A systematic literature review of CVID reveals pervasive detrimental noninfectious manifestations
Noninfectious manifestations of common variable immunodeficiency (CVID) are not formally summarized. We performed a systematic literature review to generate a comprehensive reference for the field. Splenomegaly was the most reported manifestation across 53 publications, occurring in a median of 35.2% of patients. Frequently reported digestive system manifestations included diarrhea (median 27.8%; 21 publications), hepato(spleno)megaly (median, 21.0%; 19 publications), portal hypertension (median 21.0%; 3 publications), nodular lymphoid hyperplasia (median, 17.0%; 9 publications), and enteropathy (median, 16.0%; 34 publications). Other notable manifestations included interstitial lung disease (median, 8.7%; 32 publications) and autoimmune cytopenias (median 18.0%; 21 publications). Steroids and rituximab were the most frequently reported treatments. Numerous manifestations significantly adversely affected survival, including lymphoma, granulomatous lymphocytic interstitial lung disease, splenomegaly, and liver diseases. These comprehensive data document the pervasiveness and negative impact of noninfectious manifestations in CVID and support a call to action to develop novel therapeutics.
Authors
- Sinisa Savic (ORCID: https://orcid.org/0000-0001-7910-0554)
- Kevin S. Thorneloe (ORCID: https://orcid.org/0000-0003-3640-9839)
- Lisa Forbes Satter (ORCID: https://orcid.org/0000-0001-9402-1934)
- Charlotte Cunningham‐Rundles (ORCID: https://orcid.org/0000-0003-0725-0320)
- Elena W.Y. Hsieh (ORCID: https://orcid.org/0000-0003-3969-6597)
- Ali Mojebi (ORCID: https://orcid.org/0000-0003-2318-8627)
- Rebecca Marsh (ORCID: https://orcid.org/0000-0002-9645-0617)
- Gloria Michelle Ducasa
- Hsi-en Ho (ORCID: https://orcid.org/0000-0002-7160-2960)
- Joud Hajjar (ORCID: https://orcid.org/0000-0003-4633-1043)
- Jocelyn R. Farmer (ORCID: https://orcid.org/0000-0003-4901-9848)
- Gulbu Uzel (ORCID: https://orcid.org/0009-0003-0700-694X)
- Héctor Balastegui-Martín (ORCID: https://orcid.org/0000-0002-4651-6841)
- Marta Dafne Cabañero-Navalón (ORCID: https://orcid.org/0000-0002-1447-9214)
Institutions
- Hospital Universitari i Politècnic La Fe (ES)
- National Institute for Health and Care Excellence (GB)
- Lahey Medical Center (US)
- NIHR Leeds Musculoskeletal Biomedical Research Unit (GB)
- Precision for Medicine (United States) (US)
- Michigan Surgical Hospital (US)
- NHS Digital (GB)
- National Institute of Allergy and Infectious Diseases (US)
- MD Precision (Canada) (CA)
- Texas Children's Hospital (US)
- University of Colorado Anschutz Medical Campus (US)
- Icahn School of Medicine at Mount Sinai (US)
Publication Details
- Journal
- Journal of Human Immunity
- Published
- 2025-09-26
- DOI
- https://doi.org/10.70962/jhi.20250157
- Primary Topic
- Immunodeficiency and Autoimmune Disorders
- Type
- article
- Field-Weighted Citation Impact
- 0.00