Tolosa-Hunt Syndrome as a Presentation of Sjögren's-Associated Vasculitis.
- 1Mater Dei Hospital, Msida, Malta.
Abstract
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.Learning points
'Tolosa-Hunt syndrome' is a descriptive term for an idiopathic presentation; it does not establish aetiology. Secondary causes should be considered and thoroughly worked up.The workup should extend beyond local orbital pathology to systemic autoimmune and inflammatory disorders, including potentially serious vasculitis.Sjögren's-associated vasculitis may produce neurological manifestations resembling polyarteritis nodosa; rheumatology input and treatment of the underlying vasculitis may improve the neurological syndrome.
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BibTeX
@article{vella2026tolosahunt,
title = {{Tolosa-Hunt Syndrome as a Presentation of Sjögren's-Associated Vasculitis.}},
author = {Jack Vella and Nicola Dingli and Paul John Cassar and Maria Bonello},
journal = {European journal of case reports in internal medicine},
year = {2026},
month = oct,
doi = {10.12890/2026_007497},
url = {https://europepmc.org/article/MED/43011668},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Tolosa-Hunt Syndrome as a Presentation of Sjögren's-Associated Vasculitis.
AU - Jack Vella
AU - Nicola Dingli
AU - Paul John Cassar
AU - Maria Bonello
PY - 2026
DA - 2026/10/16
JO - European journal of case reports in internal medicine
DO - 10.12890/2026_007497
UR - https://europepmc.org/article/MED/43011668
AB - 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.Learning points 'Tolosa-Hunt syndrome' is a descriptive term for an idiopathic presentation; it does not establish aetiology. Secondary causes should be considered and thoroughly worked up.The workup should extend beyond local orbital pathology to systemic autoimmune and inflammatory disorders, including potentially serious vasculitis.Sjögren's-associated vasculitis may produce neurological manifestations resembling polyarteritis nodosa; rheumatology input and treatment of the underlying vasculitis may improve the neurological syndrome.
SN - 2284-2594
ER -