Liver Involvement in Patients With PiZZ-Emphysema, Candidates for Lung Transplantation

Abstract

Information about the prevalence and nature of liver disorders in adults with alpha1-antitrypsin deficiency is scarce. At our center, systematic liver biopsy screening is part of the evaluation before lung transplantation (LT) in the emphysema patients with the PiZZ phenotype. Our aim was to report our experience with this prospective screening. Clinical, liver function, and imaging parameters as well as liver histology data were analyzed for 23 consecutive adult patients with PiZZ severe emphysema referred to our center for consideration of LT from 2006 to 2014. Overall 20 (87%) featured chronic liver disease characterized by a chronic inflammation and/or a significant portal fibrosis on histology. Two of the 23 patients (8.7%) had septal fibrosis according to the Metavir and Ishak scores and met our definition of severe chronic liver disease. They were both clinically asymptomatic with normal liver function tests. On abdominal ultrasonography, the liver appeared normal in one patient and with abnormal contours in the other. Our data indicate that in adults with PiZZ-related emphysema being evaluated for LT, most patients had some histologic involvement. The prevalence of severe liver dysfunction is <10%.

Assessment of liver fibrosis by transient elastography (Fibroscan®) in patients with A1AT deficiency

“…Our results show that, in a cohort of PiZZ adult patients, Fibroscan® can identify in a non invasive way, complementary to LFT and US, 17% of patients with suspected significant fibrosis (≥F2) and 7% of patient with cirrhosis (≥F4). These results are consistent with those of a recent study where a systematic liver evaluation with liver biopsy was performed in 23 patient candidates for lung transplantation [27]. Morer et al have shown that 13% of patients had moderate fibrosis (F2) and 8% severe fibrosis (F3 or F4).…”

European Respiratory Society statement: diagnosis and treatment of pulmonary disease in α1-antitrypsin deficiency

“…The evaluation of comorbidities is crucial in the assessment of candidates for lung transplantation, and hepatic evaluation is particularly critical in AATD [129]. Some centres perform systematic liver biopsy in candidates, although the detection of liver disease per se does not preclude lung transplant in these patients.…”

Personalizing liver targeted treatments and transplantation for patients with alpha-1 antitrypsin deficiency

“…An alternative explanation is that AATD per se is a secondary cause of hepatic steatosis. Steatosis is commonly seen in liver biopsies taken from patients with AATD, further supporting the link between them [27].…”