EASL Clinical Practice Guidelines

Management issues, quality of life, and delivery of care

Delivery of care

AIH is a rare disease and patients require access to care from medical professionals with expertise in AIH, according to the evolving evidence on best practice. Currently AIH care is not organized and this has resulted in uneven distribution of health care delivery [321]. In order to overcome this, the German rare disease plan in accordance with EU guidance calls for a tiered care model that involves assignment of three types of clinical care centres with increasing expertise [322]. According to this model, patients should have access to referral and to specialist centres. Referral is encouraged for aspects such as diagnostic uncertainties, prognostic assessment, and exploration of optimal disease management strategies and management of (liver) related complications. Depending on the complexity of the clinical issue, the patient may move up and down the ladder of care. The delivery of such care will depend on the local, regional or national healthcare services, but will improve the efficiency of healthcare provision. Coordinated care is likely to reduce the unnecessary duplication of tests, facilitate targeting of novel diagnostic and therapeutic interventions tailored to clinical need and expected benefit and has the potential for improved patient outcomes. Access to specialist care should also result in better quality standards for laboratory testing, in particular for immunoserology. There is evidence of integration benefits and impressive cost savings in the care of patients with rare diseases [323].

Practical integrated patient support

The first diagnosis contact between the patient and physician is of major importance. Anxiety and distress levels can be alleviated if the physician recognizes the patient’s psychosocial needs and conveys reassurance and support during the first consultation [324]. However, there are no studies about the ways to communicate AIH to the newly diagnosed and how patients respond to the diagnosis. There is evidence to suggest that the expertise on AIH is unevenly distributed among health care professionals which may lead to misconceptions. Patient education programs and tools for patients with liver diseases are available, but there is little research about their implementation, effectiveness, and relevance for AIH. AIH patients need simple, disease specific information initially with practical implications such as potential impact on work, insurance, lifestyle and family planning. This allows them to engage fully in shared decision-making. Patients should be provided with contacts and access to local or national support groups. Consultations with AIH should be individualised, reassuring and tailored to the patient’s literacy level and culture/language and retain a positive attitude.

Patient reported outcomes

Instruments such as Patient reported Outcome Measures (PROM) have been developed mainly as endpoints for clinical trials. PROM provides insight into the patient perspective on the impact of disease and treatment, and have the potential to identify those treatment strategies that benefit the HRQoL of most patients. The clinical trials executed in AIH have used biochemical markers of response and the evidence suggests that biochemical improvement does not immediately translate in to higher HRQoL levels. The development of PROM may assist here to track and trace the needs of AIH patients. However there is no disease specific questionnaire available nor has a validation of PROM for AIH been developed.

Quality control

Expert centres for the care of patients with autoimmune hepatitis should evaluate the quality of the clinical services by a variety of quality control measures. These should include survival statistics, critical incident reporting systems as well as peer and patient involvement in quality control measurements. Key results should be published, and clinical research projects should be undertaken and supported. Both physician training and further education as well as patient education measures should be in place, in addition to consultation services for referring physicians.

Quality of life

AIH is a chronic liver disease with flares and remissions that may impart significant medical and economic burdens on patients’ life and health care delivery systems. The physical and psychological burden to AIH patients are significant, yet incompletely characterized [325]. In a survey among members of the Dutch Liver patients’ organization, HRQoL was investigated using three instruments including the extended version of the disease specific liver disease symptom index 1.0 (LDSI 2.0), the Dutch Short Form 36, and MFI-20. Patients with AIH (n = 142) scored lower in all SF-36 scales, but particularly in scales that measured role limitations due to physical problems or general health. AIH patients report more fatigue as assessed with the MIF-20 questionnaire [326]. Another cross-sectional study in 24 children with AIH or with PSC/AIH overlap using the PedsQL 4.0 instrument demonstrated significant impairment of HRQoL which was associated with the presence of frequent liver disease related symptoms. Particularly, abdominal pain, fatigue, and mood symptoms negatively affected HRQoL results [327]. The most definitive study to date, considered HRQoL using the 12-item Short Form Health Survey (SF-12) in 103 AIH patients [36]. Some 77% patients were in biochemical remission. While physical component scores did not deviate from the general population, the mental component scores were significantly reduced. The authors observed a high rate of severe depression and anxiety symptom levels in AIH that were related to (often unwarranted) concerns about the inheritance, status and trajectory of AIH. Despite the fact that AIH was clinically stable in the majority, there was a high rate of major depressive symptoms. The authors detected a correlation between use of prednisolone and depression, suggesting that one of our treatment goals in AIH should be to develop steroid-free treatment.