Very Early-Onset Inflammatory Bowel Disease (VEO-IBD) Presenting with Recurrent Leukocytoclastic Vasculitis Preceded by Streptococcal Pharyngitis

Inflammatory bowel disease (IBD) that presents in children <6 years of age is known as very early-onset IBD (VEO-IBD). Extraintestinal manifestations in IBD, such as erythema nodosum (EN), pyoderma gangrenosum (PG), and, less likely, leukocytoclastic vasculitis (LV), are more commonly present in Crohn’s disease. Association between LV and ulcerative colitis (UC) is not commonly seen. We report a case of a 6-year-old female with a VEO-IBD UC phenotype presenting with multiple episodes of leukocytoclastic vasculitis, each preceded by streptococcal pharyngitis. Prior to the diagnosis of VEO-IBD, a skin biopsy was obtained and had shown leukocytoclastic vasculitis with a negative IgA stain. Initial laboratory results were remarkable for leukocytosis and increased anti-strep O and anti-DNase B titers. Gastrointestinal panel PCR demonstrated Clostridium difficile toxin A/B. Treatment for LV consisted of methylprednisolone IV 20 mg for four days with a weaning schedule of prednisolone for two weeks and naproxen 250 mg BID for three days. Clostridium difficile was treated with metronidazole 250 mg TID for ten days. She remained stable for three years until she presented with continuous bloody stools, newly onset chest pain, and shortness of breath. Computed tomography angiogram (CTA) was normal. Stool calprotectin was elevated at 658 mcg/gm. Abdominal magnetic resonance enterography (MRE), esophagogastroduodenoscopy, and colonoscopy confirmed a VEO-IBD ulcerative colitis phenotype. She was started on infliximab 10 mg/kg every four weeks after infliximab titers, and antibodies were obtained. Currently, the patient remains on clinical and biochemical remission, with no recent LV episodes or recurrence of streptococcal pharyngitis. Our patient is unique as no case report has been published with multiple episodes of leukocytoclastic vasculitis in association with a VEO-IBD UC phenotype.

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Natural History of Very Early Onset Inflammatory Bowel Disease in North America: A Retrospective Cohort Study

Inflammatory Bowel Diseases ◽

10.1093/ibd/izaa080 ◽

2020 ◽

Cited By ~ 2

Author(s):

Basavaraj Kerur ◽

Eric I Benchimol ◽

Karoline Fiedler ◽

Marisa Stahl ◽

Jeffrey Hyams ◽

...

Keyword(s):

Ulcerative Colitis ◽

Inflammatory Bowel Disease ◽

Crohn’S Disease ◽

Crohn's Disease ◽

Bowel Disease ◽

Early Onset ◽

Bowel Surgery ◽

Age Of Diagnosis ◽

History Of ◽

Inflammatory Bowel

Abstract Background The incidence of very early onset inflammatory bowel disease (VEOIBD) is increasing, yet the phenotype and natural history of VEOIBD are not well described. Methods We performed a retrospective cohort study of patients diagnosed with VEOIBD (6 years of age and younger) between 2008 and 2013 at 25 North American centers. Eligible patients at each center were randomly selected for chart review. We abstracted data at diagnosis and at 1, 3, and 5 years after diagnosis. We compared the clinical features and outcomes with VEOIBD diagnosed younger than 3 years of age with children diagnosed with VEOIBD at age 3 to 6 years. Results The study population included 269 children (105 [39%] Crohn’s disease, 106 [39%] ulcerative colitis, and 58 [22%] IBD unclassified). The median age of diagnosis was 4.2 years (interquartile range 2.9–5.2). Most (94%) Crohn’s disease patients had inflammatory disease behavior (B1). Isolated colitis (L2) was the most common disease location (70% of children diagnosed younger than 3 years vs 43% of children diagnosed 3 years and older; P = 0.10). By the end of follow-up, stricturing/penetrating occurred in 7 (6.6%) children. The risk of any bowel surgery in Crohn’s disease was 3% by 1 year, 12% by 3 years, and 15% by 5 years and did not differ by age at diagnosis. Most ulcerative colitis patients had pancolitis (57% of children diagnosed younger than 3 years vs 45% of children diagnosed 3 years and older; P = 0.18). The risk of colectomy in ulcerative colitis/IBD unclassified was 0% by 1 year, 3% by 3 years, and 14% by 5 years and did not differ by age of diagnosis. Conclusions Very early onset inflammatory bowel disease has a distinct phenotype with predominantly colonic involvement and infrequent stricturing/penetrating disease. The cumulative risk of bowel surgery in children with VEOIBD was approximately 14%–15% by 5 years. These data can be used to provide anticipatory guidance in this emerging patient population.

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P091 The detection of Clostridium difficile toxin A+B in inflammatory bowel disease on the background of immunosuppressive therapy

Journal of Crohn s and Colitis ◽

10.1016/s1873-9946(12)60111-1 ◽

2012 ◽

Vol 6 ◽

pp. S46

Author(s):

L. Lazebnik ◽

V. Sagynbaeva ◽

O. Knyazev ◽

L. Efremov ◽

I. Ruchkina

Keyword(s):

Inflammatory Bowel Disease ◽

Clostridium Difficile ◽

Immunosuppressive Therapy ◽

Bowel Disease ◽

Toxin A ◽

Clostridium Difficile Toxin ◽

Inflammatory Bowel ◽

Clostridium Difficile Toxin A

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Features of very early-onset inflammatory bowel disease: the experience of the Federal Pediatric Center

Voprosy detskoj dietologii ◽

10.20953/1727-5784-2021-3-5-13 ◽

2021 ◽

Vol 19 (3) ◽

pp. 5-13

Author(s):

P.V. Shumilov ◽

◽

A.E. Shchigoleva ◽

Keyword(s):

Ulcerative Colitis ◽

Inflammatory Bowel Disease ◽

Crohn’S Disease ◽

Crohn's Disease ◽

Primary Immunodeficiency ◽

Bowel Disease ◽

Early Onset ◽

Activity Index ◽

Response To Therapy ◽

Inflammatory Bowel

Objective. To clarify the incidence of monogenic IBD-like diseases and the features of clinical course and response to therapy of major types of inflammatory bowel diseases (IBD) among children under the age of 6 with manifestation of the disease. Patients and methods. The study included 135 children under the age of 6 with manifestation of IBD; in the comparison group, there were 128 children after the age of 6 with manifestation of IBD (97 children with ulcerative colitis (UC) and 31 children with Crohn’s disease (CD)) who were observed for at least 1 year. All children underwent a standard examination, including calprotectin and antineutrophil antibodies testing, determination of activity by the Pediatric Ulcerative Colitis Activity Index (PUCAI) or the Pediatric Crohn’s Disease Activity Index (PCDAI), depending on the nosology. Children with the onset of IBD under 6 years of age underwent a genetic testing using Primary Immunodeficiency Panel by next-generation sequencing. All children were analyzed for efficacy of therapy during catamnestic observation. Results. It was revealed that in the study group the incidence of monogenic IBD-like diseases was 6.7%, of UC – 71.1%, of CD – 22.2%. Major types of IBD with very early onset differed little in their clinical, endoscopic and laboratory features from the forms with manifestation at an older age. In most cases, both CD (57%) and UC (71%) were characterized by low activity. Very earlyonset CD was characterized by isolated localization of the colon (53%, p = 0.037) and a non-stenotic and non-penetrating behaviour of the disease (60% of cases). The leading clinical symptoms were diarrhea (67%) and blood in the stool (63%, p = 0.04). Very early-onset UC was characterized by total lesion of the colon (84%, p = 0.001) and the development of anemia (48%, p = 0.01). Among children with very early-onset UC, the percentage of glucocorticosteroid-dependence and glucocorticosteroid-resistance was high, but anti-TNFα therapy was prescribed late. Conclusion. It is advisable to observe children with very early-onset IBD in federal multidisciplinary clinics, where there is experience in managing patients with this pathology. Key words: inflammatory bowel disease, very early onset, Crohn’s disease, ulcerative colitis, primary immunodeficiency, treatment, children

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Antibiotics for Treatment of Clostridium difficile Infection in Hospitalized Patients with Inflammatory Bowel Disease

Antimicrobial Agents and Chemotherapy ◽

10.1128/aac.02606-13 ◽

2014 ◽

Vol 58 (9) ◽

pp. 5054-5059 ◽

Cited By ~ 30

Author(s):

Henry A. Horton ◽

Seper Dezfoli ◽

Dror Berel ◽

Julianna Hirsch ◽

Andrew Ippoliti ◽

...

Keyword(s):

Ulcerative Colitis ◽

Inflammatory Bowel Disease ◽

Clostridium Difficile ◽

Length Of Stay ◽

Bowel Disease ◽

Standard Of Care ◽

Inclusion Criteria ◽

Content Type ◽

Lengths Of Stay ◽

Inflammatory Bowel

ABSTRACTPatients with inflammatory bowel disease (IBD), namely ulcerative colitis (UC) and Crohn's disease (CD), have worse outcomes withClostridium difficileinfection (CDI), including increased readmissions, colectomy, and death. Oral vancomycin is recommended for the treatment of severe CDI, while metronidazole is the standard of care for nonsevere infection. We aimed to assess treatment outcomes of CDI in IBD. We conducted a retrospective observational study of inpatients with CDI and IBD from January 2006 through December 2010. CDI severity was assessed using published criteria. Outcomes included readmission for CDI within 30 days and 12 weeks, length of stay, colectomy, and death. A total of 114 patients met inclusion criteria (UC, 62; CD, 52). Thirty-day readmissions were more common among UC than CD patients (24.2% versus 9.6%;P= 0.04). Same-admission colectomy occurred in 27.4% of UC patients and 0% of CD patients (P< 0.01). Severe CDI was more common among UC than CD patients (32.2% versus 19.4%;P= 0.12) but not statistically significant. Two patients died from CDI-associated complications (UC, 1; CD, 1). Patients with UC and nonsevere CDI had fewer readmissions and shorter lengths of stay when treated with a vancomycin-containing regimen compared to those treated with metronidazole (30-day readmissions, 31.0% versus 0% [P= 0.04]; length of stay, 13.62 days versus 6.38 days [P= 0.02]). Patients with UC and nonsevere CDI have fewer readmissions and shorter lengths of stay when treated with a vancomycin-containing regimen relative to those treated with metronidazole alone. Patients with ulcerative colitis and CDI should be treated with vancomycin.

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THERAPEUTIC IMPLICATIONS OF CLOSTRIDIUM DIFFICILE TOXIN DURING RELAPSE OF CHRONIC INFLAMMATORY BOWEL DISEASE

The Lancet ◽

10.1016/s0140-6736(80)90939-3 ◽

1980 ◽

Vol 315 (8165) ◽

pp. 381-383 ◽

Cited By ~ 109

Author(s):

J.Thomas Lamont ◽

YvonaM. Trnka ◽

J.Thomas Lamont ◽

YvonaM. Trnka

Keyword(s):

Inflammatory Bowel Disease ◽

Clostridium Difficile ◽

Bowel Disease ◽

Chronic Inflammatory Bowel Disease ◽

Therapeutic Implications ◽

Clostridium Difficile Toxin ◽

Inflammatory Bowel ◽

Chronic Inflammatory Bowel

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Occurrence of Clostridium difficile toxin during the course of inflammatory bowel disease

Gastroenterology ◽

10.1016/0016-5085(81)90128-1 ◽

1981 ◽

Vol 80 (4) ◽

pp. 697-700 ◽

Cited By ~ 78

Author(s):

S. Meyers ◽

L. Mayer ◽

E. Bottone ◽

E. Desmond ◽

H.D. Janowitz

Keyword(s):

Inflammatory Bowel Disease ◽

Clostridium Difficile ◽

Bowel Disease ◽

Clostridium Difficile Toxin ◽

Inflammatory Bowel

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COLORECTAL ADENOCARCINOMA DEVELOPING IN A YOUNG FEMALE WITH OVERLAP SYNDROME AND VERY EARLY ONSET-INFLAMMATORY BOWEL DISEASE: A CASE REPORT

Inflammatory Bowel Diseases ◽

10.1093/ibd/izaa347.036 ◽

2021 ◽

Vol 27 (Supplement_1) ◽

pp. S14-S15

Author(s):

Prashanthi Kandavel ◽

Victoria Shakhin ◽

Mallory Chavannes ◽

Christopher Gayer ◽

Hillel Naon ◽

...

Keyword(s):

Ulcerative Colitis ◽

Inflammatory Bowel Disease ◽

Bowel Disease ◽

Early Onset ◽

Colorectal Adenocarcinoma ◽

Clinical Course ◽

Overlap Syndrome ◽

High Risk Population ◽

Endoscopic Screening ◽

Inflammatory Bowel

Abstract We present a 4-year-old female diagnosed with very-early onset inflammatory bowel disease (VEO-IBD), ulcerative colitis type, and overlap syndrome based on clinical presentation, blood and stool studies, diagnostic endoscopies and liver biopsy. Her clinical course was complicated by pervasive psychological stressors, non-compliance with medical therapy, and frequent provider changes. Initial treatment included Prednisone, 6-mercaptopurine and Ursodiol. Treatment was escalated to include multiple biological agents which were stopped prematurely due to non-adherence. She developed weight loss and growth stunting, necessitating exclusive enteral nutrition and then parenteral nutrition. Due to medically refractory disease, she underwent a total colectomy with end ileostomy at the age of 13. Pathology revealed stage III colorectal adenocarcinoma (CRC) with lymph node involvement and transmural inflammation diagnostic of Crohn’s disease (CD). Genetic testing of the resected colon noted a missense mutation of TP53. Our patient completed 6 cycles of extensive and targeted chemotherapy with no evidence of disease reccurrence. She recently underwent ileal pouch-anal anastomosis and creation of a diverting ileostomy. CRC is exceedingly rare in pediatric IBD (PIBD) patients, with less than 30 reported cases in the available literature1. Most cases occurred in males and patients with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC), where the risk of developing CRC is four times greater2. The risk of CRC increases with disease duration, with most PIBD patients developing CRC at 25 years of age1. Interestingly, mutations in TP53, a tumour suppressor gene, have been found to occur more frequently in patients with IBD-CRC compared to those with sporadic-CRC or IBD without dysplasia3. Mutations in TP53 may not only increase a patients general susceptibility to CRC but may also accelerate its development by contributing to excessive inflammation4. Our patient’s treatment refractory clinical course and transition in IBD diagnosis from UC to CD highlights the complexity of VEO-IBD management. While rare in pediatric patients, the development of CRC is a life-threatening risk and it should be considered in patients with prolonged and severely refractory IBD with concomitant autoimmune liver disease. Our patient’s young age at the time of CRC diagnosis emphasizes the need for vigilant yearly endoscopic screening and development of pediatric guidelines for dysplasia monitoring in this high risk population. Further research is needed to evaluate the role of genetic variant screening in CRC risk stratification. References: 1. Aardoom MA, et al. Inflamm Bowel Dis. 2018;24(4):732–741. 2. Soetikno RM, et al. Gastrointest Endosc. 2002;56(1):48–54. 3. Yaeger R, et al. Gastroenterology. 2016;151(2):278–287. 4. Cooks T, et al. Cancer Cell. 2013;23(5):634–46.

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