Language

English

Publication Date

1-5-2026

Journal

Journal of Human Immunity

DOI

10.70962/jhi.20250157

PMID

42170597

PMCID

PMC13177467

PubMedCentral® Posted Date

10-24-2025

PubMedCentral® Full Text Version

Post-print

Abstract

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.

Published Open-Access

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