Meta-analysis: Prevalence of significant or advanced fibrosis in adults with alpha-1-antitrypsin deficiency.

Wen Hui Lim, Rohit Loomba, Virginia C Clark, Mark Muthiah, Mei Chin Lim, Benjamin Nah, Cheng Han Ng, Margaret L P Teng, Eunice X X Tan, Elina Cho, Daniel Q Huang, Darren J H Tan, Christen En Ya Ong, Charlotte Chung Hui Ong, Elden Yen Hng Ong, Benjamin Koh, Rebecca Wenling Zeng, Caitlyn Tan, Kai En Chan

Journal: Alimentary pharmacology & therapeutics 2023;58(2):152-158

PMID: 37089038

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Abstract

BACKGROUND

The prevalence of liver fibrosis detected by non-invasive imaging in alpha-1-antitrypsin (AAT) deficiency has not been systematically assessed.

AIMS

We conducted a systematic review and meta-analysis to determine the prevalence of significant fibrosis and advanced fibrosis in AAT deficiency based on non-invasive imaging.

METHODS

Medline and Embase electronic databases were searched for studies from inception to 13 November 2022 that provided data for the prevalence of fibrosis in adults with AAT deficiency. A generalised linear mixed model with Clopper-Pearson intervals was used to pool single-arm outcomes.

RESULTS

Of the 214 records identified, 8 studies were included. Five studies assessed fibrosis using vibration-controlled transient elastography. The prevalence of significant fibrosis (defined as ≥7.1 kPA) in Z homozygosity, Z heterozygosity and non-carrier status was 22.10% (five studies, 95% CI: 17.07-28.12), 9.24% (three studies, 95% CI: 4.68-17.45) and 5.38% (one study, 95% CI: 3.27-8.73), respectively, p < 0.0001, and the prevalence of advanced fibrosis (defined as ≥9.5 kPa) was 8.13% (five studies, 95% CI: 4.60-13.96), 2.96% (three studies, 95% CI: 1.49-5.81) and 1.08% (one study, 95% CI: 0.35-3.28), respectively, p = 0.003. There were limited data regarding the use of magnetic resonance elastography or acoustic radiation force impulse to assess for fibrosis.

CONCLUSION

More than one in five adult individuals with AAT deficiency and Z homozygosity harbour significant fibrosis, and nearly 1 in 10 harbours advanced fibrosis. The risk of fibrosis increases incrementally with the frequency of Pi*Z mutations.

Address: NAFLD Research Center, Division of Gastroenterology, University of California at San Diego, La Jolla, California, USA.; Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.; Division of Gastroenterology and Hepatology, Department of Medicine, National University Health System, Singapore, Singapore.; Department of Diagnostic Imaging, National University Health System, Singapore, Singapore.; Division of Gastroenterology, Hepatology, and Nutrition, University of Florida, USA.; Division of Epidemiology, Department of Family Medicine and Public Health, University of California at San Diego, San Diego, California, USA.

DOI: 10.1111/apt.17516