Carlsson M, Eriksson L, Erwander I, Wieslander J, Segelmark M
Department of Microbiology, Immunology and Glycobiology, Lund University, Lund, Sweden.
Clin Exp Rheumatol. 2003 Nov-Dec;21(6 Suppl 32):S95-100.
Lung damage is the most common cause of death in cystic fibrosis (CF). It is induced by bacterial colonization and inflammatory activity perpetuates its course. Autoantibodies directed against BPI (bactericidal permeability increasing protein), called BPI-ANCA, have recently been associated with cystic fibrosis. Here we confirm this association and evaluate the relation between ANCA and total IgG level as they relate to bacterial colonization, pulmonary function, and musculoskeletal symptoms.
BPI-ANCA, MPO-ANCA, and PR3-ANCA were measured with ELISA in 46 adult patients with CF. Total IgG was determined by immunoturbidimetry. Results were correlated to bacterial colonization, lung function and musculoskeletal symptoms.
BPI-ANCA was found in 33 patients. In the whole group, both BPI-ANCA and total IgG were inversely correlated to lung function, but in patients chronically colonized with Pseudomonas aeruginosa (P. aeruginosa), BPI-ANCA alone was correlated to lung damage (p = 0.01). Median lung function, measured as forced expiratory volume in 1 second, in P. aeruginosa colonized patients with high levels of BPI-ANCA was 43% of the predicted value. In BPI-ANCA negative, the corresponding figure was 83%. In patients not colonized with P. aeruginosa, this relation was less evident. No correlation between ANCA and musculoskeletal symptoms was seen.
P. aeruginosa induced lung damage in CF patients is associated with the presence of BPI-ANCA. P. aeruginosa colonized patients without BPI-ANCA have almost normal lung function. We suggest that BPI-ANCA discriminate P. aeruginosa colonized CF patients with severe lung damage from those whose disease is less destructive. Vasculitis like symptoms in CF are not ANCA associated.
肺部损伤是囊性纤维化（CF）最常见的死亡原因。它由细菌定植引发，炎症活动使其病程持续。针对杀菌通透性增加蛋白（BPI）的自身抗体，即BPI-ANCA，最近被发现与囊性纤维化有关。在此我们证实这种关联，并评估ANCA与总IgG水平之间的关系，因为它们与细菌定植、肺功能和肌肉骨骼症状相关。
采用酶联免疫吸附测定法（ELISA）检测46例成年CF患者的BPI-ANCA、髓过氧化物酶-ANCA（MPO-ANCA）和蛋白酶3-ANCA（PR3-ANCA）。通过免疫比浊法测定总IgG。结果与细菌定植、肺功能和肌肉骨骼症状相关。
33例患者检测到BPI-ANCA。在整个研究组中，BPI-ANCA和总IgG均与肺功能呈负相关，但在长期被铜绿假单胞菌（P. aeruginosa）定植的患者中，仅BPI-ANCA与肺损伤相关（p = 0.01）。在BPI-ANCA水平高的铜绿假单胞菌定植患者中，以1秒用力呼气量衡量的中位肺功能为预测值的43%。在BPI-ANCA阴性患者中，相应数字为83%。在未被铜绿假单胞菌定植的患者中，这种关系不太明显。未观察到ANCA与肌肉骨骼症状之间的相关性。
铜绿假单胞菌诱导的CF患者肺损伤与BPI-ANCA的存在有关。未感染BPI-ANCA的铜绿假单胞菌定植患者肺功能几乎正常。我们认为BPI-ANCA可区分出患有严重肺损伤的铜绿假单胞菌定植CF患者和疾病破坏性较小的患者。CF中类似血管炎的症状与ANCA无关。
