Quantitative analysis of tissue inflammation and responses to treatment in immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome, and review of literature
| Autor: | Wan Chen Chung, Hans D. Ochs, Chih An Chen, Yung Chieh Lin, Yao Jong Yang, Chi Chang Shieh, Yuan Yow Chiou |
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| Rok vydání: | 2016 |
| Předmět: |
Male
0301 basic medicine Pathology Kidney medicine.disease_cause T-Lymphocytes Regulatory Autoimmunity 0302 clinical medicine Immunology and Allergy Enteropathy Intestinal Mucosa Child T helper 2 cells GATA3 FOXP3 Forkhead Transcription Factors Genetic Diseases X-Linked General Medicine Nuclear Receptor Subfamily 1 Group F Member 3 Immunohistochemistry regulatory T cells Infectious Diseases Immune System Diseases 030220 oncology & carcinogenesis Rituximab Immunosuppressive Agents Diarrhea Microbiology (medical) medicine.medical_specialty GATA3 Transcription Factor Biology Tacrolimus 03 medical and health sciences Th2 Cells Immune system immune dysregulation Immunology and Microbiology(all) medicine Humans Immunosuppression Therapy Autoimmune disease General Immunology and Microbiology X-Linked syndrome Th1 Cells Immune dysregulation IPEX syndrome medicine.disease Gastrointestinal Tract Diabetes Mellitus Type 1 030104 developmental biology enteropathy Immunology Th17 Cells polyendocrinopathy T-Box Domain Proteins |
| Zdroj: | Journal of Microbiology, Immunology and Infection. 49(5):775-782 |
| ISSN: | 1684-1182 |
| DOI: | 10.1016/j.jmii.2015.10.015 |
| Popis: | Background/Purpose Immune dysregulation, polyendocrinopathy, enteropathy, X-linked ( IPEX) syndrome is a severe autoimmune disease that is caused by regulatory T cell deficiency due to FOXP3 gene mutations. The long-term outcome can be variable depending on the extent of tissue damage caused by autoimmunity and infections, the use of immunosuppressive treatment or sequela of bone marrow transplantation. Methods We used immunohistochemical staining to analyze cell types infiltrating the tissue of affected organs from a classic IPEX patient with a splicing mutation (c.736-2A>C) in the FOXP3 gene. Expression of transcription factors that are critical for immune responses including T-bet, GATA-3, RORγt, and FOXP3 were evaluated in various tissue samples. For objective analysis of the distribution of different cell types in tissues, we used an automated microscope-based image acquiring system to assess quantitatively the different cell types by investigating the histopathological changes in the patient's biopsy samples obtained from the intestine and the kidneys before and after treatment. Results The percentages of cells expressing the T H 2-associated transcription factor GATA3 were higher in the IPEX patient before treatment than in controls, suggesting that T H 2-type cells contribute to the tissue inflammation of the gut and kidneys in IPEX syndrome. Immunosuppressive treatment effectively decreased the number of effector cells in the kidneys and intestine of the IPEX patient. Conclusion This study provides quantitative evidence that the inflamed intestinal and renal tissues of the IPEX patient contain T H 2-type immune effector cells, which decreased in number after immunosuppressive treatment was initiated and the clinical symptoms had improved. |
| Databáze: | OpenAIRE |
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