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Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus
- Source :
- Chest. 153:143-151
- Publication Year :
- 2018
- Publisher :
- Elsevier BV, 2018.
-
Abstract
- Background Pulmonary arterial hypertension (PAH) is a rare complication of systemic lupus erythematosus (SLE). Methods We identified all patients with SLE and PAH (SLE-PAH) who were enrolled in the French Pulmonary Hypertension Registry with a diagnosis confirmed by right heart catheterization (RHC). A control group of 101 patients with SLE without known PAH was selected from SLE expert centers participating in the Pulmonary Hypertension Registry. Survival was estimated by the Kaplan-Meier method. Hazard ratios associated with potential predictors of death were estimated using Cox proportional hazard models. Results Of the 69 patients with SLE-PAH identified in the French Pulmonary Hypertension Registry, 51 were included in the study. They did not differ from the control group regarding age, sex, or duration of SLE at the time of the analysis but had a higher frequency of anti-SSA and anti-SSB antibodies. The delay between SLE diagnosis and PAH diagnosis was 4.9 years (range, 2.8-12.9) years. The 3- and 5-year overall survival rates were 89.4% (95% CI, 76.2%-96.5%) and 83.9% (95% CI, 68.8%-92.1%), respectively. The survival rate was significantly better in patients with anti-U1-RNP antibodies ( P = .04). Conclusions Patients with SLE-PAH have an overall 5-year survival rate of 83.9% after the PAH diagnosis. Anti-SSA/SSB antibodies may be a risk factor for PAH, and the presence of anti-U1-RNP antibodies appears to be a protective factor regarding survival.
- Subjects :
- 030203 arthritis & rheumatology
Pulmonary and Respiratory Medicine
medicine.medical_specialty
business.industry
Hazard ratio
Interstitial lung disease
030204 cardiovascular system & hematology
Critical Care and Intensive Care Medicine
medicine.disease
Pulmonary hypertension
3. Good health
Pulmonary function testing
03 medical and health sciences
0302 clinical medicine
Internal medicine
Cardiology
Medicine
Risk factor
skin and connective tissue diseases
Cardiology and Cardiovascular Medicine
business
Prospective cohort study
Pulmonary wedge pressure
Survival rate
Subjects
Details
- ISSN :
- 00123692
- Volume :
- 153
- Database :
- OpenAIRE
- Journal :
- Chest
- Accession number :
- edsair.doi...........fa14fa0f9ec654422f68f7d6cee236b4