Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management

Shukla A 1 , Rockey DC 2 , Kamath PS 3 , Kleiner DE 4 , Singh A 1 , Vaidya A 1 Show all authors , Koshy A 5 , Goel A 6 , Dökmeci AK 7 , Meena B 8 , Philips CA 9 , Sharma CB 10 , Payawal DA 11 , Kim DJ 12 , Lo GH 13 , Han G 14 , Qureshi H 15 , Wanless IR 16 , Jia J 17 , Sollano JD 18 , Al Mahtab M 19 , Muthiah MD 20 , Sonderup MW 21 , Nahum MS 22 , Merican MIB 23 , Ormeci N 24 , Kawada N 25 , Reddy R 26 , Dhiman RK 27 , Gani R 28 , Hameed SS 29 , Harindranath S 1 , Jafri W 29 , Qi X 30 , Chawla YK 31 , Furuichi Y 32 , Zheng MH 33 , Sarin SK 34

Affiliations 

  • 1 Department of Gastroenterology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India
  • 2 Digestive Disease Research Center, Medical University of South Carolina, 96 Jonathan Lucas Street, Suite 803, MSC 623, Charleston, SC, 29425, USA
  • 3 Mayo Clinic College of Medicine and Science, Rochester, MN, USA
  • 4 National Cancer Institute, Bethesda, MD, USA
  • 5 Department of Gastroenterology, VPS Lakeshore Hospital, Kochi, Kerala, India
  • 6 Department of Hepatology, CMC, Vellore, India
  • 7 Department of Medicine, Ankara University School of Medicine, Ankara, Turkey
  • 8 Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India
  • 9 Department of Clinical and Translational Hepatology, The Liver Institute, Rajagiri Hospital, Aluva, Kerala, India
  • 10 Department of Pathology, Institute of Liver and Biliary Sciences, New Delhi, Delhi, India
  • 11 Fatima University Medical Center Manila, Manila, Philippines
  • 12 Department of Internal Medicine, Hallym University College of Medicine, Seoul, South Korea
  • 13 Department of Medical Research, E-Da Hospital, Kaohsiung, School of Medicine for International Students, I-Shou University, 1, Yi-Da Road, Kaohsiung, 824, Taiwan
  • 14 Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Diseases and Xijing Hospital of Digestive Diseases, Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
  • 15 Doctor's Plaza, Clifton, Karachi, Pakistan
  • 16 Department of Pathology, Dalhousie University, Halifax, Canada
  • 17 Liver Research Center, Beijing Friendship Hospital, Capital Medical University, 95 Yongan Road, Beijing, Mainland, China
  • 18 Department of Medicine, University of Santo Tomas, Manila, Philippines
  • 19 Department of Hepatology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
  • 20 Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore
  • 21 Division of Hepatology, Department of Medicine, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa
  • 22 Faculty of Medicine, National Autonomous University of Mexico, Mexico City, Mexico
  • 23 Board of Governors, MAHSA University, Jenjarom, Malaysia
  • 24 İstanbul Health and Technology University, Istanbul, Turkey
  • 25 Department of Hepatology, Graduate School of Medicine, Osaka City University, 1-4-3 Asahimachi, Abeno-Ku, Osaka, 545-8585, Japan
  • 26 Division of Gastroenterology and Hepatology, 2 Dulles, Liver Transplant Office, University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA, 19104, USA
  • 27 Department of Hepatology, PGIMER, Chandigarh, India
  • 28 Division of Hepatobiliary, Department of Internal Medicine, Faculty of Medicine, Cipto Mangunkusumo Hospital, Universitas Indonesia, Jakarta, Indonesia
  • 29 Department of Medicine, Aga Khan University and Hospital, Stadium Road, Karachi, 74800, Pakistan
  • 30 Center of Portal Hypertension, Department of Radiology, Zhongda Hospital, Medical School, Southeast University, Nurturing Center of Jiangsu Province for State Laboratory of AI Imaging & Interventional Radiology (Southeast University), Nanjing, China
  • 31 Department of Hepatology and Gastroenterology, Kalinga Institute of MedicalSciences, KIIT University, Bhubaneshwar, India
  • 32 Department of Clinical Laboratory and Endoscopy, Tokyo Women's Medical University, Adachi Medical Center, Tokyo, Japan
  • 33 MAFLD Research Center, Department of Hepatology, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, 325000, China
  • 34 Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India. sksarin@ilbs.in
Hepatol Int, 2024 Dec;18(6):1684-1711.
PMID: 39546143 DOI: 10.1007/s12072-024-10739-6

Abstract

Since the Asian Pacific Association for the Study of the Liver (APASL) published guidelines on non-cirrhotic portal fibrosis/idiopathic portal hypertension in 2007, there has been a surge in new information, especially with the introduction of the term porto-sinusoidal vascular disorder (PSVD). Non-cirrhotic intra-hepatic causes of portal hypertension include disorders with a clearly identifiable etiology, such as schistosomiasis, as well as disorders with an unclear etiology such as non-cirrhotic portal fibrosis (NCPF), also termed idiopathic portal hypertension (IPH). This entity is being increasingly recognized as being associated with systemic disease and drug therapy, especially cancer therapy. An international working group with extensive expertise in portal hypertension was assigned with formulating consensus guidelines to clarify the definition, diagnosis, histological features, natural history, and management of NCPF/IPH, especially in the context of PSVD. The guidelines were prepared based on evidence from existing published literature. Whenever there was paucity of evidence, expert opinion was included after detailed deliberation. The goal of this manuscript, therefore, is to enhance the current understanding and help create global consensus on the issues surrounding NCPF/IPH.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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