title: Local leukocyte proliferation as a target for cyclophosphamide in the treatment of Henoch-Schönlein purpura nephritis grade VI.
abstract: Henoch-Schönlein purpura nephritis (HSPN) is one of the most common types of chronic glomerulonephritis in children; however, there have been few reports on the pathogenesis and management of grade VI HSPN. We present the case of a 6-year-old boy with grade VI HSPN accompanied by severe nephrotic syndrome and hypocomplementaemia. Immunohistological studies revealed profound glomerular accumulation of CD45- and CD68-positive inflammatory cells. Moreover, some cells expressed the proliferating marker proliferating cell nuclear antigen. His proteinuria and general oedema persisted despite repeated high-dose steroid therapy; however, these clinical symptoms immediately improved after beginning treatment with cyclophosphamide (CyP). Grade VI HSPN was successfully treated with steroids and immunosuppressants. Among immunosuppressive drugs, CyP was considered the most effective.
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Kaneko
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: Yohei
authors.initials: Y
authors.lastname: Ikezumi
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Yamada
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: Hiroya
authors.initials: H
authors.lastname: Hasegawa
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: Utako
authors.initials: U
authors.lastname: Kaneko
authors.affiliation: Department of Pediatrics, Niigata University Medical and Dental Hospital, Niigata, Japan.
authors.forename: Akihiko
authors.initials: A
authors.lastname: Saitoh
mesh_terms: D001706:Biopsy; D049109:Cell Proliferation / Q000187:drug effects*; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005455:Fluorescent Antibody Technique; D015432:Glomerulonephritis, Membranoproliferative / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D011695:IgA Vasculitis / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007668:Kidney / Q000187:drug effects* / Q000276:immunology / Q000473:pathology; D007962:Leukocytes / Q000187:drug effects* / Q000276:immunology / Q000473:pathology; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000276:immunology; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide
keywords: glomerulonephritis; hypocomplementaemia; immunosuppressant; macrophage; nephrotic syndrome