Common variable immunodeficiency: more pathways than roads to Rome

XP Peng, A Caballero-Oteyza… - Annual Review of …, 2023 - annualreviews.org
Fifty years have elapsed since the term common variable immunodeficiency (CVID) was
introduced to accommodate the many and varied antibody deficiencies being identified in …

Autoimmune cytopenias in common variable immunodeficiency are a diagnostic and therapeutic conundrum: an update

S Chawla, P Barman, R Tyagi, AK Jindal… - Frontiers in …, 2022 - frontiersin.org
Common variable immunodeficiency (CVID) is the most common symptomatic primary
immunodeficiency (PID). CVID is a heterogenous condition and clinical manifestations may …

Beyond infections: new warning signs for inborn errors of immunity in children

G Costagliola, DG Peroni, R Consolini - Frontiers in Pediatrics, 2022 - frontiersin.org
Patients with inborn errors of immunity (IEI) are susceptible to developing a severe infection-
related clinical phenotype, but the clinical consequences of immune dysregulation …

[HTML][HTML] Clinical, immunological, molecular and therapeutic findings in monogenic immune dysregulation diseases: Middle East and North Africa registry

M Jamee, G Azizi, S Baris, E Karakoc-Aydiner… - Clinical …, 2022 - Elsevier
Monogenic immune dysregulation diseases (MIDD) are caused by defective
immunotolerance. This study was designed to increase knowledge on the prevalence and …

Therapeutic modalities and clinical outcomes in a large cohort with LRBA deficiency and CTLA4 insufficiency

N Taghizade, R Babayeva, A Kara, IS Karakus… - Journal of Allergy and …, 2023 - Elsevier
Background LPS-responsive beige-like anchor (LRBA) deficiency (LRBA–/–) and cytotoxic T-
lymphocyte–associated antigen-4 (CTLA4) insufficiency (CTLA4+/–) are mechanistically …

[HTML][HTML] Development of preclinical and clinical models for immune-related adverse events following checkpoint immunotherapy: a perspective from SITC and AACR

NL Bayless, JA Bluestone, S Bucktrout… - … for immunotherapy of …, 2021 - ncbi.nlm.nih.gov
Recent advances in cancer immunotherapy have completely revolutionized cancer
treatment strategies. Nonetheless, the increasing incidence of immune-related adverse …

Lymphadenopathy at the crossroad between immunodeficiency and autoinflammation: An intriguing challenge

G Costagliola, R Consolini - Clinical & Experimental …, 2021 - academic.oup.com
Lymphadenopathies can be part of the clinical spectrum of several primary
immunodeficiencies, including diseases with immune dysregulation and autoinflammatory …

Comparing the levels of CTLA‐4‐dependent biological defects in patients with LRBA deficiency and CTLA‐4 insufficiency

MC Catak, B Akcam, S Bilgic Eltan, R Babayeva… - Allergy, 2022 - Wiley Online Library
Background Lipopolysaccharide‐responsive beige‐like anchor protein (LRBA) deficiency
and cytotoxic T‐lymphocyte protein‐4 (CTLA‐4) insufficiency are recently described …

Paediatric‐onset Evans syndrome: Breaking away from refractory immune thrombocytopenia

N Aladjidi, T Pincez, F Rieux‐Laucat… - British Journal of …, 2023 - Wiley Online Library
Since its first description by Evans in 1951, this syndrome has been linked to chronic
immune thrombocytopenia with the concurrent or delayed onset of autoimmune haemolytic …

Expanding IPEX: inborn errors of regulatory T cells

H Wobma, E Janssen - Rheumatic Disease Clinics, 2023 - rheumatic.theclinics.com
Regulatory T cells (Tregs) play a crucial role in enforcing immune tolerance in the periphery,
preventing the activation of autoreactive cells, production of autoantibodies, and …