Distinguishing uveitis secondary to sarcoidosis from idiopathic disease: Cardiac implications

Importance: Idiopathic disease is the most frequent diagnosis in a uveitis clinic. The need to distinguish sarcoidosis from idiopathic uveitis is controversial. However, cardiac involvement in sarcoidosis can be life-threatening. Objective: To report a series of patients with uveitis and cardiac sarcoidosis to illustrate the importance of categorizing the causes of uveitis. Design, Setting, and Participants: This retrospective observational case series reviewed the medical records of 249 patients with uveitis who were referred to the Casey Eye Institute between July 1, 2008, and February 28, 2017. Main Outcomes and Measures: We describe patients who initially received a diagnosis of idiopathic uveitis but subsequently received a diagnosis of sarcoidosis. Clinical data, including ophthalmologic findings, were collected. We summarized the number of patients who initially presented with idiopathic uveitis, the number of patients who recived a classification of idiopathic uveitis after evaluation, the number of patients who underwent chest computed tomography or an electrocardiogram, and the number of patients with ocular sarcoidosis. Results: Of 33 patients with sarcoidosis, 21 (63.6%) were women and the mean (SD) age was 53.5 (13.8) years. Of 249 patients, the reerring diagnosis was idiopathic uveitis for 179 (72%). After history, examination, and laboratory testing, 127 (51%) were still considered to have idiopathic disease. Fifty-three of the 179 patients (30%) with idiopathic disease underwent chest computed tomography scanning. A diagnosis of presumed sarcoidosis, usually on the basis of a chest computed tomography scan, was made in 19 patients (36.2%). As 14 patients (5.6%) were previously known to have sarcoidosis, 33 patients (13.3%) were evaluated with definite or presumed ocular sarcoidosis. We obtained electrocardiograms as a screen for cardiac sarcoidosis on 14 (42.4%) of these patients. Nine patients with abnormal electrocardiogram results were referred to cardiologists. Four of the 19 patients (21.1%) who were referred for idiopathic uveitis but subsequently received a diagnosis of presumed sarcoidosis were found to have episodes of ventricular tachycardia that required implantable cardiac defibrillators. Distinguishing ocular sarcoidosis from idiopathic uveitis had potentially life-saving implications for these patients. Conclusions and Relevance: The present case series shows the potential utility of distinguishing sarcoidosis-associated uveitis from idiopathic uveitis. We suggest that patients older than 40 years with a history of idiopathic uveitis be evaluated with chest computed tomography and an electrocardiogram if sarcoidosis is suggested on ophthalmic examination.