Anti-synthetase syndrome associated with anti PL-12 and anti-Signal recognition particle antibodies and a necrotizing auto-immune myositis

Ashish Malkan; Cecilia Cappelen-Smith; Roy Beran; Neil Griffith; Catherine Toong; Min-Xia Wang; Dennis Cordato

doi:10.1016/j.jocn.2014.04.031

Anti-synthetase syndrome associated with anti PL-12 and anti-Signal recognition particle antibodies and a necrotizing auto-immune myositis

J Clin Neurosci. 2015 Feb;22(2):396-8. doi: 10.1016/j.jocn.2014.04.031. Epub 2014 Aug 21.

Authors

Ashish Malkan¹, Cecilia Cappelen-Smith¹, Roy Beran², Neil Griffith¹, Catherine Toong³, Min-Xia Wang⁴, Dennis Cordato⁵

Affiliations

¹ Department of Neurophysiology, Liverpool Hospital, Locked Bag 7103, Liverpool, NSW 1871, Australia; South Western Clinical School, University of New South Wales, NSW, Australia.
² 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.
³ South Western Clinical School, University of New South Wales, NSW, Australia; Immunology Department, Liverpool Hospital, Liverpool, NSW, Australia.
⁴ Department of Neurology, University of Sydney, NSW, Australia.
⁵ 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.

PMID: 25150763
DOI: 10.1016/j.jocn.2014.04.031

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.

Keywords: Anti-PL-12 antibody; Anti-SRP antibody; Anti-synthetase syndrome; Myositis-specific autoantibody testing; Necrotizing auto-immune myositis.

Publication types

Case Reports

MeSH terms

Adult
Anti-Inflammatory Agents / therapeutic use
Autoantibodies / immunology*
Autoimmune Diseases / etiology*
Autoimmune Diseases / pathology
Cyclophosphamide / therapeutic use
Female
Humans
Immunization, Passive
Immunosuppressive Agents / therapeutic use
Immunotherapy
Ligases / immunology*
Muscle Weakness / etiology
Muscle Weakness / pathology
Myositis / etiology*
Myositis / pathology
Necrosis
Prednisolone / therapeutic use
Signal Recognition Particle / immunology*
Syndrome
Treatment Outcome

Substances

Anti-Inflammatory Agents
Autoantibodies
Immunosuppressive Agents
Signal Recognition Particle
Cyclophosphamide
Prednisolone
Ligases