| Abstract | Anti-sintetaza sindrom je autoimuna sistemska bolest iz spektra idiopatskih upalnih miopatija. Ovom restrospektivnom studijom na uzorku od 16 ispitanika sa dijagnosticiranim ASS koji su liječeni na Zavodu za reumatologiju i kliničku imunologiju KBC-a Rijeka u periodu od travnja 2018. godine do listopada 2023. godine dokazano je da se bolest dva puta češće javlja u žena nego u muškaraca. Nadalje, ASS se može prvom simptomatologijom javiti u bilo kojoj životnoj dobi, međutim, najviše ispitanika se prezentiralo između 30 i 70 godine života. Od kliničkih karakteristika, najviše ispitanika imalo je prisutan artritis, miozitis i/ili ILD, dok su ”mehaničarske” ruke, Raynaudov fenomen, febrilitet i disfagija, bile su zastupljene u manjeg broja ispitanika. Miozitis se češće javljao u pacijenata s pozitivnim anti-Jo-1 protutijelima, dok se ILD češće javljala u pacijenata s pozitivnim anti-PL-7 protutijelima. Prije postavljanja dijagnoze i uvođenja terapije, većina ispitanika imala je blago povišene vrijednosti upalnih parametara (SE i CRP), blago povišene vrijednosti LDH te znatno povišene vrijednosti CK. Većina ispitanika imala je pozitivno samo jedno protutijelo, dok je samo jedan ispitanik imao pozitivna dva protutijela. Najviše ispitanika bilo je pozitivno na anti-Jo-1 protutijelo, dok su ostali ispitanici imali pozitivno anti-PL-7 ili anti-PL-12 protutijelo. Miozitis se prvenstveno dijagnosticirao na temelju anamneze, fizikalnog pregleda i povišenih vrijednosti CK, dok su EMNG i biopsija mišića provedene u manjeg broja ispitanika. S druge strane, HRCT čini zlatni standard u postavljanju dijagnoze ILD-a. Na HRCT-u su najčešće bili zastupljeni NSIP i UIP uzorak, dok je OP uzorak bio zastupljen u manjeg broja ispitanika. Pacijentima s ASS su kao prva linija liječenja uvedeni GK, a kako bi se s vremenom smanjila doza GK, u terapiju se mogu dodavati MTX, RTX, AZA, MMF i Cs. |
| Abstract (english) | Anti-synthetase syndrome is an autoimmune systemic disease from the spectrum of idiopathic inflammatory myopathies. This retrospective study on a sample of 16 subjects diagnosed with ASS who were treated at the Department of Rheumatology and Clinical Immunology of CHC Rijeka in the period from April 2018 to October 2023 proved that the disease occurs twice as often in women than in men. Furthermore, ASS can present its first symptoms at any age, however, most subjects presented between 30 and 70 years of age. Of the clinical characteristics, most subjects had arthritis, myositis and/or ILD, while "mechanic" hands, Raynaud's phenomenon, febrility and dysphagia were present in a smaller number of subjects. Myositis occurred more often in patients with positive anti-Jo-1 antibodies, while ILD occurred more often in patients with positive anti-PL-7 antibodies. Before the diagnosis and introduction of therapy, most of the subjects had slightly elevated values of inflammatory parameters (SE and CRP), slightly elevated LDH values, and significantly elevated CK values. Most of the subjects had only one positive antibody, while only one subject had two positive antibodies. Most subjects were positive for anti-Jo-1 antibody, while the rest of the subjects had positive anti-PL-7 or anti-PL-12 antibody. Myositis was primarily diagnosed based on history, physical examination and elevated CK values, while EMNG and muscle biopsy were performed in a smaller number of subjects. On the other hand, HRCT is the gold standard in the diagnosis of ILD. On HRCT, NSIP and UIP patterns were most often represented, while OP pattern was represented in a smaller number of subjects. Patients with ASS are given GK as the first line of treatment, and in order to reduce the dose of GK over time, MTX, RTX, AZA, MMF and Cs can be added to the therapy. |
