MPO-ANCA associated crescentic glomerulonephritis with numerous immune complexes: case report. 2012

Ryuji Morizane, and Konosuke Konishi, and Akinori Hashiguchi, and Hirobumi Tokuyama, and Shu Wakino, and Hiroshi Kawabe, and Matsuhiko Hayashi, and Koichi Hayashi, and Hiroshi Itoh
Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan. morizanr@da2.so-net.ne.jp

BACKGROUND Antineutrophil cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis (CGN) is a major cause of rapidly progressive glomerulonephritis (RPGN). ANCA-associated CGN is generally classified into pauci-immune RPGN, in which there are few or no immune complexes. METHODS A 78-year-old man presented with RPGN after a 7-year course of chronic proteinuria and hematuria with stable renal function. A blood examination showed a high titer of myeloperoxidase (MPO)-ANCA. A renal biopsy showed crescentic glomerulonephritis with abundant subepithelial, intramenbranous and subendothelial deposits by electron microscopy, leading to the diagnosis of ANCA-associated CGN superimposed on type 3 membranoproliferative glomerulonephritis (MPGN). CONCLUSIONS This case is unique in that type 3 MPGN and MPO-ANCA-associated CGN coexisted, and no similar case has been reported to date. Because ANCA-associated CGN has a predilection for elderly individuals and primary type 3 MPGN is rarely seen in this age group, coincidental existence appears less likely. This case may confer valuable information regarding the link between immune complex and ANCA-associated CGN.

UI MeSH Term Description Entries
D007105 Immune Complex Diseases Group of diseases mediated by the deposition of large soluble complexes of antigen and antibody with resultant damage to tissue. Besides SERUM SICKNESS and the ARTHUS REACTION, evidence supports a pathogenic role for immune complexes in many other IMMUNE SYSTEM DISEASES including GLOMERULONEPHRITIS, systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC) and POLYARTERITIS NODOSA. Hypersensitivity, Type III,Type III Hypersensitivity,Disease, Immune Complex,Diseases, Immune Complex,Hypersensitivities, Type III,Immune Complex Disease,Type III Hypersensitivities
D008297 Male Males
D009195 Peroxidase A hemeprotein from leukocytes. Deficiency of this enzyme leads to a hereditary disorder coupled with disseminated moniliasis. It catalyzes the conversion of a donor and peroxide to an oxidized donor and water. EC 1.11.1.7. Myeloperoxidase,Hemi-Myeloperoxidase,Hemi Myeloperoxidase
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005921 Glomerulonephritis Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY. Bright Disease,Kidney Scarring,Glomerulonephritides,Scarring, Kidney
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D019268 Antibodies, Antineutrophil Cytoplasmic Autoantibodies directed against cytoplasmic constituents of POLYMORPHONUCLEAR LEUKOCYTES and/or MONOCYTES. They are used as specific markers for GRANULOMATOSIS WITH POLYANGIITIS and other diseases, though their pathophysiological role is not clear. ANCA are routinely detected by indirect immunofluorescence with three different patterns: c-ANCA (cytoplasmic), p-ANCA (perinuclear), and atypical ANCA. ANCA,Anti-Neutrophil Cytoplasmic Autoantibodies,Anti-Neutrophil Cytoplasmic Autoantibody,Antineutrophil Cytoplasmic Antibodies,Neutrophil Cytoplasmic Autoantibodies,Neutrophil Cytoplasmic Autoantibody,c-ANCA,p-ANCA,Anti-Neutrophil Cytoplasmic Antibodies,Anti-Neutrophil Cytoplasmic Antibody,Antineutrophil Cytoplasmic Antibody,Anti Neutrophil Cytoplasmic Antibodies,Anti Neutrophil Cytoplasmic Antibody,Anti Neutrophil Cytoplasmic Autoantibodies,Anti Neutrophil Cytoplasmic Autoantibody,Antibody, Anti-Neutrophil Cytoplasmic,Antibody, Antineutrophil Cytoplasmic,Autoantibody, Anti-Neutrophil Cytoplasmic,Autoantibody, Neutrophil Cytoplasmic,Cytoplasmic Antibody, Anti-Neutrophil,Cytoplasmic Antibody, Antineutrophil,Cytoplasmic Autoantibody, Anti-Neutrophil,Cytoplasmic Autoantibody, Neutrophil,c ANCA,p ANCA

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