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Anti-signal recognition particle autoantibodies: marker of a necrotising myopathy
  1. G J D Hengstman1,
  2. H J ter Laak1,
  3. W T M Vree Egberts2,
  4. I E Lundberg3,
  5. H M Moutsopoulos4,
  6. J Vencovsky5,
  7. A Doria6,
  8. M Mosca7,
  9. W J van Venrooij2,
  10. B G M van Engelen1
  1. 1Department of Neurology, Neuromuscular Centre Nijmegen, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  2. 2Department of Biochemistry, Centre for Molecular Life Sciences, Radboud University Nijmegen, Nijmegen, The Netherlands
  3. 3Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden
  4. 4Department of Pathophysiology, School of Medicine, National University of Athens, Athens, Greece
  5. 5Institute of Rheumatology, Prague, Czech Republic
  6. 6Division of Rheumatology, University of Padova, Padova, Italy
  7. 7Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy
  1. Correspondence to:
    G J D Hengstman
    Department of Neurology, Neuromuscular Centre Nijmegen, University Medical Centre Nijmegen, PO Box 9101, Internal Code 935, 6500 HB Nijmegen, The Netherlands;g.hengstman{at}neuro.umcn.nl

Abstract

Objective: To elucidate the clinical importance of the anti-signal recognition particle (SRP) autoantibody in patients with myositis.

Methods: Retrospective systematic assessment of the clinical, laboratory and histological characteristics of 23 anti-SRP-positive patients from six European centres. Data were compared with a large group of anti-SRP-negative patients with myositis published previously.

Results: Clinically, patients with anti-SRP autoantibodies often had a severe symmetric proximal muscle weakness resulting in marked disability, dysphagia and highly elevated levels of serum creatine kinase. Three patients had typical dermatomyositis rashes. The disease was associated with the occurrence of extramuscular signs and symptoms including interstitial lung disease. No association was found with an increased risk of cardiac involvement, and the disease carried a reasonably favourable prognosis with most patients responding to treatment. None of the patients had the typical histological features of myositis. Most muscle biopsy specimens showed the presence of necrotic muscle fibres and no inflammatory infiltrates.

Conclusions: Anti-SRP autoantibodies are associated with a syndrome of a necrotising myopathy in the spectrum of immune-mediated myopathies that differs from typical polymyositis. Further studies are needed to elucidate the pathogenesis and to clarify the role of the anti-SRP autoantibodies in this unique disease.

  • HLA, human leucocyte antigen
  • ILD, interstitial lung disease
  • MAC, membrane attack complex
  • SRP, signal recognition particle

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Footnotes

  • Published Online First 5 May 2006

  • Competing interests: None.

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