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Juvenile temporal arteritis (JTA) is a rare benign vascular lesion limited to the branches of the external carotid artery, first recognised by Lie et al.1 Histological features of JTA include vasculitis (without giant cells and granulomas), lymphocytes infiltrate, and fibrous intimal proliferation.1 Despite frequent local arterial thrombosis associated with the lesion, no study on the coagulation pathway has been undertaken until now. We present an exceptional case of JTA occurring in a man in association with activated protein C resistance.
A 34 year old male smoker (12 cigarettes/day), without past medical history, consulted in January 2001 for right headache associated with swellings of both temples. Temporal regions were painful when he wanted to brush his hair. No visual or systemic symptoms occurred. Physical examination showed no pulse of the right temporal artery and a pulse of the left one; both arteries were thickened, tensed, indurated, and painful. Ultrasound echo Doppler disclosed arterial thrombosis of the right superficial temporal artery and normal aspect of the carotids and subclavicular arteries. Biological evaluation showed no inflammation (erythrocyte sedimentation rate 2 mm/1st h and C reactive protein <4 mg/l) and normal white blood cell count (8×109/l, with 1.4% eosinophils), haemoglobin level, and platelet count. Histopathological analysis of the right temporal artery disclosed an inflammatory infiltrate with mononuclear cells without giant cells or granuloma, and a fibrous intimal proliferation. No eosinophils were seen in the biopsy. An electrocardiogram was normal. The patient was treated with aspirin (100 mg/day) and the temporal pain resolved. He stopped smoking.
Past familial history showed multiple phlebitides and a pulmonary embolism in the grandmother, a phlebitis in the mother, and three phlebitides in the father. Blood coagulation evaluation in the patient showed activated protein C resistance with heterozygous mutation in factor V gene (Arg 506-Gln). There was no mutation in factor II gene, normal levels of antithrombin, protein S, protein C, and protein Z, no lupus anticoagulant, and no anticardiolipin antibody. Homocystine level was normal. A search for antinuclear, antineutrophil cytoplasmic antibodies, and cryoglobulinaemia was negative. Funduscopic examination was normal. Angiographic magnetic resonance imaging of the cerebral arteries only performed a few months later (when the patient was referred to our medical department) was normal. Control of ultrasound echo Doppler performed 2 years later (when the patient was still receiving aspirin treatment) showed a persistent thrombosis of the right temporal artery associated with a myointimal thickness and a non-occlusive myointimal thickness of the left artery. Carotid, vertebral, and subclavicular arteries appeared to be normal. The patient continued to receive aspirin treatment.
JTA is a very rare entity of benign course and fewer than 15 cases have been reported.1–6 It is usually unilateral, although bilateral involvement, as seen in our case, has also been reported.6 JTA occurs in children or adults under 40 years, and is characterised by (a) pain in the temporal region with arterial induration or palpable nodules; (b) no associated inflammatory symptoms; (c) no objective ophthalmic symptoms; (d) no biological inflammation; (e) possible blood eosinophilia with eosinophilic infiltrate in biopsy; and (f) no need for steroid treatment. The reported patients who were treated with steroids had systemic symptoms and an associated systemic vasculitis.3 More recently, rapid regression of the temporal pain in a patient with JTA treated with non-steroidal anti-inflammatory drugs has been reported.7
Differential diagnosis includes Kimura’s disease (angiolymphoid proliferative disorder of soft tissues with eosinophilia and raised immunoglobulin E, occurring almost exclusively in oriental men), angiolymphoid hyperplasia with eosinophilia,8 giant cell arteritis, panarteritis nodosa, Takayasu’s arteritis, and isolated vasculitis of the vasa vasorum.9 Histopathological analysis often discloses arterial thrombosis associated with non-giant cell, non-necrotising, and non-granulomatous vasculitis. A search for blood coagulation disorders has never been performed until now. Factor V Leiden is a well known risk factor for venous thrombosis but may also play a part in arterial thrombosis, particularly myocardial infarction10 or ischaemic cerebrovascular disease.11 Even if we cannot exclude a fortuitous association in our case, factor V Leiden mutation might have played a role in arterial thrombosis. This observation reminds doctors of this rare entity and highlights the need to study blood coagulation in cases of JTA.