Endocrine Abstracts

JOINT1886

Background: Despite modern evidence-based treatment, patients with primary adrenal insufficiency (PAI) experience reduced quality of life, increased fatigue, and diminished work capacity compared to healthy individuals. Patient-reported outcome measures (PROM) can provide better insights into patients’ own experiences and perceptions, which can lead to improved follow-up and treatment.

Methods: Patients included in the Register for Organ-Specific Autoimmune Diseases (ROAS) with autoimmune PAI, aged 18 and older, completed annual electronic PROM questionnaires from 2018 to 2023. Patients answered questions regarding disease progression, medication use, work capacity, stress dosage, incidence of adrenal crises, symptoms of cortisol deficiency, and quality of life. The latter was evaluated using AddiQoL, a validated questionnaire for PAI.

Results: The number of responses increased from 179 in 2018 to 478 in 2023 (response rate 54%). At the time of diagnosis, the most common symptoms were fatigue (92%), increased pigmentation (79%), and weight loss (75%). The most frequent time interval from the first symptom to diagnosis was 3–12 months (35%, n=169), while 25% (n=119) reported symptoms occurring 1–3 years before diagnosis. In 2023, 308 patients (65%) reported using cortisone acetate tablets (median daily dose 37.5 mg), while 136 (29%) used Plenadren (median daily dose 25 mg). Plenadren use increase from 20% in 2018. A majority (70%) reported temporary dose increases in the past year. Most patients (94%) felt they had received adequate information about stress dosing of glucocorticoids, and 80% had access to hydrocortisone injections. The median daily dose for fludrocortisone was 0.13 mg. Fifty-nine patients (12%) experienced an adrenal crisis in the past year. In 2023, 267 patients (56%) had paid employment, 111 (23%) were retirees, and 123 (26%) had sick pension. A total of 131 patients (49%) reported no sick leave days, while 47 (18%) had more than 30 days of sick leave. The mean AddiQoL-30 score was 86±12 in 2023, unchanged from 2018 (86±13). The AddiQoL-8 score was also unchanged (21±5 for both years).

Conclusion: Patient-reported data provide valuable insights into the health and quality of life of patients with autoimmune PAI. The triad of fatigue, increased skin pigmentation, and weight loss were the most common symptoms at onset. Nearly one-third of patients are now treated with modified release hydrocortisone (Plenadren), and over 50% are employed. Quality of life in patients with PAI remains diminished but unchanged over the 6-year observation period.