Version 2.78

Description

3-Hydroxy-3-Methylglutaryl-coenzyme A reductase (HMG-CoA reductase or HMGCR) is an enzyme in the cholesterol-synthesis pathway and is the target of HMG-CoA reductase inhibitors, also known as statins. Autoantibodies to HMGCR may be a marker of necrotizing autoimmune myopathy (NAM), which is characterized by muscle fiber necrosis with little or no inflammation and responsiveness to immunosuppressive treatment. Anti-HMGCR positive cases of NAM usually have limited extramuscular involvement, slow onset of muscle disease, and can occur in both adults and children. Due to the insidious onset, pediatric cases of NAM may be initially confused with muscular dystrophy. The anti-HMGCR antibody titer may correlate with muscle strength and creatine kinase level. Some cases of NAM have been diagnosed in patients previously exposed to statins, and in some of these cases, stopping the statin drug was the only intervention necessary for the resolution of disease. However, exposure to statins is not required to diagnose cases of NAM, and first-line treatment typically includes corticosteroids or other immunosuppressive drugs such as methotrexate. PMID: 24797170 Source: Regenstrief LOINC, PMID: 24797170

Basic Part Properties

Part Display Name
3-Hydroxy-3-Methylglutaryl-coenzyme A reductase Ab
Part Type
Component (Describes the core component or analyte measured)
Created On
2016-07-02
Construct for LOINC Short Name
3-OH-3-Methylglutaryl-coA reductase Ab

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