Elsevier

Joint Bone Spine

Volume 80, Issue 3, May 2013, Pages 324-327
Joint Bone Spine

Case report
Temporal arteritis in the young

https://doi.org/10.1016/j.jbspin.2012.09.012Get rights and content

Abstract

Temporal arteritis in the form of giant cell arteritis (GCA) is common in the elderly but is extremely rare in patients less than 50 years of age. We describe two male patients: one who presented at the age of 31 years with painful, nodular swellings of both temporal arteries and whose temporal artery biopsy demonstrated a non-giant cell panarteritis with mixed inflammatory cell infiltrate typical of juvenile temporal arteritis (JTA); another one, aged 40 years, who presented with headache and cerebral angiography consistent with an intracranial vasculitis and whose temporal artery biopsy confirmed an authentic multinucleated GCA. The first patient spontaneously improved after biopsy and the second patient has responded well to corticosteroid therapy. These two cases exemplify well two distinct but extremely rare forms of temporal arteritis in young patients. A 3rd subset is that associated with a systemic vasculitis. Few cases of JTA have been reported and, to our knowledge, we describe in this report one of the only cases of GCA with central nervous system involvement 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:

  • as a typical GCA, clinically and on histology;

  • as juvenile temporal arteritis (JTA), which is a specific and distinct entity and;

  • 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.

References (15)

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