Publication:

Collet Sicard syndrome secondary to granulomatosis with polyangiitis

Granulomatosis with polyangiitis (GPA) is a systemic vasculitis that affects small to medium-sized blood vessels. Although uncommon, cranial nerve (CN) involvement can result in CN palsy. We present a clinical case of a female with collet Sicard syndrome (CSS), displaying left-sided CN IX, X, XI, and XII involvement, which we determined to be caused by GPA. Our patient met 7 points on the American College of Rheumatology 2018 (ACR) criteria for GPA with positive ANCA, bilateral mastoiditis, and decreased sensorineural hearing. Early identification of GPA is crucial due to systemic CN involvement, as it can lead to dysphonia, dysphagia, and other complications. Early treatment can improve the functional prognosis of patients, requiring intensive induction immunosuppression due to frequent bilateral progression and worse prognosis. Despite its infrequency, timely diagnosis is critical for better patient outcomes.