The term cryptogenic cirrhosis (CC) implies that the underlying etiology is uncertain and devoid of definitive clinical, serological, and histological criteria for a specific type of cirrhosis. CC denotes a spectrum of underlying causes, among whom, the majority of evidence implicates non-alcoholic steatohepatitis (NASH) led progression as the most frequent route. CC is held accountable for around 5% of cirrhosis cases today. Cirrhosis complications like liver failure and hepatocellular carcinoma appear as the first presenting feature of the disease, as in other hepatic diseases. The hepatocarcinogenic risk of CC is substantial, with hepatocellular carcinoma’s reported annual cumulative incidence ranging between 2.6% to 3.5%. The diagnosis of CC relies on the exclusion of distinctly recognizable diseases and involves a careful clinical assessment including risk history and family history, followed by laboratory testing including serology, autoantibodies, immunoglobulin concentrations, biomarkers for Wilson’s disease and Celiac disease, and lastly histological assessment for the prominent type and inflammation distribution, and typical presenting features of cirrhotic tissues. The definitive treatment of CC involves transplantation. In cases suspected to be linked with NASH, medical management until transplantation can be beneficial to the patient. The management of CC involves collaboration of primary care with gastroenterology, hepatology, and transplant consultation, and nutritional counseling. Data regarding survival post-transplantation is limited and has shown conflicting results.
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