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. 2018 Dec 19;20(1):280.
doi: 10.1186/s13075-018-1776-8.

Primary respiratory disease in patients with systemic lupus erythematosus: data from the Spanish rheumatology society lupus registry (RELESSER) cohort

Javier Narváez  1 , Helena Borrell  2 , Fernando Sánchez-Alonso  3 , Iñigo Rúa-Figueroa  4 , Francisco Javier López-Longo  5 , María Galindo-Izquierdo  6 , Jaime Calvo-Alén  7 , Antonio Fernández-Nebro  8 , Alejandro Olivé  9 , José Luis Andreu  10 , Víctor Martínez-Taboada  11 , Joan Miquel Nolla  2 , José María Pego-Reigosa  12 ; RELESSER Study Group
Collaborators, Affiliations

Primary respiratory disease in patients with systemic lupus erythematosus: data from the Spanish rheumatology society lupus registry (RELESSER) cohort

Javier Narváez et al. Arthritis Res Ther. .

Abstract

Background: 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.

Methods: All 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.

Results: In 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.

Conclusion: Except for pleural disease, the remaining respiratory manifestations are very uncommon in SLE (<4%). Pleuropulmonary manifestations independently contributed to a decreased survival in these patients.

Keywords: Pleuropulmonary involvement; Systemic lupus erythematosus.

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Conflict of interest statement

Ethics approval and consent to participate

The study protocol was approved by the institutional ethics committee of the Hospital Universitario Doctor Negrín (Las Palmas de Gran Canaria) and subsequently by the local ethics committee of all participating centers. Informed consent was obtained from the patients, and their clinical records and information were anonymized prior to analysis. Confidential information of the patients was protected in accordance with national norms. This study was conducted in accordance with the principles of the Declaration of Helsinki and the International Conference for Harmonization.

Consent for publication

Informed consent was obtained from the patients, and their clinical records and information were anonymized prior to analysis. Confidential information of the patients was protected in accordance with national norms.

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Survival analysis (Kaplan–Meier) comparing patients with and without primary respiratory disease
See this image and copyright information in PMC

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