title: Cyclosporin A therapy for severe Henoch-Schönlein nephritis with nephrotic syndrome.
abstract: To evaluate the efficacy of cyclosporin A (CyA) for treating severe Henoch-Schönlein nephritis (HSN), seven patients with nephrotic syndrome, aged 3.9-13.8 years (mean 6.5 years), were analyzed retrospectively. Mean follow-up times were 5.5 years (range 2-9 years). All underwent renal biopsy before treatment, and follow-up renal biopsy was performed in six of the seven patients. All patients improved, with 24-h protein declining from a mean of 9.2 g/m(2)/day (range 1.5-16 g/m(2)/day) to 0.3 g/m(2)/day (range 0.03-1.2 g/m(2)/day) (p=0.016) and serum albumin increasing from a mean of 2.1 g/dl (range 1.5-2.4 g/dl) to 4.6 g/dl (range 3.5-5.3 g/dl) (p=0.016) after CyA therapy. The activity index decreased significantly at the second renal biopsies obtained at a mean interval of 11.7 months after the first (6.4+/-3.3 vs 3.5+/-1.2, p=0.042, respectively), while the chronicity index and the tubulointerstitial scores did not change. On the immunofluorescent findings at the second biopsies, the degree of deposits of immunoglobulins such as IgA, IgM, C3, and fibrinogen decreased in five of the six patients. Although this case series is without controls, our study suggests that CyA may be beneficial to a subset of HSN patients with nephrotic syndrome.
authors.affiliation: Institute of Kidney Disease, Department of Pediatrics, Yonsei University College of Medicine, Seodaemun-Ku, Seoul, Korea.
authors.forename: Jae Il
authors.initials: JI
authors.lastname: Shin
authors.forename: Jee Min
authors.initials: JM
authors.lastname: Park
authors.forename: Youn Ho
authors.initials: YH
authors.lastname: Shin
authors.forename: Ji Hong
authors.initials: JH
authors.lastname: Kim
authors.forename: Pyung Kil
authors.initials: PK
authors.lastname: Kim
authors.forename: Jae Seung
authors.initials: JS
authors.lastname: Lee
authors.forename: Hyeon Joo
authors.initials: HJ
authors.lastname: Jeong
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D005921:Glomerulonephritis / Q000188:drug therapy*; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications* / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy; D011507:Proteinuria / Q000188:drug therapy; D012189:Retrospective Studies
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine