Optic Nerve Head Granuloma, Retinal Vasculitis and Elevated Levels of Angiotensin‑converting Enzyme: Dilemma of Forme Fruste Ocular Sarcoidosis

Purpose: To report 2 cases of optic nerve head (ONH) granuloma, with raised serum angiotensin‑converting enzyme (ACE) levels not fitting into the existing criteria for ocular sarcoidosis (OS). Case Report: Fundus photography, ultrasonography, fluorescein angiography, and optical coherence tomography were performed for both patients. Systemic workup was performed for granulomatous disorders, including sarcoidosis, tuberculosis, and syphilis. Both patients had ONH granulomas and elevated ACE levels, with one of the patients also presenting retinal vasculitis. No other focus of systemic sarcoidosis was localized. Both patients were treated with oral steroids, following which they showed a marked, rapid clinical improvement. Both patients remained stable for at least one year. Conclusion: The current accepted criterion for diagnosis of OS may need changes to include such borderline cases due to lack of correlation between clinical and investigative findings.