Title Sindrom aktivacije makrofaga Title (english) Macrophage activation syndrome Author Matea Kronja Mentor Marija Jelušić (mentor)Committee member Ivan Malčić (predsjednik povjerenstva)Committee member Danko Milošević (član povjerenstva)Committee member Marija Jelušić (član povjerenstva)Granter University of Zagreb Defense date and country 2015-07-15, Croatia Scientific / art field, BIOMEDICINE AND HEALTHCARE Abstract Sindrom aktivacije makrofaga (MAS) je životno ugrožavajuća komplikacija reumatske bolesti koja se najčešće pojavljuje u pojedinaca sa sustavnim tipom juvenilnog idiopatskog artritisa (sJIA). MAS je karakteriziran visoko stimuliranim ali neučinkovitim imunološkim odgovorom što dovodi do prekomjerne proizvodnje citokina i hemofagocitoze. ----- Cilj našeg istraživanja bio je analizirati preliminarne kriterije za dijagnosticiranje MAS-a u bolesnika sa sJIA-om iz 2014. te ih usporediti s kriterijima koji su se do tada koristili za dijagnosticiranje MAS-a iz 2005., kao i s kriterijima za dijagnosticiranje HLH iz 2004., koji su prvi korišteni u tu svrhu. U istraživanje su uključena djeca u dobi od 1 do 18 godina kojima je u razdoblju od 2009. do 2014. godine u Klinici za pedijatriju, Zavodu za kliničku imunologiju i reumatologiju KBC-a Zagreb dijagnosticiran sJIA prema ILAR kriterijima. Sustavni JIA je dijagnosticiran u 30-tero djece, 11 djevojčica i 19 dječaka. Prosječna dob pri dijagnozi sustavnog JIA bila je (±SD) 8.51±5.65. Osam bolesnika (26,6%) imalo je relaps bolesti. Prema preliminarnim kriterijima iz 2005., MAS-a je razvilo 6 (20%) bolesnika sa sJIA-om i to četvero bolesnika pri postavljanju dijagnoze sJIA, a dva u relapsu bolesti. Uzimajući u obzir kriterije iz 2014. godine, MAS razvilo njih 4 (13%), dva bolesnika pri postavljanju dijagnoze sJIA, a dva u relapsu bolesti. Niti jedan bolesnik nije zadovoljio kriterije za HLH iz 2004. ----- Zaključno, prevalencija MAS-a u bolesnika sa sJIA se razlikovala s obzirom na korištene kriterije i nešto je veća u usporedbi s literaturnim podatcima. Klinička prezentacija se nije značajnije razlikovala u usporedbi s literaturom. S obzirom da ne postoji jedinstveni parametar za pravovremeno prepoznavanje MAS-a, ispravno postavljena dijagnoza znatno ovisi o iskustvu kliničara, te pažljivom praćenju relativnog pada u laboratorijskim vrijednostima. Odgađanje liječenja i čekanje razvoja pune kliničke slike moglo bi dovesti do potencijalno smrtnog ishoda.
Abstract (english) The term macrophage activation syndrome (MAS) describes a hyperinflammatory complication of systemic juvenile idiopathic arthritis (sJIA) caused by severe hypercytokinemia due to a dysregulated immune response. ----- The aim of the present study was to compare the capacity of the HLH-2004 guidelines with the capacity of the MAS guidelines from 2005, and so with the preliminary ones from 2014 in order to differentiate systemic JIA–associated MAS from active systemic JIA without MAS. The study included 30 children aged 1-18 diagnosed with systemic juvenile idiopathic arthritis who were treated at the Department of Pediatrics, Division of Immunology and Rheumatology, University Hospital Center Zagreb, in the period from 2009 to 2014 year. Out of 30 patients diagnosed with systemic juvenile idiopathic arthritis there were eleven girls and nineteen boys. The mean age at onset of the sJIA was (±SD) 8,51±5,65 years. There were eight patients (26.6%) with the relaps of the disease. With the guidelines from 2005, MAS was diagnosed in 6 (20%) patients with sJIA. In four of them it occured like the presenting manifestation of sJIA, and in the rest two it occured during relaps. With the preliminary guidelines from 2014, MAS was diagnosed in 4 (13%) patients with sJIA. In two of them it occured like the presenting manifestation of sJIA, and in the rest two it occured during relaps. MAS was not diagnosed with the HLH-2004 guidelines. ----- In conclusion, due to the use of different diagnostic guidelines we found a difference in prevalence of MAS. It was slight higher in comparison to available studies, while other researched features, such as clinical characteristics, were similar. Early recognition of MAS is often challenging as there is no single pathognomonic clinical or laboratory parameter, and is most commonly raised by clinical expert opinion and detection of subtle laboratory alterations. MAS remains a serious condition, which underscores the importance of well-established criteria that aid in making a timely diagnosis before the occurrence of full-blown clinical picture.
Keywords
sindrom aktivacije makrofaga
sustavni juvenilni idiopatski artritis (sJIA)
Keywords (english)
macrophage activation syndrome (MAS)
systemic juvenile idiopathic arthritis (sJIA)
Language croatian URN:NBN urn:nbn:hr:105:010817 Study programme Title: Medicine Type of resource Text File origin Born digital Access conditions Open access Terms of use Created on 2016-03-31 12:23:12
