labeling for IgG and IgA were also performed.

RESULTSThe mean age of patients was 39.9 years. The male-to-female ratio was 1:2.9. The patients mainly presented with proteinuria. Proteinuria of nephrotic level was seen in 7 cases (63.6%). Seven cases also had associated microscopic hematuria. None of them showed evidence of renal insufficiency. Cases with secondary diseases, such as hepatitis virus infection and systemic lupus erythematosus, were excluded from the study. Histologically, vacuolation and thickening of glomerular basement membrane was seen. There was also mild mesangial hypercellularity and increase in mesangial matrix. Occasional glomeruli with crescent formation were identified in 2 cases. Immunofluorescence study showed granular staining for IgG and C3 along glomerular capillary walls, in addition to clumps of IgA deposits in mesangium. Electron microscopy revealed subepithelial and mesangial electron-dense deposits. Immunogold labeling showed IgG and IgA localized in the subepithelial and mesangial deposits respectively.

CONCLUSIONMembranous nephropathy coexisting with IgA nephropathy possesses the clinicopathologic features of both components. It might be caused by independent occurrence of the two entities.

Adult ; Female ; Glomerular Basement Membrane ; immunology ; pathology ; ultrastructure ; Glomerular Mesangium ; immunology ; pathology ; ultrastructure ; Glomerulonephritis, IGA ; complications ; immunology ; pathology ; Glomerulonephritis, Membranous ; complications ; immunology ; pathology ; Humans ; Immunoglobulin A ; metabolism ; Immunoglobulin G ; metabolism ; Kidney Glomerulus ; immunology ; pathology ; ultrastructure ; Male ; Middle Aged ; Retrospective Studies

Measurements of serum C3 levels by the single radial immunodiffusion method using Helena plates were done in 41 patients with acute poststreptococcal glomerulonephritis and in 25 healthy children. In 28 patients the sequential determination were done until returning the level above normal range. The mean value of serum C3 concentration in this group as a who1e was lower than in the control group and the difference was statistically significant (p < 0.001). The initial reduction of serum C3 concentration did not correlate with the severity of the acute phase of the disease. In those children with acute poststreptococcal glomerulonephritis, serum C3 concentration returned to normal within six weeks.
Adolescent ; Child ; Child, Preschool ; Complement 3/analysis* ; Complement 3/deficiency ; Female ; Glomerulonephritis/etiology ; Glomerulonephritis/immunology* ; Human ; Male ; Streptococcal Infections/complications ; Streptococcal Infections/immunology*

OBJECTIVETo highlight current knowledge about M-type phospholipase A2 receptor (PLA2R) which is the first human autoantigen discovered in adult idiopathic membranous nephropathy.

DATA SOURCESRelevant articles published in English from 2000 to present were selected from PubMed. Searches were made using the terms "idiopathic membranous nephropathy, M-type PLA2R and podocyte."

STUDY SELECTIONArticles studying the role of M-type PLA2R in idiopathic membranous nephropathy were reviewed. Articles focusing on the discovery, detection and clinical observation of anti-PLA2R antibodies were selected.

RESULTSM-type PLA2R is a member of the mannose receptor family of proteins, locating on normal human glomeruli as a transmembrane receptor. The anti-PLA2R in serum samples from MN were primarily IgG4 subclass. Technologies applied to detect anti-PLA2R autoantibody are mainly WB, IIFT, ELISA and so on. Studies from domestic and overseas have identified a strongly relationship between circulating anti-PLA2R levels and disease activity.

CONCLUSIONRecent discoveries corresponding to PLA2R facilitate a better understanding on IMN pathogenesis and may provide a new tool to its diagnosis, differential diagnosis, risk evaluation, response monitoring and patient-specific treatment.

Animals ; Autoantigens ; metabolism ; Glomerulonephritis, Membranous ; immunology ; metabolism ; Humans ; Podocytes ; metabolism ; Receptors, Phospholipase A2 ; metabolism

OBJECTIVES: To investigate the possible role of mononuclear cells and their products in the pathogenesis of IgA nephropathy, in vitro expression of ICAM-1 on cultured mouse mesangial cell (MC) was examined after stimulation with mononuclear cell culture supernatant from patients with IgA nephropathy. METHODS: Peripheral blood mononuclear cells (PBMC) were isolated and cultured from 18 patients with primary IgA nephropathy, 8 normal controls and 5 patients with non-IgA nephropathy (FSGS 1, MGN 3, MPGN 1). ICAM-1 expression on cultured mouse MC by TNF-alpha, IL-1 beta and culture supernants of PBMC were analyzed using a cell ELISA method. The concentration of IL-1 beta and TNF-alpha in culture supernatants was measured by using a commercially available radioimmunoassay kit. RESULTS: Addition of human recombinant TNF-alpha induced an increased ICAM-1 expression in a dose-dependent manner. The expression of ICAM-1 was further increased after co-stimulation with TNF-alpha and IL-1 beta. Addition of PBMC culture supernatants into mouse MC induced significantly higher expression of ICAM-1 by supernatants from the patients with IgA nephropathy compared with that from normal controls. The concentration of TNF-alpha and IL-1 beta in supernatants from the patients with IgA nephropathy was significantly higher than that from those with non-IgA nephropathy. CONCLUSION: TNF-alpha and IL-1 released from mononuclear cells induced the up-regulation of ICAM-1 expression and this may be related to the immune pathogenesis of IgA nephropathy.
Animal ; Cells, Cultured ; Glomerular Mesangium/immunology ; Glomerular Mesangium/cytology ; Glomerulonephritis, IGA/immunology* ; Glomerulonephritis, IGA/etiology ; Human ; Intercellular Adhesion Molecule-1/metabolism* ; Interleukin-1/secretion ; Interleukin-1/pharmacology ; Leukocytes, Mononuclear/immunology ; Mice ; Tumor Necrosis Factor/secretion ; Tumor Necrosis Factor/pharmacology

OBJECTIVETo study the difference in clinico-pathological features between IgA nephropathy (IgAN) and Henoch-Schonlein purpura nephritis (HSPN) in children.

METHODSThe medical data of 103 children with HSPN and 61 children with IgAN were retrospectively studied.

RESULTSThere were no significant differences in age, sex and disease course between the HSPN and IgAN groups (P>0.05). Clinical classification demonstrated that more severe conditions were found in the IgAN group than in the HSPN group and gross hematuria was more common in the IgAN group (P<0.05). Serum creatinine and cholesterol levels were higher in the IgAN group than in the HSPN group (P<0.05). Fibrinogen-related antigen deposition was more common in the HSPN group, while complement 3(C3) deposition was more common in the IgAN group. Interstitial fibrosis, tubular casts and tubular inflammatory infiltration were also more common in the IgAN group (P<0.05).

CONCLUSIONSSignificant clinico-pathological differences can be found between HSPN and IgAN in children, and these differences do not support a one disease entity hypothesis.

Child ; Child, Preschool ; Female ; Glomerulonephritis, IGA ; immunology ; pathology ; Humans ; Kidney ; pathology ; Male ; Nephritis ; immunology ; pathology ; Purpura, Schoenlein-Henoch ; immunology ; pathology ; Retrospective Studies