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Turkish Journal of Medical Sciences

Abstract

Background/aim: Liver damage is a significant complication that increases mortality and morbidity in primary immunodeficiencies (PIDs). Although biopsy is the gold standard, the fibrosis-4 (FIB-4) scoring system is a noninvasive method for diagnosing liver fibrosis. In this study, we investigated the value of the FIB-4 score as an early indicator of liver damage in patients with primary antibody deficiencies (PADs).

Materials and methods: Of 52 patients, 40 had common variable immunodeficiency (CVID), 12 had immunoglobulin G subclass deficiency (IgG-SGD), and they were enrolled in the study. Patients were divided into hematology, rheumatology + dermatology, gastroenterology/hepatology, and respiratory diseases groups based on their clinical phenotypes. FIB-4 score calculation and transient elastography were used to detect liver complications.

Results: FIB-4 scores were significantly higher in CVID patients than in those with SGD (p = 0.003), and were also elevated in PAD patients with hematology and gastroenterology/hepatology phenotypes compared with rheumatology + dermatology and respiratory phenotypes (p < 0.001). In the gastroenterology/hepatology group patients, FIB-4 scores showed a strong positive correlation with elastography values (r = 0.825, p < 0.001). FIB-4 and elastography showed moderate to good agreement (κ = 0.41–0.60) for detecting significant fibrosis, and FIB-4 was also a valuable indicator in high-risk patients (≥3.25).

Conclusions: This study is the first to show that FIB-4 may serve as a practical and noninvasive indicator of liver damage in PADs.

Author ORCID Identifier

HACI UĞUR MUŞABAK: 0000-0003-1511-7634

TUBA ERDOĞAN: 0000-0002-3143-2442

EMRE ÖZBEK: 0000-0001-6345-8607

DUDU DURAK ANŞİN: 0000-0001-8461-0153

ZUMRUD MEHTİ: 0009-0005-6082-9448

DOI

10.55730/1300-0144.6219

Keywords

Common variable immunodeficiency, fibrosis-4 index, liver damage

First Page

847

Last Page

859

Publisher

The Scientific and Technological Research Council of Türkiye (TÜBİTAK)

Creative Commons License

Creative Commons Attribution 4.0 International License
This work is licensed under a Creative Commons Attribution 4.0 International License.

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