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Evaluation of patients with suspected sarcoidosis
Angiotensin converting enzyme (ACE) is integral to the renin-angiotensin system (RAS), which maintains blood pressure by regulation of fluid volume and vascular tension. Its peptidase action on the decapeptide angiotensinogen I results in the hydrolysis of a terminal histidyl leucine dipeptide and the formation of the octapeptide angiotensin II, a potent vasoconstrictor that increases blood pressure.
ACE activity is increased in sarcoidosis, a systemic granulomatous disease that commonly affects the lungs. In sarcoidosis, ACE is thought to be produced by epithelioid cells and macrophages of the granuloma.
ACE activity reflects the severity of sarcoidosis: 68% positivity in those with stage I sarcoidosis, 86% in stage II sarcoidosis, and 91% in stage III sarcoidosis.
Other conditions such as Gaucher disease, leprosy, untreated hyperthyroidism, psoriasis, premature infants with respiratory distress syndrome, adults with amyloidosis, and histoplasmosis have been associated with increased serum ACE activity.
> or =18 years: 16-85 U/L
0-17 years: ACE activity may be 20-50% higher in healthy children compared to healthy adults (16-85 U/L).
For SI unit Reference Values, see https://www.mayocliniclabs.com/order-tests/si-unit-conversion.html
An elevation in the level of serum angiotensin converting enzyme (ACE), along with radiographic evidence of infiltrates or adenopathy and organ biopsies showing noncaseating epithelial granulomas is suggestive of a diagnosis of sarcoidosis.
Normal, healthy children and infants are known to have ACE activity levels greater than the adult reference interval.
Spinal fluid angiotensin converting enzyme (ACE) activity to aid the diagnosis of neurosarcoidosis has been reported; however, there is insufficient evidence to support ACE being used for this purpose.
1. Liebermann J: Elevation of serum angiotensin-converting-enzyme (ACE) level in sarcoidosis. Am J Med. 1975;59:365-372
2. Rodriguez GE, Shin BC, Abernathy RS, Kendig EL Jr: Serum angiotensin-converting enzyme activity in normal children and in those with sarcoidosis. J Pediatr. 1981;99:68-72
3. Personal observations from a Mayo pediatric normal range study using a manual method (Hana)
4. Maguire GA, Price CP: A continuous monitoring spectrophotometric method for the measurement of angiotensin-converting enzyme in human serum. Ann Clin Biochem. 1985;22:204-210