Swigris Jeffrey J, Fischer Aryeh, Gillis Joann, Meehan Richard T, Brown Kevin K
Autoimmune Lung Disease Center, National Jewish Medical and Research Center, Denver, CO 80206, USA.
Chest. 2008 Jan;133(1):271-80. doi: 10.1378/chest.07-0079.
Systemic lupus erythematosus (SLE) is considered the archetypal systemic autoimmune disease. Clinically characterized by multisystem involvement and varied serologic abnormalities, no two patients present or have disease that evolves in exactly the same way. Viewed histologically, SLE is characterized by some combination of inflammation and fibrosis, and the clinical phenotype is dictated by the relative contributions of each and the organs affected. Tissue injury appears to be mediated by characteristic autoantibody production, immune complex formation, and their organ-specific deposition. As expected in a multisystem disease, the entire pulmonary system is vulnerable to injury. Any of its compartments-airways, lung parenchyma, vasculature, pleura, or the respiratory musculature-may be independently or simultaneously affected. This article offers the reader a comprehensive review of the numerous pulmonary and thrombotic manifestations of SLE and suggests approaches to their management.
系统性红斑狼疮(SLE)被认为是典型的全身性自身免疫性疾病。其临床特征为多系统受累及多种血清学异常,没有两个患者的临床表现或疾病演变方式完全相同。从组织学角度来看,SLE的特征是炎症和纤维化的某种组合,临床表型由每种因素的相对作用以及受影响的器官决定。组织损伤似乎是由特征性自身抗体产生、免疫复合物形成及其器官特异性沉积介导的。正如在多系统疾病中所预期的那样,整个肺部系统都易受损伤。其任何一个部分——气道、肺实质、血管系统、胸膜或呼吸肌——都可能单独或同时受到影响。本文为读者全面综述了SLE的众多肺部和血栓形成表现,并提出了相应的管理方法。