ANCA-Associated Vasculitis with Predominant Peripheral and Central Nervous System Involvement: A Case Report.

BACKGROUND

ANCA-associated vasculitis (AAV) is an immune-mediated multi-system disease. It can present with neurological involvement as its predominant manifestation. We report a case of AAV with predominant peripheral and central nervous system involvement.

CASE PRESENTATION: A 62-year-old male presented with fever and asymmetric weakness and pain in the lower limbs. Electrophysiological studies revealed asymmetric axonal damage in both lower limbs. During hospitalization, he developed acute bulbar palsy. Brain MRI confirmed bilateral basal ganglia infarction. Ancillary tests indicated involvement of the lungs, kidneys, and hematological systems, along with positive MPO-ANCA (p-ANCA), confirming the diagnosis of AAV. His symptoms gradually improved following treatment with glucocorticoids and immunosuppressants. At the 6-month follow-up, his symptoms were largely resolved.

CONCLUSION

The presence of asymmetric axonal neuropathy or atypical non-atherosclerotic cerebral infarction, particularly when accompanied by multisystem involvement, should raise suspicion for AAV. Early diagnosis and prompt treatment significantly improve patient outcomes.