Primary Pneumococcal Peritonitis can be the first presentation of a familial complement factor I deficiency.,Clinical & Experimental Immunology

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Primary Pneumococcal Peritonitis can be the first presentation of a familial complement factor I deficiency.
Clinical & Experimental Immunology ( IF 3.4 ) Pub Date : 2020-07-08 , DOI: 10.1111/cei.13490
S Ugrinovic ₁ , H Firth ₂ , D Kavanagh _{3,

4} , T Gouliouris _{5,

6} , P Gurugama ₁ , H Baxendale ₁ , P J Lachmann ₇ , D Kumararatne ₁ , E Gkrania-Klotsas ₅

Affiliation

Primary pneumococcal peritonitis is a rare infection that has been described in women but has not been previously linked with immunodeficiency. The complement system plays a central role in immune defence against Streptococcus pneumoniae and, in order to evade complement attack, pneumococci have evolved a large number of mechanisms that limit complement‐mediated opsonization and subsequent phagocytosis. We investigated an apparently immunocompetent woman with primary pneumococcal peritonitis and identified a family with deficiency for complement factor I. Primary pneumococcal peritonitis should be considered a possible primary immunodeficiency presentation.

中文翻译：

原发性肺炎球菌性腹膜炎可能是家族性补体因子 I 缺乏症的首发表现。

原发性肺炎球菌腹膜炎是一种罕见的感染，已在女性中进行了描述，但之前并未与免疫缺陷相关联。补体系统在针对肺炎链球菌的免疫防御中发挥着核心作用，为了逃避补体攻击，肺炎球菌已经进化出大量限制补体介导的调理作用和随后的吞噬作用的机制。我们调查了一名患有原发性肺炎球菌腹膜炎的明显免疫功能正常的女性，并确定了一个补体因子 I 缺乏的家庭。原发性肺炎球菌腹膜炎应被视为可能的原发性免疫缺陷表现。

更新日期：2020-07-08

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