ANCA-negative microscopic polyangiitis with neuromuscular involvement: When pathology could make the difference.

Abstract

Microscopic polyangiitis (MPA) is a necrotizing small vessel vasculitis with little or absent immune deposits (pauci-immune vasculitis), usually associated with the presence of antineutrophil cytoplasmic autoantibodies (ANCA) and a wide spectrum of organ manifestations. In our report we describe the case of a 74-year-old Asian man, who rapidly developed lower limb weakness and impaired renal and pulmonary functions. ANCA detection remained borderline throughout the disease course. Electrophysiological and instrumental studies revealed a picture of neuromuscular involvement; renal and muscle biopsies disclosed a small vessel vasculitis. He was started on a targeted immunosuppressive combination therapy and his clinical status progressively improved. In the framework of a multi-organ disease, microscopic polyangiitis should be considered as a differential diagnosis in case of acute/subacute onset of muscle weakness, even in the absence of ANCA detection.

• Authors: Francesca Vitali, Maria Ausilia Sciarrone, Giovanni Frisullo, Anna Laura Fedele, Eleonora Torchia, Giorgio Tasca, Marco Luigetti
• Journal: Clinical neurology and neurosurgery (Clin Neurol Neurosurg) Vol. 225 Pg. 107573 (02 2023) ISSN: 1872-6968 [Electronic] Netherlands
• PMID: 36608469 (Publication Type: Case Reports)
• Copyright: Copyright © 2023 Elsevier B.V. All rights reserved.

Chemical References

• Antibodies, Antineutrophil Cytoplasmic
• Immunosuppressive Agents

Topics

• Male
• Humans
• Aged
• Microscopic Polyangiitis (complications, diagnosis, drug therapy)
• Antibodies, Antineutrophil Cytoplasmic
• Immunosuppressive Agents (therapeutic use)
• Disease Progression
• Granulomatosis with Polyangiitis (drug therapy)