Editorial: non-invasive assessment of hepatic fibrosis in alpha-1 antitrypsin deficiency using magnetic resonance elastography

Background: Magnetic resonance elastography (MRE) is a promising non-invasive technique for the identification and quantification of hepatic fibrosis. This manuscript describes our early experience with MRE for the assessment of the presence and staging of liver fibrosis on a 3T magnetic resonance imaging (MRI) system.Objectives: The purpose of this study was to describe the MRE physics, procedure, interpretation and drawbacks, along with a few recommendations as per our experience.Method: Magnetic resonance elastography was performed on 85 patients with a 3T MRI and the images were analysed both qualitatively and quantitatively. Liver stiffness was assessed by drawing freehand geographic regions of interest on the elastograms to cover the maximum portion of the hepatic parenchyma within the 95% confidence maps on each slice. Correlation with histopathology was performed whenever available.Results: Of the 80 patients who met the inclusion criteria, 41 patients displayed a normal liver stiffness measurement (LSM) and 39 patients had a raised LSM. In the patients who had a raised LSM, 14 patients had Stage I–II fibrosis, 8 patients had Stage II–III fibrosis, 6 patients had Stage III–IV fibrosis, 4 patients had Stage IV fibrosis or cirrhosis and 7 patients had non-alcoholic steatohepatitis. The mean thickness of the waves increased with increasing stages of fibrosis. The waves became gradually darker medially in patients with normal LSM as compared to the patients with raised LSM. Histopathology with METAVIR scoring was available in 46 patients, which agreed with the MRE findings in all except two patients.Conclusion: Magnetic resonance elastography is a suitable non-invasive modality for the identification and quantification of hepatic fibrosis.

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Non-Invasive Assessment and Management of Liver Involvement in Adults With Alpha-1 Antitrypsin Deficiency

Chronic Obstructive Pulmonary Diseases Journal of the COPD Foundation ◽

10.15326/jcopdf.7.3.2019.0161 ◽

2020 ◽

Vol 7 (3) ◽

pp. 260-271

Author(s):

Karim Hamesch ◽

Pavel Strnad

Keyword(s):

Liver Involvement ◽

Non Invasive ◽

Antitrypsin Deficiency ◽

Alpha 1 Antitrypsin Deficiency ◽

Alpha 1 Antitrypsin

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Non-invasive testing for liver pathology in alpha-1 antitrypsin deficiency

BMJ Open Respiratory Research ◽

10.1136/bmjresp-2020-000820 ◽

2020 ◽

Vol 7 (1) ◽

pp. e000820

Author(s):

Syed Hamza Abbas ◽

Elisha Pickett ◽

David A Lomas ◽

Douglas Thorburn ◽

Bibek Gooptu ◽

...

Keyword(s):

Liver Biopsy ◽

Predictive Value ◽

Liver Stiffness ◽

Liver Function Tests ◽

Liver Pathology ◽

Non Invasive ◽

Liver Ultrasound ◽

Antitrypsin Deficiency ◽

Alpha 1 Antitrypsin Deficiency ◽

Alpha 1 Antitrypsin

BackgroundMany patients with alpha-1 antitrypsin deficiency (A1ATD) receive care in respiratory clinics without access to specialist hepatology expertise. Liver disease can develop asymptomatically, and non-invasive markers of fibrosis may help identify patients who require definitive assessment with liver biopsy. We evaluated the utility of non-invasive markers of liver fibrosis in A1ATD to guide testing in settings without ready access to hepatology expertise.MethodsPatients attending the London A1ATD service undergo assessment using blood tests to calculate the ‘APRI’ and ‘FIB-4’ score, liver ultrasound and Fibroscan. Liver biopsy is offered to patients who have abnormal liver function tests with abnormal liver ultrasound and/or liver stiffness >6 kPa on Fibroscan. Liver biopsies were assessed for the presence of A1AT, steatosis, fibrosis and inflammation.Results75 patients with A1ATD had results for analysis, 56% were female, age 16–82 years. 75% of patients had Fibroscan <6 kPa, 19% had Fibroscan 6–7.9 kPa and 6%>8 kPa. There was a significant correlation between FIB-4 and Fibroscan (r=0.244, p=0.035). Fibroscan >6 kPa corresponded to a FIB-4 score of >1.26. However, FIB-4 >1.26 had poor sensitivity (47%), specificity (32%) and positive-predictive value (PPV; 36%) to identify Fibroscan >6 kPa. The negative-predictive value (NPV) was stronger at 81%. APRI data were similar. Twelve patients underwent liver biopsy, with 11 reports available for analysis. Six had FIB-4 scores<1.26 and five had Fibroscan of <6 kPa. A1AT was present in 64% of biopsies, steatosis in 82%, mild fibrosis in 36%, moderate fibrosis in 9% and severe fibrosis in 9%.ConclusionA combination of liver ultrasound and non-invasive fibrosis tests can help identify patients with A1ATD liver injury. However, APRI and FIB-4 scores alone had poor sensitivity and specificity to justify use as an independent tool for liver pathology in A1ATD.

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