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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Monogenic autoinflammatory diseases: concept and clinical manifestations.
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Monogenic autoinflammatory diseases: concept and clinical manifestations.

机译：单一的自身炎症性疾病：概念和临床表现。

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摘要

The objective of this review is to describe the clinical manifestations of the growing spectrum of monogenic autoinflammatory diseases including recently described syndromes. The autoinflammatory diseases can be grouped based on clinical findings: 1. the three classic hereditary "periodic fever syndromes", familial Mediterranean Fever (FMF); TNF receptor associated periodic syndrome (TRAPS); and mevalonate kinase deficiency/hyperimmunoglobulinemia D and periodic fever syndrome (HIDS); 2. the cryopyrin associated periodic syndromes (CAPS), comprising familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID) or CINCA, and; 3. pediatric granulomatous arthritis (PGA); 4. disorders presenting with skin pustules, including deficiency of interleukin 1 receptor antagonist (DIRA); Majeed syndrome; pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome; deficiency of interleukin 36 receptor antagonist (DITRA); CARD14 mediated psoriasis (CAMPS), and early-onset inflammatory bowel diseases (EO-IBD); 5. inflammatory disorders caused by mutations in proteasome components, the proteasome associated autoinflammatory syndromes (PRAAS) and 6. very rare conditions presenting with autoinflammation and immunodeficiency.
机译：本综述的目的是描述在最近描述的综合征的中生自身炎性疾病的生长范围的临床表现。自身炎性疾病可以根据临床调查结果进行分组：1。三种经典遗传性“周期性发烧综合征”，家族地中海发热（FMF）; TNF受体相关的周期性综合征（陷阱）;和甲醛酸碱酶缺乏/超杂烩和周期性发烧综合征（HIDS）; 2.乳蛋白相关的周期性综合征（帽），包括家族性病自身炎症综合征（FCAS），Muckle-Wells综合征（MWS）和新生儿发作多系统炎症疾病（Nomid）或Cinca，以及; 3.儿科肉芽肿性关节炎（PGA）; 4.疾病患有皮肤脓疱，包括白细胞介素1受体拮抗剂（DIRA）的缺乏; Majeed综合症;化脓性关节炎，pyoderma gangrenosum和痤疮（爸爸）综合征;白细胞介素36受体拮抗剂（Ditra）的缺乏; CARD14介导的牛皮癣（营地）和早期发病性肠病（EO-IBD）; 5.蛋白酶体组分突变引起的炎症性疾病，蛋白酶组相关的自身炎症综合征（PRAAS）和6.具有自身炎症和免疫缺陷的非常罕见的条件。

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