Pulmonary fibrosis occurs in up to 70% of scleroderma patients and progresses to cause severe restrictive lung disease in about 15% of patients. The mechanisms that cause pulmonary fibrosis in scleroderma remain incompletely understood. Increased amounts of mRNA or protein for multiple profibrotic cytokines and chemokines have been identified in lung tissue or broncholveolar lavage samples from scleroderma patients, when compared to healthy controls. These cytokines include transforming growth factor (TGF)-beta, connective tissue growth factor (CTGF), platelet-derived growth factor (PDGF), oncostatin M (OSM), monocyte chemotactic factor-1 and pulmonary and activation-regulated chemokine (PARC). Potential cellular sources of these profibrotic cytokines and chemokines in scleroderma lung disease include alternatively activated macrophages, activated CD8+ T cells, eosinophils, mast cells, epithelial cells and fibroblasts themselves. This review summarizes the literature on involvement of cytokines and chemokines in the development of pulmonary fibrosis in scleroderma.

中文翻译：

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硬皮病中肺纤维化的细胞因子调节。

高达70％的硬皮病患者发生肺纤维化，并在约15％的患者中发展为引起严重的限制性肺疾病。导致硬皮病肺纤维化的机制仍不完全清楚。与健康对照相比，已从硬皮病患者的肺组织或支气管肺泡灌洗液样品中发现了多种促纤维化细胞因子和趋化因子的mRNA或蛋白质含量增加。这些细胞因子包括转化生长因子（TGF）-β，结缔组织生长因子（CTGF），血小板衍生生长因子（PDGF），抑瘤素M（OSM），单核细胞趋化因子1以及肺和激活调节趋化因子（PARC） 。硬皮病肺部疾病中这些促纤维化细胞因子和趋化因子的潜在细胞来源包括活化的巨噬细胞，活化的CD8 + T细胞，嗜酸性粒细胞，肥大细胞，上皮细胞和成纤维细胞本身。这篇综述总结了有关细胞因子和趋化因子参与硬皮病肺纤维化发展的文献。