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MRE identifies fibrosis in patients with alpha-1 antitrypsin deficiency

Magnetic resonance elastography appeared to accurately identify fibrosis in patients with homozygous Z genotype alpha-1 antitrypsin deficiency, according to a pilot prospective study published in Alimentary Pharmacology & Therapeutics.

“Our data show serum biomarkers most commonly elevated in individuals with other forms of metabolic liver disease are not elevated in individuals with alpha-1 antitrypsin deficiency liver disease,” Rohit G. Loomba, MD, professor in the department of medicine at the University of California at San Diego (UCSD) School of Medicine, and colleagues wrote. “We believe that magnetic resonance elastography may be an accurate, noninvasive method for diagnosis of liver fibrosis in patients with ZZ genotype alpha-1 antitrypsin.”

Rohit G. Loomba

Currently, clinicians do not look for liver disease in patients with alpha-1 antitrypsin (AAT) deficiency until it is very late, Loomba told Healio.com/hepatology.

In a pilot prospective study inspired by David A. Brenner, MD, dean of UCSD School of Medicine, Loomba and colleagues performed a study of 33 adults who underwent liver biopsy, clinical research evaluation and MRE. The researchers separated those who underwent liver biopsy into AAT, NAFLD and control groups. The researchers quantified liver stiffness with MRE and histological fibrosis with the Ishak 6-point scale. Afterward, they used receiver operating characteristic (ROC) analysis to assess MRE diagnostic performance.

After adjusting for stage of fibrosis, they found that serum alanine aminotransferase levels were similar between the control and AAT groups, but significantly lower in AAT compared with NAFLD (P = .0172). MRE-estimated stiffness in fibrosis detection had an area under the ROC curve of 0.9 (P < .0001). Further, an MRE threshold of at least 3 kPa provided 88.9% accuracy with 80% sensitivity and 100% specificity to detect the presence of fibrosis at any stage.

These results suggest that magnetic resonance elastography may be accurate for identifying fibrosis in patients with AAT deficiency, the researchers concluded.

“Clinicians with expertise in select places may apply this in their clinical practice as it is a rare disease but larger data from a multicenter setting would be needed for more widespread utilization and recommendations,” Loomba said. – by Will Offit

Disclosure: The researchers report no relevant financial disclosures.