VASKULITISI U DJEČJOJ DOBI: RETROSPEKTIVNA STUDIJA U RAZDOBLJU OD 2002. DO 2012. GODINE U KLINICI ZA PEDIJATRIJU KLINIČKOG BOLNIČKOG CENTRA ZAGREB

Marija Jelušić, Lucija Kostić, Marijan Frković, Maša Davidović, Ivan Malčić

The aim of our study was to analyze clinical features, laboratory findings, treatment, course and outcome of different types of vasculitis in children. All children aged up to 18 years that have been diagnosed with a vasculitis disorder from 2002. to 2012. at the Department of Paediatric, University Hospital Centre Zagreb according to EULAR/PRES/PRINTO criteria were included in the study. Vasculitis was diagnosed in 180 children, 101 girls and 79 boys, mean age 7.19±3.7 years, with an average follow-up of 5,58±3,28 years. Most of the children (155 or 86%) were diagnosed with Henoch-Shönlein purpura (HSP), polyarteritis nodosa (PAN) was diagnosed in 6 children (3.3%), isolated cutaneous leukocytoclastic vasculitis in 5 (2.8%), Takayasu arteritis (TA) and Kawasaki disease in 2 (1.1%) respectively, hypocomplementemic urticarial vasculitis in one patient (0.5%) and other types of vasculitis in 10 (5.5%) patients (vasculitides in systemic connective tissue disorders in 7 and unclassifi ed vasculitides in 3 patients). All patients had elevated inflammatory markers (C-reactive protein and erythrocyte sedimentation rate). Anti-neutrophil cytoplasmatic antibodies (ANCA) were positive only in one patient, suff ering from microscopic polyangiitis. Treatment modality in most patients were NSAIDs, while children with kidney or gastrointestinal system affection were treated with glucocorticoids and/or immunosuppresive drugs. Biological therapy (anti-CD20, rituximab) was used in patients with most severe symptoms. One child (0.56%), suffering from microscopic polyangiitis, died due to kidney failure during the followup. Forty patients (22.6%) had one disease relapse, while 6 (3.4%) had two relapses. In conclusion, we found some diff erences in laboratory parameters (e.g. lower incidence of elevated antistreptolysin O titer in HSP) and epidemiological data (e.g. higher prevalence of PAN in female children) in comparison to data from available studies, while other clinical features, laboratory findings, disease outcome and treatment were similar.

Cilj istraživanja bio je analizirati klinička obilježja, laboratorijske nalaze, liječenje, tijek bolesti i ishod pojedinih oblika vaskulitisa u djece. U istraživanje su bili uključeni svi bolesnici u dobi do 18 godine života kojima je u razdoblju od 2002. do 2012. godine u Klinici za pedijatriju KBC-a Zagreb utvrđena dijagnoza vaskulitisa prema kriterijima EULAR/PRES/PRINTO. Vaskulitis je dijagnosticiran u 180 djece, 101 djevojčice i 79 dječaka, u dobi 7,19±3,7 godina. Prosječno vrijeme praćenja bolesnika bilo je 5,58±3,28 godina. Henoch-Schönleinova purpura (HSP) dijagnosticirana je u 155 bolesnika (86 %), nodozni poliarteritis (PAN) u 6 (3,3 %), izolirani kutani leukocitoklastični vaskulitis u 5 (2,8 %), Takayasuov arteritis (TA) u dva (1,1 %), Kawasakijeva bolest (KB) u dva (1,1 %), hipokomplementarni urtikarijski vaskulitis u jednog (0,5 %) te drugi vaskulitisi u 10 (5,5 %) bolesnika (vaskulitisi povezani sa sistemnim bolestima veziva u sedam i neklasifi cirani u tri bolesnika). U svih bolesnika upalni laboratorijski parameteri (C reaktivni protein, sedimentacija eritrocita) bili su povišeni. Antineutrofi lna citoplazmatska protutijela (ANCA) bila su pozitivna u samo jednog bolesnika s mikroskopskim poliarteritisom. Potporne mjere liječenja u obliku nesteroidnih protuupalnih lijekova bili su način liječenja u većine bolesnika, dok su bolesnici sa zahvaćenim bubrežnim i gastrointestinalnim sustavom tretirani glukokortikoidima i/ili imunosupresivima. U bolesnika s najtežim simptomima primijenjena je i biološka terapija (anti-CD20, rituksimab). Tijekom praćenja umrlo je jedno dijete (0,56%) oboljelo od mikroskopskog poliarteritisa radi zatajenja bubrega. Četrdeset bolesnika (22,6%) je imalo jedan relaps, a šest (3,4%) dva relapsa bolesti. Zaključno, primijetili smo neke razlike u naših bolesnika u odnosu na literaturne podatke, poput niže učestalosti povišenih vrijednosti antistreptolizinskog O titra u bolesnika s HSP-om te veće učestalosti PAN-a u ženskog spola, dok se ostala klinička obilježja, laboratorijski nalazi, liječenje i ishod nisu razlikovali.