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Case report
Jo1-antisynthetase syndrome and severe interstitial lung disease with organising pneumonia on histopathology with favourable outcome on early combined treatment with corticosteroids, mycophenolate mofetil and rituximab
  1. Christine A Rüegg1,
  2. Britta Maurer2,
  3. Irène Laube3 and
  4. Dieter Scholtze3
  1. 1 Division of Pulmonology, University Hospital Zurich, Zurich, Switzerland
  2. 2 Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland
  3. 3 Division of Pulmonology, Stadtspital Triemli, Zurich, Switzerland
  1. Correspondence to Dr Christine A Rüegg, crueegg{at}gmx.li

Abstract

Antisynthetase syndrome is a rare autoimmune disease and represents a distinct entity within the idiopathic inflammatory myopathies. Its variable systemic manifestations are composed of myositis, interstitial lung disease, non-erosive arthritis, fever, Raynaud’s phenomenon, hyperkeratotic skin changes and the presence of antibodies against aminoacyl-transfer-RNA-synthetases. Interstitial lung disease is the major determinant of morbidity and mortality. The role of lung biopsy remains controversial but it might be considered on an individual basis and may provide information regarding prognosis and treatment response. An integrated clinical, radiological and pathological approach to interstitial lung disease has to be emphasised. Due to the rarity of the disease, no standardised treatment guidelines for antisynthetase syndrome exist. We discuss a patient with anti-Jo1-autoantibody antisynthetase syndrome with proximal myositis and severe, rapid onset, interstitial lung disease with a histopathological pattern of organising pneumonia on surgical lung biopsy and good response to early combined immunosuppressive treatment with corticosteroids, mycophenolate mofetil and rituximab.

  • Interstitial Lung Disease
  • Connective Tissue Disease
  • Biological Agents
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Footnotes

  • Contributors All authors were involved in the care of the patient. CAR: reviewed the medical literature and wrote the manuscript. BM: reviewed the medical literature and provided critical revisions of the manuscript. IL and DS: contributed to the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Obtained.

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