Clinical Image: Antineutrophil cytoplasmic antibody-associated vasculitis causing compressive cervicothoracic myelopathy.

Abstract

The patient, a 64-year-old man, presented with a 6-week history of upper back pain and progressive lower-limb weakness and paraesthesia. Of particular note, he had a past history of chronic sinusitis and otitis media. Examination revealed a spastic paraparesis with sustained clonus, hyperreflexia, and medical research council grade 3/5 weakness in both lower limbs. Magnetic resonance imaging of his cervicothoracic spine revealed a circumferential mass–like lesion extending from C5 to T9 with compression of the spinal cord at T2-T3 (see image). Antineutrophil cytoplasmic antibody (ANCA) testing revealed a perinuclear pattern with a strongly positive myeloperoxidase antibody. Histologic examination demonstrated a dense lymphocytic infiltrate with fibrinoid necrosis of the blood vessel wall consistent with vasculitis. A diagnosis of ANCA-associated vasculitis causing compressive myelopathy was made. Treatment with high-dose glucocorticoids and pulsed intravenous cyclophosphamide was initiated, with almost immediate improvement seen in the patient's neurological status. This highlights the importance of including ANCA-associated vasculitis within the differential causes of spinal lesions. Disclosure form Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.