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Clinical outcomes in children with Henoch–Schönlein purpura nephritis without crescents

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Abstract

Background

Henoch–Schönlein purpura is the most common vasculitis in children. Its long-term prognosis depends on renal involvement. The management of Henoch–Schönlein purpura nephritis (HSPN) remains controversial. This study reports the prognosis of children with HSPN presenting with class 2 International Study of Kidney Disease in Children (ISKDC) nephritis.

Methods

All children with HSPN class 2 diagnosed between 1995 and 2015 in four pediatric nephrology centers were included, and clinical and biological data were collected from the medical files. The primary endpoint was proteinuria remission defined as a proteinuria <200 mg/L.

Results

Ninety-two children were included in the study with a median follow-up of 36 (6–120) months; 28% had nephrotic syndrome, 31% proteinuria >3 g/L, 52% proteinuria between 1 and 3 g/L, and 18% proteinuria <1 g/L. Forty-seven percent of patients received orally treatment with steroids alone, 37% received methylprednisolone pulses followed by steroids orally, 18% received no steroids. Although 85% reached remission during follow-up, 12% did not maintain complete remission over time so that only 75% remained in complete remission by the end of the follow-up. Univariate analysis found a higher likelihood of remission in patients with higher proteinuria at disease onset (p = 0.009). This trend was not found in the multivariate analysis after adjusting for treatments, as patients with higher proteinuria were most often treated with steroids.

Conclusion

Our study shows that one fourth of patients with HSPN class 2 remain proteinuric and thus carry the risk of developing chronic kidney disease over the long term. This finding, together with the better outcome of patients treated with steroids, is in favor of using high-dose steroids orally or IV in these patients.

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Authors and Affiliations

  1. Pediatric Nephrology, Armand Trousseau Hospital, APHP, 26 Avenue du Docteur Netter, Paris, 75012, France

    Jean Daniel Delbet, Bilal Aoun, Iulia Stoica & Tim Ulinski

  2. University Pierre and Marie Curie, Paris 6, Paris, France

    Jean Daniel Delbet, Iulia Stoica, Isabelle Brocheriou & Tim Ulinski

  3. Pediatric Nephrology, Robert Debré Hospital, APHP, Paris, France

    Julien Hogan & Georges Deschênes

  4. University Paris Diderot, Paris 7, Paris, France

    Julien Hogan & Georges Deschênes

  5. Pediatric Nephrology, Necker Enfants Malades, APHP, Paris, France

    Rémi Salomon

  6. Pediatric Nephrology and Rheumatology, CHU Toulouse, Paris, France

    Stéphane Decramer

  7. Pathology Department, Tenon Hospital, APHP, Paris, France

    Isabelle Brocheriou

  8. DHU i2b, Inflammation-Immunopathology-Biotherapy, Paris, France

    Tim Ulinski

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  1. Jean Daniel Delbet
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Correspondence to Tim Ulinski.

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Delbet, J.D., Hogan, J., Aoun, B. et al. Clinical outcomes in children with Henoch–Schönlein purpura nephritis without crescents. Pediatr Nephrol 32, 1193–1199 (2017). https://doi.org/10.1007/s00467-017-3604-9

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  • DOI: https://doi.org/10.1007/s00467-017-3604-9

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