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Article Abstract

Objectives: The clinicopathological features of immune-mediated necrotizing myopathy (IMNM) sometimes mimic muscular dystrophy, complicating accurate diagnosis. The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria of idiopathic inflammatory myopathies (IIMs) are superior in terms of sensitivity and specificity; however, the sensitivity is reported to be relatively low in IMNM. We examined the clinicopathological characteristics and the prognoses of anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibody-positive and anti-signal recognition particle (SRP) antibody-positive cases that do not satisfy the EULAR/ACR classification criteria.

Methods: Seventy-one anti-HMGCR antibody-positive cases and 94 anti-SRP antibody-positive cases were included. We compared the clinicopathological characteristics of the groups that were not classified as IIMs (
Results: Nineteen anti-HMGCR antibody-positive cases (26%) and 19 anti-SRP antibody-positive cases (20%) were classified as the
Conclusions: Some IMNM cases showing a clinicopathologically mild phenotype and a benign course cannot be classified as IIMs by the EULAR/ACR classification criteria, and antibody testing is crucial for accurate diagnosis.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC12316373PMC
http://dx.doi.org/10.1093/rheumatology/keaf183DOI Listing

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