Henoch-Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide
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Nephritis in Henoch-Schönlein purpura (HSP) is the primary cause of morbidity and mortality. Although many therapeutic regimens have been reported to be effective, no therapy has been shown in a controlled trial to be beneficial. Fifty-six patients with histopathologically severe HSP nephritis were randomized to receive supportive therapy with or without cyclophosphamide, 90 mg/m2/day for 42 days. Patients were classified according to status at final follow-up: Fully Recovered 48.2%, Persistent Abnormalities 39.3%, or ESRD/Death 12.5%. There were no differences in onset data or outcome between the two trial groups or in outcome between trial and 23 non-trial patients followed concurrently. Therefore, data from trial and non-trial patients were combined for further analysis. There was no correlation between outcome and age, blood pressure, serum total protein, or serum albumin. Although rates of proteinuria did not correlate with outcome, all those with progression to ESRD had nephrotic levels of proteinuria at onset. Only five of 28 patients with nephrotic levels of proteinuria and severe onset histopathology recovered fully. No patient with crescents in 50% or more of glomeruli went on to full recovery. Recurrence of non-renal symptoms did not correlate with outcome. Nephrotic syndrome, decreased GFR, and more severe histopathology at onset, as well as persistence of urinary abnormalities for several years, are ominous signs.
KeywordsPurpura Henoch-Schönlein Cyclophosphamide Nephritis Natural history Pediatrics Clinical trial
Work performed: Albert Einstein College of Medicine. Financial support by National Institutes of Health Research Grant 1-RO1-AM18234, the National Kidney Foundation of New York, the Kidney Disease Institute of the State of New York, the William Beaumont Hospital Pathology Projects Fund, the John Rath Foundation, the National Kidney Research Foundation (United Kingdom), and the Kidney Foundation of the Netherlands. Participants in the ISKDC who contributed to this study were as follows: Central Office (New York): H.L. Barnett and C.M. Edelmann Jr. (Directors), I. Greifer (Associate Director), D.I. Goldsmith and A. Spitzer (Directors of Coordinating Center), P. Tarshish (Data Coordinator), G. Laddomada (Project Administrator), and J. Massaro (Secretarial Assistant); Regional Coordinators: I.B. Houston, R.H. Kuijten, and L.B. Travis; Directors of Participating Centers: R.H.R. White (Birmingham, Alabama), I.B. Houston (Manchester, United Kingdom), J.G. Mongeau (Montreal, Canada), N. Hallman and J. Vilska (Helsinki), S.R. Meadow (Leeds, United Kingdom), M.J. Schoeneman and R. Weiss (New York-Albert Einstein), S. Roy (Memphis, Tennessee), G. Gordillo (Mexico City, Mexico), J. Lewy (New York-New York Hospital), O. Oetliker (Bern, Switzerland), Bowman Gray Hospital (North Carolina, USA), M. Nash (New York-Columbia Presbyterian), M. McVicar (North Shore University Hospital-New York), A. Fanconi (Zurich, Switzerland); Consultants: J. Bernstein, J. Churg, R. Habib, and R.H.R. White (Pathology) and S.M. Wassertheil-Smoller (Biostatistics).
- 4.Meadow SR, Glasgow EF, White RHR, Moncrieff MW, Cameron JS, Ogg CS (1972) Schönlein-Henoch nephritis. Q J Med 163:241–258Google Scholar
- 6.Allen DM, Diamond LK, Howell DA (1960) Anaphylactoid purpura in children. Am J Dis Child 99:833–854Google Scholar
- 7.Ostergaard JR, Storm K (1991) Neurologic manifestations of Schönlein-Henoch purpura. Act Pediatr Scand 80:339–342Google Scholar
- 10.Sterky G, Thilen A (1960) Study on the onset and prognosis of acute vascular purpura (the Schönlein Henoch syndrome) in children. Act Pediatr 49:217–229Google Scholar
- 11.Vernier RL, Worthen HG, Peterson RD, Colle E, Good RA (1961) Anaphylactoid purpura. Pathology of the skin and kidney and frequency of streptococcal infection. Pediatrics 27:181–193Google Scholar
- 15.Tejani A (2001) Personal communication. NAPRTCS dataGoogle Scholar
- 16.Loirat C, Ehrich JHH, Geerlings W, Jones EHP, Landais P, Mallick NP, Margreiter R, Raine AEG, Salmela K, Selwood NH, Tufveson G, Valderrabano F (1994) Report on management of renal failure in children in Europe, XXIII, 1992. Nephrol Dial Transplant Suppl 1:26–40Google Scholar
- 17.Tanaka H, Suzuki K, Nakahata T, Ito E, Waga S (2003) Early treatment with oral immunosuppressants in severe proteinuric purpura nephritis. Pediatr Nephrol 18:347–350Google Scholar
- 27.Yoshikawa N, White RHR, Cameron AH (1981) Prognostic significance of the glomerular changes in Henoch-Schoenlein nephritis. Clin Nephrol 16:223–229Google Scholar
- 28.Bircan Z, Katar S, Batum S, Yilmaz AY, Comert S, Vitrinel A (2000) Prognosis of Henoch-Schönlein nephritis in children (abstract). Pediatr Nephrol 14:C70Google Scholar