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Therapeutic options for CTLA-4 insufficiency
D. Egg, IC. Rump, N. Mitsuiki, J. Rojas-Restrepo, ME. Maccari, C. Schwab, A. Gabrysch, K. Warnatz, S. Goldacker, V. Patiño, D. Wolff, S. Okada, S. Hayakawa, Y. Shikama, K. Kanda, K. Imai, M. Sotomatsu, M. Kuwashima, T. Kamiya, T. Morio, K....
Language English Country United States
Document type Journal Article, Research Support, Non-U.S. Gov't
Grant support
K08 CA219473
NCI NIH HHS - United States
- MeSH
- Agammaglobulinemia etiology MeSH
- CTLA-4 Antigen deficiency genetics MeSH
- Autoimmune Diseases etiology MeSH
- Child MeSH
- Adult MeSH
- Genetic Association Studies MeSH
- Transplantation, Homologous MeSH
- Lung Diseases, Interstitial etiology MeSH
- Infant MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Child, Preschool MeSH
- Aged MeSH
- Immunologic Deficiency Syndromes complications genetics therapy MeSH
- Hematopoietic Stem Cell Transplantation MeSH
- Germ-Line Mutation * MeSH
- Check Tag
- Child MeSH
- Adult MeSH
- Infant MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Child, Preschool MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
BACKGROUND: Heterozygous germline mutations in cytotoxic T lymphocyte-associated antigen-4 (CTLA4) impair the immunomodulatory function of regulatory T cells. Affected individuals are prone to life-threatening autoimmune and lymphoproliferative complications. A number of therapeutic options are currently being used with variable effectiveness. OBJECTIVE: Our aim was to characterize the responsiveness of patients with CTLA-4 insufficiency to specific therapies and provide recommendations for the diagnostic workup and therapy at an organ-specific level. METHODS: Clinical features, laboratory findings, and response to treatment were reviewed retrospectively in an international cohort of 173 carriers of CTLA4 mutation. Patients were followed between 2014 and 2020 for a total of 2624 months from diagnosis. Clinical manifestations were grouped on the basis of organ-specific involvement. Medication use and response were recorded and evaluated. RESULTS: Among the 173 CTLA4 mutation carriers, 123 (71%) had been treated for immune complications. Abatacept, rituximab, sirolimus, and corticosteroids ameliorated disease severity, especially in cases of cytopenias and lymphocytic organ infiltration of the gut, lungs, and central nervous system. Immunoglobulin replacement was effective in prevention of infection. Only 4 of 16 patients (25%) with cytopenia who underwent splenectomy had a sustained clinical response. Cure was achieved with stem cell transplantation in 13 of 18 patients (72%). As a result of the aforementioned methods, organ-specific treatment pathways were developed. CONCLUSION: Systemic immunosuppressants and abatacept may provide partial control but require ongoing administration. Allogeneic hematopoietic stem cell transplantation offers a possible cure for patients with CTLA-4 insufficiency.
Department for Clinical Rheumatology and Immunology Hannover Medical School Hannover Germany
Department of Clinical Immunology and Allergology St Anne's University Hospital Brno Czech Republic
Department of Clinical Immunology and Allergy Royal Melbourne Hospital Melbourne Australia
Department of Clinical Immunology Rigshospitalet University of Copenhagen Copenhagen Denmark
Department of Hematology and Oncology Hyogo Prefectural Kobe Children's Hospital Kobe Japan
Department of Hematology Oncology Gunma Children's Medical Center Shibukawa Japan
Department of Hematology Oslo University Hospital Oslo Norway
Department of Immunology University Hospital Southampton NHSFT Southampton United Kingdom
Department of Immunology University Hospital Zurich University of Zurich Zurich Switzerland
Department of Infectious Diseases and General Internal Medicine University Hospital of Liège
Department of Internal Medicine 3 University Hospital Regensburg Regensburg Germany
Department of Lifetime Clinical Immunology Tokyo Medical and Dental University Tokyo Japan
Department of Pediatric Hematology Oncology University of Duisburg Essen Essen Germany
Department of Pediatrics and Developmental Biology Tokyo Medical and Dental University Tokyo Japan
Department of Pediatrics Hikone Municipal Hospital Shiga Japan
Department of Pediatrics Inselspital Bern University Hospital University of Bern Bern Switzerland
Department of Pediatrics Kiryu Kosei General Hospital Kiryū Japan
Division of Clinical Immunology Montreal Clinical Research Institute Montreal Quebec Canada
Division of Immunology University Children's Hospital Zurich University of Zurich Zurich Switzerland
Division of Infection Immunology and Infection Kanagawa Children's Medical Center Yokohama Japan
Division of Pediatric Hematology Children's Hospital of Orange County Orange Calif
German Center for Infection Research Satellite Center Freiburg Freiburg Germany
Immunology Team American Insurance Montevideo Uruguay
Jena University Hospital Pediatric Gastroenterology Jena Germany
Marmara University School of Medicine Division of Pediatric Allergy and Immunology Istanbul Turkey
Montreal Clinical Research Institute Université de Montréal Montreal Quebec Canada
The Children's Hospital of Philadelphia Perelman School of Medicine University of Pennsylvania
References provided by Crossref.org
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- $a BACKGROUND: Heterozygous germline mutations in cytotoxic T lymphocyte-associated antigen-4 (CTLA4) impair the immunomodulatory function of regulatory T cells. Affected individuals are prone to life-threatening autoimmune and lymphoproliferative complications. A number of therapeutic options are currently being used with variable effectiveness. OBJECTIVE: Our aim was to characterize the responsiveness of patients with CTLA-4 insufficiency to specific therapies and provide recommendations for the diagnostic workup and therapy at an organ-specific level. METHODS: Clinical features, laboratory findings, and response to treatment were reviewed retrospectively in an international cohort of 173 carriers of CTLA4 mutation. Patients were followed between 2014 and 2020 for a total of 2624 months from diagnosis. Clinical manifestations were grouped on the basis of organ-specific involvement. Medication use and response were recorded and evaluated. RESULTS: Among the 173 CTLA4 mutation carriers, 123 (71%) had been treated for immune complications. Abatacept, rituximab, sirolimus, and corticosteroids ameliorated disease severity, especially in cases of cytopenias and lymphocytic organ infiltration of the gut, lungs, and central nervous system. Immunoglobulin replacement was effective in prevention of infection. Only 4 of 16 patients (25%) with cytopenia who underwent splenectomy had a sustained clinical response. Cure was achieved with stem cell transplantation in 13 of 18 patients (72%). As a result of the aforementioned methods, organ-specific treatment pathways were developed. CONCLUSION: Systemic immunosuppressants and abatacept may provide partial control but require ongoing administration. Allogeneic hematopoietic stem cell transplantation offers a possible cure for patients with CTLA-4 insufficiency.
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