Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach.

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.

Treatment of Raynaud's phenomenon with the selective serotonin reuptake inhibitor fluoxetine.

OBJECTIVE: To compare fluoxetine, a selective serotonin reuptake inhibitor, with nifedipine as treatment for primary or secondary Raynaud's phenomenon. METHODS: Twenty-six patients with primary and 27 patients with secondary Raynaud's phenomenon were assigned randomly to receive 6 weeks of treatment with fluoxetine (20 mg daily) or nifedipine (40 mg daily). Following a 2-week washout period, each group was crossed over to the other treatment arm. The primary outcome variable was the frequency of attacks of Raynaud's phenomenon. Self-reported attack severity, thermographic recovery from cold challenge and plasma levels of von Willebrand factor and soluble P-selectin were also measured. RESULTS: There was a reduction in attack frequency and severity of Raynaud's phenomenon in patients treated with either fluoxetine or nifedipine, but the effect was statistically significant only in the fluoxetine-treated group (P=0.0002 for attack severity and P=0.003 for attack frequency). Subgroup analysis showed that the greatest response was seen in females and in patients with primary Raynaud's phenomenon. A significant improvement in the thermographic response to cold challenge was also seen in female patients with primary Raynaud's phenomenon treated with fluoxetine but not in those treated with nifedipine. There was no significant treatment effect on von Willebrand factor or soluble P-selectin. No significant adverse effects occurred in the fluoxetine-treated group. CONCLUSION: 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.

Toxicity profile of methotrexate in rheumatoid arthritis. A preliminary survey.

A number of patients with rheumatoid arthritis attending the Rheumatology Clinic at St Luke's Hospital are currently receiving the drug methotrexate as a second line disease-modifying agent. A survey has been conducted to assess the toxicity profile of methotrexate in 33 of these patients who were followed up for at least 1 year or until they developed side effects necessitating discontinuation of treatment. Adverse effects in this group of patients included haematological ones (6%), asymptomatic elevations of liver enzymes (57%), gastrointestinal (6%) and dermatological side effects (3%). These results have been compared to larger studies performed abroad. Regular monitoring of a complete blood count and liver function tests has helped to detect the more serious side effects of methotrexate at an early stage enabling successful intervention in these patients.

Paget's disease of bone in Malta. A preliminary survey.

We present the results of a preliminary survey carried out on 46 patients with Paget's disease of bone in Malta, 40 of whom were seen at a general medical outpatient clinic and a further 6 at a primary health care centre over the first 6 months of 1997. Various aspects of the disease have been analysed: prevalence, age and sex distribution, familial aggregates, mode of presentation, complications, pattern of bone involvement, a semi-quantitative assay of disease activity as assessed by bone scintigraphy and serum alkaline phosphatase (SAP) levels, and the factors that influenced disease activity. The results have been compared to previous published surveys on Paget's disease in different countries.