Metabolic-associated fatty liver disease (MAFLD) is currently the most common chronic liver disease in the world, which may eventually progress to cirrhosis, hepatocellular carcinoma and liver failure. Fatty liver disease was once considered to be the most common cause of cryptogenic cirrhosis. Recently, a new definition of MAFLD suggests that MAFLD-related liver cirrhosis is no longer a kind of cryptogenic cirrhosis, and it belong to two different concepts and may have different liver and extrahepatic adverse outcomes. In this paper, the definition, epidemiology, diagnosis, treatment and other aspects of MALFD-related liver cirrhosis and cryptogenic liver cirrhosis are described in order to facilitate clinical practice, improve the efficiency of clinical research, and benefit clinicians and patients.
代谢相关脂肪性肝病（MAFLD）是目前全球最常见的慢性肝病，最终可进展至肝硬化、肝癌和肝功能衰竭。脂肪性肝病曾被认为是隐源性肝硬化最常见的病因，MAFLD新定义的提出，表明MAFLD相关肝硬化不再是一种隐源性肝硬化，两者隶属两个不同概念，它们可能具有不同的肝脏和肝外不良结局。现就MALFD相关肝硬化和隐源性肝硬化的定义、流行病学、诊断、治疗等方面进行阐述，旨在方便临床实践、提高临床研究效率，使临床医师和患者获益。.

Little is known about the natural course of nonalcoholic fatty liver disease (NAFLD) with advanced fibrosis. We describe long-term outcomes and evaluate the effects of clinical and histologic parameters on disease progression in patients with advanced NAFLD.
We conducted a multi-national study of 458 patients with biopsy-confirmed NAFLD with bridging fibrosis (F3, n = 159) or compensated cirrhosis (222 patients with Child-Turcotte-Pugh scores of A5 and 77 patients with scores of A6), evaluated from April 1995 through November 2013 and followed until December 2016, death, or liver transplantation at hepatology centers in Spain, Australia, Hong Kong, and Cuba. Biopsies were re-evaluated and scored; demographic, clinical, laboratory, and pathology data for each patient were collected from the time of liver biopsy collection. Cox proportional and competing risk models were used to estimate rates of transplantation-free survival and major clinical events and to identify factors associated with outcomes.
During a mean follow-up time of 5.5 years (range, 2.7-8.2 years), 37 patients died, 37 received liver transplants, 88 had initial hepatic decompensation events, 41 developed hepatocellular carcinoma, 14 had vascular events, and 30 developed nonhepatic cancers. A higher proportion of patients with F3 fibrosis survived transplantation-free for 10 years (94%; 95% confidence interval [CI], 86%-99%) than of patients with cirrhosis and Child-Turcotte-Pugh A5 (74%; 95% CI, 61%-89%) or Child-Turcotte-Pugh A6 (17%; 95% CI, 6%-29%). Patients with cirrhosis were more likely than patients with F3 fibrosis to have hepatic decompensation (44%; 95% CI, 32%-60% vs 6%, 95% CI, 2%-13%) or hepatocellular carcinoma (17%; 95% CI, 8%-31% vs 2.3%, 95% CI, 1%-12%). The cumulative incidence of vascular events was higher in patients with F3 fibrosis (7%; 95% CI, 3%-18%) than cirrhosis (2%; 95% CI, 0%-6%). The cumulative incidence of nonhepatic malignancies was higher in patients with F3 fibrosis (14%; 95% CI, 7%-23%) than cirrhosis (6%; 95% CI, 2%-15%). Death or transplantation, decompensation, and hepatocellular carcinoma were independently associated with baseline cirrhosis and mild (<33%) steatosis, whereas moderate alcohol consumption was associated with these outcomes only in patients with cirrhosis.
Patients with NAFLD cirrhosis have predominantly liver-related events, whereas those with bridging fibrosis have predominantly nonhepatic cancers and vascular events.