Background 

This case provides a warning on how easily common presentations may be dismissed, and serious diagnoses delayed if a broad list of differentials including additional systemic involvement is not considered.

This case illustrates how unexplained persistent headaches should raise the index of suspicion of atypical causes such as vasculitis. A judgement on the likelihood of vasculitis can be made at the time of clinical review with focused questions. This patient visited three different health professionals before a correct diagnosis was reached. Piecing together different symptoms and a patient timeline could have led to an earlier diagnosis. Asking ‘red flag’ questions is paramount in ensuring serious conditions are not missed. This is particularly important in systemic diseases, as patients are not necessarily aware of which symptoms are relevant. Thus, they may not volunteer the relevant clinical information unless prompted. This last point enabled the ophthalmologist to consider giant cell arteritis (GCA) as a diagnosis.

We demonstrate that although investigation results may be characteristic, they cannot always be relied on to exclude systemic diseases. In this case, inflammatory markers were not significantly raised, an unusual but well-documented occurrence. This is supported by a study that identified 167 patients fulfilling the American College of Rheumatology criteria for GCA over a 48-year period. Of these patients, nine had a reported erythrocyte sedimentation rate (ESR) of less than 40 mm/hour. Six of the nine patients had an ESR of <30 mm/hour. All nine of these patients had a positive temporal artery biopsy.1 History taking and clinical judgement should therefore always take precedence in forming a diagnosis, and diagnoses not to be ruled out on the basis of normal tests; an important message in today’s investigation-driven medical practice.

In the case of GCA, important screening questions to consider should include: scalp tenderness, temporal headaches, jaw claudication, visual disturbances, polymyalgia and constitutional symptoms.

Case presentation

An 80-year-old man with a medical history of glaucoma, hypertension and first degree heart block presented with a 3-month history of frontal headaches. A routine CT head was requested by his general practitioner (GP). This showed no intracranial masses or bony lesions but incidentally revealed a mature lacunar infarct of the left ventral thalamus. There was no radiographic cause determined for his headaches.

Alongside his headaches, the patient developed bilateral jaw pain with claudication. He independently sought advice from his dentist and returned with a diagnosis of bruxism.

Three days after the CT scan (3 months after initial presentation to healthcare), the patient visited ophthalmology for a routine glaucoma follow-up appointment. The ophthalmologist elicited episodes of diplopia, amaurosis fugax and significant weight loss in the preceding months. There was no history of scalp tenderness, fever or proximal muscle pain and stiffness. Examination revealed bilateral tender, nodular and weakly pulsatile temporal arteries. There was no relative afferent pupillary defect or anterior ischaemic optic neuropathy.

In light of the ongoing headaches, jaw claudication, diplopia, amaurosis fugax and weight loss, a diagnosis of GCA was suspected. The patient was referred the same day to the rheumatology department for prompt review.

Investigations

Inflammatory markers were not significantly raised: C-reactive protein (CRP) 11 mg/L (<10) and ESR 15 mm/hour (1–19). Recognising that normal inflammatory markers do not rule out GCA, rheumatologists arranged urgent investigation. Temporal and axillary artery ultrasound (US) demonstrated a characteristic positive halo sign in the right temporal artery (figure 1). A right temporal artery biopsy (TAB) revealed a muscular artery with focal adventitial chronic inflammation and intimal hyperplasia with mild inflammation (figure 2), consistent with GCA.

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Figure 1

Craniofacial soft tissue ultrasound. Halo sign of right temporal artery indicative of giant cell arteritis. Arrow indicates vessel wall.

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Figure 2

Right temporal artery biopsy. Mild lymphocytic infiltrate in media and around vasa vasora and intimal thickening of right temporal artery.

Differential diagnosis

Key differentials 2:

‘Primary’ headaches

• Migraine.

• Tension type.

• Cluster.

‘Dangerous’ headaches

• Intracranial haemorrhage.

• Masses (benign or malignant).

• Metastases.

Referred pain

• Temporomandibular joint dysfunction.

• Dental abscess.

• Cervical spondylosis.

• Otalgia.

• Trigeminal neuralgia.

Vision related

• Transient ischaemic attack.

• Glaucoma.

Treatment

Steroid treatment should be commenced immediately once a diagnosis of GCA is suspected, while awaiting further investigations. Sixty milligrams of prednisolone was initiated due to the presence of jaw claudication and visual symptoms in accordance with the British Society of Rheumatology guidelines.3 He was co-prescribed lansoprazole and alendronate for gastroprotection/bone protection and counselled regarding long-term steroid management and safety.

Outcome and follow-up

At 4-week follow-up, his symptoms improved significantly and inflammatory markers remained low (CRP <3, ESR 2). Oral prednisolone was tapered down gradually with frequent review of intraocular pressures to exclude glucocorticoid-induced exacerbation of glaucoma. Subsequent follow-ups confirmed inactive GCA disease, with no episodes of relapse.