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Nephrol Dial Transplant (2001) 16: 1932-1935
© 2001 European Renal Association-European Dialysis and Transplant Association

Case Reports

Henoch-Schönlein purpura with IgG PR3-ANCA in a PiZZ alpha 1-antitrypsin deficient patient

Pascal Meier1,, Eric Dayer2, Robert Lemoine3 and Edouard Blanc1

1 Division of Nephrology, Department of Medicine, Hôpital de Sion, 2 Division of Infectious Diseases and Immunology, Institut Central des Hôpitaux Valaisans, Sion, Switzerland and 3 University Institute of Pathology, Lausanne, Switzerland

Keywords: alpha 1-antitrypsin; anti-neutrophil cytoplasmic antibodies; Henoch-Schönlein purpura; IgA nephropathy; proteinase 3; systemic vasculitis



   Introduction
 
Henoch-Schönlein purpura (HSP) is a clinical syndrome that includes arthralgias, purpuric lesions, abdominal pain and nephropathies. It is histopathologically characterized by widespread leukocytoclastic vasculitis with skin IgA deposits and mesangioproliferative IgA glomerulonephritis. Renal lesions closely resemble those of Berger's disease, the renal limited form of HSP [1].

Anti-neutrophil cytoplasmic antibodies (ANCA) are essentially found in two types of vasculitides. Those which give a cytoplasmic pattern (c-ANCA) and react with proteinase 3 (PR3-ANCA) are sensitive and specific markers of Wegener's granulomatosis (WG) [2]. On the other hand, p-ANCA mostly directed against myeloperoxidase (MPO) are generally encountered in microscopic polyangiitis. The incidence of ANCA is variable in other types of vasculitis including HSP, and their pathogenic significance in HSP and IgA nephropathy (IgAN) remains unclear. IgA-ANCA have been found in patients with HSP and more occasionally in those with IgAN [3]. The finding of IgG PR3-ANCA . . . [Full Text of this Article]



   Case
 


   Discussion
 


   Acknowledgments
 


   Notes
 


   References
 

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