ÌÇÐÄvlog

QuestionÌý Are serum levels of immunoglobulin G (IgG) autoantibodies to bullous pemphigoid (BP) antigens 180 (BP180) and 230 (BP230) correlated with BP disease severity?

FindingsÌý This systematic review and meta-analysis including 14 studies found moderate to strong correlation of anti-BP180 IgG autoantibody serum levels with BP disease severity measured by enzyme-linked immunosorbent assay per the Autoimmune Bullous Skin Disorder Intensity Score or the Bullous Pemphigoid Disease Area Index. However, no correlation was found between anti-BP230 IgG autoantibody levels and BP disease severity scores.

MeaningÌý These findings indicate that anti-BP180 IgG levels may serve as an adjunctive tool for monitoring BP disease severity and informing clinical practice for BP treatment and management.

ImportanceÌý The correlation between serum levels of autoantibodies against bullous pemphigoid (BP) antigens 180 (BP180) and 230 (BP230) with BP disease severity is unclear.

ObjectiveÌý To investigate the correlation of anti-BP180 and anti-BP230 immunoglobulin G (IgG) antibody levels with BP disease severity.

Data SourcesÌý A search was performed of the Cochrane Central Register of Controlled Trials, Embase, and PubMed databases from their respective inception to April 11, 2024.

Study SelectionÌý Studies evaluating the correlation between serum levels of anti-BP180 or anti-BP230 IgG measured using enzyme-linked immunosorbent assay (ELISA) and disease severity assessed per the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) or BP Disease Area Index (BPDAI) were included. No language or geographic restrictions were imposed. Nearly 0.4% of initially identified studies met the selection criteria.

Data Extraction and SynthesisÌý One researcher extracted data and another researcher confirmed data. The risk of bias was independently assessed by these researchers using the Quality Assessment of Diagnostic Accuracy Studies 2 tool, with discrepancies resolved by discussion with a third researcher. A random-effects model meta-analysis and a subgroup analysis were conducted based on the ELISA kit manufacturers.

Main Outcomes and MeasuresÌý Pooled correlation coefficients of antibody levels with ABSIS and BPDAI.

ResultsÌý In all, 14 studies with 1226 participants were analyzed. The risk of bias of included studies was generally low. The meta-analysis found anti-BP180 autoantibody levels showed moderate correlation with objective BPDAI (r = 0.56; 95% CI, 0.46-0.64) at baseline, strong correlation (r = 0.63; 95% CI, 0.39-0.79) at 3-month follow-up, and moderate correlation (r = 0.53; 95% CI, 0.25-0.72) at 6-month follow-up. Anti-BP180 autoantibody levels also showed moderate correlation (r = 0.52; 95% CI, 0.39-0.62) with ABSIS at baseline, strong correlation (r = 0.62; 95% CI, 0.39-0.79) at 3-month follow-up, and moderate correlation (r = 0.53; 95% CI, 0.25-0.72) at 6-month follow-up. By contrast, anti-BP230 autoantibody levels showed no association with objective BPDAI and ABSIS at diagnosis and follow-up. The subgroup analysis found similar results when using different ELISA kits.

Conclusions and RelevanceÌý The findings of this systematic review and meta-analysis indicated that anti-BP180 autoantibody levels may serve as an adjunctive tool for monitoring BP disease severity and guiding clinical care for patients with BP.