NEUROLOGICAL INVOLVEMENT IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (EGPA) – IS THERE A DIFFERENCE IN BIOLOGICAL BIOMARKERS?
- Adriana Girão, Portugal
Abstract
Background and Aims
Although nervous system involvement may occur in Eosinophilic Granulomatosis with Polyangiitis (EGPA), its clinical manifestations and pathophysiology are still poorly understood. Our goals are: 1-characterize CNS/PNS involvement; 2-analyze if there is a difference in biological markers in patients with and without neurological manifestations.
Methods
Retrospective observational study, including EGPA patients with and without neurological manifestations. Demographics, clinical data and biological markers were collected. Descriptive and inferential statistics were applied.
Results
A total of 16 cases were analyzed, 9 with (group-1) and 7 without (group-2) neurological involvement. Patients from group-1 were older at EGPA diagnosis. Neurological involvement preceded EGPA diagnosis in 5 patients, and occurred during follow-up in 4 patients after a median of 4,5 years. Main CNS manifestations were stroke (n=2), bilateral central retinal artery occlusion (n=1), labyrinthine haemorrhage (n=1) and compressive dorsal myelopathy due to extradural granulation tissue (n=1). Main PNS manifestation were axonal polyneuropathy (n=3), sensorineural hearing loss (n=3) and multiplex mononeuropathy (n=1). Two patients had both PNS and CNS affected. There were no statistical differences concerning biological markers (eosinophil count, MPO titers) between the 2 groups. All patients were treated with immunosuppressive drugs, with 2 patients unresponsive to treatment belonging to group-1.
Conclusions
EGPA related nervous system manifestations can be very pleomorphic, highlighting 4 distinct neurological scenarios in our sample - peripheral neuropathy, VIII cranial nerve neuropathy, ischemic and hemorrhagic lesions and compressive myelopathy. In our cohort, patients with neurological manifestations did not have different eosinophilic count and MPO titer comparing with patients without neurological involvement.