Head and Neck

A plethora of head and neck symptoms and signs are associated with GCA. These are summarized in Table 3.

TABLE 1 The Initial Clinical Manifestations in 100 Consecutive Patients with GCA

Clinical feature

Percentage

Headache

32

Polymyalgia rheumatica

25

Fever

15

Visual symptoms without visual loss

7

Fatigue/malaise

5

Myalgias or tender artery

3

Weight loss/anorexia

2

Jaw claudication

2

Visual loss

1

Tongue claudication

1

Sore throat

1

Vasculitis on angiogram

1

Hand and wrist stiffness

1

Abbreviation: GCA, giant cell arteritis. Source: From Ref. 7.

Abbreviation: GCA, giant cell arteritis. Source: From Ref. 7.

TABLE 2 Prevalence of Selected Clinical Features in 100 Patients with GCA

Clinical feature

Percentage

Gender (female/male)

69/31

Constitutional symptoms

Weight loss or anorexia

50

Fever

42

Malaise, fatigue, or weakness

40

Polymyalgia rheumatica

39

Other musculoskeletal pains

30

Synovitis

15

Symptoms related to arteries

83

Headache

68

Visual disturbance

30

Permanent visual loss

14

Jaw claudication

45

Dysphagia or swallowing claudication

8

Tongue claudication

6

Limb claudication

4

Signs related to arteries

66

Artery tenderness

27

Decreased temporal artery pulse

46

Swollen, nodular, or erythematous scalp arteries

23

Large artery bruit

21

Diminished large artery pulse

7

Central nervous system abnormalities

3

Sore throat

15

a, duration of symptoms before diagnosis was 7 months (range 1-48 months). Abbreviation: GCA, giant cell arteritis. Source: From Ref. 7.

a, duration of symptoms before diagnosis was 7 months (range 1-48 months). Abbreviation: GCA, giant cell arteritis. Source: From Ref. 7.

TABLE 3 Head and Neck Symptoms and Findings of GCA

Organ

Symptoms and findings

Vessels

Pulselessness, thickening, pain on palpation, painful arterial nodules

Skin

Erythema, facial swelling, facial pain, and ischemic changes with

necrosis

Jaw and masticatory

Intermittent claudication of the jaw muscles, trismus, temporal

muscles

mandibular joint pain, and tooth and gum pain

Mouth, oral cavity,

Swelling and pain of the oral and nasal mucosa, dysphagia, tongue

pharynx,

pain, ischemic tongue discoloration, tongue necrosis, necrosis of

and larynx

the oral mucosa, ulcerating glossitis and pharyngitis, hoarseness,

anosmia, and ageusia

Ear

Ear pain, nystagmus, dizziness, hearing loss, and tinnitus

Salivary glands

Swelling

Lymph nodes

Swelling

and tonsils

Abbreviation: GCA, giant cell arteritis.

Abbreviation: GCA, giant cell arteritis.

Vascular and Skin. Thickening, pulselessness, pain with palpation, and the appearance of painful nodules of the affected arteries occur typically in the temporal artery but also may occur in other branches of the external carotid artery, particularly the facial, maxillary, occipital, and posterior auricular arteries (Fig. 1). Typical skin changes include erythema and local or generalized facial swelling and pain. Soft-tissue swelling may occur, particularly in the periorbital and cheek areas. Ischemic changes and necrosis of the skin may occur and may even lead to local bone destruction, but this is rare, since the temporal artery generally has good collateral formation (Fig. 2). Biopsies of the affected skin generally reveal nonspecific inflammatory changes or endarteritic obliterative changes.

Mouth and Neck. Jaw claudication is almost pathognomonic for GCA and can be severe enough to cause trismus (7). Symptoms of GCA in the oral cavity and the oropharynx are characterized especially by swelling and pain, as well as dysphagia, with burning tongue pain, pale discoloration, and swelling, which, in extreme cases, may necessitate a tracheotomy.

Tongue necrosis likely occurs when both the right and the left lingual arteries are obliterated and the collaterals become stenotic. Toothache and/or gum pain may be constant and lead to initial evaluation by a dentist. Dysarthria, dysphagia, and myosis are relatively rarely reported (7-9).

FIGURE 1 Markedly dilated temporal arteries in a 74-yr-old man with GCA. The arteries are visibly thickened and inflamed; palpation of the vessel is painful. Abbreviation: GCA, giant cell arteritis. Source: Courtesy of Lester Mertz, MD, Mayo Clinic Rochester, MN, U.S.A.
FIGURE 2 Scalp necrosis resulting from infarction of the temporal artery. There is also tongue necrosis and ptosis on the left. The patient had developed sudden hearing loss of the left ear as well. Source: Courtesy of Gene G. Hunder, MD, Mayo Clinic, Rochester, MN, U.S.A.

A disturbance in taste and smell with ageusia and anosmia are rarely described, likely due to vasculitis-related ischemia of the hippocampal or the uncinate gyrus. Rarely, salivary glands may become swollen and on biopsy reveal perivascular lymphocytic infiltrates. Some patients develop sicca symptoms, although the etiology of these changes is uncertain (9).

Audiovestibular Manifestations. Ear pain may be due to involvement of the tympanic artery and may be associated with inner ear disease. Patients may develop dizziness with nystagmus and hearing loss, which may be profound. These symptoms are due to involvement of the labyrinth or the central regions of the cochleovestibular nucleus. Tinnitus may appear.

Up to two-thirds of patients with GCA may complain of subjective hearing loss at diagnosis, while about 16% have hearing loss after three months of treatment (10,11). This compares with approximately 10% of patients who may develop subjective hearing loss with PMR (based on a small number of patients studied for this outcome, N = 10), while patients with PMR do not develop tinnitus, vertigo, dizziness, or disequilibrium (10). On audiometry, parameters of hearing may improve in approximately 30% of patients but further deteriorate at three-month follow-up in approximately 7% of patients (10). Subjective hearing loss occurs in about 60% of patients, irrespective of vestibular function. The hearing loss may be refractory to treatment. A study of 44 patients (66%) showed no significant change in hearing after three months of glucocorticosteroid treatment, while hearing was improved in 27%. This improvement was unilateral in two-thirds of cases, and hearing declined in 7% of patients (10).

A retrospective study of 271 patients with GCA revealed only four patients with concomitant sensorineural hearing loss (12). In clinical practice, significant hearing loss from GCA is likely the exception. Vestibular dysfunction may be asymptomatic but on formal vestibular testing may be present in close to 90% of patients with GCA (10). The hearing loss and vestibular symptoms may be steroid-responsive when treated early. When detected prior to onset of therapy and tested after initiation of therapy, these changes are found to be frequently reversible. After three months of glucocorticosteroid therapy, vestibular dysfunction was noted in about 30% of 44 patients studied for this outcome (10). Patients with vestibular dysfunction were more likely to have associated persistent head-shaking nystagmus, noted in 73% of patients with biopsy-proven GCA (10).

Neck. Thyroid disease has been associated with PMR/GCA in over 50 patients. In one review of 43 patients, 19 (44%) were found to have hypothyroidism, which was secondary to thyroiditis in five. Seventeen patients (40%) had thyrotoxicosis (9). Thyroid disease preceded or appeared simultaneously with GCA/PMR. However, at least two other studies evaluating these findings from a literature review of cases were not duplicated in studies specifically evaluating thyroid disease in GCA (9). Swelling of the cervical lymph glands may sometimes occur in the acute phase of GCA. Histology reveals nonspecific, inflammatory, hyperplastic changes.

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