Clinical and laboratory features of IgA vasculitis with gastrointestinal involvement: a 12-year experience of the Referral Centre for Paediatric and Adolescent Rheumatology of the Republic of Croatia

Martina Held, Mario Šestan, Nastasia Kifer, Danica Grgurić, Sara Mudri, Marijan Frković, Marija Jelušić

Introduction. IgA vasculitis (IgAV ) is the most common systemic vasculitis in childhood. More than half of IgAV patients experience gastrointestinal involvement which usually occurs in the form of abdominal pain, nausea and vomiting, and up to 5% of patients may develop serious complications such as intussusception, intestinal perforation and / or acute bleeding. The aim of our study was to determine the clinical and laboratory features of gastrointestinal involvement in IgAV . Materials and methods. Retrospective analysis of IgAV patients’ data, who were diagnosed and treated at the Referral Centre for Paediatric and Adolescent Rheumatology of the Ministry of Health of the Republic of Croatia, in the period from 2009 to 2020, and who experienced gastrointestinal involvement. Differences between categorical variables were examined using the χ2 test, and between the numerical ones using the Mann-Whitney
U-test. Results. IgAV was diagnosed in 216 patients, 116 boys and 100 girls. Gastrointestinal involvement was detected in 94 patients (43.5%), the age range at diagnosis was 6.75 (5.2 – 9), and the M : F ratio was 1.68 : 1. The most common clinical sign of gastrointestinal involvement in IgA vasculitis was abdominal pain which occurred in 45 patients (47.9%). Abdominal pain was most often located in the periumbilical region (62.5%). One patient developed ileocolic intussusception. The incidence of generalised purpuric rash (p = 0,023) and nephritis (p = 0,001) was higher in patients with gastrointestinal involvement compared to a group of patients without gastrointestinal involvement. This group of patients had statistically significantly higher values of leukocyte count (p = 0,021) and lower values of erythrocyte sedimentation rate (p = 0,039) and total serum proteins (p = 0,002). In the majority of cases, patients with gastrointestinal involvement were male (p = 0,019), their length of stay (LoS) in the hospital was longer (p < 0,001) and they had a higher frequency of relapses (p = 0,011). Conclusion. In conclusion, gastrointestinal symptoms in IgAV are most often
self-limiting, while complications are rare. We observed that, in most cases, patients with gastrointestinal symptoms were males, with longer length of stay in the hospital, and a higher frequency of nephritis and relapses.

Uvod. IgA vaskulitis (IgAV ) najčešći je sistemski vaskulitis dječje dobi. U više od polovice bolesnika s IgAV -om dolazi do zahvaćanja gastrointestinalnog sustava najčešće u vidu bolova u trbuhu, mučnine i povraćanja, a u do 5% bolesnika mogu se razviti ozbiljne komplikacije poput intususcepcije, perforacije crijeva i/ili akutnog krvarenja. Cilj istraživanja bio je utvrditi kliničke i laboratorijske značajke zahvaćenosti gastrointestinalnog sustava u IgAV -u. Materijali i metode. Retrospektivna analiza podataka bolesnika s IgAV -om, dijagnosticiranih i liječenih u Referentnom centru za pedijatrijsku i adolescentnu reumatologiju Ministarstva zdravstva RH u razdoblju od 2009. do 2020. godine, koji su imali zahvaćen gastrointestinalni sustav. Razlike između kategorijskih varijabla ispitane su pomoću χ2 testa, a one između numeričkih Mann-Whitneyevim U-testom. Rezultati. IgAV je dijagnosticiran u 216 bolesnika, od toga 116 dječaka i 100 djevojčica. Gastrointestinalni sustav bio je zahvaćen u 94 bolesnika (43,5%), raspon dobi u trenutku dijagnoze bio je 6,75 (5,2–9) godina, a omjer M:Ž 1,68:1. Najčešći klinički znak zahvaćanja gastrointestinalnog sustava u IgA vaskulitisu bila je bol u trbuhu koju je imalo 45 bolesnika (47,9%). Bolovi u trbuhu bili su najčešće locirani periumbilikalno (62,5%). Jedan bolesnik razvio je ileokoličnu invaginaciju crijeva. Učestalost generaliziranoga purpuričnog osipa (p=0,023) i pojave nefritisa (p=0,001) bila je veća u bolesnika sa zahvaćenim gastrointestinalnim sustavom u usporedbi sa skupinom bolesnika bez zahvaćenog gastrointestinalnog sustava. Ta skupina bolesnika imala je statistički značajno veći broj leukocita (p=0,021) te niže vrijednosti sedimentacije eritrocita (p=0,039) i ukupnih proteina (p=0,002). Bolesnici sa zahvaćenim gastrointestinalnim sustavom češće su bili muškog spola (p=0,019), imali su dulje trajanje hospitalizacije (p<0,001) i veću učestalost relapsa (p=0,011). Zaključak. Zaključno, gastrointestinalni simptomi
u IgAV -u najčešće su samoograničavajući, a komplikacije rijetke. Uočili smo da su bolesnici s gastrointestinalnim simptomima IgA vaskulitisa češće muškog spola, imaju duže trajanje hospitalizacije te veću pojavu nefritisa i relapsa.