寻常型天疱疮诊断和治疗专家建议(2020)

Diagnosis and treatment of pemphigus vulgaris:an expert proposal(2020)

寻常型天疱疮是一类严重威胁人类健康的自身免疫性大疱性疾病。为了规范该病的诊治,邀请国内该领域著名学者进行了反复论证,提出了该建议。根据临床表现的不同,寻常型天疱疮分为皮肤黏膜型、皮肤型和黏膜主导型。病理表现为基底层上棘层松解。根据临床表现、组织病理、免疫荧光和血清抗桥粒芯糖蛋白抗体检测等诊断。系统应用糖皮质激素是一线治疗方案,轻、中、重度患者应用不同剂量。中重度患者早期可联合应用免疫抑制剂或利妥昔单抗治疗,在应用生物制剂时建议联合静脉注射免疫球蛋白。必要时可采用血浆置换、干细胞移植等。治疗过程中要严密观察病情变化,减少不良反应发生。

Pemphigus vulgaris is a kind of autoimmune bullous disease which seriously threatens human health.In order to standardize the diagnosis and treatment of this disease,domestic famous experts in this field were invited to make the recommendations after rigorous discussion.According to clinical manifestations,pemphigus vulgaris can be divided into cutaneous-mucosal type,cutaneous type and mucosal-dominant type.Its pathological manifestations are characterized by acantholysis above the basal layer.According to clinical manifestations,histopathological features,immunofluorescence findings and serum anti-desmoglein antibody levels,the diagnosis of pemphigus vulgaris can be made.Systemic glucocorticoids are the first-line treatment,and the doses of glucocorticoids should vary with disease severity.Early combination with immunosuppressive agents or rituximab is recommended for the treatment of patients with moderate or severe pemphigus vulgaris,and intravenous immunoglobulin is recommended to be combined with biological agents.If necessary,plasma exchange and stem cell transplantation can be performed.In the course of treatment,changes of patients′condition should be closely observed to reduce the occurrence of adverse reactions.