Autoimmune Bullous Diseases Associated With Immune Checkpoint Inhibitors: An Analysis Based on a Systematic Review

BACKGROUND: Autoimmune bullous diseases (AIBDs) constitute a rare yet potentially life-threatening subset of immune-related adverse events induced by immune checkpoint inhibitors (ICIs). However, the characteristics of ICI-induced AIBDs (ICI-AIBDs) and the factors influencing patient survival remain incompletely characterized. Therefore, we aimed to synthesize the available information on ICI-AIBD patients and sought to explore factors potentially influencing the survival outcome of this population. METHODS: A systematic search of 5 databases was conducted. Cox regression analysis was used to identify potential factors affecting patient survival outcomes. RESULTS: Finally, a total of 188 studies with 319 participants were analyzed. The spectrum of AIBDs comprised bullous pemphigoid (n = 254, 79.6%), lichen planus pemphigoides (n = 34, 10.7%), mucous membrane pemphigoid (n = 11, 3.4%), pemphigus group (n = 7, 2.2%), linear IgA bullous dermatosis (n = 5, 1.6%), primarily induced by programmed cell death protein 1 inhibitors. In the exploratory analysis, female (hazard ratio [HR], 2.35; 95% confidence interval [CI], 1.10-5.02; p = 0.020) and pemphigus group (HR, 7.09; 95% CI, 1.87-26.96; p = 0.003) were potentially associated with higher mortality, whereas topical glucocorticoid therapy was potentially protective (HR, 0.44; 95% CI, 0.21-0.93; p = 0.025). CONCLUSIONS: In conclusion, we delineate the full clinical spectrum of ICI-AIBDs, tentatively exploring factors potentially affecting patients' survival, which provides insights for individualized therapy and may inform future clinical practice.