Abstract
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.
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Notes
Crescents that occupy Bowman’s space almost completely
Segmental thrombosis/necrosis, crescents, and sclerosis
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Acknowledgements
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).
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Tarshish, P., Bernstein, J. & Edelmann, C.M. Henoch-Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol 19, 51–56 (2004). https://doi.org/10.1007/s00467-003-1315-x
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DOI: https://doi.org/10.1007/s00467-003-1315-x