title: Anti-synthetase syndrome associated with anti PL-12 and anti-Signal recognition particle antibodies and a necrotizing auto-immune myositis.
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.
authors.affiliation: Department of Neurophysiology, Liverpool Hospital, Locked Bag 7103, Liverpool, NSW 1871, Australia; South Western Clinical School, University of New South Wales, NSW, Australia.
authors.forename: Ashish
authors.initials: A
authors.lastname: Malkan
authors.affiliation: Department of Neurophysiology, Liverpool Hospital, Locked Bag 7103, Liverpool, NSW 1871, Australia; South Western Clinical School, University of New South Wales, NSW, Australia.
authors.forename: Cecilia
authors.initials: C
authors.lastname: Cappelen-Smith
authors.affiliation: Department of Neurophysiology, Liverpool Hospital, Locked Bag 7103, Liverpool, NSW 1871, Australia; South Western Clinical School, University of New South Wales, NSW, Australia; Griffith University, Southport, Queensland, Australia.
authors.forename: Roy
authors.initials: R
authors.lastname: Beran
authors.affiliation: Department of Neurophysiology, Liverpool Hospital, Locked Bag 7103, Liverpool, NSW 1871, Australia; South Western Clinical School, University of New South Wales, NSW, Australia.
authors.forename: Neil
authors.initials: N
authors.lastname: Griffith
authors.affiliation: South Western Clinical School, University of New South Wales, NSW, Australia; Immunology Department, Liverpool Hospital, Liverpool, NSW, Australia.
authors.forename: Catherine
authors.initials: C
authors.lastname: Toong
authors.affiliation: Department of Neurology, University of Sydney, NSW, Australia.
authors.forename: Min-Xia
authors.initials: MX
authors.lastname: Wang
authors.affiliation: Department of Neurophysiology, Liverpool Hospital, Locked Bag 7103, Liverpool, NSW 1871, Australia; South Western Clinical School, University of New South Wales, NSW, Australia. Electronic address: dennis.cordato@sswahs.nsw.gov.au.
authors.forename: Dennis
authors.initials: D
authors.lastname: Cordato
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000209:etiology* / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007116:Immunization, Passive; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007167:Immunotherapy; D008025:Ligases / Q000276:immunology*; D018908:Muscle Weakness / Q000209:etiology / Q000473:pathology; D009220:Myositis / Q000209:etiology* / Q000473:pathology; D009336:Necrosis; D011239:Prednisolone / Q000627:therapeutic use; D018271:Signal Recognition Particle / Q000276:immunology*; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D018271:Signal Recognition Particle; D003520:Cyclophosphamide; D011239:Prednisolone; D008025:Ligases
keywords: Anti-PL-12 antibody; Anti-SRP antibody; Anti-synthetase syndrome; Myositis-specific autoantibody testing; Necrotizing auto-immune myositis