Background:Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis, affecting mainly the superior airways, lungs and kidneys. Neurological impairment is frequent, described in 22 to 54% of cases, and rarely inaugurates the disease.Objectives:The objective of our work was to evaluate its prevalence and its different aspects.Methods:A retrospective study including 11 files of GPA patients followed over a period of 10 years in The Internal Medecine Department at Tahar Sfar Hospital Mahdia TUNISIA.Results:Neurological manifestations were reported in 5 patients: a pyramidal syndrome was noted in 3 patients with a normal cerebral MRI, a disabled headache was described in one patient with a normal cerebral MRI. Peripheral neuropathy in 2 cases, and the Electroneuromyogram objectified a mononeuropathy affecting the ulnar nerve, peroneal nerve, and musculocutaneous nerve in one patient and a distal sensitivomotor poly neuropathy in one patient. Cranial nerves damage was noted in two cases, one VII (1 case) and one V (1 case).Conclusion:Our study results, show the importance of timely diagnosis of these neurological manifestations, as their evolution will depend on the early GPA treatment.References:[1]doi: 10.1016/j.autrev.2017.07.007Disclosure of Interests:None declared