Date: Monday, November 6, 2017
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: To assess the prevalence, risk factors and impact on mortality of primary respiratory disease in a large retrospective cohort.
Methods: All adult patients in the Spanish Rheumatology Society Lupus Registry (RELESSER) who meet ≥ 4 ACR-97 SLE criteria were retrospectively investigated for the presence of primary pleuropulmonary manifestations.
Results: A total of 3215 patients were included: 91% were female, their mean (SD) age at diagnosis was 37 yrs (13.5), and the median follow-up time was 136 (97.7) months.
At least one pleuropulmonary manifestation was present in 11.3% (365) of the patients (289 (79.2%) patients developed only one manifestation; the rest had two or more). The mean duration of SLE until the appearance of the first respiratory manifestation was 5.8 (SD: 8.3) years.
The most common manifestation was pleurisy, which occurred in 21.1% (680) of the patients, followed by acute lupus pneumonitis (or interstitial alveolitis/pneumonitis according with BILAG 2004 definition) in 3.6% (118); pulmonary thromboembolism (including lung infarction) 2.9% (95); primary pulmonary hypertension in 2.4% (79); diffuse interstitial lung disease (or pulmonary fibrosis according with SLICC ACR Damage Index 1996) 2% (65); alveolar hemorrhage (BILAG 2004 definition) 0.8% (28); and shrinking lung syndrome (BILAG 2004 definition) 0.8% (28).
Twenty six patients with acute lupus pneumonitis and six patients with alveolar hemorrhage finally developed interstitial lung disease/pulmonary fibrosis.
In the multivariable analysis, the variables independently associated with the presence of pleuropulmonary manifestation were older age at disease onset (OR 1.03; 95% CI 1.02-1.04), higher SLEDAI scores (OR 1.03; 95% CI 1.00-1.07), the presence of Reynaud’s phenomenon (OR 1.41; 95% CI 1.09-1.84), secondary antiphospholipid syndrome (OR 2.20; 95% CI 1.63-2.96), anti-RNP positivity (OR 1.32; 95% CI 1.00-1.75), and the previous or concomitant presence of severe lupus nephritis (including classes III, IV, V and mixed III/IV + V) (OR 1.48; 95% CI 1.12-1.95), neuropsychiatric manifestations (OR 1.49; 95% CI 1.11-2.02), primary 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).
Sixty-one (1.89%) patients with pleuropulmonary manifestations died over the follow-up period. Although the mortality rate was low, the development of respiratory disease was associated with lower survival (survival rates 95.6% versus 82.2%; p=0.030). After adjusting for known confounders in the multivariable Cox regression model pleuropulmonary manifestations remained a risk factor for diminished survival (HR: 3.13; 95% CI 1.56–6.28, p=0.001).
Conclusion: Primary respiratory disease is not uncommon in patients with SLE and independently contributed to a decreased survival in these patients. This complication occurs mainly in patients with active and severe disease (with previous or concomitant major organ involvement other that lung) and seems to be associated with the positivity of antiphospholipid and anti-Sm antibodies.
To cite this abstract in AMA style:Narváez J, Borrell H, Sánchez-Alonso F, Rúa-Figueroa I, López Longo FJ, Galindo M, Calvo-Alén J, Andreu JL, Andres M, Alegre JJ, Blanco R, Cobo-Ibáñez T, Bonilla G, Boteanu A, Diez Alvarez E, Fernandez-Nebro A, Freire M, Gantes M, Garcia de la Peña P, García-Vicuña R, Hernández Beiraín J, Horcada ML, Ibañez J, Juan A, Lozano-Rivas N, Marenco de la Fuente JL, Melero González RB, Montilla-Morales CA, Moreno M, Olivé A, Oton Sanchez MT, Pecondon-Español A, Ruiz Lucea E, Sánchez Atrio A, Santos-Soler G, Toyos F, Uriarte Isacelaya E, Vazquez Rodriguez TR, Nolla JM, Pego-Reigosa J. Primary Respiratory Disease in Patients with Systemic Lupus Erythematosus: Data from the Spanish Rheumatology Society Lupus Registry (RELESSER) Cohort [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/primary-respiratory-disease-in-patients-with-systemic-lupus-erythematosus-data-from-the-spanish-rheumatology-society-lupus-registry-relesser-cohort/. Accessed June 5, 2020.
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