Clinical outcome of infantile-onset inflammatory bowel disease in 102 patients with interleukin-10 signalling deficiency.

Abstract

BACKGROUND:

Infantile-onset inflammatory bowel disease can be caused by defects in interleukin-10 signalling. The natural history and clinical outcomes of allogeneic haematopoietic stem cell transplantation, medical treatment and surgery have not been thoroughly described.

AIMS:

This study evaluates disease progression and clinical outcome in patients with interleukin-10 signalling deficiency.

METHODS:

One hundred and nine patients with interleukin-10 signalling deficiency were retrospectively reviewed from a single tertiary centre. The Kaplan-Meier method was applied to calculate probabilities of survival and interval between transplant and stoma closure.

RESULTS:

One hundred and nine patients were reviewed, and 102 patients were included in the survival analysis. One hundred and eight patients were identified with IL10RA mutations, and one patient harboured IL10RB mutation. Seventy-three patients received haematopoietic stem cell transplantation. The overall survival after transplantation was 64.2% (95% confidence interval, 52.8 to 75.6), and without transplantation, it was 47.5% (95% confidence interval, 14.8 to 80.2, P = 0.47). The median timeframe between transplant and stoma closure was 19.6 months. The probability of survival was significantly lower in patients with perforation (P < 0.001), ileus (P = 0.038) and without thalidomide treatment (P < 0.001) among patients who did not receive haematopoietic stem cell transplantation. The survival probability was not associated with timeframe between transplant and onset, graft source and genotypes.

CONCLUSIONS:

The survival probability was not significantly different between patients with transplantation and the non-transplanted patients.

• Authors: Ziqing Ye, Lai Qian, Wenhui Hu, Shijian Miao, Yuhuan Wang, Junping Lu, Ying Zhou, Xiaolan Lu, Ye Zhang, Cuifang Zheng, Hua Sun, Wenjuan Tang, Zifei Tang, Song Sun, Kuiran Dong, Xiaowen Qian, Xiaowen Zhai, Ying Huang
• Journal: Alimentary pharmacology & therapeutics (Aliment Pharmacol Ther) Vol. 55 Issue 11 Pg. 1414-1422 (06 2022) ISSN: 1365-2036 [Electronic] England
• PMID: 35187668 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2022 John Wiley & Sons Ltd.

Chemical References

• Interleukin-10

Topics

• Hematopoietic Stem Cell Transplantation
• Humans
• Inflammatory Bowel Diseases (genetics, surgery)
• Interleukin-10 (genetics)
• Mutation
• Retrospective Studies
• Treatment Outcome