High Z polymer levels linked to fibrosis, inflammation in alpha 1 antitrypsin deficiency
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WASHINGTON — Elevated Z polymer levels may predict lung and liver disease activity in patients with alpha 1 antitrypsin deficiency, according to a presenter at The Liver Meeting.
“Homozygous ZZ alpha 1 antitrypsin deficiency, or AATD, is a common genetic metabolic disorder affecting adults and children,” Anandini Suri, MD, a GI fellow at Saint Louis University and SSM Health Cardinal Glennon Children’s Hospital, said during the presentation. “Clinical manifestations of this disease are highly variable. The exact burden of liver disease in adulthood is not well understood and predictors and biomarkers of severe disease are still being identified.”
In a prospective study, Suri and colleagues examined 93 adult patients with ZZ AATD liver disease at three U.S. sites, of whom 82 were in the liver biopsy arm and 11 were in the severe arm with cirrhosis noted at the liver biopsy. At enrollment and follow-up, researchers collected data demographic, history, physical exam, laboratory and radiologic data. They included 54 participants in the final analysis.
Researchers used the 2C1 Meso Scale Discovery ELISA-based assay to determine the Z protein polymer level. The mean Z polymer level was 12.1 µg/ml, Suri said.
According to results, higher circulating Z polymer levels were linked with increased liver fibrosis on biopsy as well as increased portal and lobular inflammation. Researchers also reported an association between the Z polymer levels and aspartate aminotransferase, but not alanine aminotransferase levels.
Additionally, no correlation was noted between increased BMI or steatosis, but there was correlation between elastography scores and polymer levels. Researchers also reported a weak, negative association between Z polymer and forced expiratory volume percent.
According to researchers, further study — including 5-year follow-up biopsy in this group of patients — is warranted, which may help determine how polymer levels can be incorporated into clinical care and study enrollments.
“High circulating Z polymer levels are associated with liver injury, fibrosis, inflammation and reduction in lung function,” Suri concluded. “This could potentially be a marker that could predict both lung and liver injury disease activity and AATD limitations.”
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Suri A, et al. Abstract 5002. Serum Z polymer is a biomarker for increased liver fibrosis in adults with Alpha-1 antitrypsin deficiency. Presented at: The Liver Meeting; Nov. 4-8, 2022; Washington (hybrid).
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