JUVENILE SPONDYLOARTHRITIS

Juvenilni spondiloartritis (jSpA) skupina je multifaktorskih bolesti u kojima dolazi do poremećenog međudjelovanja imunosnog sustava i čimbenika okoliša u ljudi s predisponirajućim genotipom, što dovodi do upale i strukturnih oštećenja ciljnog tkiva. Prvi simptomi jSpA rijetko su povezani s kralježnicom, a češće se javljaju nesimetrični oligoartritis zglobova donjih ekstremiteta, daktilitis i periferni entezitis. Postoje brojni kriteriji za klasifi kaciju jSpA, no većina pedijatrijskih reumatologa danas se koristi kriterijima Međunarodne lige reumatoloških udruženja (ILAR) prema kojima je najveći broj bolesnika s artritisom i entezitisom ili artritisom ili entezitisom uz još dvije ili više od karakteristika poput bolnosti sakroilijakalnih zglobova na dodir i/ili bolnosti kralježnice zbog upalnog procesa, HLA-B27-genotipa, bolesti povezane s HLA-B27-genotipom u jednog ili više rođaka u prvom ili drugom koljenu, uveitisa te muškog spola uz više od osam godina života, svrstan u podskupinu juvenilnog idiopatskog artritisa (JIA) koju nazivamo artritis pridružen entezitisu (ErA). Sukladno tomu, dijagnoza bolesti postavlja se uglavnom na temelju kliničke slike i anamnestičkih podataka; od laboratorijskih pretraga potrebno je odrediti antinuklearna antitijela (ANA), reumatoidni faktor (RF) te HLA-tipizaciju kako bi se utvrdila prisutnost HLA-B27, B7 i/ili DR4-genotipa. S obzirom na to da velik broj bolesnika ima i supkliničku upalu crijeva, preporučljivo je provjeriti i fekalni kalprotektin. Ako postoje simptomi perifernog entezitisa, potreban je pregled muskuloskeletnim ultrazvukom s osnaženim doplerom (PDUS), a pri znakovima zahvaćenosti kralježnice radiografsko snimanje te magnetska rezonancija (MR) s kontrastnim sredstvom. Najveći broj djece s jSpA-om liječi se fi zikalnom terapijom i nesteroidnim protuupalnim lijekovima (NSAIL), dok se refraktorni oblici periferne bolesti mogu liječiti sintetskim lijekovima koji utječu na tijek bolesti (DMARD), poput sulfasalazina. Kada bolest zahvati i kralježnicu, potrebno je liječenje biološkim DMARD-ovima poput adalimumaba, infl iksimaba i etanercepta. Usprkos mnogimJuvenile spondyloartrhritis is a group of multifactorial diseases in which a disturbed interplay occurs between the immune system and environmental factors on a predisposing genetic background, which leads to infl ammation and structural damage of the target tissue. First symptoms of jSpA rarely involve the spine, while asymmetrical oligoarthritis of lower extremities, dactylitis, and peripheral enthesitis are much more common. Th ere are many classifi cation criteria for jSpA, but the majority of pediatric rheumatologists currently use the International League Against Rheumatism (ILAR) criteria according to which most patients with jSpA are classifi ed into the enthesitis-related arthritis group of juvenile idiopathic arthritis. To meet these criteria, a patient should have arthritis and/or enthesitis, with two or more symptoms such as sacroiliac joint tenderness and/or infl ammatory back pain, HLAB27 genotype, HLA B27 genotype-associated disease in a fi rst- or second-degree relative, uveitis, and male sex with eight or more years of age. Th erefore, diagnosis is most oft en made only based on clinical examination and medical history. Antinuclear antibodies (ANA), rheumatoid factor (RF), and HLA testing with B27, B7, and DR4 alleles are preferred. Since subclinical gut infl ammation is present in many patients, it is recommended to check fecal calprotectin levels. In patients with signs of peripheral enthesitis it is warranted to perform power Doppler musculoskeletal ultrasound (PDUS), and in patients with signs of axial involvement radiographic and contrast-enhanced magnetic resonance imaging. Most patients are treated with nonsteroidal anti-infl ammatory drugs (NSAIDs) and physical therapy, while in refractory cases with peripheral disease synthetic disease- modifying antirheumatic drugs (DMARDs), such as sulfasalazine, are used. In patients with axial involvement, biological DMARDs such as adalimumab, infl iximab, and etanercept are obligatory. Although a number of studies gave us a good insight into the disease pathogenesis, the response to treatment and prognosis are still diffi cult to predict