Rare eye syndrome masking as acute glaucoma linked to Sjögren’s disease in case report

But as the work-up unfolded, the picture turned out to be far more unusual: examination revealed ciliary-body, choroidal, and retinal detachment, a pattern that pointed to non-nanophthalmic uveal effusion syndrome. The case, published as a case report in BMC Ophthalmology, is notable because the syndrome appeared in a patient who also had pre-existing primary open-angle glaucoma, and because it arrived in the dramatic disguise of a glaucoma emergency.

When the same effusive pattern appears in a normally sized eye, clinicians must hunt for other triggers such as trauma, surgery, medications, or intraocular inflammation.

Confirming the diagnosis here was itself a clinical challenge, because the patient’s congenital deaf-muteness meant he could not self-report the classic sicca symptoms that usually raise suspicion. Instead, the diagnosis rested on objective testing: laboratory results showed positive serum anti-SSA antibody, rheumatoid factor, and antinuclear antibody, together with abnormal Schirmer test results, which measure tear production. Together, these findings supported a diagnosis of primary Sjögren’s syndrome.

The report’s central message is a diagnostic warning. The authors argue that non-nanophthalmic uveal effusion syndrome should be included in the differential diagnosis for bilateral acute secondary angle-closure glaucoma with ciliochoroidal effusion — that is, when elevated intraocular pressure with a closed drainage angle occurs alongside effusions of the ciliary body and choroid. When uveal effusion syndrome arises without nanophthalmos, trauma, surgical insult, medication-related triggers, or obvious intraocular inflammation, they recommend that clinicians screen for underlying systemic autoimmune conditions such as primary Sjögren’s syndrome, even in patients who cannot communicate classic dryness symptoms.

The authors are careful about what this single case can and cannot show. As they state, this single-case observation cannot establish a definitive causal linkage between primary Sjögren’s syndrome and uveal effusion syndrome; it highlights a potential clinical association that other clinicians may want to keep in mind. What the case does demonstrate is a practical point about clinical reasoning: when a familiar emergency such as acute angle-closure glaucoma comes with atypical features — bilateral involvement, ciliochoroidal effusion, and no obvious mechanical or inflammatory trigger — the differential diagnosis should widen beyond the eye itself.

For patients who, like this one, cannot describe dry eyes or dry mouth, the authors suggest that objective autoimmune screening — antibody testing and tear-production measures — may be the only reliable route to the underlying diagnosis.

Subject of Research: A case report of non-nanophthalmic uveal effusion syndrome presenting as acute secondary angle-closure glaucoma in a patient with primary Sjögren's syndrome.

Article Title: Uveal effusion syndrome with Sjögren’s syndrome initially presenting as acute glaucoma with angle closure: case report

Article References: Du, Y., Wang, Y., Liu, Z., Gao, Y., & Du, X. (2026). Uveal effusion syndrome with Sjögren’s syndrome initially presenting as acute glaucoma with angle closure: case report. BMC Ophthalmology. https://doi.org/10.1186/s12886-026-05435-y

Image Credits: AI Generated

DOI: 10.1186/s12886-026-05435-y

Keywords: uveal effusion syndrome, Sjögren's syndrome, angle-closure glaucoma, ciliochoroidal effusion, case report, ophthalmology, autoimmune disease, intraocular pressure, choroidal detachment, BMC Ophthalmology, Uveal, effusion