Muscle weakness as a presenting symptom in ANCA-associated vasculitis

Muscle weakness is rarely a presenting symptom of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), although the disease frequently involves the lungs, skin, neurons, and kidneys. Here we describe a case of AAV presenting with muscle weakness in which only muscle biopsy could confirm the diagnosis. The literature review, including three similar cases, suggested that patients with ANCA-associated muscle vasculitis likely had myalgia, normal levels of creatine kinase, pulmonary fibrosis, rheumatoid factor, and muscle edema on MRI.