Wallace W A, Howie S E, Krajewski A S, Lamb D
Department of Pathology, Edinburgh University Medical School, U.K.
J Pathol. 1996 Mar;178(3):323-9. doi: 10.1002/(SICI)1096-9896(199603)178:3<323::AID-PATH467>3.0.CO;2-7.
Cryptogenic fibrosing alveolitis (CFA) is believed to have a pathogenesis mediated by the cellular arm of the immune system. Previous studies have, however, indicated the presence of B-lymphocyte aggregates, as well as evidence of local immunoglobulin production and increased levels of B-cell growth factors. It has recently been shown that CFA is associated with the production of circulating IgG autoantibodies to antigen(s) associated with alveolar lining cells. This prompted an examination of the immunological architecture of the B-lymphocyte aggregates, in order to assess whether they might provide histological confirmation of a local humoral immune response in these patients. Thirty-eight consecutive open lung biopsy specimens were examined from patients with CFA and aggregates of B lymphocytes were identified in 37/38. In only five cases were germinal centres seen. The morphological appearances of the aggregates were reminiscent of those observed in mucosal associated lymphoid tissue (MALT). Using immunohistochemistry, despite the low frequency of true germinal centre formation, the B-lymphocyte aggregates were shown to contain the cellular micro-environment necessary for a humoral immune response. In addition, there was evidence of lymphocyte proliferation and activation within these aggregates. These results provide evidence of a local humoral immune response associated with B-lymphocyte aggregates in the lungs of patients with CFA.
隐源性纤维性肺泡炎（CFA）被认为其发病机制是由免疫系统的细胞分支介导的。然而，先前的研究表明存在B淋巴细胞聚集物，以及局部免疫球蛋白产生的证据和B细胞生长因子水平的升高。最近研究显示，CFA与针对肺泡衬里细胞相关抗原的循环IgG自身抗体的产生有关。这促使人们对B淋巴细胞聚集物的免疫结构进行检查，以评估它们是否能为这些患者局部体液免疫反应提供组织学证据。对38例CFA患者的连续开放性肺活检标本进行了检查，在38例中有37例发现了B淋巴细胞聚集物。仅在5例中观察到生发中心。这些聚集物的形态学表现让人联想到在黏膜相关淋巴组织（MALT）中观察到的情况。使用免疫组织化学方法，尽管真正生发中心形成的频率较低，但B淋巴细胞聚集物显示含有体液免疫反应所需的细胞微环境。此外，在这些聚集物中有淋巴细胞增殖和活化的证据。这些结果为CFA患者肺部与B淋巴细胞聚集物相关的局部体液免疫反应提供了证据。
