[The anti-Jo-1 syndrome - a specific form of myositis with interstitial lung disease].
Interstitial lung disease (ILD) may be associated with systemic inflammatory disorders and autoantibody production. The development of ILD has been described in detail in patients with polymyositis and dermatomyositis. Anti-synthetase antibodies, including anti-Jo-1 antibodies (antihistidyl-tRNA syntase), are found in up to 35 % of patients with myositis, 80 % of which constitute anti-Jo-1 antibodies. The anti-Jo-1 syndrome characteristically presents with myositis, shortness of breath, fever, polyarthritis/arthralgia, mechanic's hands, dermatomyositis-like skin lesions, signs of a connective tissue disease and/or Raynaud's phenomenon. ILD is an early diagnostic sign and shows focal infiltrates and an acinar pattern in CT scan. Non-specific interstitial pneumonitis with T-lymphocytic infiltrates in lung histology (VATS) or elevated IFN-gamma-inducible chemokines are further indicators for anti-Jo-1 syndrome. Corticosteroids eventually combined with an immunosuppressant drug are often required with reported beneficial effects, although not many therapeutic studies have been performed. Here we present a review of the current literature and a case report on anti-Jo-1 syndrome.
Häussermann
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Robert-Koch-Klinik, Thoraxzentrum des Klinikums St. Georg, Leipzig. anja.haeussermann@gmx.de
Gillissen
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A
Seidel
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W
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000974:Antibodies, Antinuclear / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000276:immunology*; D009220:Myositis / Q000188:drug therapy* / Q000276:immunology*; D013577:Syndrome
D000305:Adrenal Cortex Hormones; D000893:Anti-Inflammatory Agents; D000974:Antibodies, Antinuclear; D007166:Immunosuppressive Agents; C046750:Jo-1 antibody