Transient elastography, APRI and FIB-4 scores for staging of fibrosis and cirrhosis in Wilson disease

Background & Aims:

Data on the predictive capability of cirrhosis through transient elastography (TE) in Wilsons disease (WD) is scarce. Furthermore there is no data regarding its value to monitor therapy. Aim was therefore to assess whether TE is a suitable tool to identify cirrhosis in patients (i) newly diagnosed and (i) under treatment for WD.

Methods:

Patient with WD underwent TE (results in kPa) either at the time of diagnosis or during a regular outpatient visit during treatment. Data are shown only for patients in whom a liver biopsy was available. Furthermore data on initial liver biopsy results and non-invasive fibrosis scores (APRI, FIB-4) were recorded.

Results:

55 patients were included in the study. Of those 6 were de novo patients [Male: 50%, Age: 34 ± 10, TE: 10,9 kPa (3.5 – 34.8), Cirrhosis at LBX: 50%] and 49 patients [Male: 49%, Age: 40 ± 14), TE: 7.4 kPa (3.5 – 15.4), Cirrhosis at LBX: 36.7%] had received various length of treatment with chelators. Significantly more patients under treatment were found with TE values < 12kPa (93.6% vs. 62.5%; p = 0.009) irrespective of initial biopsy results. Moreover the APRI classification found no patients (0%) with cirrhosis in the treated group (vs. 33.3% in the no-treatment group, p < 0.001). No non-cirrhotic patient worsened under treatment according to the APRI classification (cirrhosis: 0%). In untreated patients, TE, APRI and FIB-4 values almost correctly classified patients with cirrhosis, whereas in treated patients with cirrhosis at LBX (n = 18) only 16.7%, 5.6% and 27.8% where classified with cirrhosis according to TE> 12kPa, APRI and FIB-4 scores respectively (see Tab. 1) suggesting a potential reversible effect of therapy on the course of the disease.

Conclusion:

In conclusion, TE and non-invasive fibrosis scores are valid tools to discriminate cirrhosis in newly diagnosed WD patients. In cirrhotic patients on long-term treatment TE, APRI and FIB-4 were mostly below the threshold for advanced fibrosis, indicating that decoppering therapy prevents progression of liver disease.

Tab. 1

	N	kPa	kPa > 12	APRI median	APRI classification: cirrhosis	FIB-4 median	FIB-4 classification: F3/4
No-Cirrhosis in untreated pats.	3	5.3	0 (0%)	0.21	0 (0%)	0.39	0 (0%)
Cirrhosis in untreated pats.	3	34.8	3 (100%)	2.15	2 (66.7%)	7.46	2 (66.7%)
Cirrhosis at time of Dg. in treated patients	18	7.9 (3.5 – 12.4)	3 (16.7%)	0.39 (0.23 – 1.27)	1 (5.6%)	1.25 (0.49 – 4.28)	5 (27.8%)
No cirrhosis at time of Dg. In treated patients	31	6.9 (3.5 – 15.3)	2 (6.5%)	0.25 (0.17 – 0.83)	0 (0%)	0.88 (0.28 – 4.23)	2 (6.5%)