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Abstract Details

A Neurologic Presentation of ANCA Positive Vasculitis
Autoimmune Neurology
P8 - Poster Session 8 (5:30 PM-6:30 PM)
14-009
Granulomatosis with polyangiitis (GPA) is a rare autoimmune inflammatory small vessel vasculitis involving the respiratory tract and kidneys. GPA can also involve the eyes, skin, gastrointestinal tract, heart, and the central or peripheral nervous system (PNS). Upper respiratory involvement is present in up to 90% of patients with GPA, while PNS involvement is present in only 15%. Diagnosis requires clinical features and the presence of specific antibodies. Proteinase-3 antineutrophil cytoplasmic antibody (PR3-ANCA) and cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) are positive in 80% of cases of GPA and only 35% of cases of other small vessel vasculitides.
To present a rare presentation of granulomatosis with polyangiitis as new onset peripheral neuropathy.
NA
A 52-year-old female presented with a three-week history of severe asymmetric burning pain and paresthesias of her hands and feet. She was initially prescribed two separate courses of prednisone in the outpatient setting. She had improvement; however, symptoms returned upon tapering and cessation. Initial outpatient workup showed mildly elevated ESR and CRP but was otherwise unremarkable. Due to the progressive development of bilateral dorsiflexion weakness she presented to the emergency department and was admitted to the hospital for comprehensive Neurologic evaluation. MRI of the neuraxis and CSF studies were unremarkable. Upon further discussion, she endorsed several months of intermittent blurred vision, oral and nasal sores, jaw fatigue, temporal scalp tenderness, and chronic sinusitis. She was treated with high dose steroids and achieved significant improvement in her symptoms. EMG was consistent with an axonal motor neuropathy. Further analysis revealed a positive c-ANCA and PR3 autoantibodies and elevated urine protein:creatinine ratio, leading to the most likely diagnosis of GPA.
Length-dependent polyneuropathy as the primary presenting symptom of GPA is rare. This case demonstrates the importance of keeping a broad differential for peripheral neuropathies.
Authors/Disclosures
Lauren K. Offerle, MD (Corewell Health - Grand Rapids/Michigan State University Neurology)
PRESENTER
Dr. Offerle has nothing to disclose.
Sarosh Irfan Madhani (Corewell Health) No disclosure on file
Taylor Struble No disclosure on file
Austin VanVelsen No disclosure on file
Jessica Feistel, DO Dr. Feistel has nothing to disclose.