[Adult case of severe Henoch-Schönlein purpura associated with steroid-resistant nephrotic syndrome successfully treated with intravenous cyclophosphamide].
Henoch-Schönlein purpura (HSP) is a systemic disorder characterized by small vessel vasculitis with the deposition of IgA immune complexes. Renal involvement is the major cause of morbidity and mortality in patients with HSP. We report here a 37-year-old female patient with HSP nephritis (HSPN) associated with steroid-resistant nephrotic syndrome and renal dysfunction despite conventional therapy. The patient was successfully treated with intravenous cyclophosphamide following treatment with intravenous pulse methylprednisolone and oral prednisolone. The combination therapy resulted in a significant decrease in proteinuria, together with improvement of renal function. The patient finally reached a stage of clinical remission.
Namba
Tomoko
T
Division of Nephrology, Osaka Rosai Hospital, Osaka, Japan.
Yamamoto
Takeshi
T
Matsuda
Jun
J
Kadoya
Hiroyuki
H
Takeji
Masanobu
M
Yamauchi
Atsushi
A
D000328:Adult; D000936:Antigen-Antibody Complex / Q000378:metabolism; D003520:Cyclophosphamide / Q000008:administration & dosage*; D004351:Drug Resistance; D005260:Female; D005920:Glomerular Mesangium / Q000378:metabolism; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications* / Q000188:drug therapy*; D007070:Immunoglobulin A / Q000378:metabolism; D007262:Infusions, Intravenous; D008775:Methylprednisolone / Q000008:administration & dosage; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000209:etiology*; D011239:Prednisolone / Q000008:administration & dosage; D011507:Proteinuria / Q000188:drug therapy / Q000209:etiology; D020551:Pulse Therapy, Drug; D012720:Severity of Illness Index; D016896:Treatment Outcome
D000936:Antigen-Antibody Complex; D007070:Immunoglobulin A; D003520:Cyclophosphamide; D011239:Prednisolone; D008775:Methylprednisolone