title: Plasma exchange combined with immunosuppressive treatment in a child with rapidly progressive IgA nephropathy.
abstract: Although diffuse crescentic formation in immunoglobulin A (IgA) nephropathy, histologically characterized by extensive extracapillary proliferation, is assumed to have a poor prognosis, there has still been no established treatment because of the low prevalence of the condition, especially in pediatric patients. This paper reports on a 5-year-old boy with rapidly progressive IgA nephropathy requiring dialysis for 1 month. He had been treated with plasma exchange (PE) combined with immunosuppressive treatment, including steroids and mizoribine, because renal function deteriorated rapidly despite initial treatment with intravenous methylprednisolone pulse. The histological findings at that time revealed IgA nephropathy, with large circumferential cellular crescent formation in approximately 80% of the glomeruli. Three weeks after PE initiation, serum levels of creatinine and IgA-containing immune complexes returned to normal, and urinary protein excretion gradually decreased. The second renal biopsy taken 7 months later demonstrated mild IgA nephropathy with small fibrocellular crescents. This case report indicates that PE combined with immunosuppressive treatment may benefit children with rapidly progressive IgA nephropathy, even when extensive crescent formations are present.
authors.affiliation: Division of Nephrology, Saitama Children's Medical Center, 2100 Magome, Iwatsuki-ku, Saitama-city Saitama, 339 8551, Japan. f_shuich@d2.dion.ne.jp
authors.forename: Shuichiro
authors.initials: S
authors.lastname: Fujinaga
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Ohtomo
authors.forename: Daisuke
authors.initials: D
authors.lastname: Umino
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Mochizuki
authors.forename: Hitohiko
authors.initials: H
authors.lastname: Murakami
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Shimizu
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Yamashiro
authors.forename: Kazunari
authors.initials: K
authors.lastname: Kaneko
mesh_terms: D002675:Child, Preschool; D004359:Drug Therapy, Combination; D005920:Glomerular Mesangium / Q000737:chemistry / Q000473:pathology / Q000648:ultrastructure; D005922:Glomerulonephritis, IGA / Q000175:diagnosis / Q000628:therapy*; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D010951:Plasma Exchange; D016896:Treatment Outcome
chemical_list: D007070:Immunoglobulin A; D007166:Immunosuppressive Agents