Rheumatology (Oxford, England) · 2025
Clinicopathological features of anti-HMGCR and anti-SRP myopathies that do not satisfy the EULAR/ACR criteria of inflammatory myopathies.
СтатьяResearch Support, N.I.H., IntramuralНазвание и аннотация: Перевод в очереди
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Komaki Shogo, Kubota Akatsuki, Kitamura Asuka, Maeda Meiko, Kadoya Masato, Hokkoku Keiichi, Kobayashi Shunsuke, Bannai Taro, Shiio Yasushi, Uchio Naohiro, Uesaka Yoshikazu, Kikuchi Junko, Sugimoto Izumi, Mikata Takashi, Okudera Rena, Suzuki Kazushi, Arai Noritoshi, Sakiyama Yoshio, Tokushige Shin-Ichi, Ichikawa Yaeko, Yajima Nobuyuki, Nakashima Ran, Mimori Tsuneyo, Shimizu Jun, Komai Toshihiko, Tsuchiya Haruka, Fujio Keishi, Toda Tatsushi
Аннотация
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 (<possible group) with those classified as IIMs (definite-possible group) on the basis of the EULAR/ACR classification criteria. For the <possible group whose follow-up data were available, their clinical features were assessed up to 24 months.
RESULTS: Nineteen anti-HMGCR antibody-positive cases (26%) and 19 anti-SRP antibody-positive cases (20%) were classified as the <possible group. The <possible group had milder weakness, less frequent dysphagia, lower serum creatine kinase and aldolase levels and fewer necrotic fibres and granular p62-positive fibres than the definite-possible group. Of the 26 cases in the <possible group that could be followed up, only three cases were classified as the definite-possible group, and the other cases remained in the <possible group during the follow-up period.
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.
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 (<possible group) with those classified as IIMs (definite-possible group) on the basis of the EULAR/ACR classification criteria. For the <possible group whose follow-up data were available, their clinical features were assessed up to 24 months.
RESULTS: Nineteen anti-HMGCR antibody-positive cases (26%) and 19 anti-SRP antibody-positive cases (20%) were classified as the <possible group. The <possible group had milder weakness, less frequent dysphagia, lower serum creatine kinase and aldolase levels and fewer necrotic fibres and granular p62-positive fibres than the definite-possible group. Of the 26 cases in the <possible group that could be followed up, only three cases were classified as the definite-possible group, and the other cases remained in the <possible group during the follow-up period.
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.