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[Anti-SRP myopathy: different entity from myositis]
1Department of Neurology, Keio University School of Medicine.
Abstract:
Anti-signal recognition particle (SRP) antibody, detected in 5-8% of patients with clinical diagnosis of myositis, had been associated with severe and refractory myositis. However, it has been accepted that anti-SRP myopathy should be separated from myositis based on histological features of necrotizing myopathy. We reviewed clinical features of 27 patients with anti-SRP myopathy, and analyzed disease progression and neurological outcome. Anti-SRP antibodies in serum were detected by RNA immunoprecipitation assay using extracts of K562 cells. Of the 27 patients, 5 (18.5%) showed chronic progressive muscle weakness as well as atrophy of limbs and trunk muscles from a younger age with more severe neurological outcomes compared to the other 22 patients with the subacute form. A subset of patients with anti-SRP myopathy can show a chronic progressive form associated with severe clinical deficits.
Insights
Anti-signal recognition particle (SRP) myopathy can present as a chronic progressive condition. This subset of patients experiences severe muscle weakness and atrophy, leading to poorer neurological outcomes.
Area of Science:
- Neurology
- Immunology
- Genetics
Context:
- Anti-signal recognition particle (SRP) antibodies are found in 5-8% of myositis patients.
- Anti-SRP myopathy is characterized by necrotizing myopathy, distinct from typical myositis.
- Previous understanding associated anti-SRP antibodies with severe, refractory myositis.
Purpose:
- To review clinical features of anti-SRP myopathy.
- To analyze disease progression and neurological outcomes in anti-SRP myopathy patients.
- To identify distinct clinical phenotypes within anti-SRP myopathy.
Summary:
- A review of 27 anti-SRP myopathy patients identified a chronic progressive form in 18.5% of cases.
- This chronic form presents with early-onset muscle weakness and atrophy, and more severe neurological deficits.
- The study utilized RNA immunoprecipitation assay for anti-SRP antibody detection.
Impact:
- Highlights a distinct chronic progressive phenotype of anti-SRP myopathy.
- Suggests that anti-SRP myopathy requires tailored management strategies based on disease course.
- Contributes to a better understanding of the clinical spectrum and prognosis of anti-SRP myopathy.
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