Pulmonary Involvement in Systemic Lupus Erythematosus: A Potentially Overlooked Condition

Abstract

Systemic lupus erythematosus (SLE) is a pleiotropic disease that can present in numerous forms, ranging from mild mucocutaneous symptoms to severe manifestations affecting multiple organs. SLE has the potential to impact any segment of the respiratory system, exhibiting a range of severity levels throughout the different stages of the disease. Pulmonary manifestations in SLE patients can be classified as primary (i.e., directly related to SLE and to immune-mediated damage), secondary to other SLE manifestations (e.g., nephrotic syndrome, renal failure, congestive heart failure), and comorbidities (e.g., infections, cancers, overlapping primary respiratory diseases). Understanding and correctly managing lung involvement in SLE is crucial because pulmonary complications are common and can significantly impact morbidity and mortality in affected patients. Early recognition and appropriate treatment can prevent irreversible lung damage, improve quality of life, and reduce the risk of life-threatening complications. Treatment algorithms are based on the suppression of inflammation, with or without the need for dedicated, supportive care. According to disease severity, available treatments include nonsteroidal anti-inflammatory drugs, corticosteroids, immunosuppressants, and biological agents. In this review, we aim to summarize the current knowledge on lung involvement in SLE and then focus on the management and treatment approaches available for the different forms.

Keywords: autoimmunity; interstitial lung disease; pleuritis; pulmonary involvement; systemic erythematous lupus.

Figure 1

Axial non-contrast thorax computed tomography scan in a patient with systemic lupus erythematosus. The exam revealed abnormal accumulations of fluid within the pleural space (asterisk).

Figure 2

Diagnostic flowchart and suggested management for patients with systemic lupus erythematosus (SLE) presenting with respiratory symptoms. ALP, acute lupus pneumonitis; AZA, azathioprine; CYC, ciclophosphamide; CCBs, calcium channel blockers; CT, computed tomography; DAH, diffuse alveolar hemorrhage; ERAs, endothelin receptor antagonists; Hb, hemoglobin; HRTC, high-resolution computed tomography; ILD, interstitial lung disease; IVIG, intravenous immunoglobulin; MMF, mycophenolate mofetil; NSAIDs, nonsteroidal anti-inflammatory drugs; PAH, pulmonary arterial hypertension; PPF, progressive pulmonary fibrosis; PDE-5is: phosphodiesterase 5 inhibitors.

Figure 3

Treatment strategies available for pleuropulmonary involvement in systemic lupus erythematosus patients. AZA, azathioprine; CYC, cyclophosphamide; CCBs, calcium channel blockers; DAH, diffuse alveolar hemorrhage; ERAs, endothelin receptor antagonists; ILD, interstitial lung disease; IVIG, intravenous immunoglobulin; MMF, mycophenolate mofetil; NSAIDs, nonsteroidal anti-inflammatory drugs, PAH, pulmonary arterial hypertension; PDE-5is, phosphodiesterase 5 inhibitors.

Figure 4

Axial non-contrast thorax computed tomography scan in a patient with systemic lupus erythematosus (SLE). The exam revealed abnormal permanent enlargement of the airspaces distal to the terminal bronchioles (arrows), accompanied by the destruction of the alveolar wall and subpleural interstitial involvement (arrowheads).