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Giant cell arteritis (GCA) often presents atypically. An under-recognised presentation is with speech or respiratory involvement. We report a case of GCA with dysphonia and visual loss, a previously unreported combination.
A 67 year old woman presented with sudden, non-progressive, painless blurring of the vision in her right eye for 4 days. Her left eye, which had been poor since childhood, was unchanged. She had no other ophthalmic symptoms or history. She smoked and was on treatment for hypertension. On questioning, she reported pain in her throat for 3 weeks, worse on swallowing, which had not responded to oral antibiotics. Her voice had been markedly hoarse for the same period. She denied other respiratory symptoms. She did not have anorexia, weight loss, myalgia, muscle stiffness, scalp tenderness, or jaw claudication, but had been experiencing a dull ache all over her head for 6 weeks.
On examination, she had deep tissue tenderness on the anterolateral aspect of her neck, bilaterally. She had no scalp tenderness, and her temporal arteries were pulsatile. Her corrected visual acuity was 6/24 in both eyes. Her right optic disc was swollen, consistent with an anterior, ischaemic, optic neuropathy. The left optic disc was normal. The remaining ophthalmic and systemic examinations were unremarkable. Blood tests revealed an erythrocyte sedimentation rate of 130 mm in the first hour, a C reactive protein of 125 mg/l, and a mild, normochromic, normocytic anaemia. A diagnosis of right anterior, ischaemic optic neuropathy secondary to giant cell arteritis (GCA) was made, and she was admitted for pulse, intravenous methylprednisolone and high dose oral prednisolone. Temporal artery biopsy confirmed the diagnosis of GCA (fig 1).
The following day, her headache and throat pain were much better, her right optic disc was less swollen, and her inflammatory markers began to fall. She was referred to an otorhinolaryngologist regarding her prolonged hoarse voice. Examination and flexible laryngoscopy showed no abnormalities. Chest radiograph was normal. On day 2, her voice began to improve, and by day 3 it was back to normal. She was discharged on oral prednisolone.
Giant cell (temporal) arteritis is the most common of the vasculitides, and presents with varied and often non-specific symptoms. Diagnosis may be further hindered by the possibility of non-elevated inflammatory markers1 and negative temporal artery biopsy.2 Ophthalmic artery involvement may cause irreversible, bilateral blindness, and may occur even in the absence of systemic symptoms and signs, a scenario termed occult GCA.3
Speech and respiratory features in giant cell arteritis have been described only infrequently.4,5 They include cough, sore throat, pain on swallowing, anterior neck tenderness, and dysphonia (hoarse voice). Nevertheless, it has been estimated that 4% of patients have respiratory symptoms as the initial presentation of GCA, and that as many as 9% will display them at some time during the course of the disease.4
Voice changes have been reported in eight patients with GCA, seven with hoarseness,4–7 and one with a broken, falsetto voice.8 None of these were reported to have had visual involvement. It had been suggested that the vasculitis in cases of GCA with speech or respiratory features might show a preference for branches of the external carotid artery (which supplies the larynx) over the internal carotid (which supplies the eye).5 Our case, however, demonstrates the possibility of dual involvement.
In considering the diagnosis of GCA, non-classic features, such as speech and respiratory symptoms, can be easily overlooked. In the absence of classic symptoms they should be specifically asked about. The label of occult GCA, therefore, should not be applied without first excluding the whole spectrum of recognised GCA features. In addition, GCA should be considered in cases of prolonged dysphonia with concomitant visual symptoms.