Pulmonary Manifestations of Systemic Lupus Erythematosus

Shikha Mittoo, MD, MHS, FRCPC; Charlene D. Fell, MD, MSc, FRCPC, FCCP

Disclosures

Semin Respir Crit Care Med. 2014;35(2):249-254. 

In This Article

Abstract and Introduction

Abstract

Systemic lupus erythematosus (SLE) is a systemic inflammatory disease, characterized serologically by an autoantibody response to nucleic antigens, and clinically by injury and/or malfunction in any organ system. During their disease course, up to 50% of SLE patients will develop lung disease. Pulmonary manifestations of SLE include pleuritis (with or without effusion), inflammatory and fibrotic forms of interstitial lung disease, alveolar hemorrhage, shrinking lung syndrome, pulmonary hypertension, airways disease, and thromboembolic disease. Two major themes inform our understanding of SLE-associated pulmonary manifestations: first, the presence of specific autoantibodies correlates with the presence of certain pulmonary manifestations and second, vascular injury marks a common pathophysiologic thread among the various SLE-related lung diseases. This review will focus on the clinical presentation, pathogenesis, pathology, management, and prognosis of these SLE-associated lung conditions.

Introduction

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with a predilection for young women. Lung involvement is a known complication of the disease and/or its treatments; up to 50% of SLE patients experience such involvement during their disease course.[1] Broadly speaking, pulmonary complications of SLE can be divided into three categories: infectious, malignant, and disease related. Although pulmonary infections typically affect the airways and/or parenchyma, complications attributable to SLE can affect all compartments of the lungs and include pleuritis (with or without effusion), interstitial lung disease (ILD), alveolar hemorrhage, shrinking lung syndrome (SLS), pulmonary hypertension (PH), airways disease, and thromboembolic disease. A heightened awareness of SLE-related pulmonary manifestations among clinicians and the evolution of more sensitive tools which capture lung involvement have also lead to the recognition that subclinical lung involvement occurs at a greater frequency than clinically identifiable involvement. This review will focus primarily on the clinical presentation, pathogenesis, pathology, management, and outcome of clinically identifiable pulmonary manifestations attributable to SLE.

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