[Bronchiectasis worsening by p-ANCA (bactericidal/permeability-increasing protein) positive vasculitis. A case report and review of the literature].

Abstract

A 67-year-old woman was hospitalized with recurrent fever, arthralgia and erythema of the arms and legs. She had suffered from bronchiectasis for the previous 4 years, and Pseudomonas was persistently detected in her sputum. During the course of her illness, she developed distal sensitive and motor polyneuropathy. Serum test was positive for myeloperoxidase and bactericidal/permeability-increasing protein antineutrophil cytoplasmic antibodies. Nerve biopsy showed vasculitis infiltration of the vasa nervorum. She started immunoglobulin therapy, and after methylprednisolone and pulse cyclophosphamide therapy once monthly for 6 months she showed a good response and a reduction in symptoms.