[Ocular and neuro-ophthalmic manifestations of sarcoidosis: retrospective study of 30 cases]

Abstract

Introduction: The purpose of this study was to describe the diagnostic strategy and therapeutic approach when sarcoidosis of the visual pathways is suspected, by way of a descriptive study of sarcoidosis patients in the ophthalmology services in Tours, Rennes and Angers.

Patients and methods: A multicenter, retrospective chart review was performed for 30 patients diagnosed with sarcoidosis and followed at the university hospitals of Tours, Rennes and Angers between January 1997 and August 2011. The diagnosis of sarcoidosis was based on a combination of clinical and ancillary tests, including pathologic confirmation and/or hyperlymphocytosis with a CD4/CD8 ratio greater than 5 on bronchoalveolar lavage.

Results: We identified 30 patients with ocular and/or neuro-ophthalmic sarcoidosis (25 female [83%], five male [17%], median age 48 years (36-87). Histologic confirmation of epithelioid granuloma was obtained in 24 (80%) of the 30 included patients. Sarcoid uveitis was present in 83% of patients; neurosarcoidosis was found in 37% of patients. Additional complaints at presentation included dyspnea (31%), arthralgia (41%), myalgia (24%) and paresthesia (27%). Bilateral ocular involvement was found in 89% of cases. In 83% of patients, there was an active anterior uveitis on slit lamp examination at initial presentation. Funduscopic abnormalities included vitritis in 67%, snowbanking in 13%, snowballs in 27% and disc edema in 30%. Eight patients (27%) had retinal vasculitis and nine patients (30%) were diagnosed with multifocal choroiditis on angiography. An elevated angiotensin-converting enzyme was present in 13 (52%) of 25 patients tested, with an average of 71 (range 36-241). Evidence of sarcoidosis was present on chest CT in 22 (79%) of 28 patients. Neuroimaging abnormalities on brain MRI were found in eight of ten tested patients. The 10 IU tuberculin skin test was negative in all tested patients (12). Median lymphocytosis in the bronchoalveolar lavage fluid (BALF) was 0.3 (0.2-0.7) in the 15 tested patients. BALF CD4/CD8 ratio was positive in all tested patients (12). Oral corticosteroids were initiated at 1mg/kg per day and then tapered in 20 (67%) of 30 patients. Median duration of steroid treatment was 12 months (5-127), resulting in rapid improvement in 84% of cases. However, nearly half of patients experienced a recurrence of the disease.

Conclusion: Positive diagnosis of sarcoidosis requires a careful history and clinical examination, ancillary testing, and collaboration among several medical specialties. Normal angiotensin-converting enzyme levels do not rule out the diagnosis, since in our series, they were normal in almost half the cases. The tests with highest sensitivity included: a negative tuberculin skin test (100%), an elevated CD4/CD8 ratio in the BALF (100%), radiographic evidence of sarcoidosis on chest CT (79%) and brain MRI (80%). Other factors contributing to the diagnosis were: female gender (83%), age over 40 (67%), arthralgia (41%), asthenia (50%), vitritis (67%) and multifocal choroiditis (30%). In our study, treatment with corticosteroids resulted in significant improvement in over 80% of patients.