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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Hilhorst, Marc Van Paassen, Pieter Tervaert, Jan Willem Cohen |
| Organization | Limburg Renal Registry |
| Description | Country affiliation: Netherlands Author Affiliation: Hilhorst M ( Clinical and Experimental Immunology, Maastricht University, Maastricht, The Netherlands.); van Paassen P ( Clinical and Experimental Immunology, Maastricht University, Maastricht, The Netherlands.); Tervaert JW ( Clinical and Experimental Immunology, Maastricht University, Maastricht, The Netherlands jw.cohentervaert@maastrichtuniversity.) |
| Abstract | In patients with GN or vasculitis, ANCAs are directed against proteinase 3 (PR3) or myeloperoxidase (MPO). The differences between PR3-ANCA-associated vasculitis (AAV) and MPO-AAV described in the past have been supplemented during the last decade. In this review, we discuss the differences between these two small-vessel vasculitides, focusing especially on possible etiologic and pathophysiologic differences. PR3-AAV is more common in northern parts of the world, whereas MPO-AAV is more common in southern regions of Europe, Asia, and the Pacific, with the exception of New Zealand and Australia. A genetic contribution has been extensively studied, and there is a high prevalence of the HLA-DPB1*04:01 allele in patients with PR3-AAV as opposed to patients with MPO-AAV and/or healthy controls. Histologically, MPO-AAV and PR3-AAV are similar but show qualitative differences when analyzed carefully. Clinically, both serotypes are difficult to distinguish, but quantitative differences are present. More organs are affected in PR3-AAV, whereas renal limited vasculitis occurs more often in patients with MPO-AAV. For future clinical trials, we advocate classifying patients by ANCA serotype as opposed to the traditional disease type classification. |
| File Format | HTM / HTML |
| ISSN | 10466673 |
| e-ISSN | 15333450 |
| Journal | Journal of the American Society of Nephrology |
| Issue Number | 10 |
| Volume Number | 26 |
| Language | English |
| Publisher | American Society of Nephrology |
| Publisher Date | 2015-10-01 |
| Publisher Place | United States |
| Access Restriction | Open |
| Subject Keyword | Discipline Nephrology Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis Enzymology Myeloblastin Metabolism Peroxidase Animals Diagnosis Drug Therapy Disease Models, Animal Comparative Study |
| Content Type | Text |
| Resource Type | Article |
| Subject | Nephrology |
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