1. Anti-synthetase syndrome associated with anti PL-12 and anti-Signal recognition particle antibodies and a necrotizing auto-immune myositis
- Author
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Neil Griffith, Roy G. Beran, Cecilia Cappelen-Smith, Catherine Toong, Min-Xia Wang, Ashish Malkan, and Dennis Cordato
- Subjects
Adult ,Pathology ,medicine.medical_specialty ,Proximal muscle weakness ,medicine.medical_treatment ,Prednisolone ,Anti-Inflammatory Agents ,Autoimmune Diseases ,Ligases ,Necrosis ,Physiology (medical) ,Medicine ,Humans ,Cyclophosphamide ,Myositis ,Autoantibodies ,Signal recognition particle ,Muscle Weakness ,biology ,business.industry ,Interstitial lung disease ,Autoantibody ,Immunization, Passive ,General Medicine ,Immunotherapy ,Syndrome ,medicine.disease ,Treatment Outcome ,Neurology ,Immunology ,biology.protein ,Surgery ,Female ,Neurology (clinical) ,Antibody ,business ,Signal Recognition Particle ,Immunosuppressive Agents ,medicine.drug - Abstract
We report a 37-year-old woman with a 2 month history of proximal muscle weakness and extremely high creatine kinase (21,808 U/L) due to necrotizing auto-immune myositis (NAM) in association with anti-synthetase syndrome. Myositis-specific auto-immune antibody panel was positive for anti-Signal recognition particle and anti-PL-12. CT scan of the chest confirmed interstitial lung disease. Prednisolone, intravenous immunoglobulin and cyclophosphamide therapy was given with gradual improvement. This patient is notable for the unusual combination of NAM and anti-synthetase syndrome with the rare finding of two myositis-specific autoantibodies, which directed testing for associated extramuscular features and management with more aggressive immunotherapy.
- Published
- 2014