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Updated: Aug 8, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Prevalence of Major Pulmonary Complications Among Patients With Systemic Lupus Erythematosus: A Systematic Review and
Athanasios Vassilopoulos1, Stephanos Vassilopoulos1, Markos Kalligeros2
1Department of Internal Medicine, Rhode Island Hospital, Warren Alpert Medical School of Brown University, Providence, Rhode Island.
Objective:
The prevalence of major pulmonary complications of systemic lupus erythematosus (SLE) such as interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), diffuse alveolar hemorrhage (DAH), shrinking lung syndrome (SLS), and acute lupus pneumonitis (LP) has been less studied. We aimed to calculate the prevalence of those complications in patients with SLE.
Methods:
We searched PubMed and Embase for observational studies evaluating major pulmonary complications in individuals with SLE. We calculated the pooled prevalence of interstitial lung abnormalities (ILAs/radiographic ILD) and clinical ILD, as well as PAH, DAH, SLS, and LP among individuals with SLE with data available from eligible prospective and retrospective studies. We used 95% confidence intervals (CIs). Our study was registered on PROSPERO (CRD420251118717).
Results:
From 1,347 abstracts, we identified 40 studies with relevant data. We estimated that the pooled prevalence of ILAs was 12.39% (95% CI 9.68%-15.36%) whereas for PAH, LP, DAH, and SLS they were 2.30% (95% CI 1.84%-2.81%), 3.05% (95% CI 1.53%-4.85%), 1.37% (95% CI 0.70%-2.22%), and 1.25% (95% CI 0.05%-2.19%), respectively. Regarding clinical ILD, the pooled prevalence was 2.92% (95% CI 1.50%-4.46%). As for ILD radiographic patterns, the prevalence of nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP) was 52.47% (95% CI 37.36%-67.37%) and 12.09% (95% CI 5.01%-21.24%), respectively, among patients with SLE with ILD.
Conclusion:
Major pulmonary complications in SLE are more prevalent than previously recognized, especially radiographic findings of ILAs. ILD with an NSIP pattern is the most common, although it is reassuring that DAH, SLS, and LP remain relatively rare. Targeted screening with high-resolution computed tomography or right heart catheterization for ILD and PAH, respectively, along with timely intervention in high-risk or symptomatic patients could improve outcomes.
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