Association of systemic immune complexes, complement activation, and antibodies to Pseudomonas aeruginosa lipopolysaccharide and exotoxin A with mortality in cystic fibrosis.
Association of systemic immune complexes, complement activation, and antibodies to Pseudomonas aeruginosa lipopolysaccharide and exotoxin A with mortality in cystic fibrosis.
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全身免疫复合物、补体激活以及铜绿假单胞菌脂多糖和外毒素 A 抗体与囊性纤维化死亡率的关联。
DOI：
10.1164/arrd.1986.133.4.648
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发表时间：
1986
期刊：
影响因子：
--
通讯作者：
Larrick,JW
中科院分区：
文献类型：
--
作者：
Moss,RB;Hsu,YP;Lewiston,NJ;Curd,JG;Milgrom,H;Hart,S;Dyer,B;Larrick,JW
循环免疫复合物、血浆补体激活和抗铜绿假单胞菌离散抗原的血清抗体与囊性纤维化（CF）患者临床病程的相关性尚不清楚。我们将这些因素与49例被P定植的CF患者的结局相关联。铜绿假单胞菌，比较14名死于肺病的患者与35名年龄和定植持续时间相似的幸存者，以及9名未定植的CF患者，24名其他支气管炎性肺病患者和10名健康对照受试者。CF患者定植有P.死亡的铜绿假单胞菌免疫复合物的发生率高于存活的铜绿假单胞菌（71%比40%，P < 0.05）。此外，C4活化与免疫复合物和死亡率高度相关（p < 0.001）。那些死亡的人也有更高水平的IgG抗体。与存活的P.铜绿假单胞菌（分别为p < 0.005和p = 0.01），而两组的P。aerosasonicate、弹性蛋白酶、碱性蛋白酶和内毒素核心抗体。我们的结论是，血清抗P抗体IgG水平的增加。嗜水气单胞菌LPS和外毒素A以及全身免疫复合物和补体激活的存在与CF的不良预后相关，并可能为研究CF肺病的可能免疫发病机制提供有用的非侵入性标志物。
The relevance of circulating immune complexes, plasma complement activation, and serum antibodies against discrete antigens ofPseudomonas aeruginosa,to the clinical course in patients with cystic fibrosis (CF) is unknown. We related these factors to outcome in 49 patients with CF colonized byP. aeruginosa,comparing 14 who died of lung disease with 35 survivors of similar age and duration of colonization, as well as 9 uncolonized patients with CF, 24 patients with other bronchorrheic lung disease, and 10 healthy control subjects. The patients with CF colonized byP. aeruginosawho died had a higher incidence of immune complexes than did survivors (71 versus 40%, p < 0.05). Moreover, C4 activation was highly associated with immune complexes and mortality (p < 0.001 for each). Those who died also had much higher levels of IgG antibodies toP. aeruginosalipopolysaccharide (LPS) and exotoxin A than did survivors colonized byP. aeruginosa(p < 0.005 and p = 0.01, respectively), whereas both groups had similar levels ofP. aeruginosasonicate, elastase, alkaline protease, and endotoxin core antibodies. We conclude that increasing levels of serum IgG antibodies toP. aeruginosaLPS and exotoxin A and the presence of systemic immune complexes and complement activation are associated with poor prognosis in CF, and may provide useful noninvasive markers for studying the possible immunopathogenesis of CF lung disease.