Background Pulmonary arterial hypertension (PAH) affects 5-15% of all systemic sclerosis (SSc) patients and is associated with up to 30% mortality at 3 years. Early diagnosis improves the outcomes of PAH. We examined two cohorts: (1) The Pulmonary Hypertension Assessment and Recognition Outcomes in Scleroderma (PHAROS); a prospective cohort of SSc patients at risk for PAH or with incident PAH (1-2) and (2) An inception Paris Cochin Rheumatology cohort evaluating SSc patients suspected of having PAH (3).
Objectives To evaluate various routinely collected clinical parameters and determine the number of cases missed by a single parameter versus a combination of parameters as non-invasive diagnostic screen for PAH (WHO Group I) in SSc patients.
Methods Non-invasive tests including,- patient characteristics, systolic pulmonary arterial pressure (sPAP) on echocardiogram (TTE), FVC%/DLCO% ratio, and DLCO% predicted were evaluated alone and in combination for their ability to diagnose PAH in SSc patients as compared to right heart catheterization (RHC), the gold standard for diagnosis.
Results In the PHAROS cohort (N=206), 59 had PAH. In the Cochin cohort (N=141), 10 had PAH. Various sPAP cut-off’s resulted in missed cases of RHC PAH. By also including DLCO or FVC/DLCO ratio, 50%>78% of patients with RHC PAH missed by TTE were captured by PFT. (Table) In a multivariant logistic regression model (PAH vs No-PH) of the combined data, sPAP>40mmHg (OR 8.1 [95%CI 2.9,25.1]) was the only significant determinant after adjusting for age, gender, disease duration, FVC/DLCO≥ 1.6, anti-centromere antibody and anti-SCL-70 antibody.
Conclusions Using two large scleroderma cohorts, a combination of TTE with PFT complement each other for diagnosis of PAH. PFT with DLCO should be performed to screen for PAH in SSc.
Hinchcliff, M., A. Fischer, et al. (2011). “Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS): baseline characteristics and description of study population.” J Rheumatol 38(10): 2172-2179.
Bae, S., R. Saggar, et al. (2012). “Baseline characteristics and follow-up in patients with normal haemodynamics versus borderline mean pulmonary arterial pressure in systemic sclerosis: results from the PHAROS registry.” Ann Rheum Dis 71(8): 1335-1342.
Avouac, J., P. Airo, et al. (2010). “Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies.” J Rheumatol 37(11): 2290-2298
Disclosure of Interest None Declared
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