Treatment Pattern of Pemphigus Vulgaris in UK : A Retrospective Cohort Study Using Clinical Practice Research Datalink (CPRD) and Hospital Episode Statistics (HES)

Speaker(s)

Blein C1, De Ruyck F2, Davidson J3, Rice C4, Wittlin B2, Iannazzo S2, Agalarov Y2
1argenx BVBA, Zwijnaarde, Belgium, 2argenx BVBA, Ghent, Belgium, 3Corevitas, Altrincham,, CHE, UK, 4Corevitas, Bristol, BST, UK

OBJECTIVES: Pemphigus diseases are life-threatening, chronic, autoimmune blistering diseases. This research aims to explore treatment pathway over time among pemphigus vulgaris (PV) in the UK.

METHODS: This retrospective cohort study included patients with PV using CPRD-HES data from January 2010 to December 2020. Eligible patients require at least two diagnosis for PV (ICD-10: L10.0 or relevant SNOMED code), aged ≥18 years, have no diagnosis of any non-vulgaris form of pemphigus (ICD-10 code L10.1, L10.2, L10.3, L10.4, L10.5, L10.8, L10.9) in HES or relevant SNOMED code in the 1 year pre-index period, and at least 12 months follow-up period. We describe the treatment pathway for incident patients, defining ,>20mg prednisolone (or equivalent) per day for a period of at least 14 days as patients under regular high dose steroids, steroid dose of less than 10mg prednisolone (or equivalent) per day as minimum remission (CRmin) and off therapy as complete remission (CR).

RESULTS: Among 339 incident patients, 65.3% started with a monotherapy treatment, mostly corticosteroid (CS 48.8%) of which only 8.1% achieved a CR and 11.5% a CRmin. As second line, 58.8% of patients still had a monotherapy, mostly CS (23.2%) with less than 5 % achieving a CR and 14.6% CRmin. As third line, 66.2% continued with monotherapy splitted into 18.6% CS and 18.6% immunosuppressants of which around 20% achieved a CR and another 20% CRmin. Finally, 72.6% have received at least a CS over the time, and 149 patients (60.6%) with regular high dose. We also observed that 62.3 % of the patients have a mean daily dose superior to 10 mg, 45.2% superior to 15 mg and 3.3% superior to 40 mg.

CONCLUSIONS: Corticosteroids are the more frequently used of therapy for PV and with a regular high dose administration. Such high doses are associated with significant side effects demonstrated in literature.

Code

EPH258

Topic

Study Approaches

Disease

Rare & Orphan Diseases, Systemic Disorders/Conditions (Anesthesia, Auto-Immune Disorders (n.e.c.), Hematological Disorders (non-oncologic), Pain)