Other therapies
Fatigue is a nonspecific but often-reported symptom in PBC, and represents an unmet need since no therapies are licensed. A structured approach to management, quantifying fatigue and its impacts (through the use of disease-specific tools such as the PBC-40 quality-of-life measures), addressing contributing and exacerbating factors, and supporting patients to cope with its impact is effective. Drugs such as coenzyme Q and rituximab have been shown to be ineffective. A graded programme of exercise helps some individuals. People with PBC may have poor lipid-dependent absorption of oil-soluble vitamins (A, D, E, and K). Appropriate supplementation is recommended when bilirubin is elevated. People with PBC are at elevated risk of developing osteoporosis as compared to the general population and others with liver disease. Screening and treatment of this complication is an important part of the management of PBC. As in all liver diseases, consumption of alcohol should be restricted or eliminated. In patients with advanced liver disease, the only curative therapy is liver transplant. Outcomes are favourable, with five-year patient survival rates better than for most other indications for LT (80–85%). Prognosis
The introduction of UDCA has dramatically changed the pattern and the course of the disease. Numerous trials and observational studies have demonstrated its efficacy on liver biochemistry, histological progression, and transplant-free survival.Among the UDCA-treated patients, the degree of the liver biochemistry improvement, i.e. the UDCA-response, identifies patients with different long-term prognosis.
Airway management
Airway management has been found to be the most important factor in treating patients with Ludwigs Angina, i.e. it is the “primary therapeutic concern”. Airway compromise is known to be the leading cause of death from Ludwigs Angina. The basic method to achieve this is to allow the patient to sit in an upright position with supplemental oxygen provided by masks or nasal prongs. Patients airway can rapidly deteriorate and therefor close observation and preparation for more invasive methods such as endotracheal intubation or tracheostomy if needed is vital. If the oxygen saturation levels are adequate and antimicrobials have been given, simple airway observation can be done. This is a suitable method to adopt in the management of children, as a retrospective study described that only 10% of children required airway control. However, a tracheostomy was performed on 52% of those affected with Ludwigs Angina over 15 years old. If more invasive or surgical airway control is necessary, there are multiple things to considerFlexible nasotracheal intubation require skills and experience. If nasotracheal intubation is not possible, cricothyrotomy and tracheostomy under local anaesthetic can be done. This procedure is carried out on patients with advanced stage of Ludwigs Angina. Endotracheal intubation has been found to be in association with high failure rate with acute deterioration in respiratory status. Elective tracheostomy is described as a safer and more logical method of airway management in patients with fully developed Ludwigs Angina. Fibre-optic nasoendoscopy can also be used, especially for patients with floor of mouth swellings.