Sami Naveed, Bhol Kailash C, Ahmed Razzaque A
Center for Blistering Diseases, New England Baptist Hospital, and Department of Dermatology, Harvard Medical School, 188 Longwood Avenue, Boston, MA 02115, USA.
Eur J Dermatol. 2003 Jul-Aug;13(4):377-81.
Pemphigus vulgaris (PV) is an autoimmune mucocutaneous blistering disease. Recently, patients with mucosal involvement have been described to have autoantibodies to desmoglein 3 (dsg), while patients with mucocutaneous disease have autoantibodies to dsg 1 and dsg 3. The objective of this study was to prospectively analyze, over a 24-month period, the influence of intravenous immunoglobulin (i.v.Ig) therapy on autoantibody titers to dsg 3 and dsg 1, in two groups of patients with severe PV. Group A consisted of 11 patients with mucocutaneous involvement and group B consisted of 10 patients with only mucosal involvement. Levels of autoantibodies to dsg 3 and 1 were measured by ELISA, at monthly intervals. Prior to therapy initiation, group A patients' sera showed a high ELISA index value of both dsg 3 and 1 antibodies, while group B patients had a high index value to only dsg 3. During i.v.Ig therapy, a progressive decline in the ELISA index values was observed in all patients. After the initiation of i.v.Ig therapy, in group A, a statistically significant reduction (p < 0.05) in ELISA index value to dsg 3 and 1 was detected after four and five months, respectively. In Group B, a significant decline in the mean autoantibody titer values to dsg 3 (p < 0.05) was observed after six months of i.v.Ig therapy. Group A patients had a negative ELISA index value to dsg 3 and 1 after a mean period of 21 and 20 months, respectively. Group B patients had a negative dsg 3 score after a mean period of 22 months. These results indicate that autoantibody titers to dsg 3 and 1, as measured by ELISA, can be used to monitor the serological response to treatment in PV patients. A sustained serological remission is observed in patients treated with i.v.Ig therapy. .
寻常型天疱疮（PV）是一种自身免疫性黏膜皮肤水疱病。最近，有报道称黏膜受累的患者存在针对桥粒芯糖蛋白3（dsg）的自身抗体，而黏膜皮肤疾病患者则存在针对dsg 1和dsg 3的自身抗体。本研究的目的是在24个月的时间里，前瞻性分析静脉注射免疫球蛋白（i.v.Ig）疗法对两组重度PV患者中dsg 3和dsg 1自身抗体滴度的影响。A组由11例黏膜皮肤受累患者组成，B组由10例仅黏膜受累患者组成。每月通过酶联免疫吸附测定（ELISA）法检测dsg 3和1的自身抗体水平。在开始治疗前，A组患者血清中dsg 3和1抗体的ELISA指数值均较高，而B组患者仅dsg 3的指数值较高。在i.v.Ig治疗期间，所有患者的ELISA指数值均呈逐渐下降趋势。在开始i.v.Ig治疗后，A组中，分别在4个月和5个月后检测到dsg 3和1的ELISA指数值有统计学意义的降低（p < 0.05）。在B组中，i.v.Ig治疗6个月后观察到dsg 3的平均自身抗体滴度值有显著下降（p < 0.05）。A组患者在平均21个月和20个月后dsg 3和1的ELISA指数值均为阴性。B组患者在平均22个月后dsg 3评分为阴性。这些结果表明，通过ELISA法检测的dsg 3和1的自身抗体滴度可用于监测PV患者对治疗的血清学反应。接受i.v.Ig治疗的患者可观察到持续的血清学缓解。
