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Immunosuppression for uveal effusion syndrome – a report of two cases
Author(s) -
Staniszewski B.,
Forrester J.V.,
Kuffova L.
Publication year - 2017
Publication title -
acta ophthalmologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.534
H-Index - 87
eISSN - 1755-3768
pISSN - 1755-375X
DOI - 10.1111/j.1755-3768.2017.0t081
Subject(s) - medicine , azathioprine , immunosuppression , methylprednisolone , cyclophosphamide , uveitis , disease , plasmapheresis , surgery , dermatology , ophthalmology , chemotherapy , immunology , antibody
Purpose Uveal effusion syndrome (UES) is a rare inflammatory disease characterised by massive choroidal detachment due to accumulation of fluid in the suprachoroidal space. There may also be an associated exudative retinal detachment (ERD). The cause of UES is not known but can be primary due to connective tissue disease (1) or secondary to inflammatory disease. We report two cases of secondary UES cases which were refractory to treatment and discuss further management. Methods A case series of two patients presenting to a hospital in United Kingdom with ocular symptoms secondary to UES. Results Case 1 presented with reduced vision and photopsia in one eye without obvious signs of inflammation while case 2 presented with a painful red eye associated with epiphora and increased intraocular pressure. Large uveal effusions and ERD were clinically apparent on fundoscopy in both patients and an inflammatory etiology was presumed. Both patients suffered a severe relapsing course requiring repeated adjustments to their immunosuppressive therapies ‐ several pulses of intravenous methylprednisolone for exacerbations of the disease plus mycophenolate mofetil (MMF) (case 1) and azathioprine (case 2). Failure to respond required a cycle of cyclophosphamide (pulse 15 mg/kg/infusion) which settled the disease in case 2. However, case 1 failed to respond and is treated with 8‐weekly course of Remsima infusions (anti‐TNFa, 5 mg/kg/infusion) which led to essentially complete resolution of the effusion while maintaining patient's vision. Conclusions UES is a rare condition which is difficult to manage. In cases of secondary inflammatory UES a range of immunosuppressants may be required to achieve resolution of the disease. 1. Forrester et.al (1990) The uveal effusion syndrome and trans‐scleral flow. Eye 4: 354‐65.