Behera Biswanath, Palit Aparna, Sahu Suchanda, Sahoo Soumya S, Sethy Madhusmita
Department of Dermatology, Venereology and Leprology, All India Institute of Medical Sciences, Bhubaneswar, Bhubaneswar, IND.
Department of Dermatology and Venereology, All India Institute of Medical Sciences, Kalyani, Kalyani, IND.
Cureus. 2025 Jun 10;17(6):e85679. doi: 10.7759/cureus.85679. eCollection 2025 Jun.
Background Pemphigus is a rare autoimmune blistering disorder characterized by involvement of the skin and mucous membranes, primarily due to autoantibodies targeting desmogleins. Emerging evidence has pointed to a potential pathogenic role of antimuscarinic acetylcholine receptor (anti-M-AChR) antibodies and reported a correlation between their titers and pemphigus disease activity. However, data on this association, particularly in relation to different phases of pulse therapy in the Indian context, remain limited. Aim This study aimed to evaluate the correlation between anti-M-AChR antibody titers and pemphigus disease activity at baseline and after Phase I of dexamethasone-cyclophosphamide/azathioprine pulse therapy. Materials and methods This prospective longitudinal observational study included newly diagnosed cases of pemphigus confirmed through histopathology and direct immunofluorescence from April 2019 to March 2021. Pemphigus Disease Area Index (PDAI) scores were recorded, and eligible patients received dexamethasone-cyclophosphamide or dexamethasone-azathioprine pulse therapy as appropriate. Results A total of 29 patients were enrolled: 23 (79%) with pemphigus vulgaris, five (17%) with pemphigus foliaceus, and one (3.4%) with pemphigus erythematosus. Patient ages ranged from 21 to 69 years, with a male-to-female ratio of 0.7:1. By the end of Phase I therapy, 20 patients (69%) completed follow-up. The mean baseline anti-M-AChR antibody titer was 76.48 ± 48.12 U/mL, which decreased to 53.61 ± 30.97 U/mL after Phase I. At that point, PDAI scores showed a moderate correlation with anti-M-AChR antibody levels (r = 0.51, p = 0.02). The reduction in antibody titers from baseline to the end of Phase I was statistically significant (p = 0.05). Conclusions While serum anti-M-AChR antibody titers may not reliably indicate disease activity at the time of diagnosis, their levels appear to reflect changes in disease activity following treatment. This suggests their potential use as a prognostic marker during the course of therapy.
天疱疮是一种罕见的自身免疫性水疱性疾病,其特征为皮肤和黏膜受累,主要是由于针对桥粒芯糖蛋白的自身抗体所致。新出现的证据表明抗毒蕈碱型乙酰胆碱受体(anti-M-AChR)抗体具有潜在的致病作用,并报道了其滴度与天疱疮疾病活动度之间的相关性。然而,关于这种关联的数据,特别是在印度背景下与脉冲治疗不同阶段相关的数据仍然有限。
本研究旨在评估地塞米松-环磷酰胺/硫唑嘌呤脉冲治疗I期前后,anti-M-AChR抗体滴度与天疱疮疾病活动度之间的相关性。
这项前瞻性纵向观察性研究纳入了2019年4月至2021年3月期间通过组织病理学和直接免疫荧光确诊的新诊断天疱疮病例。记录天疱疮疾病面积指数(PDAI)评分,符合条件的患者根据情况接受地塞米松-环磷酰胺或地塞米松-硫唑嘌呤脉冲治疗。
共纳入29例患者:寻常型天疱疮23例(79%),落叶型天疱疮5例(17%),红斑型天疱疮1例(3.4%)。患者年龄在21至69岁之间,男女比例为0.7:1。到I期治疗结束时,20例患者(69%)完成了随访。基线时anti-M-AChR抗体平均滴度为76.48±48.12 U/mL,I期后降至53.61±30.97 U/mL。此时,PDAI评分与anti-M-AChR抗体水平呈中度相关性(r = 0.51,p = 0.02)。从基线到I期结束时抗体滴度的降低具有统计学意义(p = 0.05)。
虽然血清anti-M-AChR抗体滴度在诊断时可能无法可靠地指示疾病活动度,但其水平似乎反映了治疗后疾病活动度的变化。这表明它们在治疗过程中作为预后标志物的潜在用途。