Case reportTemporal arteritis in the young
Introduction
Age greater than 50 years old is one of the criteria for a patient with vasculitis to be classified as having giant cell arteritis (GCA) according to the 1990 American College of Rheumatology (ACR) classification criteria and Chapel Hill nomenclature. However, vasculitis of the temporal arteries can be observed, although rarely, in younger patients with three main forms:
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as a typical GCA, clinically and on histology;
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as juvenile temporal arteritis (JTA), which is a specific and distinct entity and;
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as temporal artery involvement secondary to systemic vasculitis.
Whilst the clinical presentation of each can be vastly different leaving no diagnostic uncertainty, there are cases in which the precise diagnosis can be elusive.
Here we describe two cases of temporal arteritis in young patients with two of these distinct pathologies, in order to emphasize the importance, in all young patients presenting with possible temporal artery vasculitis, of identifying the underlying diagnosis as it will confer information on prognosis and therapeutic options.
Section snippets
Case 1
A 31-year-old male with a background of mild asthma presented in 2009 with mild right-sided temporal headache, fatigue and subtle swelling in the region of his right temporal artery. With the exception of a prominent right temporal artery, physical examination was unremarkable and a full blood profile, including ESR and differential WBC, was normal. Magnetic resonance imaging (MRI) demonstrated dilatation of the right temporal artery (left temporal artery normal) (Fig. 1). Magnetic resonance
Discussion
Whilst temporal arteritis in the form of GCA is not uncommon in the elderly [1], vasculitis of the temporal arteries in the young (< 50 years) is extremely rare with less than 40 cases reported in the literature. Temporal arteritis in the young can be sub classified into three groups according to the underlying pathology and clinical presentation (JTA, GCA and temporal arteritis as a manifestation of a systemic vasculitis), which are important to recognize because each one has a different
Conclusion
Temporal arteritis in the young is rare and can generally be classified into one of three subsets. As with our 2nd case there can often be significant clinical and pathological overlap between different disease entities, making management and prognosis challenging. JTA confers the best prognosis, all reported cases to date have resolved with excision of the affected temporal artery without the need for systemic therapy. It remains unclear whether full excision biopsy of the affected artery is
Disclosure of interest
The authors declare that they have no conflicts of interest concerning this article.
Acknowledgment
Authors thank Dr. Thierry Généreau (Nouvelles Cliniques Nantaises, Nantes, France) for his insights on these cases.
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