Spectrum and features of extraintestinal manifestations of inflammatory bowel disease in children at the stage of disease onset: a retrospective single-center study

Objective. The purpose of the study is to determine the spectrum and frequency of extra-intestinal manifestations in children with inflammatory bowel disease. Patients and methods. A retrospective single-center study included 165 patients with inflammatory bowel disease – 92 with Crohn's disease (CD) and 73 with ulcerative colitis (UC). The diagnosis of IBD was established according to Porto criteria ESPGHAN 2014. Groups were comparable by sex and age. The study evaluated clinical and laboratory activity in both nosologies. Extra-intestinal manifestations (EIM's), their spectrum, frequency and timing relative to intestinal symptoms were assessed. Results. Clinical activity was more frequent in CD and less frequent in UC. Diarrhea (p < 0.001), blood impurities in stool (p = 0.047), and nocturnal bowel movement episodes (p < 0.001) were significantly more common in patients with CD. Nonspecific symptoms were more typical of CD: subfebrile fever, and weight loss. A high frequency of EIM's was found in the early stages of IBD – 55.2% of patients had them. The most common type of EIM's was joint syndrome, which was found in 41.8% of patients, and was more likely to be associated with more moderate clinical activity in those with peripheral arthritis (Me = 21.3 (IQR 15–34.4) vs. Me = 27.5 (IQR 20–40); p = 0.034). Skin manifestations accounted for only 15.2%, which is a lower percentage than in other studies, but aphthous stomatitis was more common in those with CD (14.1% vs. 2.7%, p = 0.011). Erythema nodosum was significantly more common before and simultaneously with the appearance of intestinal manifestations (2.4%). Later, the appearance of nodular erythema became less common (1.2%), (p = 0.026). Primary sclerosing cholangitis (PSC) was detected in 13.7% of patients with UC and in 3.3% of those with CD (p = 0.013). Most often, PSC was detected simultaneously with intestinal manifestations (4.3%), (p < 0.001). Conclusion. The results obtained reflect the high frequency of extra-intestinal manifestations of IBD at the stage of the onset of the disease. Timely assessment of extra-intestinal manifestations as a possibility for the onset of IBD should be carried out by gastroenterologists together with other specialists, especially rheumatologists, since they may be the first to encounter systemic manifestations of inflammatory bowel diseases. Key words: inflammatory bowel diseases, Crohn's disease, ulcerative colitis, children, extra-intestinal manifestations