Search

AIM: Vascular damage and fibrotic process represent the pathophysiological hallmarks of systemic sclerosis (SSc). Peripheral microangiopathy can be early detected by nailfold videocapillaroscopy (NVC), whereas recent studies suggest that ultrasound real time elastosonography (US-RTE) can be a useful tool to evaluate the reduction of skin elasticity. The aim of our study was to investigate possible correlations between NVC microvascular alterations and finger tissue stiffness evaluated with US-RTE in SSc subjects. METHODS: 20 subjects (16 women and 4 men) who met the ACR criteria for SSc were recruited. All subjects underwent complete clinical examination, NVC and US-RTE evaluation. US-RTE was carried out on both the hands, at the level of the palmar surface of the distal phalanx of the fingers. RESULTS: 10 subjects showed limited disease subset (LSS) and 10 showed diffuse disease subset (DSS). Eight patients had 'Early/Active' NVC pattern and 12 had 'Late' NVC pattern. DSS subjects showed highest skin stiffness values when compared to both LSS (p = 0.03) and healthy subjects (p = 0.002). Multiple regression analysis shows an independent association between Late NVC pattern and skin stiffness (R2 = 0.24, p = 0.027). CONCLUSION: This study demonstrates, for the first time, a strong relationship between microvascular alterations and skin stiffness, evaluated with US-RTE, in SSc patients.

Summary Introduction The term scleroderma derives from the Greek words skleros , which means hard, and derma , which means skin . It refers to an acquired systemic inflammatory disease of the connective tissue --also known as systemic sclerosis (SSc)-- characterized by excessive collagen deposition in the skin and the internal organs that results in fibrosis. The typical vascular lesion in SSc leads to narrowing of the vessel lumen, intimal thickening, medial hypotrophy, and adventitial fibrosis of small muscular vessels, collagen deposition in the other matrix components of interstice, and the Raynaud phenomenon secondary to these widespread microvascular abnormalities. All these characteristics lead to a connective tissue re-modeling. Discussion Several clinical studies utilize the American Rheumatology Association's 1980 classification. However, these diagnostic criteria are unsatisfactory because they fail to take into consideration part of the disease spectrum. Early-phase SSc is characterized by the Raynaud phenomenon (in 90% of all patients), sclerodactyly, and positivity for SSc-specific autoantibodies (antinuclear antibodies, anti-topoisomerase I antibodies, anti-RNA polymerase I and III antibodies, anti-centromere antibodies, anti-fibrillarin antibodies, anti-PM-SCL antibodies).. It is necessary to reduce delays in the diagnosis of SSc. Conclusions Patients with red-flag positivity (Raynaud phenomenon and digital edema) require Phase I SSc screening, which consists in capillaroscopic assessment of possible microvascular abnormalities. The work-up will then focus on inflammatory indices, renal function tests, and internal organ involvement (echocardiography, high-resolution computed tomography of the chest, diffusing capacity of the lungs for carbon monoxide).

Background Digital ulcers (DU) affect 50% of systemic sclerosis (SSc) patients, representing a challenging clinical problem. Despite a high negative predictive value, capillaroscopic scores proposed to select patients at risk for DU show an inadequate positive predictive value, especially in patients without previous DU. Aim of this study To increase the predictive value for DU development of capillaroscopy, through a predictive risk chart taking into account capillaroscopic, demographic, and clinico-serological parameters. Patients and methods Two hundred and nineteen unselected SSc patients from 8 Italian Rheumatology Centers were consecutively enrolled during a 6-month period. Demographic, clinical, serological and instrumental data and capillaroscopy skin ulcers risk index (CSURI) were collected. Results A multivariate logistic regression analysis showed a significant positive association between DU appearance and male gender, DU history, altered CSURI, and ESR. A prediction risk chart of the development of DU within 6 months were built on the basis of the above parameters. According to the risk level, four risk classes were identified: low (≤19.3%); medium (>19.3%, ≤58.6%); high (>58.6%, ≤89.2%), and very high risk (>89.2%). Conclusions The systematic evaluation of the above parameters can be helpful to identify patients at risk to develop DU optimizing preventive vasoactive therapy.

Background Patients with peripheral arterial disease (PAD) at stage IIb, pain-free walking distance (PFWD) less than 100 m and unsuitable for revascularization have both impaired quality of life and severe clinical outcome. Aim of the study was to evaluate the efficacy of the prostacyclin analogue iloprost, added to standard therapy, in these patients. Material and methods Patients were randomized to receive standard medical therapy (Group A) or standard therapy plus iloprost (Group B), for 1 year. Iloprost was administered for 10 days every 3 months. Treadmill test was performed every 3 months, in Group B before starting the 10-day iloprost cycle. Results Fifty patients in Group A and 51 in Group B were enrolled. Mean baseline and 12-month PFWD values were 75·4 ± 21·3 and 128·9 ± 62·9 for iloprost group and 70·3 ± 21·6 and 99·6 ± 62·6 m for controls. Patients treated with iloprost had significantly higher PFWD at 9 and 12 months. This finding was confirmed after carrying forward the last valid observation (124·7 ± 63·4 vs. 88·4 ± 63·1 m, P

IntroductionThe early detection of systemic sclerosis (SSc) patients at high risk of developing digital ulcers could allow preventive treatment, with a reduction of morbidity and social costs. In 2009, a quantitative score, the capillaroscopic skin ulcer risk index (CSURI), calculated according to the formula ‘D×M/N2’, was proposed, which was highly predictive of the appearance of scleroderma digital ulcers within 3 months of capillaroscopic evaluation.ObjectivesThis multicentre study aims to validate the predictive value and reproducibility of CSURI in a large population of SSc patients.MethodsCSURI was analysed in 229 unselected SSc patients by nailfold videocapillaroscopy (NVC). All patients were re-evaluated 3 months later with regard to the persistence and/or appearance of new digital ulcers.Results57 of 229 patients presented with digital ulcers after 3 months. The receiver operating characteristic curve analysis showed an area under the curve of 0.884 (95% CI 0.835 to 0.922), with specificity and sensitivity of 81.4% (95% CI 74.8 to 86.89) and 92.98% (95% CI 83.0 to 98.0), respectively, at the cut-off value of 2.96. The reproducibility of CSURI was validated on a random sample of 81 patients, with a κ-statistic measure of interrater agreement of 0.8514.ConclusionsThe role of CSURI was confirmed in detecting scleroderma patients with a significantly high risk of developing digital ulcers within the first 3 months from NVC evaluation. CSURI is the only method validated to predict the appearance of digital ulcers and its introduction into routine clinical practice might help optimise the therapeutic strategy of these harmful SSc complications.

Background Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by vascular damage and severe fibroproliferative phenotype, which affects skin and internal organs. To date, the modified Rodnan Skin Score (mRSS) is the most employed technique to assess cutaneous involvement in SSc. However, recent studies consider the employment of ultrasound elastosonography (UE), highlighting its ability to identify the reduction of strain in the skin. Currently, SSc can be classified into two different subsets: limited (LSS) and diffuse (DSS) disease. The former is usually confined to specific regions of the body, with no internal organ involvement, the latter is characterized by diffuse skin involvement, severe internal organ involvement and poor prognosis. Objectives The present study was aimed to explore possible differences in skin stiffness between LSS and DSS by using UE. Methods 20 SSc patients (10 with LSS and 10 with DSS) and 10 healthy subjects, were recruited for the present study. They underwent clinical examination and ultrasound evaluation. UE was performed at palmar surface of each finger tip. Images were obtained by applying repetitive skin compression. The elastogram, which reflects the relative elasticity of the tissues, was created as a color coded map (i.e. blue = great stiffness, red = low stiffness). Images were analyzed by a proprietary software (Esaote elastosonography module, Esaote, Inc., Italy) that allows to obtain a numeric evaluation of stiffness expressing the global percentage of hardness. “Skin stiffness” was computed by considering the average of eight fingers for each patient. Results Table 1 reports clinical and ultrasound features of SSc group and control group. As shown, mean age was 61±14 years in SSc subjects and 55±13.9 years in healthy subjects. Overall, SSc subjects showed higher skin stiffness values than control group (p Conclusions The present investigation has two main results. First, ultrasound with elastosonography seem to discriminate skin stiffening in SSc patients compared to healthy subjects, thus confirming the findings of previous investigations. Second, among SSc patients, those with DSS show a higher skin stiffness compared to LSS subjects, supporting the more severe course of DSS compared to LSS. References Iagnocco A et al (2010) J Rheumatol 37: 1688-91 Ostojic P et al (2006) Clin Rheumatol 25:453-7 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.1280