Retrospective Analysis of a Single- Kuwaiti Center Clinical Experience Toward Development of Proper Controlling Treatment of 84 Pemphigus Patients with a Long-Term Follow-Up: Efficacy and Safety of the Multidrug Protocol Combining Intravenous Immunoglobulin with the Cytotoxic Immunosuppressor Drugs

I. Almasry, V. Lazarević, F. Alkhawaja, Jihan M Rajy, N. Musami, Nadia Alnaki, R. Dvořák, Aliaa Sadek, Humoud Al Sabah, Atlal Al-Lafi
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Abstract

Pemphigus is the name of a group of autoimmune pathological entities characterized by the formation of intraepithelial blisters in the skin and/or mucosa. Pemphigus vulgaris (PV) and Pemphigus foliaceus (PF) are considered as classical forms. Rare, non-classical forms of Pemphigus include: Pemphigus herpetiformis, IgA pemphigus, Paraneoplastic pemphigus, IgG/IgA pemphigus. Aim: Aim of our study is to explore the prognostic influence of clinical, immunological and therapeutic drugs on disease course and remission in different pemphigus variants in Kuwait and to establish a safe and effective multidrug protocol. Plan of the study is to make a correlation between epidemiological factors, therapy used and clinical and immunological remission, as well as, follow up of comorbidities in our patients. 84 patients were evaluated, from 1st January 1990 to 31st December 2013. IVIG is used as a third-line adjuvant treatment initially flanked by high-dose systemic corticosteroids and steroid- sparing immune-suppressants. Thus, this multidrug IVIG regimen made it possible to achieve a rapid control of the pemphigus symptoms, with progression to a stable disease remission, while maintenance an overall safety of treatment.
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回顾性分析科威特中心对84例天疱疮患者进行长期随访的临床经验:静脉注射免疫球蛋白联合细胞毒性免疫抑制药物的多药方案的有效性和安全性
天疱疮是一组自身免疫性病理实体的名称,其特征是皮肤和/或粘膜上皮内水疱的形成。寻常型天疱疮(PV)和叶状天疱疮(PF)被认为是经典形式。罕见的,非经典形式的天疱疮包括:疱疹样天疱疮,IgA天疱疮,副肿瘤天疱疮,IgG/IgA天疱疮。目的:探讨临床、免疫和治疗药物对科威特不同类型天疱疮病程和缓解的预后影响,并建立安全有效的多药治疗方案。本研究的目的是研究流行病学因素、所采用的治疗方法与临床和免疫缓解的相关性,并对患者的合并症进行随访。从1990年1月1日至2013年12月31日对84例患者进行了评估。IVIG最初被用作三线辅助治疗,与大剂量全身皮质类固醇和保留类固醇的免疫抑制剂同时使用。因此,这种多药IVIG方案可以实现天疱疮症状的快速控制,并进展到稳定的疾病缓解,同时保持治疗的总体安全性。
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