Anti-signal recognition particle positive necrotizing myopathy-sjogren’s syndrome overlap syndrome: a descriptive study on clinical and myopathology features
BMC musculoskeletal disorders(2023)
摘要
Background and objective The aim of this study was to elucidate the clinical and myopathological characteristics of patients with anti-signal recognition particle (SRP) positive immune-mediated necrotizing myopathy (IMNM) overlap Sjogren’s syndrome (SS). Materials and methods We retrospectively analyzed the data of anti-SRP positive IMNM patients admitted in the Neurology Department of Tongji Hospital between January 2011 to December 2020. Patients were divided into two groups: anti-SRP IMNM overlap SS group and anti-SRP IMNM control group. The clinical features, laboratory results, histological features, treatment, and prognosis were compared between the two groups. Results A total of 30 patients with anti-SRP IMNM were included, including six anti-SRP IMNM overlap SS patients (two males, four females), with a median age of 39 years, and 24 anti-SRP IMNM patients (ten males, fourteen females), with a median age of 46 years. The anti-SRP IMNM overlap SS group had a lower prevalence of muscle atrophy (0 vs 50%, p = 0.019), and a higher prevalence of extramuscular manifestations, including cardiac abnormalities and ILD (Interstitial lung disease). CD4 + and CD68 + inflammatory infiltrations were significantly increased in anti-SRP IMNM overlap SS patients, with an increased presence of CD4 + cells in both necrotic( p = 0.023) and endomysial areas ( p = 0.013), and more CD68 + cells ( p = 0.016) infiltrated the endomysial area. Deposition of membrane attack complex (MAC) on sarcolemma ( p = 0.013) was more commonly seen in the anti-SRP IMNM overlap SS group. Conclusion Our data revealed that anti-SRP IMNM-SS overlap patients may present with milder muscular manifestation, but worse extramuscular manifestations compared to anti-SRP IMNM patients without SS. CD4 + and CD68 + inflammatory infiltrations and MAC deposition were remarkably increased in anti-SRP IMNM-SS overlap patients.
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关键词
Anti-signal recognition particle antibodies,Immune-mediated necrotizing myopathy,Overlap syndrome,Sjogren’s syndrome
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