Abstract
BackgroundThe purpose of this study was to assess the prevalence, associated factors, and impact on mortality of primary respiratory disease in a large systemic lupus erythematosus (SLE) retrospective cohort.MethodsAll adult patients in the RELESSER-TRANS (Registry of Systemic Lupus Erythematosus Patients of the Spanish Society of Rheumatology [SER], cross-sectional phase) registry were retrospectively investigated for the presence of primary pleuropulmonary manifestations.ResultsIn total 3215 patients were included. At least one pleuropulmonary manifestation was present in 31% of patients. The most common manifestation was pleural disease (21%), followed by lupus pneumonitis (3.6%), pulmonary thromboembolism (2.9%), primary pulmonary hypertension (2.4%), diffuse interstitial lung disease (2%), alveolar hemorrhage (0.8%), and shrinking lung syndrome (0.8%).In the multivariable analysis, the variables associated with the development of pleuropulmonary manifestation were older age at disease onset (odds ratio (OR) 1.03, 95% confidence interval (CI) 1.02–1.04), higher SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) scores (OR 1.03, 95% CI 1.00–1.07), the presence of Raynaud’s phenomenon (OR 1.41, 95% CI 1.09–1.84), secondary antiphospholipid syndrome (OR 2.20, 95% CI 1.63–2.97), and the previous or concomitant occurrence of severe lupus nephritis, (OR 1.48, 95% CI 1.12–1.95) neuropsychiatric manifestations (OR 1.49, 95% CI 1.11–2.02), non-ischemic cardiac disease (OR 2.91, 95% CI 1.90–4.15), vasculitis (OR 1.81, 95% CI 1.25–2.62), hematological manifestations (OR 1.31, 95% CI 1.00–1.71), and gastrointestinal manifestations, excluding hepatitis (OR 2.05, 95% CI 1.14–3.66). Anti-RNP positivity had a clear tendency to significance (OR 1.32, 95% CI 1.00–1.75; P = 0.054).The development of pleuropulmonary manifestations independently contributes to a diminished survival (hazard ratio of 3.13). However, not all complications will influence the prognosis in the same way. Whereas the occurrence of pleural disease or pulmonary thromboembolism has a minimal impact on the survival of these patients, the remaining manifestations have a major impact on mortality.ConclusionExcept for pleural disease, the remaining respiratory manifestations are very uncommon in SLE (<4%). Pleuropulmonary manifestations independently contributed to a decreased survival in these patients.
Highlights
The purpose of this study was to assess the prevalence, associated factors, and impact on mortality of primary respiratory disease in a large systemic lupus erythematosus (SLE) retrospective cohort
The spectrum of pulmonary manifestations caused by SLE includes pleural disease, upper and lower airway dysfunction, primary pulmonary hypertension, pulmonary thromboembolism, acute reversible hypoxemia, diffuse interstitial lung disease, acute lupus pneumonitis, diffuse alveolar hemorrhage, and shrinking lung syndrome [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16]
Pleuropulmonary manifestations analyzed in this study included (1) pleural disease, including patients with episodes of pleurisy according with the SELENA-SLEDAI (Systemic Lupus Erythematosus Disease Activity SLICC Systemic Lupus International Collaborating Clinics (Index)) definition [19], or pleural fibrosis according with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR)-damage index (SDI) [20] or both; (2) acute lupus pneumonitis or interstitial alveolitis/pneumonitis [21, 22]; (3) diffuse interstitial lung disease or pulmonary fibrosis (SDI definition); (4) pulmonary hemorrhage (BILAG 2004 definition); (5) shrinking lung syndrome (BILAG 2004 definition); (6) primary pulmonary hypertension (SDI definition); and (7) pulmonary thromboembolism, including the cases registered with pulmonary infarction in accordance with the Systemic Lupus International Collaborating Clinics-Damage Index (SDI) definition
Summary
The purpose of this study was to assess the prevalence, associated factors, and impact on mortality of primary respiratory disease in a large systemic lupus erythematosus (SLE) retrospective cohort. Primary respiratory disease in systemic lupus erythematosus (SLE) seems not uncommon, its exact prevalence is still unknown, since it has been reported to occur in 5% to 90% of the patients [1,2,3,4,5,6]. This wide range in prevalence may be explained by the characteristics of the patients studied (early or established disease), whether only symptomatic patients were included or systematic screening was done, the methodology used for diagnosis (clinical manifestations and imaging or autopsy findings), and the different study designs. The severity of these respiratory complications is highly variable and ranges from subclinical to potentially life-threatening conditions
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