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.

Sera from 735 patients with systemic sclerosis were classified according to antinuclear antibody (ANA) pattern as follows: centromere (25%), homogeneous (26%), fine speckled (21%), fine speckled with nucleolar (14%), coarse speckled (7%), nucleolar only (3%) and cytoplasmic only (3%). Immunoprecipitations using 35S-labelled HeLa cell antigen extract were performed using sera from 374 of these patients to detect the systemic sclerosis-specific antibodies to RNA polymerases I and III. The sera were selected to represent each ANA group, but focused on those giving fine speckled nucleoplasmic staining (with or without nucleolar staining) where all 86 sera positive for these antibodies were concentrated. Immunoprecipitates from a further 93 sera from patients with ANA-positive autoimmune diseases other than systemic sclerosis did not precipitate RNA polymerases. In addition, all sera were tested for antibodies to the extractable nuclear antigens topoisomerase I, nRNP, Ro, La and PM-Scl. Sera positive for antibodies to these antigens gave clear correlations with ANA patterns but, of the examples tested, none contained antibodies precipitating RNA polymerase I or III. Thus, sera containing antibodies to RNA polymerases I and III were exclusive of both anticentromere and anti-topoisomerase I, and formed a major serological subgroup (11.7%). Clinically, 77% were patients with diffuse cutaneous disease reflected by higher skin scores and a significantly higher incidence of renal involvement (33%) than patients with antibodies to topoisomerase I (3%).

A total of 12 weeks of varenicline followed by a further 12-week course for successful quitters is a highly cost-effective alternative compared with currently available smoking cessation options.

Objective. To identify combinations of easily acquired clinical variables, at first presentation with scleroderma, that would predict subsequent mortality. Methods. In this prospective study of all new patients at one major scleroderma center, 280 patients with definite scleroderma (according to the American College of Rheumatology criteria) whose disease onset occurred from 1982 to 1991 and who were followed up to the end of 1996 were identified. Standardized data collection was performed at entry to obtain data on major clinical and laboratory variables. Vital status was determined by linkage to the National Death Index. Results. At 5 years, 55 (26%) of 215 women and 21 (32%) of 65 men had died. Univariate analysis showed that older age, diffuse skin disease, higher skin score, low carbon monoxide diffusing capacity, abnormal elec-trocardiogram findings, proteinuria, hematuria, low he-moglobin level, elevated erythrocyte sedimentation rate (ESR), and presence of antitopoisomerase antibody were all associated with increased mortality. A logistic regression model, validated by Monte Carlo simulation, identified 3 factors, proteinuria, elevated ESR, and low carbon monoxide diffusing capacity, that in combination , had an accuracy of >80% in predicting mortality. The absence of these 3 factors was associated with 93% survival. Conclusion. A simple model has been developed that appears to accurately predict mortality over 5 years in a cohort of patients newly presenting with sclero-derma.

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