Background/Purpose: Pleuritis remains the most common pulmonary manifestation in patients with SLE.  The prevalence of other less common complications of SLE such as: interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), diffuse alveolar hemorrhage (DAH), shrinking lung syndrome (SLS) and acute interstitial pneumonia (AIP) has been less studied. We aimed to calculate the prevalence of PAH, DAH, SLS, AIP among individuals with SLE with data available from prospective and retrospective studies.

Methods: We searched PubMed and EMBASE for cohorts evaluating pulmonary complications in individuals with SLE. In order to be considered eligible for our inclusion criteria,  studies should have met the following diagnostic criteria: PAH had to be diagnosed by right heart catheterization (RHC), ILD and AIP by high-resolution or standard computed tomography, respectively, SLS according to definition by Hoffbrand et al (dyspnea, associated with reduced lung volumes on imaging and a restrictive pattern on pulmonary function tests) (1), while DAH was diagnosed by a combination of clinical, laboratory, imaging, and pathologic findings.

Results: Thirty studies provided data on 31,448 patients with SLE and reported the prevalence of pulmonary complications. Studies were published between 1995 and 2023 with 23 being retrospective and 7 prospective (11 with data on ILD, 9 on DAH, 7 on SLS, 3 on PAH and 3 on AIP). ILD (n=6,044) was the most common manifestation with a prevalence of 14.47% (95% CI: 11.19-18.08%) followed by AIP (n=4,422) 3.60% (95% CI: 1.41-6.68%), PAH (n=3,993) 2.30% (95% CI: 1.84-2.81%), DAH (n=9,208) 1.35% (95% CI: 0.61-2.32%) and finally SLS (n=1,054) 1.25% (95% CI: 0.54-2.19%).

Conclusion: In this meta-analysis of 30 studies of SLE patients, the prevalence of ILD was higher than previously reported, at 15%, while PAH diagnosed by RHC was present in 2.3% of patients. Reassuringly, DAH and SLS occurred in less than 2% of patients. As newer therapeutic interventions are available for ILD and PAH associated with connective tissue diseases, such as SLE, an appropriate early diagnostic strategy is highly recommended for all SLE patients presenting with pulmonary symptoms.

Reference

1. Hoffbrand B I, Beck E R. “Unexplained” Dyspnoea and Shrinking Lungs in Systemic Lupus Erythematosus Br Med J 1965; 1 :1273

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/prevalence-of-pulmonary-complications-among-patients-with-systemic-lupus-erythematosus-sle-a-systematic-review-and-meta-analysis/