[Inflammatory myopathy: unusual presentation of a case of Wegener disease]

The case is presented of a 66-year-old man who developed an inflammatory myopathy picture predominantly consisting in leg weakness. Wegener's granulomatosis was diagnosed on the basis of suggestive thoracic imaging, microhematuria and necrotizing arteritis with granulomas in muscular biopsy. An increasing titer of anti-neutrophilic cytoplasm auto-antibodies (ANCA) confirmed the diagnosis. Rapid resolution was obtained by classic immunosuppressor treatment (cyclophosphamide and corticoids), without worsening of renal function. In discussion we emphasize the importance of ANCA measurement for diagnosis and follow-up.