ஜர்னல் ஆஃப் கிளினிக்கல் & எக்ஸ்பெரிமென்டல் டெர்மட்டாலஜி ரிசர்ச்
திறந்த அணுகல்

சுருக்கம்

Outcomes of Pemphigus Treatments at the Royal Melbourne Hospital over 5 Years on Immune Suppressive Therapies used over this Time: A Retrospective Analysis

Sonja Sokolovska, George A Varigos and Laura Scardamaglia

Background: Pemphigus is a rare, autoimmune blistering condition, resulting in significant morbidity and mortality. It warrants treatment by various immunosuppressive agents, which also contribute to significant morbidity and mortality. Newer treatment agents such as intravenous rituximab may be more effective or better tolerated. The aim of this study is to determine the patient responses to various Pemphigus treatments, and how these treatments have affected the patient’s quality of life during their course of management.

Methods: All patients who have been treated for biopsy confirmed Pemphigus vulgaris at the Royal Melbourne Hospital qualified to have their medical records retrieved for the purposes of this quality assurance audit. The medical records of the entire cohort of 21 Pemphigus patients treated at the Royal Melbourne Hospital for Pemphigus vulgaris between May 2009 and May 2014 (inclusive) were retrieved for analysis. Relevant data pertaining to their particular Pemphigus treatment and how it affected their quality of life was extracted, as well as details regarding potential confounding factors, including medical co morbidities and concurrent medications. The PDAI (Pemphigus disease activity index) (Murrell et al. 2008) and the ABSIS (autoimmune bullous skin disorder intensity score) (Pfutze et al. 2007) were retrieved from each patient’s record, with their scores being determined by the lesion extent and severity that was documented in the patient record template (Department of Dermatology, Royal Melbourne Hospital). PDAI and ABSIS values were recorded in Excel Database format as documented for each patient before their Pemphigus treatment was commenced, and upon their most current treatment review appointment. Both pre and post treatment ABSIS and PDAI scores were then analysed using SPSS (IBM, Armonk, NY, USA) statistical software.

Results: With the exception of 2 severe Pemphigus cases where treatment is ongoing, all of the Royal Melbourne Hospital patients have their Pemphigus treatments documented as significantly improving their overall quality of life. The relevant scoring of disease severity was markedly reduced across all subjects.

Conclusions: The treatment of Pemphigus vulgaris at The Royal Melbourne Hospital leads to significant improvement in the quality of life of its Pemphigus patients. Potential implications of this study may provide foundations for the development of a formal Pemphigus disease and treatment registry to enable adequate patient follow up and monitor their ongoing care. There is also potential for newer measures of Pemphigus treatment outcomes, which may be rigorously applied in dermatology clinics to allow a more objective measure of clinical treatment endpoints.