Journal article
Autoimmune Enteropathy: An Updated Review with Special Focus on Stem Cell Transplant Therapy
Digestive diseases and sciences, Vol.64(3), pp.643-654
03/01/2019
DOI: 10.1007/s10620-018-5364-1
PMCID: 8260026
PMID: 30415406
Abstract
Autoimmune enteropathy (AIE) is a complex disease affecting both children and adults. Although associated with significant morbidity and mortality, the pathophysiology of the disease and its treatment havenot been well characterized. This study aims to review the medical literature available on this rare but clinically significant ailment, to help establish a better understanding of its pathophysiology and enumerate the available diagnostic and treatment modalities. A literature search was conducted on PubMed using key terms related to autoimmune enteropathy and intractable diarrhea, with no restrictions on the date of publication or language. We found a total of 98 reports of AIE published in the form of case reports and case series. The evidence reviewed suggests that AIE is a multifaceted disorder that requires a high index of suspicion in the appropriate clinical setting to be able to make an early diagnosis. Current evidence supports the use of supportive care to correct nutritional and metabolic deficiencies, and immunosuppressives and immunomodulators as directed therapies. Hematopoietic stem cell transplant is an aggressive, but successful curative modality for patients with AIE as part of immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome. Cumulative clinical experience with management of AIE has allowed improved outcomes in transplanted and non-transplanted AIE patients even though morbidity and mortality with are still high in patients with this condition. More research is needed to further define the role of new therapies for AIE, and a central registry with participation of multiple institutions might help share and standardize care of patients with this rare but serious condition.
Details
- Title: Subtitle
- Autoimmune Enteropathy: An Updated Review with Special Focus on Stem Cell Transplant Therapy
- Creators
- Zunirah Ahmed - Auburn University at MontgomeryAamer Imdad - SUNY Upstate Medical UniversityJames A. Connelly - Vanderbilt University Medical CenterSari Acra - Vanderbilt University Medical Center
- Resource Type
- Journal article
- Publication Details
- Digestive diseases and sciences, Vol.64(3), pp.643-654
- DOI
- 10.1007/s10620-018-5364-1
- PMID
- 30415406
- PMCID
- 8260026
- NLM abbreviation
- Dig Dis Sci
- ISSN
- 0163-2116
- eISSN
- 1573-2568
- Publisher
- Springer Nature
- Number of pages
- 12
- Grant note
- RC2 DK118640 / NIDDK NIH HHS; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Institute of Diabetes & Digestive & Kidney Diseases (NIDDK)
- Language
- English
- Date published
- 03/01/2019
- Academic Unit
- Stead Family Department of Pediatrics; Gastroenterology, Hepatology, Pancreatology, and Nutrition
- Record Identifier
- 9984446428702771
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