title: Chronic glomerulonephritis associated with IgG subclass deficiency.
abstract: We experienced two patients with IgG subclass deficiency who suffered from chronic glomerulonephritis (GN). Patient 1 was a 17-year-old girl with IgG subclass deficiency (combined deficiency of IgG2 and IgG4). Renal biopsy was performed when she was aged 16 years, and she was diagnosed with membranoproliferative GN. Patient 2 was a 16-year-old girl with IgG subclass deficiency (combined deficiency of IgG2, IgG3, and IgG4). Renal biopsy was performed when she was aged 15 years, and she was diagnosed with membranous nephropathy. We examined the glomerular deposition patterns of their IgG subclasses. Furthermore, we compared their clinical and laboratory findings with those of three patients with IgG subclass deficiency without GN. Patients with GN suffered infections more frequently than those without GN. The serum levels of IgG (especially IgG1) and IgM were higher in patients with GN than in those without GN. In patient 1 IgG1 and IgG3 were deposited in a mesangiocapillary pattern, but, in patient 2, only IgG1 was deposited in a capillary pattern. Thus, the different patterns of IgG subclass deficiency between the two patients may be responsible for their different glomerular pathologies. To the best of our knowledge, this is the first report of chronic GN in patients with IgG subclass deficiency.
authors: Department of Nephrology, National Children's Medical Center, National Center for Child Health and Development, 10-1, Okura 2chome, Setagaya-ku, Tokyo, 157-8535, Japan.
authors: Koichi
authors: K
authors: Kamei
authors: Atsuko
authors: A
authors: Nakagawa
authors: Yasufumi
authors: Y
authors: Otsuka
authors: Makiko
authors: M
authors: Nakayama
authors: Shinichi
authors: S
authors: Kobayashi
authors: Kentaro
authors: K
authors: Matsuoka
authors: Kazumoto
authors: K
authors: Iijima
mesh_terms: D000293:Adolescent; D000328:Adult; D047228:Angiotensin II Type 1 Receptor Blockers / Q000008:administration & dosage / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D001562:Benzimidazoles / Q000008:administration & dosage / Q000627:therapeutic use; D001706:Biopsy; D001713:Biphenyl Compounds; D016022:Case-Control Studies; D002908:Chronic Disease; D005260:Female; D005455:Fluorescent Antibody Technique; D005500:Follow-Up Studies; D015432:Glomerulonephritis, Membranoproliferative / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D015433:Glomerulonephritis, Membranous / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology / Q000601:surgery; D006801:Humans; D017099:IgG Deficiency / Q000097:blood* / Q000145:classification / Q000175:diagnosis; D007132:Immunoglobulin Isotypes / Q000276:immunology; D007075:Immunoglobulin M / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007678:Kidney Glomerulus / Q000276:immunology; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D010503:Periodic Acid-Schiff Reaction / Q000379:methods; D020551:Pulse Therapy, Drug; D013777:Tetrazoles / Q000008:administration & dosage / Q000627:therapeutic use; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D047228:Angiotensin II Type 1 Receptor Blockers; D000893:Anti-Inflammatory Agents; D001562:Benzimidazoles; D001713:Biphenyl Compounds; D007132:Immunoglobulin Isotypes; D007075:Immunoglobulin M; D016756:Immunoglobulins, Intravenous; D013777:Tetrazoles; C081643:candesartan; D008775:Methylprednisolone