关键词: 天疱疮, 桥粒芯糖蛋白质1, 桥粒芯糖蛋白质3, 临床表型, 疾病活动度

Abstract: 【Abstract】 Objective To investigate associations of anti-desmoglein （Dsg1 and Dsg3） antibodies detected by enzyme-linked immunosorbent assay （ELISA） with clinical phenotypes and disease activity in pemphigus patients, and to explore their change patterns. Methods A total of 111 patients with pemphigus were enrolled from Hospital for Skin Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College between January 2015 and January 2018. ELISA was performed to detect serum levels of anti-Dsg1 and anti-Dsg3 antibodies in these patients with different clinical types of pemphigus at different stages, including onset stage, control stage （no erythema or vesicles occurred in the last 2 or more weeks, and primary lesions began to regress）, maintenance stage （the condition had been stable for ≥ 1 month, and treatment was maintained with a low dose of glucocortiroids ［prednisone equivalent of < 15 mg/d］）, and recurrence stage, and the change patterns of serum levels of anti-Dsg1 and anti-Dsg3 antibodies were analyzed. Statistical analysis was carried out with SPSS 22 software by using one-way analysis of variance for the comparison among groups, and least significant difference （LSD）-t test for multiple comparisons. Results At the disease onset stage, control stage, maintenance stage and recurrence stage, 92, 53, 33, and 9 patients respectively completed the detection. Among the 92 patients with initial onset of pemphigus, the positive rates of anti-Dsg1 and anti-Dsg3 antibodies were 100% and 2.77% respectively in 36 patients with pemphigus foliaceus, 20% and 80% respectively in 10 with mucosal-dominant pemphigus vulgaris, and 97.82%, 95.65% respectively in 46 with mucocutaneous pemphigus vulgaris. The serum levels of anti-Dsg1 antibodies in the patients with pemphigus foliaceus significantly differed among the disease onset stage, control stage, maintenance stage and recurrence stage（［137.43 ± 77.74］, ［13.94 ± 14.81］, ［21.50 ± 58.33］, ［121.13 ± 86.89］U/ml, respectively）, the serum levels of anti-Dsg3 antibodies in the patients with mucosal-dominant pemphigus vulgaris also significantly differed among the above clinical stages（［125.61 ± 94.81］, ［34.5 ± 16.26］, 0.6, 258 U/ml, respectively）, and the serum levels of anti-Dsg1 and anti-Dsg3 antibodies in patients with mucocutaneous pemphigus vulgaris both significantly differed among the above clinical stages（anti-Dsg1 antibody: ［115.39 ± 70.62］, ［15.74 ± 25.10］, ［3.62 ± 12.09］, ［78.60 ± 92.25］U/ml, respectively; anti-Dsg3 antibody: ［137.98 ± 81.25］, ［58.14 ± 63.46］, ［29.26 ± 64.70］, ［136.9 ± 101.47］ U/ml, respectively）. Additionally, the serum levels of anti-Dsg1 antibodies in the patients with pemphigus foliaceus, as well as the serum levels of anti-Dsg3 antibodies in the patients with mucosal-dominant pemphigus vulgaris and those with mucocutaneous pemphigus vulgaris, were both significantly lower at the disease control stage and maintenance stage than at the disease onset stage and recurrence stage （all P < 0.05）. During the treatment, epitope spreading occurred in 2 patients, and high-titer anti-Dsg antibodies were observed in 4 patients at the stable stage. Conclusion Anti-Dsg antibody spectrum is associated with clinical phenotypes of pemphigus, and its serum levels measured by ELISA can be applied to disease activity monitoring and evaluation of therapeutic efficacy.

Key words: Pemphigus, Desmoglein 1, Desmoglein 3, Clinical phenotype, Disease activity