Abstract
Purpose: :
Sarcoidosis is an idiopathic inflammatory condition that commonly involves the lungs, eyes and skin. Definitive diagnosis is made with pathologic confirmation of non-caseating granulomas. In the case of intraocular inflammation, the risks of obtaining a biopsy are significant. We describe diagnostic tests used in our patients with intraocular inflammation and biopsy-proven sarcoidosis to determine which combination of tests might allow for a presumptive diagnosis of sarcoidosis in the absence of a tissue diagnosis.
Methods: :
Medical records of patients with ocular inflammation and biopsy-proven sarcoidosis seen between 1995 and 2007 were reviewed to identify patients who underwent diagnostic testing including serum angiotensin converting enzyme (ACE) levels, serum lysozyme levels, chest x-ray and/or chest computerized tomography (CT).
Results: :
42 patients with biopsy-proven sarcoidosis had available ancillary test results. ACE was high in 12/29 patients; lysozyme in 9/21 patients. 20 patients had both ACE and lysozyme levels measured. 13/20 patients had at least one elevated marker. 3 had elevated ACE and lysozyme, 5 only elevated ACE, 5 only elevated lysozyme, and 7 had neither marker elevated. 3 of the patients with normal ACE were on ACE inhibitors. 3/4 patients on oral prednisone had negative serum markers. Chest imaging consistent with sarcoidosis was observed by x-ray in 24/35 patients and by CT in 19/19 patients. 5 of 14 patients who underwent both imaging studies had a negative x-ray and positive CT; 4/5 were Caucasian females over 50 years of age. 18/19 patients who underwent testing for both serum markers and chest imaging had at least one diagnostic test consistent with sarcoidosis.
Conclusions: :
Serum levels of ACE and lysozyme are most useful in patients who are not taking ACE inhibitors (ACE level) or oral immunosuppressants. Chest x-ray, chest CT and elevation of ACE and/or lysozyme were each positive in at least 65% of patients with sarcoidosis. Chest CT may be the appropriate test for Caucasian women over 50 years of age. The combination of chest imaging and elevated serum levels of either ACE or lysozyme identified 95% of patients with ocular sarcoidosis and may be useful in patients who are not able to undergo confirmatory biopsy.
Keywords: immunomodulation/immunoregulation • imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound) • uveitis-clinical/animal model