What are Autoimmune and cholestatic liver diseases?
Autoimmune and cholestatic liver diseases are a group of rare medical conditions that affect the liver and bile ducts. The major conditions in this group are:
Autoimmune hepatitis (AIH) is an autoimmune condition where the body’s own immune system causes inflammation of the liver (hepatitis). This can result in abnormal liver tests on blood work, may cause jaundice and can lead to long term scarring of the liver (cirrhosis). It is commonly treated with tablet medications to suppress the immune system, including steroids (such as prednisolone) and other drugs such as azathioprine (Imuran) or mycophenolate (CellCept).
Primary biliary cholangitis (PBC) is a condition where the lining of the bile ducts, which normally drain bile from the liver to the gut, becomes damaged and inflamed. This results in slowing of the bile flow (cholestasis) and can cause symptoms including itching of the skin, fatigue and brain fog. Primary biliary cholangitis can cause liver blood tests to be abnormal and can lead to jaundice and scarring of the liver (cirrhosis) in some patients. It is commonly treated with a tablet medication called ursodeoxycholic acid (“Urso” or UDCA), or in some cases obeticholic acid (OCA) or bezafibrate.
Primary sclerosing cholangitis (PSC) also affects the bile ducts causing reduced bile flow, and also causes scarring and narrowing of the bile ducts (called strictures). This can lead to infection of the bile ducts (cholangitis), itching, jaundice and scarring of the liver (cirrhosis). Patients often, but not always, have inflammatory bowel disease (often called colitis). Management is focused on treatment of symptoms and active trials of new treatments are underway.
As well as these three conditions there are a range of other rarer conditions related to the immune system that affect the liver and bile ducts including IgG-4 disease, autoimmune sclerosing cholangitis and immunodeficiency syndromes, all of which have their own treatment approaches.
What do we offer in the autoimmune and cholestatic liver diseases services?
Clinical care: we offer a full, multidisciplinary service for patients with consultant led clinics by Dr Marshall and Professor Thorburn, and specialist nurse-led and pharmacist-led treatment clinics. In addition our clinical nurse specialists provide in-person and remote-access patient support and advice. The clinics are supported by specialist multi-disciplinary teams including histopathology, radiology, interventional radiology, hepatobiliary endoscopy (for ERCP and endoscopic ultrasound) and are closely linked to the Royal Free Hospital liver transplant service.
Regular, dedicated, specialist multi-disciplinary meetings are held to review patients with complex AIH, PSC or PBC on a weekly or fortnightly basis, allowing us access to wide-ranging expert opinion.
We are an Operational Delivery Network hub for the specialist provision of advanced and new treatments for PBC and closely link with national specialist networks including the national IgG-4 and primary immunodeficiency multi-disciplinary meetings.
Research focus: in addition to specialist clinical care we also offer a portfolio of clinical trials and a rage of research projects exploring the causes, management and outcomes of these conditions and their impact on patients’ lives. If they wish, patients may be eligible to take part in these studies to contribute to research, to donate samples (such as blood) to a research archive (biobank) and potentially to access novel treatments in clinical trials across these conditions.
We are also a founding member of the European Reference Network for Rare Liver Diseases. This is a Europe-wide network of liver specialists with an interest in rare liver diseases, including the cholestatic and autoimmune liver diseases. The network aims to improve clinical knowledge and patient care, produces clinical guidance, offers international expert panels to discuss complex cases and provides information for patients.
As part of our commitment to education and practice development, the autoimmune and cholestatic liver diseases service hosts national and international meetings for clinicians and frequently hosts international clinician observers and visiting practicing clinicians from across the globe. We also offer patient education days, organised by patient charities, allowing patients the opportunity to discuss the background to their illnesses, latest research advances and standards of care with expert clinicians and researchers.
Referral to the Autoimmune and cholestatic liver diseases clinic
Patients can be referred directly to the clinic by their GP for suspected or confirmed immune-mediated liver diseases, or by gastroenterologists and hepatologists from other UK hospitals. We are happy to manage patients under joint care with their local hospital to minimise the inconvenience of travel to our centre if they live some distance away. We are also happy to review patients who are interested in clinical trials that are not available at their local centre.
Telephone: 020 3758 2000 X31142
Email – administration: firstname.lastname@example.org
Email – clinical nurse specialists (for current patients): email@example.com
Pager – clinical nurse specialists (for current patients): bleep 4176
Patient support organisations:
- The British Liver Trust: The British Liver Trust is a UK-wide health charity that promotes liver health, campaigns to raise awareness about liver diseases and provides information and support for patients with liver diseases.
- AIH support: AIH support is a patient run charity that provides support and information relating to AIH
- The PBC foundation: The PBC foundation is a patient support organisation that offers information and advice to patients with PBC
- PSC support: PSC support is a patient-led charity that provides information and advice relating to PSC
Research organisations: There are three national patient research platforms with which we are actively involved. Their websites include information on research in autoimmune and cholestatic diseases. They were all formed as collaborations between patient groups, doctors, scientists and industry to improve understanding and treatment of AIH, PBC and PSC.