1. Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort: results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature
- Author
-
Ragnar Gunnarsson, Villy Johnsen, Anne Prøven, Arne K. Andreassen, Torhild Garen, Øyvind Molberg, Liv-Turid Bertelsen, Karen Irgens, Åse Stavland Lexberg, Lamya Samir Noori Garabet, Jan Tore Gran, Kari Time, Jan Leidulf Nordeide, Sonja Pedersen, Øyvind Palm, Alvilde Dhainaut, Andrea Becker-Merok, Inge-Margrethe Gilboe, and Trond Mogens Aaløkken
- Subjects
Adult ,Male ,medicine.medical_specialty ,Hypertension, Pulmonary ,Pulmonary function testing ,Cohort Studies ,Mixed connective tissue disease ,Rheumatology ,Internal medicine ,medicine.artery ,Prevalence ,medicine ,Humans ,Pharmacology (medical) ,Mixed Connective Tissue Disease ,Norway ,business.industry ,Interstitial lung disease ,medicine.disease ,Pulmonary hypertension ,Surgery ,Cross-Sectional Studies ,Blood pressure ,Echocardiography ,Heart catheterization ,Cohort ,Pulmonary artery ,Female ,Tomography, X-Ray Computed ,business - Abstract
OBJECTIVES The aim of this study was to assess the overall prevalence of pulmonary hypertension (PH) in an unselected MCTD cohort and review the current knowledge with a systematic database search. METHODS A nationwide multicentre cohort of 147 adult MCTD patients were initially screened for PH by echocardiography, high-resolution computed tomography (HRCT), pulmonary function tests and N-terminal pro-brain natriuretic peptide (NT-proBNP) and then followed up for a mean of 5.6 years. Right-sided heart catheterization was performed when estimated pulmonary artery systolic pressure was >40 mmHg on echocardiography. PH was diagnosed according to the 2009 European Society of Cardiology and European Respiratory Society guidelines. RESULTS At inclusion, 2.0% (3/147) had established PH. Two additional PH patients were identified during follow-up, giving a total PH frequency in the cohort of 3.4% (5/147). All five had elevated serum NT-proBNP. Two had isolated pulmonary arterial hypertension (PAH) and three PH associated with interstitial lung disease (PH-ILD). Three PH patients died during follow-up. Nine other patients in the cohort also died, but none of them had echocardiographic signs of PH prior to death. CONCLUSION The data from the current unselected MCTD cohort suggest that the prevalence of PH is much lower than expected from previous studies but confirm the seriousness of the disease complication.
- Published
- 2013
- Full Text
- View/download PDF