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

• Published in: Arthritis research & therapy Vol. 20; no. 1; p. 280
• Main Authors: Narváez, Javier, Borrell, Helena, Sánchez-Alonso, Fernando, Rúa-Figueroa, Iñigo, López-Longo, Francisco Javier, Galindo-Izquierdo, María, Calvo-Alén, Jaime, Fernández-Nebro, Antonio, Olivé, Alejandro, Andreu, José Luis, Martínez-Taboada, Víctor, Nolla, Joan Miquel, Pego-Reigosa, José María
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 19.12.2018; BioMed Central; BMC
• Subjects: Adult; Aged; Aged, 80 and over; Comorbidity; Complications and side effects; Cross-Sectional Studies; Development and progression; Female; Humans; Kaplan-Meier Estimate; Lung Diseases - diagnosis; Lung Diseases - epidemiology; Lupus eritematós; Lupus erythematosus; Lupus Erythematosus, Systemic - epidemiology; Lupus Nephritis - epidemiology; Malalties de la pleura; Malalties dels pulmons; Male; Middle Aged; Pleural disease; Pleuropulmonary involvement; Prevalence; Prognosis; Pronòstic mèdic; Proportional Hazards Models; Pulmonary diseases; Registries - statistics & numerical data; Respiratory tract diseases; Retrospective Studies; Risk factors; Spain - epidemiology; Statistics; Systemic lupus erythematosus; Young Adult; Spain; Pleuropulmonary involvement; Systemic lupus erythematosus
• ISSN: 1478-6362; 1478-6354; 1478-6362
• DOI: 10.1186/s13075-018-1776-8

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. 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. 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. 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.