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利妥昔单抗治疗天疱疮的经验:一项来自喜马拉雅山次区域某邦的三年回顾性研究。

Experience of Rituximab Therapy in Pemphigus: A Three-Year Retrospective Study from a Sub-Himalayan State.

作者信息

Verma Ghanshyam, Sharma Aditi, Rattan Renu, Negi Ajeet, Gupta Mudita, Sharma Rajni

机构信息

Department of Dermatology, Indira Gandhi Medical College, Shimla, HP, India.

出版信息

Indian J Dermatol. 2022 Jul-Aug;67(4):477. doi: 10.4103/ijd.ijd_169_22.

Abstract

BACKGROUND

Pemphigus is a group of auto-immune blistering disorders, characterised clinically by mucocutaneous blisters and erosions and histopathologically by intra-epidermal acantholysis. It was traditionally associated with high morbidity and mortality. The use of rituximab has brought upon a new dawn in the treatment of pemphigus.

AIM

A retrospective analysis to ascertain the efficacy, tolerance, adverse effect profile, remission, and relapse with the use of rituximab.

MATERIAL AND METHODS

A retrospective analysis of all diagnosed pemphigus patients who received rituximab therapy over a period of 3 years was performed. The patient's baseline characteristics, disease duration, clinical presentations, mucosal involvement, disease-severity assessment, and adverse events with rituximab were noted. The outcomes were evaluated based on the definitions of the disease-outcome parameters as early and late endpoints.

RESULTS

Of the 17 pemphigus patients, there were 14 females (82.4%) and three males (17.6%) with a mean age of 35.9 ± 16.5 years (range: 9-65 years). Pemphigus vulgaris (PV) was the predominant type in 11 (64.7%) patients. After rituximab infusion, the 17 patients attained the end of consolidation phase (ECP) within 15 days to 3 months, and the mean duration was 1.24 months. The complete remission (CR on/off) ranged from 0.5 to 35 months, and the mean duration of remission was 21.7 months. Within a median time of 4.2 months, almost 80% patients achieved CR on therapy. Nine (53%) patients were in CR without any therapy till the end of the study period, and eight (47%) were in remission while on minimal therapy.

CONCLUSION

Rituximab is an efficacious therapeutic agent for pemphigus and is better tolerated and safer to all the previous medications used in the treatment.

摘要

背景

天疱疮是一组自身免疫性水疱性疾病,临床特征为黏膜皮肤水疱和糜烂,组织病理学特征为表皮内棘层松解。传统上,它与高发病率和死亡率相关。利妥昔单抗的使用为天疱疮的治疗带来了新的曙光。

目的

进行一项回顾性分析,以确定使用利妥昔单抗的疗效、耐受性、不良反应情况、缓解情况和复发情况。

材料与方法

对在3年期间接受利妥昔单抗治疗的所有已确诊天疱疮患者进行回顾性分析。记录患者的基线特征、病程、临床表现、黏膜受累情况、疾病严重程度评估以及利妥昔单抗的不良事件。根据疾病结局参数的定义,将结局评估为早期和晚期终点。

结果

17例天疱疮患者中,女性14例(82.4%),男性3例(17.6%),平均年龄35.9±16.5岁(范围:9 - 65岁)。寻常型天疱疮(PV)是11例(64.7%)患者中的主要类型。利妥昔单抗输注后,17例患者在15天至3个月内达到巩固期末(ECP),平均持续时间为1.24个月。完全缓解(CR on/off)范围为0.5至35个月,平均缓解持续时间为21.7个月。在中位时间4.2个月内,近80%的患者在治疗中达到CR。9例(53%)患者在研究期结束时未经任何治疗处于CR状态,8例(47%)患者在最小治疗量下处于缓解状态。

结论

利妥昔单抗是治疗天疱疮的一种有效治疗药物,与以往用于治疗的所有药物相比,耐受性更好且更安全。

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