Objective: To determine the prevalence of systemic sclerosis associated pulmonary arterial hypertension (SScPAH), evaluate outcome, and identify predictors of mortality in a large patient cohort. Methods: A prospective four year follow up study of 794 patients (722 from our own unit and 72 referrals). All patients screened for PAH using a combination of echocardiography, lung function testing, and clinical assessment. Patients with suspected raised pulmonary artery systolic pressures of .35 mm Hg, carbon monoxide transfer factor (TLCO) ,50% predicted, or a precipitous fall in TLCO .20% over a one year period with no pulmonary fibrosis, and patients with SSc with breathlessness with no pulmonary fibrosis found were investigated with right heart catheterisation. All patients with SScPAH were treated in accordance with current best practice. Results: The prevalence of PAH was 12% (89/722) by right heart catheter. The survival was 81%, 63%, and 56% at 1, 2, and 3 years from the diagnosis (in 89 patients from our own cohort and 59/72 referrals). Haemodynamic indices of right ventricular failure-raised mRAP (hazard ratio 21), raised mPAP (hazard ratio 20), and low CI (hazard ratio 11) predicted an adverse outcome There was no significant difference in survival between patients with SScPAH with (n = 40) and without (n = 108) pulmonary fibrosis (p = 0.3). Conclusions: The prevalence of SScPAH in this cohort was similar to that of other catheter based studies and lower than that of previous echo based studies. The 148 patients with SScPAH actively treated had comparable outcomes to those of the cohorts with primary pulmonary hypertension. A high mRAP was the strongest haemodynamic predictor of mortality. To improve prognosis, future treatments need to be implemented at an earlier disease stage to prevent right ventricular decompensation.

Pulmonary hypertension (PHT) is an important complication of systemic sclerosis (SSc). Echocardiography can be used to detect PHT and, with Doppler echocardiography, the pulmonary arterial systolic pressure (PASP) can often be estimated. We have undertaken a study to compare echocardiographic assessment with right heart catheterization (RHC) in 33 SSc patients in whom clinical assessment [including ECG, chest X-ray, lung function tests and high-resolution computed tomography (HRCT) had raised strong suspicion of PHT. The mean (S.D.) interval between echocardiography and RHC was 1.8 (2.3) months. Twenty-one patients (64%) had PHT (PASP > or = 30 mmHg) on RHC, and echocardiography correctly identified 19 of these (sensitivity 90%). Of the 12 patients without PHT on RHC, nine were correctly identified by echocardiography (specificity 75%). The five incorrectly classified patients all had PASP in the borderline normal/abnormal range. The presence of tricuspid regurgitation allowed Doppler measurement of PASP in 20 patients (61%) and this correlated significantly with RHC values (r = 0.83, P < 0.001). We conclude that echocardiography is a reliable method for detecting PHT and it may be particularly useful for the early detection and monitoring of this potentially fatal complication in SSc.

Objective-To develop criteria for disease activity in systemic sclerosis (SSc) that are valid, reliable, and easy to use. Methods-Investigators from 19 European centres completed a standardised clinical chart for a consecutive number of patients with SSc. Three protocol management members blindly evaluated each chart and assigned a disease activity score on a semiquantitative scale of 0-10. Two of them, in addition, gave a blinded, qualitative evaluation of disease activity ("inactive to moderately active" or "active to very active" disease). Both these evaluations were found to be reliable. A final disease activity score and qualitative evaluation of disease activity were arrived at by consensus for each patient; the former represented the gold standard for subsequent analyses. The correlations between individual items in the chart and this gold standard were then analysed. Results-A total of 290 patients with SSc (117 with diVuse SSc (dSSc) and 173 with limited SSc (lSSc)) were enrolled in the study. The items (including -factorsthat is, worsening according to the patient report) that were found to correlate with the gold standard on multiple regression were used to construct three separate 10-point indices of disease activity:

Our results demonstrate that thermography is a promising diagnostic tool when associated with clinical examination in discriminating disease activity, as long as it is applied to lesions without severe atrophy of the skin and subcutaneous fat. Further evaluation is needed to determine whether thermography can predict the future progression of lesions.

This pilot study confirms the tolerability of fluoxetine and suggests that it would be effective as a novel treatment for Raynaud's phenomenon. Larger and placebo-controlled trials are warranted to assess fluoxetine's therapeutic potential further in this vasospastic condition.