Giant Cell Arteritis (Temporal Arteritis) Clinical Presentation

Updated: Aug 29, 2018
  • Author: Mythili Seetharaman, MD; Chief Editor: Herbert S Diamond, MD  more...
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Presentation

History

The onset of giant cell arteritis (GCA) may be either abrupt or insidious. [50] GCA may begin with constitutional manifestations such as anorexia, fever, malaise, myalgia, night sweat, and weight loss. These prodromal symptoms may occur for a few days and may even stretch out to weeks.

The most commonly reported symptoms in patients with GCA are as follows:

  • Headache (initial symptom in 33%, present in 72%)
  • Neck, torso, shoulder, and pelvic girdle pain that is consistent with polymyalgia rheumatica (PMR; initial in 25%, present in 58%)
  • Fatigue and malaise (initial in 20%, present in 56%)
  • Jaw claudication (initial in 4%, present in 40%)
  • Fever (initial in 11%, present in 35%)

The headache of GCA has no pathognomonic features, but typically—and most importantly—the headache is either new, in a patient without a history of headaches, or of a new type, in a patient with a history of chronic headache. The headache is usually localized to the temporal or occipital area. Less often, the pain may be predominantly occipital or occipitonuchal; occasionally it is diffuse.

The headache is usually throbbing and continuous. Other descriptions of the pain include dull, boring, and burning. Focal tenderness on direct palpation is typically present. The patient may note scalp tenderness with hair combing, or with wearing a hat or eyeglasses.

Patients with mild GCA may complain only of generalized muscle aches and pains and unusual fatigue. [51, 52] These may be mistaken for symptoms of PMR.

Jaw claudication is noted as fatigue or discomfort of the jaw muscles during chewing of firm foods such as meat, chewing gum, or prolonged speaking. Jaw claudication is highlly predictive of temporal arteritis, and it is a result of ischemia of the maxillary artery supplying the masseter muscles. Tongue infarction is almost pathognomonic for GCA. [156]

Nonspecific symptoms of cough and sore throat occur in 17% and 11% of patients, respectively, but are rarely the presenting complaints. Amaurosis fugax occurs in 10% overall (initially in 2%), and some degree of permanent visual loss occurs in 8% (initial symptom in 3%).

Less common symptoms, which are almost never the presenting complaint, include the following:

  • Limb claudication (8%)
  • Transient ischemic attacks (TIAs) or stroke (4-7%)
  • Scintillating scotoma (5%)
  • Carpal tunnel syndrome (5%) or other peripheral neuropathies
  • Tongue or throat claudication (4%)
  • Diplopia or ptosis (2%)
  • Tongue numbness (2%)
  • Myelopathic symptoms (< 1%)
  • Affective or psychotic symptoms [53, 54] /li>
  • Facial pain (rare)
  • Scalp necrosis (rare) [55, 56, 57, 58]
  • Tongue necrosis (rare) [59, 60]

Ophthalmologic symptoms

Around 50% of patients with GCA experience visual symptoms over the course of the disease. Initial visual symptoms may be transient and intermittent, typically consisting of unilateral visual blurring or vision loss, often painless, or occasionally diplopia. Alternatively, a partial field defect may progress to complete blindness over days. Patients may experience visual hallucinations, which may precede permanent loss of vision. [61]

Transient repeated episodes of blurred vision are usually reversible, but sudden irreversible loss of vision may occur, especially if treatment is not started promptly. If GCA remains untreated in patients with unilateral vision loss, the second eye may become affected within 1-2 weeks.

Other symptoms

Vertebrobasilar events sometimes present as acute confusional states or coma as opposed to discrete focal syndromes. Presumably, cognitive changes reflect thalamic, mesial temporal, and mesencephalic involvement in most cases. Acute encephalopathy is a rare complication of GCA and is a poor prognostic sign. Many patients with this complication progress to coma and die.

In 2003, Amor-Dorado et al [62] reported a previously unrecognized high incidence of audiovestibular disturbances such as vestibular dysfunction and/or hearing impairment in their GCA patients.

Most patients have poorly localized tenderness over the joints, especially the shoulders and hips. Synovitis was once excluded as a feature of giant cell arteritis, but moderate bland effusions can develop in the knees and occasionally other joints (eg, shoulders, wrists).

Involvement of the posterior auricular artery may manifest as pain in the ear canal, pinna, or parotid gland.

Symptoms related to large artery involvement

Certain clinical characteristics distinguish large-vessel GCA from cranial GCA. Approximately 88% of large-vessel involvement occurs in women. Patients typically have a younger age at onset, fewer constitutional symptoms, and a longer interval until diagnosis.

Next:

Physical Examination

Physical signs parallel symptoms and depend on the organ systems that are damaged by vasculitic ischemia. The clinician should conduct a head and neck, ophthalmologic, and neurologic examination and assess vital signs and blood pressure in both arms to rule out aortic arch involvement.

Approximately half of patients have signs of superficial temporal artery inflammation, including erythema, pain on palpation, nodularity, thickening, or reduced pulsation on the affected side (see the image below). The examiner may be able to roll an affected temporal artery between the fingers and the skull.

Prominent temporal artery is visible on the temple Prominent temporal artery is visible on the temple of a 76-year-old woman with temporal arteritis. Courtesy of ScienceSource (https://www.sciencesource.com/).

Gentle pressure on the scalp may elicit focal or generalized tenderness. This differs from the hypersensitivity or hyperesthesia (unusual discomfort from a very mild stimulus, such as gently stroking the patient's hair) that is commonly found with migraine. [63]

Tenderness to pressure on the carotid artery (carotodynia) occurs in about 15% of patients. Cranial nerve palsies, particularly of the sixth nerve, should also be noted. The carotid, axillary, and brachial arteries should be assessed for bruits. Carotid bruits occur in 10-20% of patients with giant cell arteritis (GCA) and are frequently bilateral.

Ophthalmologic findings

A complete eye examination should be performed, including the following:

  • Visual acuity
  • Pupillary exam
  • Visual fields
  • Motility
  • Slit lamp exam
  • Fundoscopy

Special attention should be paid to the optic nerve and central retinal artery. The retina should be carefully examined for nerve fiber layer infarcts.   

The most common cause of vision loss in GCA is anterior ischemic optic neuropathy (AION), which is caused by vasculitis of the posterior ciliary artery. [64, 65, 66, 67, 68, 69, 70, 71, 28, 72] Vision loss may precede the funduscopic changes of optic nerve infarction by roughly 36 hours. [157]

Ophthalmoscopy in acute AION may show sludging of blood in retinal arterioles, which can be orthostatically sensitive. The optic disc may show chalky white pallor and edema, with or without splinter hemorrhages along the disc margin. See the image below.

Anterior ischemic optic neuropathy. Image courtesy Anterior ischemic optic neuropathy. Image courtesy of Richard Kho, MD, Q.C. Eye Center, Quezon City, Philippines.

As AION evolves, the absolute amount of disc elevation tends to be modest (< 3 diopters in most cases), with infrequent areas of disc hemorrhage. Edema resolves within 10 days or so; within 2-4 weeks, it is replaced by optic atrophy.

Other important vascular ophthalmic presentations of GCA include the following [73, 74, 75, 76] :

  • Posterior ischemic (retrobulbar) optic neuropathy
  • Central retinal artery occlusion
  • Branch retinal artery occlusion
  • Choroidal ischemia [77, 78, 79]

With retrobulbar involvement (posterior ischemic optic neuropathy), optic pallor and atrophy gradually develop without antecedent papillitis. Central retinal artery occlusion causes pallor and diffuse retinal edema with a cherry-red spot because there are no ganglion cells at the foveal center (see the image below). Occlusion of the central retinal artery or its branches occurs in fewer than 10% of GCA cases with eye involvement. [80]   Nerve fiber layer infarcts may be a sign of retinal ischemia.

Central retinal artery occlusion (CRAO). Central retinal artery occlusion (CRAO).

Neuro-ophthalmic manifestations [81] of GCA include the following:

  • Diplopia
  • Ptosis [82]
  • Nystagmus
  • Internuclear ophthalmoplegia (INO) [83]
  • Pupillary abnormalities [84]

The oculomotor apparatus can be involved at any level, including the extraocular muscles (EOM), nerves, and brain stem. However, the most common site is the EOM. A characteristic feature of EOM involvement is daily fluctuation of the ocular motility disorder. Horner syndrome is uncommon, judging from a review of several large series; however, ptosis and miosis may occur together, separately, or in conjunction with other oculomotor disturbances.

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