AB0746 Juvenile temporal arteritis: Report of a case and review of the literature. (23rd January 2014)

• Record Type: Journal Article
• Authors: Campochiaro, C.; Guglielmi, B.; Cavalli, G.; Berti, A.; Sabbadini, M.G.; Dagna, L.
• Is Part Of: Annals of the rheumatic diseases. Volume 71(2012)Supplement 3
• Page Start: 681
• Page End: 681
• Publication Date: 2014-01-23
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2012-eular.746
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: Juvenile temporal arteritis (JTA) is an extremely rare non-granulomatous, non giant-cell inflammatory disease of the temporal arteries of unknown etiology with typical histological features. JTA can affect older children or young adults with a history of eosinophilia. JTA is usually limited to a single lesion in the temporal artery and it is not associated with systemic symptoms Objectives: We discuss the case of a patient with juvenile temporal arteritis involving both temporal arteries and provide a review of the medical literature on this topic Methods: A 41-year-old man presented with a painless, nodular lesion in the region of the left superficial temporal artery. He reported the occurrence of a similar, contra-lateral manifestation 1 month before. Physical examination disclosed two subcutaneous pulsating nodules located in the temporal regions. Laboratory tests (including inflammatory markers) were negative, except for significant eosinophilia (19%). High-resolution MR angiography showed thickened temporal arteries, strongly enhanced by the contrast media (both in and around the arterial wall). Total-body MR angiography was negative. A biopsy of the right temporal artery showed an encapsulated, well defined soft nodule of the subcutis containing the left superficial temporal artery. Histology showed a non-granulomatous panarteritis with eosinophilic infiltrate and several lymphomonocytic cells, extending to the perivascular connective tissue. The intimal wall was thickened and internal elastic lamina was severely disrupted. No necrosis, giant cells or luminal thrombosis could be observed. We searched the English-language medical literature indexed in PubMed using the keywords "juvenile arteritis" or "eosinophilic arteritis". Results: Since now only 40 cases of JTA have been described. JTA affects males more than females and is typically limited to a single lesion in the temporal arteries. The lack of headache, ocular involvement and symptoms consistent with polymyalgia rheumatica is a specific characteristic of JTA, in contrast to giant cell arteritis. Usually no systemic symptoms are present. Laboratory tests are usually unremarkable. Among the 15 patients described in English literature with univocal JTA, only 4 had eosinophilia and one had both eosinophilia and elevated levels of serum IgE. Histology is always necessary to confirm the diagnosis and to rule out other diagnostic possibilities. Conclusions: Vasculitides of the temporal arteries are a rare disease of the young, often but not always associated with peripheral eosinophila. JTA is often asymptomatic with a benign clinical course (only one patient had a recurrence). JTA is classically unilateral, and only three cases of bilateral JTA have been described in literature. The excision of the affected vessel for diagnostic purposes may be curative. Since the clinical course and treatment of JTA is different from other large vessel vasculitides, physicians must be aware of this condition in the differential diagnosis of large vessel vasculitis in the young. References: Nesher G et al. Vasculitis of the temporal arteries in the young. Semin Arthritis Rheum 2009. Kolman OK et al. Juvenile temporal arteritis. J Am Acad Dermatol 2010. Disclosure of Interest: None Declared