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IgA血管炎的多系统表现。

Multisystemic manifestations of IgA vasculitis.

作者信息

Du Lina, Wang Panpan, Liu Chang, Li Shaojing, Yue Shuang, Yang Yan

机构信息

Department of Chinese Medicine, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, No. 56 Nanlishi Road, Beijing, 100045, China.

出版信息

Clin Rheumatol. 2021 Jan;40(1):43-52. doi: 10.1007/s10067-020-05166-5. Epub 2020 Jun 16.

Abstract

Immunoglobulin A vasculitis (IgAV), also known as Henoch-Schönlein Purpura, is one of the most common kind of systemic vasculitis in children, and due to the involvement of small blood vessels throughout the body, this disease can cause a variety of symptoms in different organs. Our aim was to review the data on various systemic manifestations of IgAV. A research of the literature was performed in PubMed database, utilizing the MeSH terms "IgA vasculitis" and "Henoch Schönlein Purpura". According to the predetermined structure of the manuscript, we extracted and sorted out the relevant data. Clinically, almost all the patients will present with palpable skin purpura, together with arthritis, gastrointestinal tract involvement, or kidney damage. Other rare systemic manifestations include neurological symptoms, scrotal involvement, and cardiopulmonary disease. When uncommon complications occur, patients may be misdiagnosed as other diseases, thus delaying treatment. Although the course of IgAV is mostly self-limited, misdiagnosis can also lead to a poor prognosis. A comprehensive awareness to the clinical manifestations of IgAV is the necessary prerequisite for its timely diagnosis. Prompt diagnosis and adequate treatment are essential for optimal results.

摘要

免疫球蛋白A血管炎(IgAV),也称为过敏性紫癜,是儿童最常见的系统性血管炎之一。由于全身小血管受累,这种疾病可在不同器官引起多种症状。我们的目的是回顾关于IgAV各种全身表现的数据。利用医学主题词“免疫球蛋白A血管炎”和“过敏性紫癜”在PubMed数据库中进行文献检索。根据预先确定的手稿结构,我们提取并整理了相关数据。临床上,几乎所有患者都会出现可触及的皮肤紫癜,同时伴有关节炎、胃肠道受累或肾脏损害。其他罕见的全身表现包括神经症状、阴囊受累和心肺疾病。当出现罕见并发症时,患者可能被误诊为其他疾病,从而延误治疗。尽管IgAV的病程大多为自限性,但误诊也可能导致预后不良。对IgAV临床表现的全面认识是及时诊断的必要前提。及时诊断和充分治疗对于取得最佳效果至关重要。

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