{
  "abstract": "Background Cryptogenic cirrhosis (CC), has been inadequately documented globally. We aimed to assess the prevalence of CC worldwide and its clinical outcomes stratified by WHO regions and clinical presentation.Methods A systematic review and meta-analysis of articles and abstracts published between 1st of January 1989 and 31st December2024, in any language, retrieved from MEDLINE, Embase, Preprints, Scopus, Global Health, Global Index Medicus, and Web of Science. Inclusion criteria: adult or pediatric populations of any age, diagnosed with cirrhosis using histological, clinical or non-invasive methods, and fully investigated for chronic liver disease (CLD) including metabolic disorders as confounding factors of CC. We used a random-effects model to estimate the prevalence of CC worldwide and among subgroups of patients stratified by WHO regions and hepatic presentation at diagnosis.Results Of 26,410 articles and abstracts identified, 1030 were screened and 68 were included in this analysis: 58 studies in adults (1 case-control study, 57 cohort studies) and 10 in children (all cohort studies). A total of 1,731,881 adults (median age 54 [IQR 49–61]) and 1,164 children (median age 8 [8–8.4]) were analyzed. In adults, CC was diagnosed following liver transplantation (LT) (13 studies, 22%), or investigation of ACLF (3 studies, 5%), cirrhosis (27 studies, 47%), CLD (11 studies, 19%), or hepatocellular carcinoma (HCC) (4 studies, 7%). In children, the diagnosis was mainly made after LT (6 studies, 60%). The overall prevalence of CC was 7% [95% CI: 4–9] in adults and 14% [4–29] in children. The lowest prevalence (5%) was observed in the WHO Western Pacific Region and the highest prevalence (32%) in the WHO African Region. According to the stage of liver disease at diagnosis, the prevalence ranged between 4% in LT, 9% in ACLF and cirrhotic patients, and 10% in HCC patients ( figure 1). The pooled mortality rate (based on 9 cohort studies) was 30.6% [27–36] after a median follow-up of 9 years [9–16]. In subgroups of patients with CLD and cirrhosis, the proportions of HCC were 11.5% and 18%, after median follow-ups of 11.5 years [10.25–12.75] and 8 years [6.5–10.25], respectively. Hepatic decompensation was reported in eight studies, 27.4% as ascites decompensation (median follow-up: 8 years [4.5–10.25]); 16.5% as variceal bleeding (8 years [4.5–9.8]); and 62.1% as encephalopathy (9 years [6–11.75]).Conclusion Cryptogenic cirrhosis is frequently observed and may significantly contribute to the burden of liver disease worldwide. Its clinical impact and pathophysiology need to be further investigated.Abstract P85 Figure 1",
  "authors": [
    {
      "affiliations": [
        "Department of Metabolism, Digestion and Reproduction,Queen Elizabeth the Queen Mother Wing (QEQM), St Mary’s Campus, Imperial College, London, UK",
        "APHP, Henri Mondor Hospital , Paris , France"
      ],
      "name": "Anna Sessa"
    },
    {
      "affiliations": [
        "Department of Metabolism, Digestion and Reproduction,Queen Elizabeth the Queen Mother Wing (QEQM), St Mary’s Campus, Imperial College, London, UK"
      ],
      "name": "Monika Prabhaker-Kaushal"
    },
    {
      "affiliations": [
        "Department of Metabolism, Digestion and Reproduction,Queen Elizabeth the Queen Mother Wing (QEQM), St Mary’s Campus, Imperial College, London, UK"
      ],
      "name": "Robert Goldin"
    },
    {
      "affiliations": [
        "Unité d’Epidémiologie des Maladies Emergentes, Institut Pasteur, Université Paris Cité, Paris, France"
      ],
      "name": "Yusuke Shimakawa"
    },
    {
      "affiliations": [
        "APHP, Henri Mondor Hospital , Paris , France"
      ],
      "name": "Vincent Leroy"
    },
    {
      "affiliations": [
        "Department of Metabolism, Digestion and Reproduction,Queen Elizabeth the Queen Mother Wing (QEQM), St Mary’s Campus, Imperial College, London, UK"
      ],
      "name": "Lemoine Maud"
    }
  ],
  "title": "P85 Global prevalence and clinical outcomes of cryptogenic cirrhosis: a systematic review and meta-analysis",
  "uid": "9749ff52-e1c1-5712-8573-2226a8b538d6"
}