Background: In pemphigus, loss of epidermal adhesion is induced by binding of circulating autoantibodies to the desmosomal cadherins desmoglein 3 (Dsg3) in pemphigus vulgaris (PV) and desmoglein 1 (Dsg1) in pemphigus ...Background: In pemphigus, loss of epidermal adhesion is induced by binding of circulating autoantibodies to the desmosomal cadherins desmoglein 3 (Dsg3) in pemphigus vulgaris (PV) and desmoglein 1 (Dsg1) in pemphigus foliaceus (PF), respectively. Therapeutic removal of Dsg- reactive autoantibodies by immunoadsorption (IA) has been demonstrated to exert clinical remission of the disease. Objectives: The aim of this intervention study was to evaluate the efficacy and safety of the peptide- based Globaffin adsorber system in the treatment of severe pemphigus cases. Patients and Methods: We applied IA in 4 PV and 2 PF patients with severe chronic disease resistant to conventional immunosuppressive therapy. IA was performed on 4 consecutive days, representing 1 treatment cycle, followed by a 4- week treatment- free interval. Serum samples for determining serum IgG and anti- Dsg1Dsg3 IgG autoantibodies were drawn daily before and after IA, respectively. During follow- up, patients were examined carefully, and laboratory parameters were controlled monthly for up to 1 year. Results: IA led to excellent clinical responses. Skin and mucosal lesions cleared almost completely within weeks. One IA cycle reduced anti- Dsg1 and anti- Dsg3 autoantibodies by an average of 50- 70% as determined by ELISA. Conclusions: Using the Globaffin adsorber system, IA represents an effective and safe treatment opportunity in severe and therapy- resistant pemphigus.展开更多
Objective: To evaluate the role of the enzyme-linked immunosorbent assay (ELISA) test for the detection of antibodies to desmoglein 1 (dsg1) and desmoglein 3 (dsg3) in the diagnosis of pemphigus vulgaris (PV), and its...Objective: To evaluate the role of the enzyme-linked immunosorbent assay (ELISA) test for the detection of antibodies to desmoglein 1 (dsg1) and desmoglein 3 (dsg3) in the diagnosis of pemphigus vulgaris (PV), and its correlation with disease severity and clinical presentation (mucosal PV, cutaneous PV, mucocutaneous PV). Methods: Twenty-seven active PV patients and 26 controls with other dermatologic disorders were included in the study. The severity of oral and cutaneous involvement was assessed and recorded. ELISA test for the measurement of anti-dsg1 and anti-dsg3 antibodies was performed (Medical and Biological Laboratories Co. Ltd., Nagoya, Japan). The cut-off ELISA value for both anti-dsg1 and anti-dsg3 was taken as 20. Results: Of the 27 patients, 26 were ELISA positive for anti-dsg1 antibodies and 23 for anti-dsg3 antibodies. Of the controls, two were positive for anti-dsg1 and none for anti-dsg3 antibodies. The sensitivity and specificity of ELISA for anti-dsg1 in the diagnosis of PV were 96.3% and 92.3% , respectively. For anti-dsg3, they were 85.2% and 100% , respectively. The different morphologic types of PV could not be differentiated on the basis of antibody profile; however, a direct correlation between anti-dsg3 titers and the severity of oral disease was noted, and also between antidsg1 titers and the severity of cutaneous disease. Conclusions: ELISA (dsg1 and dsg3) is an efficient tool for confirming the diagnosis of PV. Specific antibody titers correlate with disease severity; however, desmoglein testing cannot differentiate between the various morphologic subtypes of PV.展开更多
天疱疮疾病面积评分(Pemphigus disease area index,PDAI)是评估天疱疮病情活动度众多评分体系中的可靠指标;而抗桥粒芯糖蛋白(Desmoglein,Dsg)抗体,大多学者认为与病情活动度呈正相关,但目前对此尚未达成共识。因此或许可以通过验证PDA...天疱疮疾病面积评分(Pemphigus disease area index,PDAI)是评估天疱疮病情活动度众多评分体系中的可靠指标;而抗桥粒芯糖蛋白(Desmoglein,Dsg)抗体,大多学者认为与病情活动度呈正相关,但目前对此尚未达成共识。因此或许可以通过验证PDAI值与抗Dsg抗体滴度的相关性来证实抗体滴度与病情活动度的关系,并进一步界定出病情轻中重度所对应的抗Dsg抗体滴度区间,指导治疗。国内尚无类似文献报道,还需要进一步的临床研究来证实。展开更多
文摘Background: In pemphigus, loss of epidermal adhesion is induced by binding of circulating autoantibodies to the desmosomal cadherins desmoglein 3 (Dsg3) in pemphigus vulgaris (PV) and desmoglein 1 (Dsg1) in pemphigus foliaceus (PF), respectively. Therapeutic removal of Dsg- reactive autoantibodies by immunoadsorption (IA) has been demonstrated to exert clinical remission of the disease. Objectives: The aim of this intervention study was to evaluate the efficacy and safety of the peptide- based Globaffin adsorber system in the treatment of severe pemphigus cases. Patients and Methods: We applied IA in 4 PV and 2 PF patients with severe chronic disease resistant to conventional immunosuppressive therapy. IA was performed on 4 consecutive days, representing 1 treatment cycle, followed by a 4- week treatment- free interval. Serum samples for determining serum IgG and anti- Dsg1Dsg3 IgG autoantibodies were drawn daily before and after IA, respectively. During follow- up, patients were examined carefully, and laboratory parameters were controlled monthly for up to 1 year. Results: IA led to excellent clinical responses. Skin and mucosal lesions cleared almost completely within weeks. One IA cycle reduced anti- Dsg1 and anti- Dsg3 autoantibodies by an average of 50- 70% as determined by ELISA. Conclusions: Using the Globaffin adsorber system, IA represents an effective and safe treatment opportunity in severe and therapy- resistant pemphigus.
文摘Objective: To evaluate the role of the enzyme-linked immunosorbent assay (ELISA) test for the detection of antibodies to desmoglein 1 (dsg1) and desmoglein 3 (dsg3) in the diagnosis of pemphigus vulgaris (PV), and its correlation with disease severity and clinical presentation (mucosal PV, cutaneous PV, mucocutaneous PV). Methods: Twenty-seven active PV patients and 26 controls with other dermatologic disorders were included in the study. The severity of oral and cutaneous involvement was assessed and recorded. ELISA test for the measurement of anti-dsg1 and anti-dsg3 antibodies was performed (Medical and Biological Laboratories Co. Ltd., Nagoya, Japan). The cut-off ELISA value for both anti-dsg1 and anti-dsg3 was taken as 20. Results: Of the 27 patients, 26 were ELISA positive for anti-dsg1 antibodies and 23 for anti-dsg3 antibodies. Of the controls, two were positive for anti-dsg1 and none for anti-dsg3 antibodies. The sensitivity and specificity of ELISA for anti-dsg1 in the diagnosis of PV were 96.3% and 92.3% , respectively. For anti-dsg3, they were 85.2% and 100% , respectively. The different morphologic types of PV could not be differentiated on the basis of antibody profile; however, a direct correlation between anti-dsg3 titers and the severity of oral disease was noted, and also between antidsg1 titers and the severity of cutaneous disease. Conclusions: ELISA (dsg1 and dsg3) is an efficient tool for confirming the diagnosis of PV. Specific antibody titers correlate with disease severity; however, desmoglein testing cannot differentiate between the various morphologic subtypes of PV.
文摘天疱疮疾病面积评分(Pemphigus disease area index,PDAI)是评估天疱疮病情活动度众多评分体系中的可靠指标;而抗桥粒芯糖蛋白(Desmoglein,Dsg)抗体,大多学者认为与病情活动度呈正相关,但目前对此尚未达成共识。因此或许可以通过验证PDAI值与抗Dsg抗体滴度的相关性来证实抗体滴度与病情活动度的关系,并进一步界定出病情轻中重度所对应的抗Dsg抗体滴度区间,指导治疗。国内尚无类似文献报道,还需要进一步的临床研究来证实。
