Reumatizam, Vol. 59 No. 2, 2012.
Stručni rad
Anti-synthetase syndrome
Srđan Novak
; Odjel za reumatologiju i kliničku imunologiju, Klinika za internu medicinu, Klinički bolnički centar Rijeka, Rijeka, Hrvatska
Sažetak
Antysynthetase syndrome is considered as a group of idiopathic inflammatory myositis with charcteristic serologic hallmark - antibodies which recognise the aminoacyl-tRNA synthetasses (ARS). Clinical picture of those patients contains myositis and/or intersticial lung disease (ILD) and/or arthritis and/or fever and/or Raynaud phenomenon and sometimes characteristic look of mechanic´s hands. Myositis can be overt, sometimes even absent, while IBP is major cause of morbidity and determines the outcome of the disease. Untill now eight different any-synthetase autoantibodies are recognised, and most frequent are findings of anti-histidyl-tRNa synthetase antibodies. Patients with other ARS autoantibodies usually have severe ILD. Drug of choice are steroids in dosage of 1 mg/kg with immunosupresive agent (azatioprin or methotrexate) while in severe IBP cyclophosphamide is needed. Recently succsesful treatment with rituximab in combination with cyclophosphamide is reported.
Ključne riječi
antisynthetase syndrome; dermatomyositis; intersticial lung disease; polymyositis; treatment
Hrčak ID:
124042
URI
Datum izdavanja:
25.10.2012.
Posjeta: 2.844 *