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IgA vaskulitis (IgAV) je sistemski vaskulitis koji zahvaća male krvne žile. IgA vaskulitis je najčešći vaskulitis dječje dobi, u većine slučajeva je blagog tijeka i ne ostavlja nikakve posljedice. Najčešći gastrointestinalni simptomi su abdominalni bolovi tipa kolika, povraćanje, proljev i gastrointestinalno krvarenje koje se može očitovati kao melena, hematemeza i hematokezija. Cilj ovog rada je prikazati gastrointestinalne simptome i usporediti kliničku sliku i laboratorijske nalaze bolesnika sa zahvaćenim gastrointestinalnim sustavom s preostalim bolesnicima s IgAV. U ovo retrospektivno istraživanje uključena su djeca u dobi do 18 godina kojima je u razdoblju od siječnja 2009. do lipnja 2020. godine dijagnosticiran i liječen IgAV u Zavodu za kliničku imunologiju, reumatologiju i alergologiju Klinke za pedijatriju Kliničkog bolničkog centra Zagreb, Referentnom centru za pedijatrijsku i adolescentnu reumatologiju Republike Hrvatske. Od ukupno 216 bolesnika, 94 (43,5%) je imalo gastrointestinalne simptome. Medijan dobi prilikom dijagnoze bio je 6,75 godina s tim da je najmlađi bolesnik imao 2,3 godina, a najstariji je imao 16,3 godina. Gastrointestinalne simptome imalo je 59 dječaka i 35 djevojčica. Najčešći gastrointestinalni simptom bila je bol u trbuhu u 45 bolesnika (47,9%), s najčešćom lokalizacijom periumbilikalno (62,5%). Sedamdeset četiri bolesnika (80%) imalo je blaži stadij zahvaćanja gastrointestinalnog sustava u vidu bolova u trbuhu sa ili bez prisutnog okultnog krvarenja. Trideset četiri bolesnika (36,2%) je imalo gastrointestinalne simptome prije pojave osipa, a u 54 bolesnika (57,4%) pronađena je infekcija koja je prethodila bolesti. Ultrazvučni nalaz je u 62,8% slučajeva bio uredan, a najčešća vidljiva promjena bila je mezenterijski limfadenitis (22,3%) i zadebljana stijenka crijeva uz edem (14,9%). Jedan bolesnik imao vidljivu intususcepciju. Bolesnici su najčešće liječeni kortikosteroidima (79%), zatim nesteroidnim protuupalnim lijekovima (40%) i imunosupresivima (8,5%). Dvadeset bolesnika (21,3%) imalo jednu ponovljenu epizodu IgA vaskulitisa., IgA vasculitis is a systemic vasculitis which affects small vessels. IgA vasculitis is the most common vasculitis in children and in most cases the disease is mild and does not have long term consequences. Gastrointestinal symptoms are colicky abdominal pain, vomiting, diarrhea and gastrointestinal bleeding like melena, hematemesis. and hematochezia. The purpose of this study is to present and compare clinical presentation and laboratory results of patients with gastrointestinal symptoms and the one without gastrointestinal symptoms in the same period. This retrospective study presents children under the age of 18 who are diagnosed and treated at the Department of Pediatrics, Division of Clinical Immunology, Rheumatology and Allergology, University Hospital Centre Zagreb, Centre of Reference for Pediatric and Adolescent Rheumatology of the Ministry of Health of the Republic of Croatia, in the period from 2009. to 2020. The total number of patients was 216 and 94 (43.5%) of them presented with gastrointestinal symptoms. There were 59 boys and 35 girls with ages ranging from 2.3 to 16.3 years (median 6.75 years). The most common gastrointestinal presentation was abdominal pain in 45 patients (47.9%), located periumbilically (62.5%). Eighty percent of our patients had mild disease like abdominal pain with or without occult hemorrhage. Gastrointestinal symptoms occurred before the manifestation of the rash in 34 patients (36.2%) and 54 patients (57.4%) had infection before IgA vasculitis onset. In 62.8% of patients ultrasound was normal, while most common findings were mesenteric lymphadenitis (22.3%) and intestinal mucosal oedema (14.9%). One patient had visible intussusception. Patients were treated with corticosteroids in 79% of cases, then with non-steroid anti-inflammatory drugs in 40% of cases and 8.5% patients were treated with immunosuppressants. Only 20 patients (21.3%) had recurrent episode of IgA vasculitis.

Henoch Schönleinova purpura (HSP) je sistemski IgA vaskulitis. Iako je većinom blaga i izlječiva dječja bolest, komplikacije i morbiditet do kojih može doći isključivo su povezane sa zahvaćanjem bubrega. Prema EULAR/PRINTO/PRES kriterijima, bubrežna bolest u HSP se može očitovati kao proteinurija, hematurija ili patohistološki kao nefritis s predominantno IgA depozitima u mezangiju glomerula. Cilj ovog rada je upravo istražiti vrstu bubrežne komplikacije i način liječenja u bolesnika kojima je dijagnosticirana HSP te primijeniti postojeću Oxfordsku i Haasovu klasifikaciju u onih pacijenata koji su imali biopsiju bubrega. U naše istraživanje uključena su sva djeca s HSP koja su u razdoblju od 2006. do 2016. godine razvila bubrežnu bolest i koja su liječena u KBC Zagreb na Klinici za pedijatriju u Zavodu za kliničku imunologiju i reumatologiju. Od 166 pacijenata s dijagnosticiranom HSP kroz navedeno razdoblje, njih 28 (16,8%) odgovara EULAR/PRINTO/PRES kriterijima za bubrežnu bolest. Prosječna dob prilikom dijagnoze HSP je bila 6.8±3.35, a bubrežna bolest je bila zastupljena u 14 djevojčica i 14 dječaka. Najveći broj pacijenata je imao hematuriju (53,6%), potom hematuriju i proteinuriju (39,3%), a najmanje samo proteinuriju (7,1%). Biopsija bubrega je napravljena u 9 pacijenata (32,1%), od čega su dvije trećine bili pacijenti s hematurijom i proteinurijom. Prema nalazima biopsije, 4 pacijenta odgovaraju stupnju III klasifikacije prema Haasu, 3 odgovara stupnju I, a 2 pacijenata stupnju II Haasove klasifikacije. 21 pacijent je liječen kortikosteroidima (75%), 13 NSAID (46,4%), 6 ACE inhibitorima (21,4%), a 5 pacijenata imunosupresivnim lijekovima (17,8%). U usporedbi s drugim literaturnim podacima, ovo istraživanje ima slične rezultate učestalosti bubrežnih komplikacija, nalaza biopsija bubrega i liječenja imunosupresivnim lijekovima., Henoch Schönlein purpura (HSP) is an IgA, systemic vasculitis. Although HSP is mostly a benign and curable children's disease, complications and morbidity that can occur are exclusively related to renal damage. According to EULAR/PRINTO/PRES criteria for HSP, kidney disease can manifest as proteinuria, hematuria, or patohistologically as nephritis with predominantly IgA deposits in the glomerular mesangium. The aim of this study is to investigate the types of renal complications and medical treatment in patients with diagnosed HSP and to apply the existing Oxford and Haas classifications in patients with renal biopsy. In our study are include children with HSP who are developed renal complications during the period from 2006 to 2016 and are treated at the Department of Pediatrics, Division of Clinical Immunology and Rheumatology, University Hospital Center Zagreb. Out of 166 patients with HSP, there were 28 with EULAR/PRINTO/PRES criteria for kidney damage. The mean age at disease onset was 6.8 ± 3.35 and renal complications were present in 14 girls and 14 boys. The largest number of patient had hematuria (53,6%), then hematuria and proteinuria (39,3%), and the smallest had only proteinuria (7,1%). Renal biopsy was preformed in 9 patients (32,1%), of which two thirds were patients with both hematuria and proteinuria. According to biopsy findings, 4 patients had subclass III according to Haas, 3 had subclass I according to Haas and 2 had subclass II according to Haas. 21 patient were managed with corticosteroids (75%), 13 with NSAIDs (46,4%), 6 with ACEi (21,4%) and 5 patients were on immunomodulators (17,8%). To conclude, in comparison to available studies, this study has similar results in types of renal complications, in kidney biopsies and in immunomodulator managment.