Chapter

Vasculitis (ANCA negative)

Rosanna Coppo and Alessandro Amore

in Rheumatology and the Kidney

Second edition

Published on behalf of Oxford University Press

Published in print April 2012 | ISBN: 9780199579655
Published online February 2013 | e-ISBN: 9780191763472 | DOI: http://dx.doi.org/10.1093/med/9780199579655.003.0078

Series: Oxford Clinical Nephrology Series

Vasculitis (ANCA negative)

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1. A perfect diagnosis: IgA-dominant immune deposits in small vessels, involving skin, gut and glomeruli with clinical features of palpable purpura, associated with arthralgia and abdominal pain. 2. Renal involvement at onset presents mostly with haematuria and acute nephritic syndrome, with renal function impairment and proteinuria. 3. In children renal involvement is less frequent and less severe than in adults, moreover it often recovers in few weeks. 4. When renal involvement is severe and persistent over years, the long term prognosis of IgAN related to HSP is similar in children and adults. 5. Aberrantly glycosylated IgA1 circulating molecules are detectable in both HSP and primary IgAN: additional factors should favour the development of vasculitic lesions. 6. No prevention of severe renal involvement can be obtained administering prednisone when extra-renal signs only are present. 7. Short-term treatment with prednisone can control extra-renal signs when severe, particularly in presence of serious gastrointestinal involvement. 8. Risk factors for progression include severe proteinuria, extensive crescent formations and advanced chronic histological signs (tubular-interstitial damage). 9. Long-term therapy should be modulated according to clinical and histological features, ranging from prednisone alone in milder cases to alkylating agents (cyclophosphamide) and plasma exchanges in rapidly progressive ones. 10. After renal transplantation recurrence of IgA nephropathy occurs, whereas purpura bouts are extremely infrequent.

Chapter.  5754 words. 

Subjects: Nephrology

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