Q:

As part of the evaluation, serum angiotensin-converting enzyme (ACE) level is drawn. What state- ment is true regarding the use of ACE levels in sarcoidosis?

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A LJ 1 -year-old African American female patient is referred for evaluation of leukopenia. She reports she is generally in good health . but she has noted increasing shortness of breath in recent weeks. She has a history of ovarian carci noid tumor that was trea ted surgical ly. Complete blood count shows leukocyte count 2.1 00/fLL. hemoglobin 1 2 .2 g/dl. and platelet count 2 30,000/ fLl. Physical exam shows no splenomegaly or lymphadenopathy. I mages below are from CT chest and bone marrow biopsy.

As part of the evaluation, serum angiotensin-converting enzyme (ACE) level is drawn. What state-ment is true regarding the use of ACE levels in sarcoidosis?


  1. ACE levels are elevated in > 90% of patients with active sarcoidosis
  2. ACE levels greater than 1 50% of normal correlate with extrapulmonary sarcoidosis
  3. False positive results limit the usefulness of the assay in diagnosis and therapy
  4. Patients taking amiodarone should hold the medication for 48 hours prior to testing

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C. While false-positive ACE elevations are not frequently encountered, the frequency is great enough to argue against the usage of ACE level to guide firm decisions in the diagnosis or treat-ment of sarcoidosis. In general, the ACE level is elevated in around 75% of patients with active disease.

No clear relationship between specific cutoffs in ACE elevation and extrapulrnonary sarcoid has been demonstrated. Amiodarone does not affect the ACE level (Ann Clin Biochem. 1989;26(Pt 1):13).

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