Occurrence of C3 nephritic factor and C4 nephritic factor in membranoproliferative glomerulonephritis (MPGN). 1994

H Ohi, and T Yasugi
Department of Internal Medicine II, Nihon University School of Medicine, Tokyo, Japan.

One hundred patients diagnosed with hypocomplementaemic MPGN (C3 < 40%) were studied to determine the presence of C3 nephritic factor (C3NeF) and/or C4 nephritic factor (C4NeF). Of those studied, 12 were C3NeF-positive, nine were C4NeF-positive and 10 were positive for both C3NeF and C4NeF. In the 10 patients both C3NeF- and C4NeF-positive, a marked decrease in C3 and C5 levels and a decrease in levels of late components from C6 to C9 were observed. This observation was in contrast to that seen in patients who were either C3NeF- or C4NeF-positive. Patients positive for both C3NeF and C4NeF continued to exhibit hypocomplementaemia after therapy. Immunofluorescent findings revealed heavy C3 immunoglobulin deposits in the 10 patients who were both C3NeF- and C4NeF-positive, whereas no such deposits were found in those patients who were either C3NeF- or C4NeF-positive only. When those patients who were both C3NeF- and C4NeF-positive were compared with those who were either C3NeF- or C4NeF-positive, nephritic syndrome and a poor prognosis were observed more frequently. This study demonstrates a correlation between clinical outcome and hypocomplementaemic MPGN. Further investigations of MPGN as an autoimmune disease are necessary.

UI MeSH Term Description Entries
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003165 Complement System Proteins Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY). Complement Proteins,Complement,Complement Protein,Hemolytic Complement,Complement, Hemolytic,Protein, Complement,Proteins, Complement,Proteins, Complement System
D003176 Complement C3 A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase. C3 Complement,C3 Precursor,Complement 3,Complement C3 Precursor,Complement Component 3,Precursor-Complement 3,Pro-C3,Pro-Complement 3,C3 Precursor, Complement,C3, Complement,Complement, C3,Component 3, Complement,Precursor Complement 3,Precursor, C3,Precursor, Complement C3,Pro C3,Pro Complement 3
D003178 Complement C3 Nephritic Factor An IgG autoantibody against the ALTERNATIVE PATHWAY C3 CONVERTASE, found in serum of patients with MESANGIOCAPILLARY GLOMERULONEPHRITIS. The binding of this autoantibody to C3bBb stabilizes the enzyme thus reduces the actions of C3b inactivators (COMPLEMENT FACTOR H; COMPLEMENT FACTOR I). This abnormally stabilized enzyme induces a continuous COMPLEMENT ACTIVATION and generation of C3b thereby promoting the assembly of MEMBRANE ATTACK COMPLEX and cytolysis. C3 NeF,C3 NeF IgG Autoantibodies,C3 NeF IgG Autoantibody,C3NeF IgG Autoantibodies,C3NeF IgG Autoantibody,C 3 Nephritic Factor,C3 Nephritic Factor,Complement 3 Nephritic Factor,Autoantibody, C3NeF IgG,IgG Autoantibody, C3NeF
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001323 Autoantibodies Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them. Autoantibody
D015432 Glomerulonephritis, Membranoproliferative Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS, increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Various subtypes are classified by their abnormal ultrastructures and immune deposits. Hypocomplementemia is a characteristic feature of all types of MPGN. C3G Complement 3 Glomerulopathy,Complement 3 Glomerulopathies,Complement 3 Glomerulopathy,Glomerulonephritis, Mesangiocapillary,MPGN Membranoproliferative Glomerulonephritis,Membranoproliferative Glomerulonephritis,Mesangiocapillary Glomerulonephritis,DDD MPGNII,Dense Deposit Disease,Glomerulonephritis, Hypocomplementemic,MPGNII,Membranoproliferative Glomerulonephritis Type II,Membranoproliferative Glomerulonephritis, Type I,Membranoproliferative Glomerulonephritis, Type II,Membranoproliferative Glomerulonephritis, Type III,Mesangiocapillary Glomerulonephritis, Type I,Mesangiocapillary Glomerulonephritis, Type II,Subendothelial Membranoproliferative Glomerulonephritis,Type II MPGN,DDD MPGNIIs,Glomerulonephritides, MPGN Membranoproliferative,Glomerulonephritides, Membranoproliferative,Glomerulonephritis, MPGN Membranoproliferative,Glomerulopathies, Complement 3,Glomerulopathy, Complement 3,Hypocomplementemic Glomerulonephritides,Hypocomplementemic Glomerulonephritis,MPGN Membranoproliferative Glomerulonephritides,MPGN, Type II,MPGNII, DDD,MPGNIIs,Membranoproliferative Glomerulonephritides,Membranoproliferative Glomerulonephritides, MPGN,Membranoproliferative Glomerulonephritis, MPGN,Membranoproliferative Glomerulonephritis, Subendothelial,Mesangiocapillary Glomerulonephritides,Type II MPGNs

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