Minami-Otsuka Institute of Technology, Minami-Otsuka Clinic, 2-41-9 Minami-Otsuka, Toshima-ku, Tokyo, 170-0005, Japan.
Expert Rev Clin Pharmacol. 2012 Mar;5(2):219-27. doi: 10.1586/ecp.12.9.
A variety of interstitial lung diseases (ILDs) have been reported in association with connective tissue diseases (CTDs). ILD is commonly associated with multiple CTDs and accounts for significant morbidity and mortality in these conditions. In rheumatoid arthritis and systemic sclerosis, ILD commonly occurs in the course of these disorders (incidence: 20-44%). The pathological findings of ILDs are similar to those of idiopathic interstitial pneumonia. A wide variety of histopathologic features, such as various types of interstitial pneumonia and airway involvement, have been observed that are specific for ILDs in rheumatoid arthritis, and this high variety makes its pathology complicated. The diagnosis of ILD is generally based on clinical presentation, bronchioalveolar lavage fluid and high-resolution computed tomography, among others. The most important differential diagnosis is infection, especially pneumocystis pneumonia, and treatment-related toxic damage. The immunosuppressive agents most widely used for the treatment of ILDs are cyclophosphamide, azathioprine, mycophenolate mofetil and calcineurin inhibitors. Other therapeutic strategies are currently being extensively studied, such as antifibrotic agents, endothelin-1 receptor antagonists, tyrosine kinase inhibitors and newer biological agents. In this article, we describe novel therapies for ILDs associated with CTDs.
已报道多种间质性肺疾病（ILDs）与结缔组织疾病（CTDs）相关。ILD 常与多种 CTD 相关，并在这些疾病中导致显著的发病率和死亡率。在类风湿关节炎和系统性硬化症中，ILD 常发生于这些疾病的病程中（发生率：20-44%）。ILD 的病理学表现与特发性间质性肺炎相似。已观察到多种组织病理学特征，如各种类型的间质性肺炎和气道受累，这些特征是类风湿关节炎中 ILD 的特异性表现，这种多样性使其病理学变得复杂。ILD 的诊断通常基于临床表现、支气管肺泡灌洗液和高分辨率计算机断层扫描等。最重要的鉴别诊断是感染，特别是肺孢子菌肺炎和治疗相关的毒性损伤。最常用于治疗 ILD 的免疫抑制剂是环磷酰胺、硫唑嘌呤、霉酚酸酯和钙调神经磷酸酶抑制剂。目前正在广泛研究其他治疗策略，如抗纤维化药物、内皮素-1 受体拮抗剂、酪氨酸激酶抑制剂和新型生物制剂。在本文中，我们描述了与 CTD 相关的 ILD 的新型治疗方法。
