SynthesisFrontiers in immunology2021
Lessons Learned From the Clinical Presentation of Common Variable Immunodeficiency Disorders: A Systematic Review and Meta-Analysis.
Synthesis in Frontiers in immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 51 citations in OpenAlex.
- Temporal windowing of recurrent sinusitis improves EHR-based immunodeficiency classification.Journal of human immunity · 2026Article
- 25-Hydroxyvitamin D Sufficiency Is Associated With Reduced Atelectasis in Common Variable Immunodeficiency: A Cross-Sectional Study.Medical science monitor : international medical journal of experimental and clinical research · 2026Article
- Pseudomonal sternal abscess and osteomyelitis as an initial manifestation of common variable immunodeficiency.ASM case reports · 2026Article
- Investigating serum free light chains in patients with common variable immunodeficiency disorder in compare with other immunodeficiency diseases.Scientific reports · 2026Article
- Liver Disease in Common Variable Immunodeficiency: Current Evidence and Knowledge Gaps.International journal of molecular sciences · 2026Review
- A systematic literature review of CVID reveals pervasive detrimental noninfectious manifestations.Journal of human immunity · 2026Article
- Common Variable Immunodeficiency Disorders: A perspective from New Zealand.Clinical reviews in allergy & immunology · 2025Review
- T follicular helper cells in primary immune regulatory disorders.The Journal of allergy and clinical immunology · 2025Review
- CVID Enteropathy Associated With Chronic Norovirus Infection: Background, Clinical Features, and Therapeutic Aspects.Reviews in medical virology · 2025Review
- Lymphoproliferative Disorders Mimicking Rheumatologic Disease: A Clinical Reasoning Perspective.Current allergy and asthma reports · 2025Review
- Gastrointestinal Manifestations of Common Variable Immunodeficiency: A Mentored Review.Digestive diseases and sciences · 2025Review
- Common Variable Immunodeficiency Disorder: A Decade of Insights from a Cohort of 150 Patients in India and the Use of Machine Learning Algorithms to Predict Severity.Journal of clinical immunology · 2025Article
- Association of serum copeptin levels with pulmonary complications and heart right ventricular functions in common variable immunodeficiency.BMC immunology · 2025Article
- Genetics in a Danish Common Variable Immunodeficiency Cohort.Journal of clinical immunology · 2025Article
- Granulomatous lymphocytic interstitial lung disease in common variable immune deficiency: an in-depth clinical, immunological, functional and radiological exploration with a focus on its management, challenged by chronic CMV infection.Frontiers in immunology · 2025Article
- Immunogenetic Landscape in Pediatric Common Variable Immunodeficiency.International journal of molecular sciences · 2024Review
- The Horse with Recurrent Fevers and Infections: Approach to Common Variable Immunodeficiency.The Veterinary clinics of North America. Equine practice · 2024Review
- Article
- Decoding mutational hotspots in human disease through the gene modules governing thymic regulatory T cells.Frontiers in immunology · 2024Article
- Investigating pulmonary and non-infectious complications in common variable immunodeficiency disorders: a UK national multi-centre study.Frontiers in immunology · 2024Article
Corrections and comments
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Authors and funding
3 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Background: Diagnostic delay in common variable immunodeficiency disorders (CVID) is considerable. There is no generally accepted symptom-recognition framework for its early detection. Objective: To systematically review all existing data on the clinical presentation of CVID. Methods: PubMed, EMBASE and Cochrane were searched for cohort studies, published January/1999-December/2019, detailing the clinical manifestations before, at and after the CVID-diagnosis. Results: In 51 studies (n=8521 patients) 134 presenting and 270 total clinical manifestations were identified. Recurrent upper and/or lower respiratory infections were present at diagnosis in 75%. Many patients had suffered severe bacterial infections (osteomyelitis 4%, meningitis 6%, septicemia 8%, mastoiditis 8%). Bronchiectasis (28%), lymphadenopathy (27%), splenomegaly (13%), inflammatory bowel disease (11%), autoimmune cytopenia (10%) and idiopathic thrombocytopenia (6%) were also frequently reported. A bimodal sex distribution was found, with male predominance in children (62%) and female predominance in adults (58%). 25% of CVID-patients developed other manifestations besides infections in childhood, this percentage was much higher in adults (62%). Immune-dysregulation features, such as granulomatous-lymphocytic interstitial lung disease and inflammatory bowel disease, were more prominent in adults. Conclusions: The shift from male predominance in childhood to female predominance in adults suggests differences in genetic and environmental etiology in CVID and has consequences for pathophysiologic studies. We confirm the high frequency of respiratory infections at presentation, but also show a high incidence of severe bacterial infections such as sepsis and meningitis, and immune dysregulation features including lymphoproliferative, gastrointestinal and autoimmune manifestations. Early detection of CVID may be improved by screening for antibody deficiency in patients with these manifestations.
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