Nail-fold capillary abnormalities are associated with anti-centromere antibody and severity of digital ischaemia.

OBJECTIVES: Advances in nail-fold capillaroscopy allow capillary abnormalities to be quantified. Our aim was to investigate, in patients with SSc, the relationships between the degree of nail-fold capillary abnormality and disease subtypes (lcSSc and dcSSc), duration of RP and the presence of (i) severe digital ischaemia (as defined by previous i.v. vasodilators, debridements or amputations), (ii) a positive ACA, (iii) clinically evident calcinosis, (iv) pulmonary arterial hypertension and (v) telangiectases. METHODS: This was a retrospective study of 176 patients. Six capillary measurements (four semi-automated and two manual) were calculated (automated width, distance between capillaries, tortuosity and derangement, and manual distance and density). Relationships between these measurements and the different clinical features of SSc were examined using multiple linear regressions (adjusted for age, sex and smoking status). RESULTS: One hundred and forty-two patients had lcSSc and 34 had dcSSc. Sixty-eight (39%) had a history of severe digital ischaemia, 66 (38%) were anti-centromere positive, 53 (30%) had clinically evident calcinosis and 26 (15%) had an estimated pulmonary artery pressure of >30 mmHg. Positive associations were found between both automated and manually measured distance between capillaries and (i) presence of severe digital ischaemia and (ii) positive ACA, and reduced density was also associated with positive anti-centromere. Patients with moderate/severe telangiectases had wider capillaries compared with those with 'mild' lesions. CONCLUSIONS: Both severe digital ischaemia and positive ACA are associated with measurable nail-fold capillaroscopic change, which has the potential of being an outcome measure for the structural microvascular disease associated with SSc-spectrum disorders.

Observational study of treatment outcome in early diffuse cutaneous systemic sclerosis.

OBJECTIVE: Randomized clinical trials in early diffuse cutaneous systemic sclerosis (dcSSc) are challenging. We used an observational approach to estimate the relative effectiveness of different current treatment approaches, capturing entry and outcome data in a standardized way. METHODS: Patients with dcSSc within 3 years of the onset of skin thickening were included. Standardized entry and followup data were collected in relation to the first disease-modifying treatment at baseline and 4-6 weeks, then 3, 6, 12, 18, 24, 30, and 36 months. The 5 different protocols were (1) intravenous cyclophosphamide followed by mycophenolate mofetil (MMF); (2) antithymocyte globulin followed by MMF; (3) MMF alone; (4) no disease-modifying treatment; (5) other immunosuppressant treatment. The primary outcome measure was the modified Rodnan skin score (mRSS). Inverse probability of treatment weights were used to allow for differing patient characteristics between groups. RESULTS: The study included 147 patients from 12 centers. Numbers of patients starting on Protocols 1 to 5 were 29, 25, 61, 19, and 13, respectively. mRSS decreased over time from 24 (IQ 19-32) at baseline to 15.5 (IQ 9-24.5) at 3 years. Although there were differences in the magnitude of the change for different protocols, there were no significant differences between protocols in the rate of change of mRSS over time (p = 0.43). When inverse probability weights were applied, the results remained nonsignificant (p = 0.41). CONCLUSION: Using this observational approach, there were no obvious differences in outcome between groups after allowing as far as possible for baseline differences in treatment allocations.