Case description

A 37-year old Asian woman presented with a six-month history of dry eyes, dry mouth, bilateral dilated pupils and episodic blurred vision. On further questioning, the left pupil became dilated 3 years previously, the right 6 months previously. On examination, slit lamp examination revealed irregular shaped pupils and subtle segmental contraction. There was no direct light reflex in the left eye and a sluggish response in the right (Fig. 1A). The near vision reaction was slow. Pharmacological test with dilute solution(0.05%) of pilocarpine resulted in strong constriction of both pupils (Fig. 1B), confirming the presence of Adie’s tonic pupil. Deep tendon reflexes were absent. Ophthalmoscopy and peripheral nerve conduction were normal. Serologic testing revealed positive anti-Ro/SS-A antibodies and elevated rheumatoid factor. A salivary-gland biopsy revealed lymphocytic sialadenitis (Fig. 1C). A diagnosis of primary Sjögren syndrome was thus made. Prednisone and hydroxychloroquine were prescribed. Her sicca symptoms were relieved but Adie’s tonic pupil persisted.

Fig. 1
figure 1

Adie’s pupil in Sjögren syndrome. A: No response to light in the left pupil and impaired response in the right. B: Strong constriction to light in both pupils after instillation of a dilute (0·05%) solution of pilocarpine. C: Lymphocytic sialadenitis in salivary-gland biopsy

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Discussion

In addition to glands, Sjögren syndrome can also affect ganglion cells and cause peripheral neuropathy [1]. Adie’s pupil is is a rare neurological disorder characterized by enlargement of the pupil with poor reaction to light and light-near dissociation. Tendon areflexia is often associated with this condition, in which it is termed Adie’s syndrome [2]. The disorder is usually benign and idiopathic; however, it can also be a sign of systemic diseases [3]. Adie’s pupil has been occasionally reported in patients with Sjögren syndrome [1, 4, 5], and ciliary ganglionitis might be the underlying mechanism. In this case, Adie’s pupil preceded the development of sicca symptoms, and therefore was the initial sign of Sjögren syndrome, which was consistent with a previous report [5]. Hence Sjögren syndrome should be looked for in all patents with Adie’s pupil. Timely recognition of this rare manifestation is critical for an early diagnosis and proper management.