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BioDrugs

, Volume 15, Issue 2, pp 99–138 | Cite as

Schönlein-Henoch Purpura in Children and Adults

Diagnosis, Pathophysiology and Management
  • Guy Rostoker
Review Article

Abstract

Schönlein-Henoch syndrome is a form of systemic small-vessel vasculitis,characterised by vascular and/or mesangial immunoglobulin A1 deposits. The main clinical manifestations are vascular purpura, predominating on the lower limbs, and articular, gastrointestinal and renal symptoms. Pulmonary, cardiac, genital and neurological symptoms have also be observed. The syndrome usually affects children, whereas it is rare in adults.

The frequency of renal involvement varies between individual studies (from 20 to 100%). Renal manifestations are usually mild and transient, although chronic nephropathies may occur. Overall, an estimated 2% of children with Schönlein-Henoch purpura progress to renal failure and up to 20% of children with nephritis treated in specialised centres require haemodialysis. The renal prognosis appears to be worse in adults. Aetiological investigations are required, as a triggering factor is found in approximately half the patients (e.g. viral, bacterial and parasitic infections, drugs, toxins, systemic diseases and cancer).

Dapsone has beneficial effects on cutaneous, gastrointestinal and articular manifestations in adults, especially those with chronic forms. Corticosteroids may be useful for refractory abdominal pain. Methylprednisolone pulse therapy, immunosuppressive drugs (e.g. cyclophosphamide and azathioprine), plasma exchange and polyclonal immunoglobulin therapy are beneficial in very rare life-threatening forms of the disease and in rare instances where renal function is compromised.

Keywords

Vasculitis Nephritis Kawasaki Disease Familial Mediterranean Fever Renal Involvement 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Acknowledgements

This work was supported by grants from Association Quincy Recherche Clinique et Thérapeutique, Centre Hospitalier Privé Claude Galien and Compagnie Génèrale de Santé. The author is grateful to Mrs Mireille Griuncelli for her technical assistance.

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Copyright information

© Adis International Limited 2001

Authors and Affiliations

  • Guy Rostoker
    • 1
  1. 1.Service de Néphrologie et de DialyseCentre Hospitalier Privé Claude GalienQuincy Sous SenartFrance

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