ANTI-SINTETAZA SINDROM

Srđan Novak

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 anysynthetase autoantibodies are recognised, and most frequent are fi ndings 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.

Anti-sintetaza sindrom obuhvaća skupinu idiopatskih upalnih miozitisa uz karakteristično serološko obilježje - protutijela na aminoacil tRNA sintetazu (ARS). U kliničkoj slici takvih bolesnika javlja se miozitis i/ili intersticijska bolest pluća (IBP) i/ili artritis i/ili povišena temparatura i/ili Raynaudov sindrom te ponekad i karakteristični izgled ruku tzv. mehaničarske ruke. Miozitis u takvih bolesnika može biti prikriven a nekad i potpuno odsutan dok je IBP glavni uzrok morbiditeta i determinira prognozu bolesti. Do danas je opisano osam različitih anti-sintetaza protutijela a najčešća su anti-histidil tRNA protutijela. Bolesnici s drugim anti-ARS protutijelima uobičajeno imaju teže oblike IBP. Lijek izbora su glukokortikoidi u dozi 1 mg/kg uz imunosupresivni lijek (azatioprin ili metotreksat) dok je u težih oblika IBP potrebno i liječenje ciklofosfamidom. U novije vrijeme opisana je uspješna primjena rituksimaba u kombinaciji s ciklofosfamidom.