Abatacept alleviates severe autoimmune symptoms in a patient carrying a de novo variant in CTLA-4 Sangmoon Lee, MD, Jin Soo Moon, MD, PhD, Cho-Rong Lee,

Slides:

Advertisements

Similar presentations

Endoplasmic reticulum stress influences bronchial asthma pathogenesis by modulating nuclear factor κB activation So Ri Kim, MD, PhD, Dong Im Kim, MS, Mi.

Advertisements

Clinical Significance of IGFBP-3 Methylation in Patients with Early Stage Gastric Cancer Seung Tae Kim, Hye-Lim Jang, Jeeyun Lee, Se Hoon Park, Young.

Interaction between IL13 genotype and environmental factors in the risk for allergic rhinitis in Korean children Woo Kyung Kim, MD, PhD, Ji-Won Kwon,

Defective natural killer–cell cytotoxic activity in NFKB2-mutated CVID-like disease Vassilios Lougaris, MD, Giovanna Tabellini, PhD, Massimiliano Vitali,

Vitamin D supplementation during pregnancy: Effect on the neonatal immune system in a randomized controlled trial Eve Hornsby, PhD, Paul E. Pfeffer,

Kyoung-Bok Min, MD, PhD, Jin-Young Min, PhD

Human circulating group 2 innate lymphoid cells can express CD154 and promote IgE production Laura Maggi, PhD, Gianni Montaini, BSc, Alessio Mazzoni,

Hyoung Yoon Chang, MD, PhD, MPH, Dong In Suh, MD, Song-I

Abatacept alleviates severe autoimmune symptoms in a patient carrying a de novo variant in CTLA-4 Sangmoon Lee, MD, Jin Soo Moon, MD, PhD, Cho-Rong Lee,

Santa Jeremy Ono, BA, PhD, Mark B. Abelson, MD

CD4+ T cells in patients with chronic inflammatory rheumatic disorders show distinct levels of exhaustion Theresa Frenz, PhD, Elena Grabski, PhD, Daniela.

Distinct TLR-mediated pathways regulate house dust mite–induced allergic disease in the upper and lower airways Ji-Hwan Ryu, PhD, Jung-Yeon Yoo, BS,

Bruton tyrosine kinase mediates TLR9-dependent human dendritic cell activation Vassilios Lougaris, MD, Manuela Baronio, PhD, Massimiliano Vitali, PhD,

FOXP3+ CD4 T-cell maturity and responses to microbial stimulation alter with age and associate with early-life gut colonization Sophia Björkander, Maria.

Increased blood levels of NKG2D+CD4+ T cells in patients with alopecia areata Yong Hyun Jang, MD, PhD, Jin Kyeong Choi, PhD, Yun Hwan Jang, MD, Sun Young.

Low vitamin D levels are associated with atopic dermatitis, but not allergic rhinitis, asthma, or IgE sensitization, in the adult Korean population Hui.

Pre-Engraftment Syndrome after Unrelated Cord Blood Transplantation: A Predictor of Engraftment and Acute Graft-versus-Host Disease Meerim Park, Soo.

Chronic rhinosinusitis with polyps and without polyps is associated with increased expression of suppressors of cytokine signaling 1 and 3 Se Jin Park,

IL-2 consumption by highly activated CD8 T cells induces regulatory T-cell dysfunction in patients with hemophagocytic lymphohistiocytosis Stéphanie.

Serum ferritin and transferrin levels as serologic markers of methylene diphenyl diisocyanate–induced occupational asthma Gyu-Young Hur, MD, PhD, Gil-Soon.

Heleen D. de Koning, MD, Joost Schalkwijk, PhD, Jos W. M

Expression of 11β-hydroxysteroid dehydrogenase 1 and 2 in patients with chronic rhinosinusitis and their possible contribution to local glucocorticoid.

Tofacitinib relieves symptoms of stimulator of interferon genes (STING)–associated vasculopathy with onset in infancy caused by 2 de novo variants in.

Cysteinyl leukotriene E4 activates human group 2 innate lymphoid cells and enhances the effect of prostaglandin D2 and epithelial cytokines Maryam Salimi,

Human dendritic cell subset 4 (DC4) correlates to a subset of CD14dim/−CD16++ monocytes Federica Calzetti, BS, Nicola Tamassia, PhD, Alessandra Micheletti,

Late Referral to Palliative Care Services in Korea

Lieuwe D. Bos, MSc, PhD, Peter J. Sterk, MD, PhD, Stephen J

Claudin-1 polymorphism modifies the effect of mold exposure on the development of atopic dermatitis and production of IgE Ho-Sung Yu, MS, Mi-Jin Kang,

Programmed cell death ligand 1 alleviates psoriatic inflammation by suppressing IL-17A production from programmed cell death 1–high T cells Jong Hoon.

Blocking Activator Protein 1 Activity in Donor Cells Reduces Severity of Acute Graft- Versus-Host Disease through Reciprocal Regulation of IL-17–Producing.

Kathleen R. Bartemes, BA, Gail M. Kephart, BS, Stephanie J

Differential expression of functional chemokine receptors on human blood and lung group 2 innate lymphoid cells Cathryn A. Weston, PhD, Batika M.J. Rana,

Cross-talk between human mast cells and epithelial cells by IgE-mediated periostin production in eosinophilic nasal polyps Dae Woo Kim, MD, PhD, Marianna.

Mark Gorelik, MD, Satya D. Narisety, MD, Anthony L

Bcl2-like protein 12 plays a critical role in development of airway allergy through inducing aberrant TH2 polarization Zhi-Qiang Liu, MD, PhD, Ying Feng,

Katherine G. MacDonald, BSc, Nicholas A. J

Fibronectin is a TH1-specific molecule in human subjects

Benjamin T. Prince, MD, Msci, Ashley L. Devonshire, MD, MPH, Kristin A

Brent E. Palmer, PhD, Douglas G. Mack, PhD, Allison K

Thymic stromal lymphopoietin–activated invariant natural killer T cells trigger an innate allergic immune response in atopic dermatitis Wen Hao Wu, PhD,

Interaction between IL13 genotype and environmental factors in the risk for allergic rhinitis in Korean children Woo Kyung Kim, MD, PhD, Ji-Won Kwon,

TNF-α induces the late-phase airway hyperresponsiveness and airway inflammation through cytosolic phospholipase A2 activation Il-Whan Choi, PhD, Sun-Kim,

Early decreases in blood eosinophil levels with reslizumab

Fibronectin is a TH1-specific molecule in human subjects

Association between Genetic Variants in Pre-MicroRNAs and Survival of Early-Stage NSCLC Mi Jeong Hong, MS, Yi Young Choi, MS, Ji-Ae Jang, MS, Hye-Jin.

The relative contribution of IL-4 and IL-13 to human IgE synthesis induced by activated CD4+ or CD8+ T cells Juha Punnonen, MD, PhD, Hans Yssel, PhD,

Baricitinib treatment in a patient with a gain-of-function mutation in signal transducer and activator of transcription 1 (STAT1) Kornvalee Meesilpavikkai,

Fms-like tyrosine kinase 3 ligand increases a lung DC subset with regulatory properties in allergic airway inflammation Zhifei Shao, MD, Arpita S. Bharadwaj,

Cysteinyl leukotriene upregulates IL-11 expression in allergic airway disease of mice Kyung Sun Lee, PhD, So Ri Kim, MD, PhD, Hee Sun Park, MD, PhD, Seoung.

Josée Lamoureux, PhD, Jana Stankova, PhD, Marek Rola-Pleszczynski, MD

Liam O’Mahony, PhD, Mübeccel Akdis, MD, PhD, Cezmi A. Akdis, MD

Association Between Age at Diagnosis and the Presence of EGFR Mutations in Female Patients with Resected Non-small Cell Lung Cancer Yoon Hee Choi, MD,

Autophagy: Nobel Prize 2016 and allergy and asthma research

Prostaglandin D2 activates group 2 innate lymphoid cells through chemoattractant receptor-homologous molecule expressed on TH2 cells Luzheng Xue, PhD,

Sara Paveglio, PhD, MS, Erin Bennett, MS, Kelly L. Hawley, PhD, Adam P

News Beyond Our Pages Journal of Allergy and Clinical Immunology

Levels of circulating IL-33 and eosinophil cationic protein in patients with hypereosinophilia or pulmonary eosinophilia Hak-Ryul Kim, MD, Chang-Duk.

Reply Journal of Allergy and Clinical Immunology

TH9 immunodeficiency in patients with hyper-IgE syndrome

CD25 deficiency causes an immune dysregulation, polyendocrinopathy, enteropathy, X- linked–like syndrome, and defective IL-10 expression from CD4 lymphocytes

IL-10–producing monocytes differentiate to alternatively activated macrophages and are increased in atopic patients Antje Prasse, MD, Martin Germann,

The Editors' Choice Journal of Allergy and Clinical Immunology

Cytokine modulation of atopic dermatitis filaggrin skin expression

Thymic stromal lymphopoietin does not activate human basophils

A Functional Polymorphism on Chromosome 15q25 Associated with Survival of Early Stage Non–Small-Cell Lung Cancer Guang Jin, MD, PhD, Eun Young Bae, BS,

IgG4 production is confined to human IL-10–producing regulatory B cells that suppress antigen-specific immune responses Willem van de Veen, MSc, Barbara.

News & Notes Journal of Allergy and Clinical Immunology

News Beyond Our Pages Journal of Allergy and Clinical Immunology

Perturbations of natural killer cell regulatory functions in respiratory allergic diseases Francesca Scordamaglia, MD, Mirna Balsamo, PhD, Antonio Scordamaglia,

Der p 1–induced CD4+FOXP3+GATA3+ T cells have suppressive properties and contribute to the polarization of the TH2-associated response Lieke Reubsaet,

Presentation transcript:

Abatacept alleviates severe autoimmune symptoms in a patient carrying a de novo variant in CTLA-4 Sangmoon Lee, MD, Jin Soo Moon, MD, PhD, Cho-Rong Lee, MS, Hye-Eun Kim, PhD, Sun-Mi Baek, BS, Solha Hwang, MS, Gyeong Hoon Kang, MD, PhD, Jeong Kee Seo, MD, PhD, Choong Ho Shin, MD, PhD, Hyoung Jin Kang, MD, PhD, Jae Sung Ko, MD, PhD, Sung Gyoo Park, PhD, Murim Choi, PhD Journal of Allergy and Clinical Immunology Volume 137, Issue 1, Pages 327-330 (January 2016) DOI: 10.1016/j.jaci.2015.08.036 Copyright © 2015 The Authors Terms and Conditions

Fig 1 De novo CTLA-4 mutation in early-onset multiorgan autoimmune disease. A and B, Disease course and pedigree of the patient. C, The evolutionary conservation of the mutated reside. D, Structure of CTLA-4 homodimer (right)5 and interaction with CD80 (left).6 E, Overexpressed CTLA4P138T lost the ability to suppress IL-2 production in Jurkat cells. A.m., Alligator mississippiensis; B.t., Bos taurus; D.r., Danio rerio; G.g., Gallus gallus; H.s., Homo sapiens; M.m., Mus musculus; O.c., Oryctolagus cuniculus; X.l., Xenopus laevis. ∗∗P < .005. Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig 2 CTLA-4-Ig agent abatacept controls immune reactivity of the patient. A, Treg-cell (CD4+CD25+FOXP3+) population in healthy donors (HD) and the patient. B, CTLA-4 expression level in CD4+CD25+FOXP3+ cells. C, Quantitative RT-PCR of Treg-cell markers from the patient-isolated Treg cells. D, Concentration of cytokines from patient-derived PBMC culture supernatant. E and F, Patient blood levels of hemoglobin and platelet (gray and smoothened black lines) plotted against RBC and platelet transfusion rates (gray bars). Period and actual administration of abatacept treatment are depicted as a black line with dots. MFI, Mean fluorescence intensity. ∗P < .05 and ∗∗P < .005. Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig E1 Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig E2 Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig E3 Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig E4 Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig E5 Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig E6 Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig E7 Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig E8 Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig E9 Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig E10 Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig E11 Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions

Fig E12 Journal of Allergy and Clinical Immunology 2016 137, 327-330DOI: (10.1016/j.jaci.2015.08.036) Copyright © 2015 The Authors Terms and Conditions