Bilateral Tonic Pupils as the Initial Manifestation of Sjögren's Syndrome

Abstract

Adie's pupil is characterised by pupil dilatation, segmental iris palsy, light-near dissociation, and slow re-dilatation. Most commonly, tonic pupils are unilateral and idiopathic, but can be caused by orbital disorders and autonomic neuropathies. There are only a few case reports of tonic pupils in patients with Sjögren's syndrome, caused by an autoimmune ciliary ganglionitis. The authors report on two cases with bilateral tonic pupils as the initial manifestation of primary Sjögren's syndrome. Both patients presented with blurred vision, bilateral tonic pupils, and sicca symptoms. The findings suggest that Sjögren's syndrome should be considered in patients presenting with bilateral Adie's tonic pupils.

Keywords: Adie’s tonic pupil; Sjögren’s syndrome.

FIGURE 1

Images of pupils, Case 1. Bilateral dilated pupils (A, B) that are non-reactive to direct light (C, D), but constrict on accommodation with slow re-dilatation suggesting tonicity (E, F), and their response to 0.1% pilocarpine demonstrating denervation supersensitivity (G, H), consistent with bilateral Adie’s pupils.

FIGURE 2

Images of pupils, Case 2. Bilateral dilated pupils (A, B) that are non-reactive to direct light (C, D), but constrict on accommodation with slow re-dilatation suggesting tonicity (E, F), and their response to 0.1% pilocarpine demonstrating denervation supersensitivity (G, H), consistent with bilateral Adie’s pupils.