Chest

Chest

Volume 133, Issue 1, January 2008, Pages 271-280
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Special Feature
Pulmonary and Thrombotic Manifestations of Systemic Lupus Erythematosus

https://doi.org/10.1378/chest.07-0079Get rights and content

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.

Section snippets

Pulmonary Involvement

In the 50 years since Sante and Wyatt7 described a lack of lung involvement “until the terminal stages of the disease,” significant advances in our understanding of SLE-related lung involvement have been made. The earliest reports describe a “waxing and waning, migrating bronchopneumonia,”8 and a diffuse, noninfectious, inflammatory lung disease termed primary atelectizing pneumonitis.910 In more recent reports, the frequency and characteristics of lung involvement have depended on the clinical

Thrombosis

Antiphospholipid antibodies (aPL), a family of acquired autoantibodies that are associated with vascular thrombosis and pregnancy morbidity, may be present in up to two thirds of patients with SLE.7879 The two most well-known and clinically important are the lupus anticoagulant (LA) and anticardiolipin antibodies (aCL). These antibodies bind serum proteins such as prothrombin, various protein/phospholipid complexes, and β2 glycoprotein I (β2GPI).8081

Conclusion

The respiratory and thrombotic manifestations of SLE are numerous, complex, and present diagnostic and therapeutic challenges for the clinician. Many of these manifestations are more common than previously thought and may contribute greatly to symptoms, including impaired exercise tolerance. Several manifestations are life threatening; the pathogenesis of each is generally uncertain; and definitive therapies are uncommon. In general, we add a glucocorticoid-sparing immunomodulatory agent to an

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    Funding for this work was provided by the Farkas Family Lupus Fund.

    The authors have no actual or potential conflicts of interest with the information discussed in this article.

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    Copyright © 2008 The American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.