• This record comes from PubMed

Pulmonary arterial hypertension associated with systemic sclerosis in the Czech Republic

. 2012 Mar ; 31 (3) : 557-61. [epub] 20111122

Language English Country Germany Media print-electronic

Document type Journal Article, Research Support, Non-U.S. Gov't

BACKGROUND: Systemic sclerosis (SSc) is an important cause of pulmonary arterial hypertension (PAH), with an estimated prevalence of 7.85-26.7%. OBJECTIVE: Our aim was to estimate the prevalence of PAH among patients with SSc in the Czech Republic and to compare haemodynamics in SSc patients diagnosed with PAH through screening with those diagnosed previously, based on symptoms. METHODS: During 2007, SSc patients in the Czech Republic, without significant pulmonary function impairment or cardiac disease, underwent screening for PAH with transthoracic echocardiography. Those with a tricuspid regurgitant (TR) jet gradient suggestive of PAH (>30 mmHg) underwent subsequent right heart catheterisation (RHC) to confirm the diagnosis (mean pulmonary arterial pressure, mPAP, ≥25 mmHg; pulmonary capillary wedge pressure, ≤15 mmHg). Haemodynamics in patients diagnosed with PAH in this way were compared with those in patients diagnosed previously, based on symptoms. RESULTS: Two hundred and three SSc patients (mean age, 53.8 ± 13 years; 82.3% women) from 26 rheumatology practices were screened. Among these, 17 had a TR jet gradient >30 mmHg and underwent RHC; PAH was confirmed in six patients. These six patients were found to have significantly lower mPAP than nine patients diagnosed previously with PAH, based on symptoms (31.17 ± 5.56 vs. 46.89 ± 9.48 mmHg, p = 0.0014). CONCLUSION: Prevalence of PAH in our SSc cohort was 7.08%. SSc patients diagnosed with PAH through screening have less advanced disease in terms of haemodynamics than those with PAH diagnosed previously based on symptoms; their prognosis is therefore likely to be more favourable.

See more in PubMed

Arthritis Rheum. 2005 Dec;52(12):3792-800 PubMed

Arthritis Rheum. 2003 Feb;48(2):516-22 PubMed

Rheumatology (Oxford). 2005 Mar;44(3):366-71 PubMed

Rheumatology (Oxford). 2001 Apr;40(4):453-9 PubMed

Eur Heart J. 2009 Oct;30(20):2493-537 PubMed

Chest. 1997 Jan;111(1):36-43 PubMed

Chest. 2003 Feb;123(2):344-50 PubMed

J Rheumatol. 2003 Nov;30(11):2398-405 PubMed

Br J Rheumatol. 1996 Oct;35(10):989-93 PubMed

Ann Rheum Dis. 2003 Feb;62(2):97-9 PubMed

Cardiol Clin. 2004 Aug;22(3):383-99, vi PubMed

Arthritis Rheum. 2005 Jul;52(7):2125-32 PubMed

Br J Rheumatol. 1997 Feb;36(2):239-43 PubMed

Ann Rheum Dis. 2003 Nov;62(11):1088-93 PubMed

J Rheumatol. 2005 Jul;32(7):1273-8 PubMed

Find record

Citation metrics

Loading data ...

Archiving options

Loading data ...