TERAPIJSKI PRISTUP BOLESNIKU S ANTISINTETAZNIM SINDROMOM – PRIKAZ BOLESNIKA

Dora Uršić, Ivana Kovačević, Željka Kardum, Kristina Kovačević Stranski, Ana Marija Masle, Ana Kovač, Mirna Lucić, Višnja Prus, Jasminka Milas Ahić

Introduction: Antisynthetase syndrome (ASS) is a rare autoimmune systemic disease characterised by myositis, arthritis, Raynaud’s syndrome, mechanic’s hands, and interstitial lung disease. Materials and methods: We present the case of a 50-year-old patient with predominant signs of polymyositis, pulmonary interstitium involvement and positive anti-Jo1 antibodies with suspected antisynthetase syndrome. Results: He was treated with glucocorticoids and following that the treatment was continued with an additional immunosuppressive therapy drug, azathioprine, which was discontinued due to a liver lesion. The treatment was then continued with cyclophosphamide. In the later course of the disease, radiological regression of pulmonary changes was monitored, and mycophenolate mofetil was introduced as a maintenance therapy. Conclusion: If left unrecognised, ASS can lead to the development of irreversible pulmonary fibrosis. However, timely application of immunosuppressive therapy can improve the quality of life and the prognosis of patients with ASS.

Uvod: Antisintetazni sindrom (ASS) je rijetka autoimunosna sustavna bolest koju karakteriziraju miozitis, artritis, Raynaudov sindrom, mehaničarske ruke i intersticijska bolest pluća. Materijali i metode: Prikazujemo 50-godišnjeg bolesnika sa znacima polimiozitisa i zahvaćanja plućnog intersticija uz pozitivna anti-Jo1 protutijela kod kojega je postavljena sumnja na antisintetazni sindrom. Rezultati: Liječen je glukokortikoidima, a potom je kao dodatna imunosupresivna terapija uveden azatioprin koji je isključen zbog jetrene lezije, a liječenje je nastavljeno ciklofosfamidom. U kasnijem tijeku bolesti pratila se radiološka regresija plućnih promjena te se kao terapija održavanja uvede mikofenolat mofetil. Zaključak: Ako ostane neprepoznat ASS dovodi do razvoja ireverzibilne plućne fibroze, međutim pravovremenom primjenom imunosupresivne terapije može se poboljšati kvaliteta života i prognoza bolesnika s ASS-om.