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

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. ●Peripheral nervous system involvement could be the first clinical manifestation of AAV.●Patients with pathology consistent with AAV may be ANCA negative on testing.●When laboratory tests result negative, a biopsy of involved organs should be performed.