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We describe a 65-year-old female patient who presented with idiopathic retroperitoneal fibrosis and polyarteritis nodosa. In addition, antineutrophil cytoplasmatic antibodies were found with specificity for myeloperoxidase (p-ANCA; anti-MPO). Following immunosuppressive treatment, regression of signs and symptoms was observed, together with the disappearance of these specific antimyeloperoxidase antibodies...