Mortality and Prognostic Factors Associated With Bullous Pemphigoid



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• A total of 572 patients with bullous pemphigoid (BP) were included in this retrospective study from Italy to investigate the disease mortality rates and potential predictors of mortality. The authors emphasized the prognostic value of anti-BP180 and anti-BP230 autoantibody levels, specifically anti-BP180 levels >72 U/mL and anti-BP230 levels >3 U/mL. Additionally, they proposed a BP mortality tool using these biomarker levels, age (particularly age >78 years), and multiple comorbidities among other risk factors.

• Combining anti-BP180 and anti-BP230 autoantibody levels with specific risk factors may allow clinicians to provide patients with BP with a more accurate prognosis.

–  Ruth McTighe, MD

Abstract 

INTRODUCTION

Bullous pemphigoid is the most common autoimmune bullous dermatosis. In recent years several studies have tried to identify the main factors of the disease related with an increased risk of death. The aim of this multicenter Italian study was to assess the risk score of death considering epidemiologic, clinical, immunological, and therapeutic factors in a cohort of patients affected by bullous pemphigoid and try to identify the cumulative survival up to 120 months.

METHODS

We retrospectively reviewed the medical records of patients with bullous pemphigoid who were diagnosed between 2005 and 2020 in the 12 Italian centers. Data collected included sex, age at the time of diagnosis, laboratory findings, severity of disease, time at death/censoring, treatment, and multimorbidity.

RESULTS

A total of 572 patients were included in the study. The crude mortality rate was 20.6%, with an incidence mortality rate of 5.9 × 100 person/year. The mortality rate at 1, 3, 5, and 10 years was 3.2%, 18.2%, 27.4% and 51.9%, respectively. Multivariate model results showed that the risk of death was significantly higher in patients older than 78 years, in presence of multimorbidity, anti-BP180 autoantibodies >72 U/mL, or anti-BP230 > 3 U/mL at diagnosis. The variables jointly included provided an accuracy (Harrel's Index) of 77% for predicting mortality.

CONCLUSION

This study represents the first nationwide Italian study to have retrospectively investigated the mortality rates and prognostic factors in patients with bullous pemphigoid. A novel finding emerged in our study is that a risk prediction rule based on simple risk factors (age, multimorbidity, steroid-sparing drugs, prednisone use, and disease severity) jointly considered with two biomarkers routinely measured in clinical practice (anti-BP230 and anti-BP180 autoantibodies) provided about 80% accuracy for predicting mortality in large series of patients with this disease.

Journal of the European Academy of Dermatology and Venereology: JEADV

Mortality and prognostic factors in patients with bullous pemphigoid: a retrospective multicenter Italian study

J Eur Acad Dermatol Venereol 2022 Jul 20;[EPub Ahead of Print], F Bardazzi, F Filippi, MA Chessa, M Iommi, C Loi, A Campanati, G Rizzetto, C Tagliati, L Atzori, S Muratori, G Genovese, P Gisondi, D Schena, R Balestri, G Rech, C Feliciani, C Lasagni, L Bigi, C De Simone, G Di Zenzo, F Moro, A Borghi, V Di Lernia, G D'Arrigo, G Tripepi, M Gori, A Pitino 

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Benjamin Hidalgo-Matlock
Skin Care Physicians of Costa Rica

Clinica Victoria en San Pedro: 4000-1054
Momentum Escazu: 2101-9574

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