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To the best of our knowledge, no cases of ulcerative colitis (UC) mimicking Henoch-Schönlein purpura (HSP) have been reported so far. During a 28-year period 5635 patients were followed up at our Pediatric Rheumatology Unit and 357 had HSP according to the European League Against Rheumatism, the Paediatric Rheumatology International Trials Organisation and the Paediatric Rheumatology European Society validated classification criteria. At the same period, 148 patients with IBD according to the European Society for Paediatric Gastroenterology, Hepatology and Nutrition criteria were followed up at the Pediatric Gastroenterology Unit in our University Hospital. Only two of them had vasculitis, as an extra intestinal manifestation of UC mimicking HSP, and fulfilled both disease criteria. A 2-year old girl had bloody diarrhoea, severe abdominal pain, arthritis in ankles, petechiae and palpable purpura not related to thrombocytopenia in lower limbs. A 5-year old boy had bloody diarrhoea, palpable purpura in buttocks, lower limbs, penis and scrotum associated with arthritis in knees, orchitis in right testicle and periarticular swelling in hands and feet. Their ileocolonoscopy showed diffuse mucosal erythema, oedema, friability and multiple irregular ulcers, and histopathological examination of colonic specimen revealed diffuse chronic mucosal inflammation, crypt distortion and crypt abscesses suggesting ulcerative colitis. There were no signs of intestinal vasculitis in both cases. In conclusion, this is the first study in a paediatric population that evidenced palpable purpura associated with UC mimicking HSP.

BACKGROUND: Inflammatory bowel diseases (IBD), comprising Crohn’s disease and ulcerative colitis, are chronic inflammatory diseases of the gastrointestinal tract that often have their onset among adolescents and young adults (AYA). IBD are characterized by episodes of active disease interspersed with periods of remission, and its activity is inversely correlated with health-related quality of life (HRQL). OBJECTIVE: This study aimed to determine whether AYA in remission or with low IBD activity would exhibit HRQL similar to that of age-matched healthy individuals, and whether demographic and disease factors could affect HRQL using a ‘patient-reported outcome’ instrument. METHODS: This study enrolled only AYA with IBD, with low activity. This research included five multidisciplinary clinics of two academic hospitals: Paediatric Gastroenterology, Gastroenterology, Coloproctology, Paediatric Rheumatology and Adolescent divisions, São Paulo, Brazil. A total of 59 AYA with IBD (age, 13-25 years) and 60 healthy AYA (age, 13-25 years) completed the Pediatric Quality of Life Inventory 4.0 and 36-Item Short-Form Health Survey questionnaires and the visual analogue scale (VAS) for pain. Demographic data, extra-intestinal manifestations, treatment, and outcomes regarding CD and UC were evaluated. RESULTS: AYA with IBD and healthy controls were similar with respect to median ages (18.63 [13.14-25.80] years vs 20.5 [13.68-25.84] years, P=0.598), proportion of female sex (42% vs 38%, P=0.654), and percentage of upper middle/middle Brazilian socioeconomic classes (94% vs 97%, P=0.596). The school/work score was significantly lower in AYA with IBD than in healthy controls (70 [10-100] vs 75 [5-100], P=0.037). The ‘general health-perception’ score was significantly lower in AYA with IBD than in healthy controls (50 [10-80] vs 0 [25-90], P=0.0002). The median VAS, FACES pain rating scale, and total VAS scores were similar between the two groups (2 [0-10] vs 3 [0-9], P=0.214). No association between HRQL and clinical and demographic parameters was identified among IBD patients. CONCLUSION: AYA with low IBD activity reported poor HRQL in school/work and general health perception domains, which highlights a disability criterion in this vulnerable population.

BACKGROUND: Crohn's disease (CD) is a chronic inflammatory disease that can affect all segments of the gastrointestinal tract. With various phenotypes and progressive course, it can be complicated with fistulas, abscesses or stenosis. Treatment targets includes clinical remission and mucosal healing in order to avoid complicated outcomes. Double balloon enteroscopy (DBE) allows complete evaluation of the small intestine, providing diagnoses and the possibility of interventions such as biopsies, with low complication rates. Some authors suggest that involvement of the proximal small bowel may indicate worse long-term therapeutic outcomes. This study aims to correlate jejunal endoscopic findings observed in baseline DBE with the activity of CD after 10 years of follow-up. METHODS: Twenty paediatric refractory CD patients (2–17 years old) performed DBE by a single experienced specialist after radiological evaluation for stenosis exclusion between 2007 and 2010. After 10 years, the degree of CD activity was assessed by the Harvey-Bradshaw Index (HBI), C-Reactive Protein (CRP) serum dosage, and current therapy through collection of electronic medical records and personal communication. RESULTS: Sixteen 16/20 patients (80%) completed the 10 years follow up period. Two patients (2/16) changed diagnosis (1- monogenic disease, IL-10 and / or IL10R gene mutation; 1 - ulcerative colitis). Previous findings of DBE of the 14 patients corresponded to: 2 active duodenojejunal ulcers, 4 duodenojejunal scar retractions, 1 retraction and pseudopolyps in the proximal ileum, 6 hyperemia and / or edema and / or granularity of the jejunal mucosa, and 1 without alteration. After 10 years, 6/14 patients have abnormal CRP (>5 mg/L), with 3 of them having HBI between 5 and 7, 2 between 8 and 16 and 1 of 4. All of them presented previous jejunal alterations, 5/6 are currently in use of biologic and 3/6 are steroid dependent. The 2 patients who had active ulcers in baseline DBE remain steroid dependent and with higher rates of HBI activity. Eight patients (8/14) have normal CRP, all presenting HBI lower than 5. Of these, 3 are using combined therapy with immunosuppressant and biologic, 1 with biologic monotherapy, 2 with immunosuppressant, and 2 without medication. CONCLUSION(S): Active jejunal disease, observed through DBE, may indicate a more severe phenotype in CD after long-term follow-up, suggesting a distinct phenotype. Evaluation of the proximal small intestine should be considered in refractory paediatric CD patients or those with diagnostic uncertainty.