Sir,

Primary biliary cirrhosis and hypothyroidism are autoimmune diseases with a female preponderance. Giant cell arteritis (GCA) is a vasculitis, possibly of autoimmune aetiology. It is a rare cause of third nerve palsy. We report a case of biopsy-proven GCA causing painful third nerve palsy in a patient with biopsy-proven primary biliary cirrhosis and hypothyroidism. We discuss GCA as part of a spectrum of autoimmune disease.

Case report

A 68-year-old lady, with hypothyroidism and primary biliary cirrhosis (PBC) (Figure 2a), presented to eye emergency clinic with painful third nerve palsy (Figure 1) without pupillary involvement. The ESR was elevated at 68 mm. The diagnosis was presumed to be due to temporal arteritis, and high-dose oral steroid was started. A temporal artery biopsy (Figure 2b) performed next day was positive for GCA. Headaches improved steadily on treatment. ESR (Erythrocyte sedimentation rate) dropped significantly to normal levels 1 week after starting high-dose steroid. Third nerve palsy fully recovered by the fifth month.

Figure 2
figure 2

(a) High-power photomicrograph of a granuloma within a portal tract. (b) Medium-power photomicrograph of temporal arterial wall showing intimal proliferation, inflammation, and giant cells.

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Figure 1
figure 1

Total ptosis of right eye due to third nerve palsy.

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Comment

GCA is the most common form of systemic vasculitis in adults,1 affecting medium and large-sized arteries. Immunological processes have been implicated in the development of GCA. Deposits of immune complexes and complement have been found in some temporal artery biopsies.2 Anti-IgG activity has also been identified in artery biopsy specimens.3 Recently, a model for the pathogenesis of GCA proposed by Weymann and Goronzy4 suggests a cell-mediated aetiology. Approximately 30% of patients with GCA have neurologic manifestations.5

PBC is an autoimmune disease, leading to progressive destruction of small intrahepatic bile ducts. A survey among a cohort of patients with PBC showed that it is associated with an increased risk of other autoimmune disorders.6 A survey of thyroid function in patients with PBC revealed the presence of thyroid antibodies in 26% of patients.7 A nationwide survey in Japan found autoimmune thyroiditis to be associated with primary biliary cirrhosis in 5.8% of cases.8 Gordon and Isenberg suggest that there is an overlap between polmyalgia rheumatica (PMR) and GCA with autoimmune thyroid dysfunction.9 Dent and Edwards,10 in their series of 250 patients with autoimmune thyroiditis, noted PMR or GCA in 2.8% of patients. Gagnerie et al11 report PBC, GCA, and PMR in a single patient. The common histopathological feature of PBC and GCA is granulomatous inflammation. Thus, even though occurrence of GCA along with PBC and hypothyroidism is rare, awareness of this association may aid the management of this potentially blinding condition in elderly patients.