• Title/Summary/Keyword: Antisynthetase Syndrome

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Acute Respiratory Distress Syndrome as the Initial Clinical Manifestation of an Antisynthetase Syndrome

  • Kim, Seo-Hyun;Park, I-Nae
    • Tuberculosis and Respiratory Diseases
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    • v.79 no.3
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    • pp.188-192
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    • 2016
  • Antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibody to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, mechanic's hand-like cutaneous involvement, Raynaud phenomenon, and polyarthritis. Lung disease is the presenting feature in 50% of the cases. We report a case of a 60-year-old female with acute respiratory distress syndrome (ARDS), which later proved to be an unexpected and initial manifestation of anti-Jo-1 antibody-positive antisynthetase syndrome. The present case showed resolution of ARDS after treatment with high-dose corticosteroids. Given that steroids are not greatly beneficial in the treatment of ARDS, it is likely that the improvement of the respiratory symptoms in this patient also resulted from the prompt suppression of the inflammatory systemic response by corticosteroids.

A Case of Antisynthetase Syndrome (항 Synthetase 증후군 1예)

  • Kim, Min-Jeong;Kim, Min Ah;Kim, Eung-Gyu;Kim, Chan-Hwan;Kim, Sang-Jin
    • Annals of Clinical Neurophysiology
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    • v.8 no.2
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    • pp.196-198
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    • 2006
  • It has been reported that antisynthetase syndrome belongs to the idiopathic myositis group which includes pulmonary interstitial disease, arthritis, Raynaud's phenomenon, and mechanic's hand, associated with the anti-Jo1 antibody. A 60- year-old man presented with one month history of lower limbs weakness, rapidly progressive exertional dyspnea, and arthralgia. A markedly increased titers of anti-Jo1 antibodies were found. Chest CT showed idiopathic pulmonary fibrosis. Muscle biopsies were consistent with polymyositis. A high dose corticosteroids and cyclosporine were not effective. We report a case of antisynthetase syndrome, in which immunosuppressive agents could not rescue the deteriorating disease course.

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