후천성 수포 박리증 환자에서 type VII collagen의 NC1 과 NC2 recombinant protein을 이용한 ELISA 수치와 임상적 중증도의 비교

의학과/석사Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune subepidermal bullous disease characterized by circulating autoantibodies against type VII collagen. Detecting these autoantibodies is crucial for the diagnosis of this disease, and is also useful for measuring disease severity. Enzyme linked immunosorbent assay (ELISA), a quantitative method to measure anti-type VII collagen antibody levels, is currently available to diagnose EBA. The aim of this study was to validate the usefulness of type VII collagen ELISA in diagnosing EBA and monitoring disease severity. Sera from patients with EBA (n=30), bullous pemphigoid (n=20), anti-laminin γ1 pemphigoid (n=9), and healthy donors (n=24) were tested by ELISA using the recombinant noncollagenous 1 (NC1) and 2 (NC2) domains of type VII collagen. Relationships between clinical characteristics, indirect immunofluoroscence (IIF) titers and ELISA values were investigated. The sensitivity and specificity of the type VII collagen ELISA were 96.7% and 98.1%, respectively. There was no significant difference between ELISA results for classic and inflammatory types (72.6 vs. 47.4 U/ml, p=0.15). The severity of skin involvement was positively correlated with both ELISA index value (r=0.87, p<0.01) and IIF titer (r=0.59, p