Tolosa-Hunt syndrome as a presentation of Sjögren’s-associated vasculitis

Tolosa-Hunt syndrome is a clinical quadrad consisting of unilateral headache, ipsilateral cranial nerve palsy, granulomatous inflammation on imaging or biopsy and no other headache syndrome to explain the diagnosis. It may be secondary to systemic inflammatory disease, and clinicians should aggressively search for underlying causative pathology. A 73-year-old patient presented with this quadrad, in whom workup identified a systemic large-vessel vasculitis secondary to Sjögren’s disease. The patient was treated with rituximab and steroids, with considerable improvements in both her orbital pathology and vasculitis. The quadrad of Tolosa-Hunt syndrome should prompt investigation for systemic autoimmune and inflammatory disease rather than being assumed to be idiopathic.