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Prevalence, Treatment, and Outcomes of Coexistent Pulmonary Hypertension and Interstitial Lung Disease in Systemic Sclerosis
- Source :
- Arthritis Rheumatol
- Publication Year :
- 2019
- Publisher :
- Wiley, 2019.
-
Abstract
- Objective Systemic sclerosis (SSc) is associated with interstitial lung disease (ILD) and pulmonary hypertension (PH). This study was undertaken to determine the prevalence, characteristics, treatment, and outcomes of PH in a cohort of patients with SSc-associated ILD. Methods Patients with SSc-associated ILD on high-resolution computed tomography (HRCT) were included in a prospective observational cohort. Patients were screened for PH based on a standardized screening algorithm and underwent right-sided heart catheterization (RHC) if indicated. PH classification was based on hemodynamic findings and the extent of ILD on HRCT. Summary statistics and survival using the Kaplan-Meier method were calculated. Results Of the 93 patients with SSc-associated ILD included in the study, 76% were women and 65.6% had diffuse cutaneous SSc. The mean age was 54.9 years, and the mean SSc disease duration was 8 years. Twenty-nine patients (31.2%) had RHC-proven PH; of those 29 patients, 24.1% had PAH, 55.2% had World Health Organization (WHO) Group III PH, 34.5% had WHO Group III PH with pulmonary vascular resistance >3.0 Wood units, 48.3% had a PH diagnosis within 7 years of SSc onset, 82.8% received therapy for ILD, and 82.8% received therapy for PAH. The survival rate 3 years after SSc-associated ILD diagnosis for all patients was 97%. The survival rate 3 years after PH diagnosis for those with SSc-associated ILD and PH was 91%. Conclusion In a large cohort of patients with SSc-associated ILD, a significant proportion of patients had coexisting PH, which often occurs early after SSc diagnosis. Most patients were treated with ILD and PAH therapies, and survival was good. Patients with SSc-associated ILD should be evaluated for coexisting PH.
- Subjects :
- Adult
Male
Cardiac Catheterization
medicine.medical_specialty
Hypertension, Pulmonary
Immunology
Comorbidity
Kaplan-Meier Estimate
030204 cardiovascular system & hematology
Gastroenterology
Article
03 medical and health sciences
0302 clinical medicine
Rheumatology
Internal medicine
Prevalence
medicine
Humans
Immunology and Allergy
Prospective Studies
skin and connective tissue diseases
Prospective cohort study
Survival rate
030203 arthritis & rheumatology
Scleroderma, Systemic
integumentary system
business.industry
Interstitial lung disease
Middle Aged
respiratory system
medicine.disease
Pulmonary hypertension
respiratory tract diseases
Survival Rate
body regions
Treatment Outcome
medicine.anatomical_structure
Heart catheterization
Cohort
Vascular resistance
Female
Lung Diseases, Interstitial
Tomography, X-Ray Computed
business
Subjects
Details
- ISSN :
- 23265205 and 23265191
- Volume :
- 71
- Database :
- OpenAIRE
- Journal :
- Arthritis & Rheumatology
- Accession number :
- edsair.doi.dedup.....ba0bca332ed36594167fdac1f7713b4f