Department of Immunology, Cambridge University Hospital NHS Trust, Cambridge, UK.
Department of Clinical Genetics, Cambridge University Hospital NHS Trust, Cambridge, UK.
Clin Exp Immunol. 2020 Dec;202(3):379-383. doi: 10.1111/cei.13490. Epub 2020 Jul 24.
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.
原发性肺炎球菌性腹膜炎是一种罕见的感染，虽已有相关女性病例报道，但此前与免疫缺陷并无关联。补体系统在抵抗肺炎链球菌的免疫防御中起着核心作用，为了逃避补体攻击，肺炎球菌进化出了大量限制补体介导调理作用和随后吞噬作用的机制。我们研究了一位表现为原发性肺炎球菌性腹膜炎的看似免疫功能正常的女性，并发现了一个补体因子 I 缺陷的家族。原发性肺炎球菌性腹膜炎应被视为一种可能的原发性免疫缺陷表现。
