HENOCH-SCHÖNLEINOV PURPURNI NEFRITIS U DJECE: ISKUSTVA DVAJU TERCIJARNIH CENTARA ZA PEDIJATRIJSKU I ADOLESCENTNU REUMATOLOGIJU REPUBLIKE HRVATSKE U RAZDOBLJU OD 2006. DO 2017. GODINE

Nastasia Čekada, Mario Šestan, Martina Held, Marijan Frković, Saša Sršen, Kristina Vrljičak, Jasna Slaviček, Luka Stričević, Vitomir Metličić, Mateja Batnožić Varga, Marijana Ćorić, Merica Glavina Durdov, Danica Galešić Ljubanović, Marijan Saraga, Danko Milošević, Ivan Malčić, Ma-rija Jelušić

Cilj istraživanja: Valjalo je utvrditi vrste bubrežnih komplikacija, indikacije za biopsiju, načine liječenja te moguće ishode u bolesnika s Henoch-Schönleinovim purpurnim nefritisom (HSPN). Ispitanici i metode: Retrospektivna analiza podataka bolesnika s Henoch-Schönleinovom purpurom (HSP) liječenih u Klinikama za pedijatriju Kliničkoga bolničkog centra Zagreb i Kliničkoga bolničkog centra Split, u razdoblju od 2006. do 2017. godine, u kojih se razvila bubrežna bolest prema kriterijima EULAR/PRINTO/PRES-a. Rezultati: Od 378 bolesnika s HSP-om u 81 (21,4%) razvila se bubrežna bolest, u 43 dječaka (53,1%) i 38 djevojčica (46,9%), s medijanom dobi 8,4 godine (2,5 – 16,8). Uočen je statistički značajan porast broja bolesnika s HSP-om tijekom jeseni i zime. Dob bolesnika pri dijagnozi pokazala se kao mogući prognostički čimbenik bubrežne bolesti. S povišenjem dobi bolesnika veći su izgledi za razvoj bubrežne bolesti, pri čemu se u dobi od 1 do 17 godina sa svakom godinom vjerojatnost razvoja nefritisa povećava u prosjeku oko 2,25%. Jednak broj bolesnika imao je istodobno hematuriju i proteinuriju (44,45%) te izoliranu hematuriju (44,45%), a njih samo 11,1% izoliranu proteinuriju. Biopsija bubrega obavljena je u 37 bolesnika (45,7%), a najviše bioptiranih bolesnika bilo je iz skupine s istodobnom hematurijom i proteinurijom (70,3%). Djeca s izoliranom hematurijom odnosno s izoliranom proteinurijom najčešće su liječena glukokortikoidima, dok su djeca s istodobnom hematurijom i proteinurijom najčešće liječena glukokortikoidima i inhibitorima enzima koji konvertira angiotenzin. Imunosupresivnim lijekovima liječeno je ukupno 16% bolesnika, među kojima je najviše onih iz skupine s istodobnom hematurijom i proteinurijom (69,2%). Samo se u jednog bolesnika (1,2%) razvilo bubrežno zatajenje uz potrebu za primjenom dijalize, dok je ishod ostalih ocijenjen kao dobar. Zaključak: Najvažniji čimbenik konačnog ishoda bolesnika s HSP-om jesu bubrežne komplikacije. Da bi se ovi bolesnici mogli optimalno liječiti, potrebno je donijeti konsenzus o indikacijama za biopsiju bubrega u HSPN-u te validirati postojeće patohistološke klasifikacije radi utvrđivanja koja od njih najbolje korelira s nepovoljnim ishodom bolesti.

Aim: To determine types of renal complications, indications for biopsy, treatment methods and possible outcomes in patients with Henoch-Schönlein purpura nephritis. Patients and methods: Retrospective analysis of medical data of HSP patients treated at the Department of Paediatrics, University Hospital Centre Zagreb, and the Department of Paediatrics, University Hospital Centre Split, during the period from 2006 to 2017, who developed kidney disease according to EULAR/PRINTO/PRES criteria. Results: Out of 378 patients diagnosed with HSP, 81 (21.4%) developed kidney disease, out of whom 43 boys (53.1%) and 38 girls (46.9%), with the median age 8.4 years (2.5–16.8).. A statistically significant increase of patients with HSP was during autumn and winter. There is a greater chance of nephritis with the increase of patients age at diagnosis. For every year (from 1 to 17 years) the chances increase by around 2.25%. Equal number of patients, 44.45%, had both hematuria and proteinuria or isolated hematuria, and only 11.1% isolated proteinuria. Renal biopsy was performed in 37 patients (45.7%), and most biopted patients were from concurrent hematuria and proteinuria group (70.3%). Children with isolated hematuria or with isolated proteinuria were most often treated with corticosteroids while children with hematuria and proteinuria were most often treated with corticosteroids and angiotensin-converting enzyme inhibitors. Immunosuppressives were used in 16% of the patients, including most of those in the group with concurrent hematuria and proteinuria (69.2%). Only one patient (1.2%) developed renal insufficiency with the need for dialysis, while the outcome of the others was evaluated as good. Conclusion: Renal complications are the most important factor in the outcome of patients with HSP. For the optimal treatment of these patients it is necessary to bring consensus about the indications for kidney biopsy in HSP, and also to validate the existing pathohistological classification to affirm which one best correlates with the adverse outcome of the disease.