Diagnosis of Sarcoidosis With an Increased ACE Level and a Normal Chest X-Ray?

John J. Cush, MD


June 13, 2000


How can a diagnosis of sarcoidosis be made in a patient with an elevated angiotensin-converting enzyme (ACE) level and a normal chest x-ray?

Response from John J. Cush, MD

ACE is produced by endothelial cells, epithelial cells, and activated macrophages. ACE levels are usually elevated in patients with sarcoidosis (60%-80%), especially those with pulmonary involvement. Elevated levels may also be seen in a wide variety of disorders, including other forms of chronic interstitial lung disease (silicosis, asbestosis, histoplasmosis), malignancies (ie, Hodgkin's disease), infections (leprosy, histoplasmosis) and patients with autoimmune disease (eg, scleroderma).

Thus, one cannot rely solely on ACE levels to establish or refute a diagnosis of sarcoidosis. In patients without suggestive pulmonary findings or other compatible symptoms (eg, uveitis, fever, parotitis, arthritis, skin findings [erythema nodosum]), the diagnosis may rely on histopathologic evidence of noncaseating granuloma in involved tissue(s). Blind biopsies from asymptomatic sites are not advised and should only be considered where clinical or imaging findings suggest pathology.


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