Adie’s tonic pupil in primary Sjögren syndrome

Excerpt

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.

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