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References from Advances in the Phenotyping and Management of Common Variable Immunodeficiency. Local targets link to admitted publications; unresolved targets remain external evidence.
Diagnostic criteria for common variable immunodeficiency disorders
10.1016/j.jaip.2016.02.023 · 2016 · External reference
State-of-the-art diagnostic evaluation of common variable immunodeficiency
10.1016/j.anai.2021.03.005 · 2021 · External reference
The European Society for immunodeficiencies (ESID) registry working definitions for the clinical diagnosis of inborn errors of immunity
10.1016/j.jaip.2019.02.004 · 2019 · External reference
International Consensus document (ICON): common variable immunodeficiency disorders
10.1016/j.jaip.2015.07.025 · 2016 · External reference
Update in understanding common variable immunodeficiency disorders (CVIDs) and the management of patients with these conditions
10.1111/j.1365-2141.2009.07669.x · 2009 · External reference
Primary immunodeficiency diseases in Australia and New Zealand
10.1007/s10875-007-9105-z · 2007 · External reference
Global distribution of common variable immunodeficiency (CVID) in the light of the UNDP human development index (HDI): a preliminary perspective of a rare disease
10.1155/2020/8416124 · 2020 · External reference
Morbidity and mortality in common variable immune deficiency over 4 decades
10.1182/blood-2011-09-377945 · 2012 · External reference
Identification and characterization of a nationwide danish adult common variable immunodeficiency cohort
10.1111/sji.12551 · 2017 · External reference
Gastrointestinal manifestations of common variable immunodeficiency: a mentored review
10.1007/s10620-025-09106-8 · 2025 · External reference
A systematic literature review of CVID reveals pervasive detrimental noninfectious manifestations
10.70962/jhi.20250157 · 2026 · External reference
Malignancy in common variable immunodeficiency: a systematic review and meta-analysis
10.1080/1744666x.2019.1658523 · 2019 · External reference
Common variable immunodeficiency disorders: division into distinct clinical phenotypes
10.1182/blood-2007-11-124545 · 2008 · External reference
Common variable immunodeficiency and autoimmune diseases: a 10-year single-center experience
10.46497/archrheumatol.2024.10729 · 2024 · External reference
A cross-sectional study of health-related quality of life in patients with predominantly antibody deficiency
10.1007/s10875-024-01781-y · 2024 · External reference
Health-related quality of life in primary immunodeficiencies: impact of delayed diagnosis and treatment burden
10.1016/j.clim.2022.108931 · 2022 · External reference
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Economic burden of common variable immunodeficiency: annual cost of disease
10.1586/1744666x.2015.1029457 · 2015 · External reference
Common variable immunodeficiency: more pathways than roads to rome
10.1146/annurev-pathmechdis-031521-024229 · 2023 · External reference
Monitoring patients with uncomplicated common variable immunodeficiency: a systematic review
10.1186/s13223-022-00661-7 · 2022 · External reference
Uncovering risk factors of premature mortality in common variable immunodeficiency
10.1016/j.jaip.2025.03.009 · 2025 · External reference
Effectiveness of immunoglobulin replacement therapy on clinical outcome in patients with primary antibody deficiencies: results from a multicenter prospective cohort study
10.1007/s10875-011-9511-0 · 2011 · External reference
Unresolved reference
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10.3389/fimmu.2017.01740
10.3389/fimmu.2017.01740 · External reference
Memory B cells in common variable immunodeficiency: clinical associations and sex differences
10.1016/j.clim.2008.02.013 · 2008 · External reference
T cell phenotypes in patients with common variable immunodeficiency disorders: associations with clinical phenotypes in comparison with other groups with recurrent infections
10.1111/j.1365-2249.2012.04643.x · 2012 · External reference
B-Cell and T-Cell phenotypes in CVID patients correlate with the clinical phenotype of the disease
10.1007/s10875-010-9424-3 · 2010 · External reference
Late-onset combined immune deficiency: a subset of common variable immunodeficiency with severe T cell defect
10.1086/606059 · 2009 · External reference
Circulating CD21low B cells in common variable immunodeficiency resemble tissue homing, innate-like B cells
10.1073/pnas.0901984106 · 2009 · External reference
Loss of B cell tolerance at the T2/T3a B cell transition is a convergent pathogenic mechanism in common variable immunodeficiency
2025 · External reference
Unresolved reference
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Clinical utility of measuring CD4+ T follicular cells in patients with immune dysregulation
10.1016/j.jaut.2023.103088 · 2023 · External reference
A decreased frequency of regulatory T cells in patients with common variable immunodeficiency
10.1371/journal.pone.0006269 · 2009 · External reference
Regulatory T cell dysfunction in subjects with common variable immunodeficiency complicated by autoimmune disease
10.1016/j.clim.2008.12.006 · 2009 · External reference
Functions of tfh cells in common variable immunodeficiency
10.3389/fimmu.2020.00006 · 2020 · External reference
Utility of serum cytokine testing to differentiate complicated common variable immunodeficiency in resource limited settings
2025 · External reference
BAFF-driven B cell hyperplasia underlies lung disease in common variable immunodeficiency
10.1172/jci.insight.122728 · 2019 · External reference
Portal hypertension in common variable immunodeficiency disorders - a single center analysis on clinical and immunological parameter in 196 patients
10.3389/fimmu.2023.1268207 · 2023 · External reference
Targeted proteomics reveals inflammatory pathways that classify immune dysregulation in common variable immunodeficiency
10.1007/s10875-020-00908-1 · 2021 · External reference
Soluble interleukin-2 receptor is a promising serum biomarker for granulomatous disease in common variable immune deficiency
10.1007/s10875-020-00947-8 · 2021 · External reference
Undetectable IgE association with noninfectious complications of common variable immune deficiency
2025 · External reference
Practice parameter for the diagnosis and management of primary immunodeficiency
10.1016/j.jaci.2015.04.049 · 2015 · External reference
Interlaboratory variability in multiplexed pneumococcal antibody testing
10.1016/j.jaci.2018.10.057 · 2019 · External reference
Questioning the accuracy of currently available pneumococcal antibody testing
10.1016/j.jaci.2018.06.013 · 2018 · External reference
Application of the diagnostic criteria for common variable immunodeficiency in resource-limited settings
10.15586/aei.v50i4.496 · 2022 · External reference
Update: vaccines in primary immunodeficiency
10.1016/j.jaci.2017.12.980 · 2018 · External reference
2025 inborn errors of immunity practice parameter
10.1016/j.anai.2025.10.026 · 2026 · External reference
Functional testing of humoral immunity in the prevnar 20 era
10.1016/j.anai.2024.12.011 · 2025 · External reference
Seeking relevant biomarkers in common variable immunodeficiency
10.3389/fimmu.2022.857050 · 2022 · External reference
10.3389/fimmu.2023.1257398
10.3389/fimmu.2023.1257398 · External reference
Current genetic landscape in common variable immune deficiency
10.1182/blood.2019000929 · 2020 · External reference
Establishing the molecular diagnoses in a cohort of 291 patients with predominantly antibody deficiency by targeted next-generation sequencing: experience from a monocentric study
10.3389/fimmu.2021.786516 · 2021 · External reference
Genetics and clinical phenotypes in common variable immunodeficiency
2023 · External reference
Disparities in genetic testing for inborn errors of immunity
10.1016/j.jaip.2024.11.011 · 2025 · External reference
Therapeutic modalities and clinical outcomes in a large cohort with LRBA deficiency and CTLA4 insufficiency
10.1016/j.jaci.2023.08.004 · 2023 · External reference
A randomized, placebo-controlled phase 3 trial of the PI3Kδ inhibitor leniolisib for activated PI3Kδ syndrome
10.1182/blood.2022018546 · 2023 · External reference
Clinical and immunologic phenotype associated with activated phosphoinositide 3-kinase δ syndrome 2: a cohort study
10.1016/j.jaci.2016.03.022 · 2016 · External reference
Clinical spectrum and features of activated phosphoinositide 3-kinase δ syndrome: a large patient cohort study
10.1016/j.jaci.2016.06.021 · 2017 · External reference
Subcutaneous versus intravenous immunoglobulin for chronic autoimmune neuropathies: a meta-analysis
10.1002/mus.25409 · 2017 · External reference
Differentiating characteristics and evaluating intravenous and subcutaneous immunoglobulin
2019 · External reference
Improving current immunoglobulin therapy for patients with primary immunodeficiency: quality of life and views on treatment
10.2147/ppa.s60771 · 2014 · External reference
Vivaglobin study group. Home-based subcutaneous immunoglobulin G replacement therapy under real-life conditions in children and adults with antibody deficiency
10.1186/2047-783x-15-6-238 · 2010 · External reference
Immunoglobulin replacement therapy in patients with primary and secondary immunodeficiencies: impact of infusion method on immunoglobulin-specific perceptions of quality of life and treatment satisfaction
10.1186/s13223-024-00939-y · 2025 · External reference
Intravenous and subcutaneous immunoglobulin G replacement therapy
10.2500/aap.2016.37.3987 · 2016 · External reference
Acute renal failure, translocational hyponatremia and hyperkalemia following intravenous immunoglobulin therapy
10.1159/000104424 · 2007 · External reference
Practical guidance for the diagnosis and management of secondary hypogammaglobulinemia: a work group report of the AAAAI primary immunodeficiency and altered immune response committees
10.1016/j.jaci.2022.01.025 · 2022 · External reference
Impact of IVIG vs. SCIG on IgG trough level and infection incidence in primary immunodeficiency diseases: a systematic review and meta-analysis of clinical studies
10.1016/j.waojou.2019.100068 · 2019 · External reference
Impact of trough IgG on pneumonia incidence in primary immunodeficiency: a meta-analysis of clinical studies
10.1016/j.clim.2010.06.012 · 2010 · External reference
Evaluation of correlation between dose and clinical outcomes in subcutaneous immunoglobulin replacement therapy
10.1111/j.1365-2249.2012.04594.x · 2012 · External reference
Higher doses of subcutaneous IgG reduce resource utilization in patients with primary immunodeficiency
10.1007/s10875-011-9631-6 · 2012 · External reference
Granulomatous lymphocytic Interstitial Lung Disease (GLILD) in common variable immunodeficiency (CVID): a multicenter retrospective study of patients from Italian PID referral centers
10.3389/fimmu.2021.627423 · 2021 · External reference
False positive serum levels of (1-3)-ß-D-Glucan after infusion of intravenous immunoglobulins and time to normalisation
10.1016/j.jinf.2017.10.017 · 2018 · External reference
Transmission of hepatitis B core antibody and galactomannan enzyme immunoassay positivity via immunoglobulin products: a comprehensive analysis
10.1093/cid/ciw222 · 2016 · External reference
Challenge of hepatitis B testing following intravenous immunoglobulin therapy in patients with autoimmune skin diseases
10.1111/1346-8138.16500 · 2022 · External reference
Intravenous immunoglobulin significantly reduces exposure of concomitantly administered anti-C5 monoclonal antibody tesidolumab
10.1111/ajt.15922 · 2020 · External reference
2022 American college of rheumatology guideline for vaccinations in patients with rheumatic and musculoskeletal diseases
10.1002/acr.25045 · 2023 · External reference
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Unresolved reference
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Preventing infections in children and adults with asplenia
10.1182/hematology.2020000117 · 2020 · External reference
Efficacy and safety of rituximab in common variable immunodeficiency-associated immune cytopenias: a retrospective multicentre study on 33 patients
10.1111/j.1365-2141.2011.08880.x · 2011 · External reference
The efficacy and safety of systemic corticosteroids as first line treatment for granulomatous lymphocytic interstitial lung disease
10.1016/j.jaci.2022.12.813 · 2023 · External reference
Rituximab monotherapy is effective as first-line treatment for granulomatous lymphocytic interstitial lung disease (GLILD) in CVID patients
10.1007/s10875-023-01587-4 · 2023 · External reference
Rituximab and antimetabolite treatment of granulomatous and lymphocytic interstitial lung disease in common variable immunodeficiency
10.1016/j.jaci.2020.07.021 · 2021 · External reference
Anti-tumour necrosis factor-alpha therapy for severe enteropathy in patients with common variable immunodeficiency (CVID)
10.1111/j.1365-2249.2007.03481.x · 2007 · External reference
Infliximab therapy for crohn’s-like disease in common variable immunodeficiency complicated by massive intestinal hemorrhage: a case report
10.1186/1756-0500-7-382 · 2014 · External reference
Infliximab for treatment of granulomatous disease in patients with common variable immunodeficiency
10.1007/s10875-014-0079-3 · 2014 · External reference
Autoimmune cytopenias in common variable immunodeficiency are a diagnostic and therapeutic conundrum: an update
10.3389/fimmu.2022.869466 · 2022 · External reference
Treatment of inflammatory complications in common variable immunodeficiency (CVID): current concepts and future perspectives
10.1080/1744666x.2023.2198208 · 2023 · External reference
CVID enteropathy is difficult to treat and shows a heterogeneous histopathology
10.1007/s10875-025-01920-z · 2025 · External reference
Treatment strategies for GLILD in common variable immunodeficiency: a systematic review
10.3389/fimmu.2021.606099 · 2021 · External reference
Mechanism-based strategies for the management of autoimmunity and immune dysregulation in primary immunodeficiencies
10.1016/j.jaip.2016.08.004 · 2016 · External reference
Abatacept alleviates severe autoimmune symptoms in a patient carrying a de novo variant in CTLA-4
10.1016/j.jaci.2015.08.036 · 2016 · External reference
Congenital T cell activation impairs transitional to follicular B cell maturation in humans
2024 · External reference
Abatacept restores dysregulated transcriptomic and proteomic profile in disorders of CTLA-4 insufficiency
10.1016/j.jaci.2025.08.027 · 2025 · External reference
Interim analysis: open-label extension study of leniolisib for patients with APDS
10.1016/j.jaci.2023.09.032 · 2024 · External reference
Hepatic manifestations in common variable immunodeficiency associated with mortality and worse Hospital outcomes in nationwide analysis using national readmission database
10.1016/j.jaip.2025.09.021 · 2025 · External reference
Severe deficiency of switched memory B cells (CD27(+)IgM(-)IgD(-)) in subgroups of patients with common variable immunodeficiency: a new approach to classify a heterogeneous disease
10.1182/blood.v99.5.1544 · 2002 · External reference
Common variable immunodeficiency patient classification based on impaired B cell memory differentiation correlates with clinical aspects
10.1023/a:1025373601374 · 2003 · External reference
The EUROclass trial: defining subgroups in common variable immunodeficiency
10.1182/blood-2007-06-091744 · 2008 · External reference
T-bethighCD21low B cells: the need to unify our understanding of a distinct B cell population in health and disease
10.1016/j.coi.2023.102300 · 2023 · External reference
Expansion of a distinct cytotoxic CD4 TFH-cell cluster in lymph nodes of patients with complicated common variable immunodeficiency
10.1016/j.jaci.2025.09.032 · 2026 · External reference
Convergence of cytokine dysregulation and antibody deficiency in common variable immunodeficiency with inflammatory complications
10.1016/j.jaci.2021.06.008 · 2022 · External reference
Role of B cell activating factor in CVID lung disease
10.1016/j.jaci.2015.12.722 · 2016 · External reference
Plasma protein profiling reflects T(H)1-driven immune dysregulation in common variable immunodeficiency
10.1016/j.jaci.2020.01.046 · 2020 · External reference
Systematic benchmark of state-of-the-art variant calling pipelines identifies major factors affecting accuracy of coding sequence variant discovery
10.1186/s12864-022-08365-3 · 2022 · External reference
Genome-wide association identifies diverse causes of common variable immunodeficiency
10.1016/j.jaci.2011.02.039 · 2011 · External reference
Histocompatibility complex status and mendelian randomization analysis in unsolved antibody deficiency
10.3389/fimmu.2020.00014 · 2020 · External reference
Cytomegalovirus drives Vδ1+ γδ T cell expansion and clonality in common variable immunodeficiency
10.1038/s41467-024-48527-3 · 2024 · External reference
Splenomegaly in CVID patients associates with CMV replication and alterations of immune cells and functions
10.1016/j.imlet.2025.107058 · 2025 · External reference
Influence of cytomegalovirus infection on immune cell phenotypes in patients with common variable immunodeficiency
10.1016/j.jaci.2012.02.011 · 2012 · External reference
Autoimmunity and inflammation in CVID: a possible crosstalk between immune activation, gut microbiota, and epigenetic modifications
10.1007/s10875-018-0574-z · 2019 · External reference
The role of chronic norovirus infection in the enteropathy associated with common variable immunodeficiency
10.1038/ajg.2014.432 · 2015 · External reference
CVID enteropathy associated with chronic norovirus infection: background, clinical features, and therapeutic aspects
10.1002/rmv.70081 · 2025 · External reference
Revealing disease subtypes and heterogeneity in common variable immunodeficiency through transcriptomic analysis
10.1038/s41598-024-74728-3 · 2024 · External reference
Unresolved reference
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Role of advanced practice providers in the cardiac intensive care unit team
10.1016/j.jacc.2023.08.061 · 2023 · External reference
Nurse practitioners and physician assistants: an underestimated workforce for older adults with cancer
10.1111/jgs.15931 · 2019 · External reference
Principles supporting dynamic clinical care teams: an American College of Physicians position paper
10.7326/0003-4819-159-9-201311050-00710 · 2013 · External reference
Unresolved reference
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The status of US allergy/immunology physicians in the 21st century: a report from the American Academy of Allergy, Asthma & Immunology Workforce Committee
10.1016/j.jaci.2007.01.040 · 2007 · External reference
Rural health disparities in allergy, asthma, and immunologic diseases: the current state and future direction for clinical care and research
10.1016/j.jaip.2023.11.030 · 2024 · External reference
Access to specialty healthcare in urban versus rural US populations: a systematic literature review
10.1186/s12913-019-4815-5 · 2019 · External reference
Telehealth and allergy services in rural and regional locations that lack specialty services
10.1016/j.jaip.2022.06.025 · 2022 · External reference
Physician awareness and understanding of primary immunodeficiency disorders: a web-based study in Japan
10.1080/25785826.2022.2137966 · 2023 · External reference
Experiences with diagnostic delay among underserved racial and ethnic patients: a systematic review of the qualitative literature
10.1136/bmjqs-2024-017506 · 2025 · External reference
Racial and ethnic disparities in early mortality among patients with inborn errors of immunity
10.1016/j.jaci.2023.09.036 · 2024 · External reference
Clinical disparity of primary antibody deficiency patients at a safety net hospital
10.1016/j.jaip.2021.03.021 · 2021 · External reference
Primary immunodeficiencies in a mesoregion of São Paulo, Brazil: Epidemiologic, clinical, and geospatial approach
10.3389/fimmu.2020.00862 · 2020 · External reference
10.3389/fimmu.2017.00678
10.3389/fimmu.2017.00678 · External reference
Electronic health record signatures identify undiagnosed patients with common variable immunodeficiency disease
10.1126/scitranslmed.ade4510 · 2024 · External reference
A predictive model for identification of pediatric individuals with common variable immunodeficiency through electronic medical records
10.1016/j.jaci.2025.02.032 · 2025 · External reference
Large-scale deep learning analysis to identify adult patients at risk for combined and common variable immunodeficiencies
10.1038/s43856-023-00412-8 · 2023 · External reference