Granulomatosis With Polyangiitis Masquerading as Bilateral Trigeminal Neuralgia

Vivek Joseph, Rahul Anil, Neil Gonter


A 32-year-old woman presented with symptoms of bilateral trigeminal neuralgia (TN) that has been refractory to empiric therapy. Imaging showed normal caliber trigeminal nerves with no evidence of nerve compression. Autoimmune etiology was considered due to unnatural presentation of symptoms. Workup revealed elevated perinuclear antineutrophil cytoplasmic antibody (P-ANCA) and elevated myeloperoxidase (MPO) antibodies suggestive of localized ANCA-related vasculitis. A literature review revealed one case, which illustrates granulomatosis with polyangiitis (GPA) causing the classical symptoms of TN in absence of its more common symptoms. Patient was started on methotrexate 15 mg weekly and low-dose prednisone daily with significant improvement in symptoms. Ear-nose-throat (ENT) manifestations of rheumatologic disorders represent a diagnostic challenge. ENT symptoms can often represent the initial sign of an otherwise asymptomatic and undiagnosed autoimmune disease process, which often calls for prompt and aggressive immunosuppressive treatment which leads to not only better control of TN symptoms but also we speculate that early immunosuppressive therapy will control the progression of the underlying autoimmune disease.

J Med Cases. 2016;7(9):384-385


Rheumatology; Mixed connective tissue disorders; Vasculitis; Trigeminal neuralgia; Immunosuppressive therapy

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