Abstract | 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. |
Abstract (english) | 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. |