Combined Cyclophosphamide and Corticosteroid-Induced Remission in Severe Glomerulopathy Associated with Systemic Vasculitis

Abstract

Three patients with systemic vasculitis and severe renal disease as major manifestations are reported. In 2 cases, rapidly progressive glomerulonephritis presented as oliguric renal failure. In the third case, the clinical picture was severe nephrotic syndrome with decreased renal function. Combined cyclophosphamide and corticosteroid treatment resulted in dramatic improvement of renal function and remission of nephrotic syndrome. In 2 cases, histological improvement was documented by repeated kidney biopsy. The optimal duration of cyclophosphamide therapy has to be determined.

Therapy for Children with Henoch-Schonlein Purpura Nephritis: A Systematic Review

“…Cyclophosphamide should be also used in association with steroids. This combination resulted in an improvement of renal function and remission of NS during systemic vasculitis [32]. In a retrospective clinical trial, the treatment of HSP GN with oral cyclophosphamide (2 mg/kg/day, 12 weeks) and pulse methylprednisolone (or oral prednisone) significantly reduced the severe proteinuria [33].…”

Answer

“…Nephrotic syndrome has been occasionally described in PAN [11,12,13]. One would expect an elevated serum creatinine in a patient with extensive fibrinoid necrosis and occasional glomerular crescents.…”

Effects of Cyclophosphamide Pulse Therapy on the Clinical and Histopathological Findings, Particularly Crescent Formation, in a Patient with Adult-onset Steroid-refractory Henoch-Schönlein Purpura Nephritis

“…In contrast, if the patient also shows signs of organ dysfunction, such as renal damage, steroid therapy is recommended (4)(5)(6). Patients refractory to steroids may be successfully treated with immunosuppressive agents, as evidenced by alleviation of their clinical symptoms (7)(8)(9)(10)(11)(12)(13)(14); however, to our knowledge, there are few reports of the findings of histopathological evaluations of the effectiveness of immunosuppressive therapy for HSPN.…”