Crestani B, Aubier M
Unité INSERM 408, Faculté Xavier-Bichat, Paris.
Rev Mal Respir. 1996;13(1):15-20.
Pulmonary fibrosis is a frequent and serious complication of scleroderma whose pathophysiology remains poorly understood. The alveolar structures are infiltrated by activated chronic inflammatory cells, alveolar macrophages and polymorphonuclear neutrophils in particular and these could play a determining role. We have studied the state of activation of alveolar macrophages and monocytes circulating in these patients who presented with scleroderma and interstitial pulmonary involvement and also in healthy subjects. The neutrophil alveolitis observed in the patients is accompanied by a raised level of interleukin-8 secretion by the alveolar macrophages compared to the healthy subjects. Interleukin-8 is an important chemotactic molecule for polymorphonuclear neutrophils in the lung. The neutrophil alveolitis is accompanied by a breakdown in the equilibrium of elastase-antielastase which could participate in the development of alveolar lesions leading to fibrosis. In addition to the activation of macrophages, there is an activation of monocytes marked by the increase in secretion of interleukin-6 and interleukin-8 in vitro during the progression of the disease of scleroderma. Thus, alveolar inflammation is integrated with the overall systemic inflammation whose causes remain unknown.
肺纤维化是硬皮病常见且严重的并发症，其病理生理学仍知之甚少。肺泡结构被活化的慢性炎症细胞浸润，尤其是肺泡巨噬细胞和多形核中性粒细胞，它们可能起决定性作用。我们研究了这些患有硬皮病和间质性肺受累的患者以及健康受试者体内循环的肺泡巨噬细胞和单核细胞的活化状态。与健康受试者相比，患者中观察到的中性粒细胞肺泡炎伴随着肺泡巨噬细胞白细胞介素-8分泌水平的升高。白细胞介素-8是肺中多形核中性粒细胞的重要趋化分子。中性粒细胞肺泡炎伴随着弹性蛋白酶-抗弹性蛋白酶平衡的破坏，这可能参与导致纤维化的肺泡病变的发展。除了巨噬细胞的活化外，单核细胞也被活化，其标志是在硬皮病病程中体外白细胞介素-6和白细胞介素-8分泌增加。因此，肺泡炎症与病因不明的全身性炎症相关。
