Juvenile Temporal Arteritis

Juvenile temporal arteritis

Authors: Dr Maria Melachrinou and Prof Andrew P. Andonopoulos Creation date: January 2005

Scientific Editor: Prof Loic Guillevin

University of Patras School of Medicine, Department of Medicine, 265 00 Rion, Patras, Greece. [email protected]

Abstract Keywords Disease name / synonyms Definition Histopathology Diagnostic criteria of JTA Differential diagnosis Etiology Clinical description Diagnostic methods Epidemiology Unresolved questions References

Abstract Juvenile temporal arteritis (JTA) is an extremely uncommon vasculitis of unknown etiology (eleven documented cases have been reported in the literature) affecting older children and young adults. In contrast to the classic form of temporal arteritis, it is not a systemic disease nor does it cause local symptoms at the temporal area. JTA has a benign clinical course and it is curable by surgical excision. The term JTA was coined by Lie and his colleagues, in 1975, when they reported four cases of an otherwise asymptomatic disease presenting with a painless nodule at the temporal region in older children and young adults. No one case had evidence of systemic disease or history of trauma to the temporal region, and the excisional biopsy of the lesions had revealed a non-giant cell granulomatous inflammation of the temporal arteries with eosinophilic infiltration, intimal proliferation and microaneurysmal disruption of the media. JTA is treated by surgical excision and does not recur.

Keywords Juvenile temporal arteritis – Temporal arteritis – Kimura’s disease – Angiolymphoid hyperplasia with eosinophilia - Thromboangiitis obliterans with eosinophilia Disease name / synonyms Histopathology Disease: Juvenile temporal arteritis Histologically, involved temporal artery shows Synonym: Non-giant cell granulomatous remarkable thickening of the wall with temporal arteritis with eosinophilia. constriction or obliteration of the lumen. There is panarteritis, with or without fibrinoid necrosis, Definition characterized by significant intimal proliferation JTA is a nongranulomatous nonnecrotizing and dense, intravascular and perivascular, eosinophilic vasculitis, presenting as painless infiltration by numerous eosinophils, temporal nodule in older children and young lymphocytes and plasma cells. Giant cells are adults; it is completely unrelated to the classic not seen. Lymphoid follicles and newly formed giant cell temporal arteritis (Andonopoulos AP capillaries surround the vessel. Elastica van et al., 2004; Bollinger A et al., 1986; Fujimoto M Gieson staining may reveal focal disruption of et al., 1996; Lie JT et al., 1975; Lie JT, 1995; the internal elastic lamina. Tomlinson FH et al., 1994; Wakugawa M et al., 1999; Watanabe C et al., 2002). Diagnostic criteria of JTA 1- Occurrence in children and young adults;

Melachrinou M and Andonopoulos AP. Juvenile temporal arteritis, Orphanet encyclopedia. January 2005: http://www.orpha.net/data/patho/GB/uk-juvenile-temporal-arteritis.pdf 1

2- Absence of associated features such as Etiology myalgia, visual disturbance, fever, anemia; The etiology of JTA remains unknown. 3- Manifested as painless temporal nodule; 4- Normal erythrocyte sedimentation rate; Clinical description 5- Eosinophilic panarteritis and thrombosis with Clinically, JTA manifests as asymptomatic or without microaneurysmal disruption of the temporal nodule and affects older children and artery; young adults (Andonopoulos AP et al., 2004; 6- Intimal proliferation, disruption of the media, Bollinger A et al., 1986; Fujimoto M et al., 1996; and extensive infiltrate consisting of Lie JT et al., 1975; Lie JT, 1995; Wakugawa M lymphocytes, eosinophils and plasma cells; et al., 1999; Watanabe C et al., 2002). Bilateral 7-Absence of granulomatous infiltration and JTA has been reported in one case (Lie JT, giant cells. (Tomlinson FH et al., 1994). 1995). The systemic symptoms that occur in the classic temporal arteritis such as headache, Differential diagnosis myalgia, visual disturbance and fever are JTA should be differentiated from conditions that absent. The hematologic tests and the may occur in the temporal regions and/or be erythrocyte sedimentation rate are usually associated with non-giant cell eosinophilic normal (Andonopoulos AP et al., 2004; Bollinger temporal areritis. A et al., 1986; Fujimoto M et al., 1996; Lie JT et al., 1975; Lie JT, 1995; Tomlinson FH et al., Kimura’s disease 1994; Wakugawa M et al., 1999; Watanabe C et It is an inflammatory disorder of unknown al., 2002). Peripheral blood eosinophilia may be etiology seen in an endemic form in Orient. It present (Bollinger A et al., 1986; Fujimoto M et usually presents as a subcutaneous mass of the al., 1996; Watanabe C et al., 2002). head and neck region, which may persist or recur, and is associated with peripheral Diagnostic methods eosinophilia and elevated IgE. Lesional biopsy. Histopathologically, early lesions show a prominent vascular proliferation and dense Epidemiology lymphocytic infiltration with numerous JTA is rare. Fewer than 20 cases have been eosinophils, while late lesions reveal a nodular reported in the literature (Andonopoulos AP et lymphoid hyperplasia and fibrosis (Kung IT et al., al., 2004; Bollinger A et al., 1986; Fujimoto M et 1984). Though, it is not known whether Kimura’s al., 1996; Lie JT et al., 1975; Lie JT, 1995; disease involves a specific artery, the vascular Tomlinson FH et al., 1994; Wakugawa M et al., changes of this condition may resemble those of 1999; Watanabe C et al., 2002). JTA. The report of Watanabe et al. describes an elderly patient in whom JTA preceded a typical Management (treatment) manifestation of Kimura’s disease. In this JTA is treated by surgical excision and does not report, the authors suggest that JTA may be recur. (Lie JT et al., 1975; Lie JT, 1995). an accessory lesion of Kimura’s disease (Watanabe C et al., 2002). Unresolved questions Is JTA an expression of Kimura’s disease? Angiolymphoid hyperplasia with eosinophilia (ALHE) References It is a dermal and subcutaneous lesion occurring Andonopoulos AP, Melachrinou M, more frequently in the head and neck. Yiannopoulos G, Meimaris N. Juvenile temporal Histolopathologically, the lesion is characterized arteritis: A case report and review of the by prominent vascular proliferation with literature. Clin Exp Rheumatol 2004;22:379-80. histiocyte-like endothelial cells, and a nodular Bollinger A, Leu HJ, Brunner U. Juvenile and diffuse lymphocytic infiltrate with eosinophils arteritis of extracranial arteries with (Olsen TG et al., 1985). hypereosinophilia. Klin Wochenschr 1986;64:526-9 Thromboangiitis obliterans with eosinophilia Fujimoto M, Sato S, Hayashi N, Wakugawa M, (Buerger’s disease) of the temporal arteries Tsushida T, Tamaki K. Juvenile temporal arteritis It is an unusual form of temporal arteritis in with eosinophilia: a distinct clinicopathological young cigarette smokers, unassociated with entity?. Dermatology 1996;192:32-5. vascular involvement of either upper or lower Kung IT, Gibson JB, Bannatyne PM. Kimura’s extremities. This variant of Buerger’s disease disease: A clinicopathologic study of 21 cases mimics clinically the classic temporal arteritis and its distinction from angiolymphoid and histopathologically Kimura’s disease and hyperplasia with eosinophilia. Pathology JTA (Lie JT et al.1988). 1984;16:39-44.

Melachrinou M and Andonopoulos AP. Juvenile temporal arteritis, Orphanet encyclopedia. January 2005: http://www.orpha.net/data/patho/GB/uk-juvenile-temporal-arteritis.pdf 2

Lie JT, Gordon LP, Titus JL. Juvenile temporal Tomlinson FH, Lie JT, Nienhuis BJ, Konzen arteritis. Biopsy study of four cases. JAMA KM, Groover RV. Juvenile temporal arteritis 1975;234:496-9. revisited. Mayo Clin Proc 1994;69:445-7. Lie JT, Michet CJ Jr. Thromboangiitis obliterans Wakugawa M, Kawabata Y, Asahina A, et al. A with eosinophilia (Buerger’s disease) of the case of juvenile temporal arteritis with temporal arteries. Hum Pathol 1988;19:598-602. eosinophilia. Rinsho Derma (Tokyo) Lie JT. Bilateral juvenile temporal arteritis. J 1999;41:2027-30. Rheumatol 1995;22:774-6. Watanabe C, Koga M, Honda Y, Oh-I T. Olsen TG, Helwig EB. Angiolymphoid Juvenile temporal arteritis is a manifestation of hyperplasia with eosinophilia: A clinicopathologic Kimura disease. Am J Dermatopathol study of 116 patients. J Am Acad Dermatol 2002;24:43-9. 1985;12:781-96.

Melachrinou M and Andonopoulos AP. Juvenile temporal arteritis, Orphanet encyclopedia. January 2005: http://www.orpha.net/data/patho/GB/uk-juvenile-temporal-arteritis.pdf 3