ABSTRACT
Introduction: Patients with cholestatic diseases may develop fatigue and cognitive symptoms. The impact of symptom burden may be significant in some patients. To date, there are no effective pharmacological therapies to improve cognitive symptoms or fatigue in cholestasis and we are wholly reliant on supportive approaches. Area covered: This review provides an overview of cognitive symptoms and fatigue in the cholestatic liver disease primary biliary cholangitis (PBC), including pathophysiology and our approach to the management of these symptoms. Expert opinion: The impact of fatigue and cognitive symptoms on the perceived quality of life can be profound for patients with PBC. The pathophysiology of these symptoms is complex and poorly understood, making the development of therapeutic trials of symptom-directed therapies challenging. The current recommended management for fatigue and cognitive symptoms is mainly supportive.
Subject(s)
Cognition Disorders/physiopathology , Fatigue/physiopathology , Liver Cirrhosis, Biliary/physiopathology , Cholestasis/complications , Cholestasis/physiopathology , Cholestasis/therapy , Cognition Disorders/diagnosis , Cognition Disorders/etiology , Cognition Disorders/therapy , Fatigue/etiology , Fatigue/therapy , Humans , Liver Cirrhosis, Biliary/complications , Liver Cirrhosis, Biliary/therapy , Pruritus/etiology , Pruritus/therapy , Quality of LifeABSTRACT
These guidelines on the management of primary sclerosing cholangitis (PSC) were commissioned by the British Society of Gastroenterology liver section. The guideline writing committee included medical representatives from hepatology and gastroenterology groups as well as patient representatives from PSC Support. The guidelines aim to support general physicians, gastroenterologists and surgeons in managing adults with PSC or those presenting with similar cholangiopathies which may mimic PSC, such as IgG4 sclerosing cholangitis. It also acts as a reference for patients with PSC to help them understand their own management. Quality of evidence is presented using the AGREE II format. Guidance is meant to be used as a reference rather than for rigid protocol-based care as we understand that management of patients often requires individual patient-centred considerations.
Subject(s)
Biliary Tract Neoplasms , Cholangitis, Sclerosing , Diagnostic Techniques, Digestive System , Immunoglobulin G4-Related Disease/diagnosis , Patient Care Management/methods , Biliary Tract Neoplasms/diagnosis , Biliary Tract Neoplasms/etiology , Cholangitis, Sclerosing/complications , Cholangitis, Sclerosing/diagnosis , Cholangitis, Sclerosing/etiology , Diagnosis, Differential , Humans , Prognosis , United KingdomABSTRACT
We report a case of ileo-colonic Histoplasmosis without apparent respiratory involvement in a patient who had previously undergone an orthotopic liver transplant (OLT) for primary biliary cholangitis 15 years earlier. The recipient lived in the United Kingdom, a non-endemic region for Histoplasmosis. However, she had previously lived in rural southern Africa prior to her OLT. The patient presented with iron deficiency anaemia, diarrhoea, abdominal pain and progressive weight loss. She reported no previous foreign travel, however, it later became known that following her OLT she had been on holiday to rural southern Africa. On investigation, a mild granulomatous colitis primarily affecting the right colon was identified, that initially improved with mesalazine. Her symptoms worsened after 18 mo with progressive ulceration of her distal small bowel and right colon. Mycobacterial, Yersinia, cytomegalovirus and human immunodeficiency virus infections were excluded and the patient was treated with prednisolone for a working diagnosis of Crohn's disease. Despite some early symptom improvement following steroids, there was subsequent deterioration with the patient developing gram-negative sepsis and multi-organ failure, leading to her death. Post-mortem examination revealed that her ileo-colonic inflammation was caused by Histoplasmosis.
Subject(s)
Histoplasma/isolation & purification , Histoplasmosis/diagnosis , Immunosuppressive Agents/adverse effects , Liver Transplantation/adverse effects , Travel-Related Illness , Abdominal Pain/blood , Abdominal Pain/diagnosis , Abdominal Pain/microbiology , Africa, Southern , Aged , Anemia, Iron-Deficiency/blood , Anemia, Iron-Deficiency/diagnosis , Anemia, Iron-Deficiency/microbiology , Cholangitis, Sclerosing/surgery , Crohn Disease/diagnosis , Diagnosis, Differential , Diarrhea/blood , Diarrhea/diagnosis , Diarrhea/microbiology , Fatal Outcome , Female , Histoplasmosis/blood , Histoplasmosis/microbiology , Humans , Immunocompromised Host , Intestinal Mucosa/microbiology , Intestinal Mucosa/pathology , Multiple Organ Failure/blood , Multiple Organ Failure/diagnosis , Multiple Organ Failure/microbiology , Time Factors , Weight LossABSTRACT
Pruritus (itch) is an important symptom of primary biliary cirrhosis (PBC), an archetypal cholestatic liver disease. Cholestatic pruritus can be a debilitating symptom causing significant deterioration in patients' quality of life. Effective management of pruritus in PBC involves awareness among clinicians to adequately assess its severity, and treatment with specific drug therapies in line with current practice guidelines. In PBC, antipruritic drugs are not universally effective and/or have significant side effects, and despite best efforts with various combinations of drugs, some patients remain significantly symptomatic, eventually opting for invasive or experimental treatments. Therefore, there is a clear unmet need for better alternative treatments for patients with refractory or intractable cholestatic pruritus. Recent advances in the understanding of pathogenesis of cholestatic pruritus and bile acid physiology have raised hopes for novel therapies, some of which are currently under trial. In this review, we aim to provide a practical guide to the management of this important and complex problem, discussing current knowledge and recent advances in the pathogenesis, summarise the evidence base for available therapeutic approaches and update potential novel future therapies for the management of pruritus in PBC.
