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Evolving Practice and Changing Phenotype in Pediatric Autoimmune Liver Disease: Outcomes From an Australian Center

Harveen Singh1, Fariha Balouch1, Charlton Noble1

  • 1Department of Gastroenterology, Hepatology and Transplant, Lady Cilento Children's Hospital.

Journal of Pediatric Gastroenterology and Nutrition
|February 23, 2018
PubMed

Insights

Autoimmune liver disease (AILD) diagnoses, particularly autoimmune sclerosing cholangitis (ASC), are increasing in children. Diagnostic tool use and inflammatory bowel disease (IBD) detection also rose, impacting treatment and transplant considerations.

Area of Science:

  • Pediatric Gastroenterology and Hepatology
  • Autoimmune Liver Diseases
  • Inflammatory Bowel Disease

Background:

  • Autoimmune liver disease (AILD) encompasses primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH), and autoimmune sclerosing cholangitis (ASC), a condition overlapping AIH and PSC.
  • Understanding trends in AILD diagnosis and patient phenotypes is crucial for adapting clinical practice.
  • This study examines changes in AILD diagnosis and characteristics in children over two distinct time periods.

Purpose of the Study:

  • To investigate shifts in the diagnosis and presentation of autoimmune liver diseases in children.
  • To analyze changes in the utilization of diagnostic modalities and the prevalence of associated inflammatory bowel disease (IBD).
  • To identify risk factors for liver transplant and colectomy in pediatric AILD patients.

Main Methods:

  • Retrospective chart review of 75 children diagnosed with AILD between January 2000 and December 2016.
  • Data divided into two 8-year cohorts: 2000-2007 (C1) and 2008-2016 (C2).
  • Analysis included AILD type, diagnostic tool usage (magnetic resonance cholangio-pancreatography, colonoscopy), liver disease severity, and outcomes (transplant, colectomy).

Main Results:

  • The proportion of autoimmune hepatitis (AIH) decreased from 65% in C1 to 45% in C2, while autoimmune sclerosing cholangitis (ASC) increased from 14% to 18%.
  • Use of magnetic resonance cholangio-pancreatography (MRCP) rose significantly (34% to 65%), as did colonoscopy performance (48% to 63%).
  • Diagnosis of AILD with co-existing inflammatory bowel disease (IBD) increased from 31% to 52%; advanced liver disease was present in 53% at presentation.

Conclusions:

  • Primary sclerosing cholangitis (PSC) and autoimmune sclerosing cholangitis (ASC) are increasingly diagnosed in pediatric patients, often alongside inflammatory bowel disease (IBD).
  • Increased utilization of advanced diagnostic tools like MRCP and colonoscopy correlates with these diagnostic trends.
  • Large duct PSC and ASC associated with IBD, and female gender, are identified as risk factors for colectomy and liver transplantation, respectively.
Abstract

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