Cryptogenic cirrhosis is diagnosed in 5% to 30% of cirrhotic patients overall and 7% of patients who undergo liver transplantation for cirrhosis.
Dr Jean-Charles Duclos-Valléea and colleagues from France re-examined their series of patients transplanted for cryptogenic cirrhosis.
The researchers, in an attempt to identify the aetiology, re-examined pre-transplant clinical and histological data and the post-transplant course.
Of 881 patients transplanted between 1987 and 2000, 28 patients with a median age of 46 years at transplantation were classified as having cryptogenic cirrhosis.
The team excluded 2 patients where intense ischemic lesions caused by chemoembolization and hepatitis B prevented histological analysis.
The researchers divided the patients into 4 including 14 patients with chronic inflammatory liver disease with autoimmune features in Group 1.
| Autoimmune liver disease emerged as the main etiology|
Group 2 included 3 patients with features suggestive of non-alcoholic fatty liver disease.
There were 3 patients with incomplete septal cirrhosis and vascular liver disease in Group 3 and 6 patients with unresolved cryptogenic cirrhosis in Group 4.
The team found that in the autoimmune liver disease group, the median International Autoimmune Hepatitis score was 13 after re-evaluation.
Review of the post-transplantation course confirmed the diagnosis with the occurrence of active graft hepatitis in 9 patients, with auto-antibodies in 5.
The researchers observed that the vascular group was characterized by lesions of obliterative portal venopathy and incomplete septal cirrhosis in all native livers.
The team reported that diagnosis of non-alcoholic fatty liver disease was based on the clinical background of obesity and/or type 2 diabetes.
In addition, diagnosis of non-alcoholic fatty liver disease was based on the presence of steatosis or steatohepatitis in native livers and graft biopsies.
Dr Duclos-Valléea's team concluded, “A definite etiological diagnosis can be achieved in the majority of patients initially diagnosed with cryptogenic cirrhosis.”
“Autoimmune liver disease emerged as the main etiology and frequently recurred on the grafted liver.”
“In all cases a precise diagnosis is obviously of practical interest for better management of post-transplant survey and treatment.”