Dr Hirschfield suspects that the poor early detection rate may also be due to an over-emphasis on lifestyle liver diseases, such as alcoholic liver disease. PBC is not associated with lifestyle factors, such as alcohol consumption or obesity, and GPs may be unlikely to suspect liver disease in women who have healthy lifestyles. According to research by the PBC Foundation, sufferers are frequently misdiagnosed with other conditions, particularly stress, depression or anxiety.

“We estimate that around 8,000 people are currently living undiagnosed with the condition in the UK, which is unacceptable,” says Collette Thain OBE, founder and CEO of the PBC Foundation UK, who herself went misdiagnosed for over 15 years. “There needs to be more commitment to raising awareness of the condition among GPs and frontline healthcare professionals, so that when high-risk women come in complaining of these symptoms, they are not assumed to be menopausal or neurotic but are tested and treated correctly.”

The charity is hoping to raise awareness of PBC among healthcare professionals and the public and is encouraging GPs and pharmacists to be alert to patients, particularly middle-aged women who may have other autoimmune conditions, presenting with the symptoms. Due to its prevalence, the average GP surgery or pharmacy is likely to have patients with PBC.

“An important challenge is for patients to be diagnosed as soon as possible by their GPs and also for everyone to recognise that there are many important liver diseases that have nothing to do with alcohol use,” says Dr Hirschfield. Pharmacists also have a role in improving patient outcomes following diagnosis through supporting adherence to medication.

According to Dr Riadh Jazrawi, former hepatologist and medical director of Dr Falk Pharma UK, outcomes are worse in the UK than in other parts of Europe, possibly due to the fact that “the majority of patients are managed by gastroenterologists rather than hepatologists”. Adherence to ursodeoxycholic acid can be affected by factors such as depression, dementia and polypharmacy, while weight changes may impact on dosage.

“Even if patients are given the correct dose initially, their weight might increase between hospital appointments and thus the initial correct dose becomes inadequate,” says Dr Jazrawi. “Pharmacists should be aware of this.”