Buhl R, Meier-Sydow J, Vogelmeier C
Pneumologie und Allergologie, Medizinische Klinik II, Universität Frankfurt.
Immun Infekt. 1995 Jun;23(3):92-6.
Idiopathic pulmonary fibrosis or lone cryptogenic fibrosing alveolitis is an interstitial lung disease of unknown origin carrying an unfavorable prognosis. A yet unidentified hazard triggers a chronic inflammatory infiltration of the lung parenchyma characterized by an accumulation of alveolar macrophages, neutrophil and eosinophil granulocytes, and lymphocytes. Cytokines released by the activated cells modulate the inflammatory events. Oxidants and proteases, mainly released by alveolar macrophages and neutrophil granulocytes, mediate the injury to the lung parenchyma, leading to loss of alveolar-capillary units. The ensuing repair process, mesenchymal cell proliferation and up-regulation of synthesis of collagen fibers and other components of connective tissue matrix, replaces lung parenchyma by fibrotic tissue, leading to irreversible pulmonary dysfunction.
特发性肺纤维化或特发性隐源性纤维性肺泡炎是一种病因不明的间质性肺疾病，预后不佳。一种尚未明确的危险因素引发了肺实质的慢性炎症浸润，其特征为肺泡巨噬细胞、中性粒细胞、嗜酸性粒细胞和淋巴细胞的积聚。活化细胞释放的细胞因子调节炎症反应。主要由肺泡巨噬细胞和中性粒细胞释放的氧化剂和蛋白酶介导对肺实质的损伤，导致肺泡 - 毛细血管单位丧失。随后的修复过程，即间充质细胞增殖以及胶原纤维和结缔组织基质其他成分合成上调，使肺实质被纤维组织替代，导致不可逆的肺功能障碍。
