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SP0215 Prospective Correlation of Angiogenetic Factors, Microvascular Abnormalities, Organ Involvement in Systemic Sclerosis
  1. G. Lepri1,2
  1. 1Rheumatology A department, Paris Descartes University, Paris, France
  2. 2Department of Clinical and Experimental Medicine, Section of Rheumatology, University of Florence, Florence, Italy

Abstract

Prospective correlations of microvascular worsening detected bycapillaroscopy and internal organ involvement aggravation in systemic sclerosis The aim was the evaluation of NVC may be considered as a useful follow-up tool of SSc patients from the very early to the advanced phase. Among 172 SSc patients, 103/172 subjects with at least 2 capillaroscopic evaluations were enrolled in the study. Mean time between the two evaluations was 13,88±5,05 months (±sd). Qualitative and quantitative evaluation of NVC images was performed. The decrease of mean and max capillaries per finger seemed to be the most frequent event, followed by the appearance of neaongiogenesis. Regarding internal organ worsening, the reduction of FVC>10% and DLCO>10% from the baseline value were the most frequent event, occurring in 15,59% and 20,39% of the study population respectively. Our preliminary results showed that worsening of NVC pattern occurred in about 11% of patients after a mean follow-up of 12 months. The semi-quantitative scoring may result an useful tool to quantify SSc microangiopathy during the follow up: the decrease of mean number of capillaries/finger and the appearance of enlarged capillaries were the more frequent events, but no correlation with clinical worsening was found. This is probably because these events are the first NVC detectable alterations in SSc patients. Our study may suggest the importance of the appearance of neoangiogenesis, as this event may correlate with clinical worsening after a mean follow-up of about 12 months (new DUs and sPAP>40 mmHg at the echocardiography) (p-value<0,05). The statistical analysis is still in progress, data have to be confirmed by further analysis.

Preliminary quantification of EPCs levels in VEDOSS patients The evaluation of EPCs levels in VEDOSS patients has was started in a small pilot population (n=7), not suitable for any statistical analysis or comparision with data already obtained in control and established-SSc population. All patients presented Raynaud's phenomenon and ANA positivity; nailfold videocapillaroscopy was normal in 4/7 patients.EPC quantification has been expressed per million Lin- mononuclear cells. The mean value was: 14,06±9,18 (x106 Lin- mononuclear cells). Min value: 0,29 and 25,90 (106 Lin-mononuclear cells).

Rituximab effects on interstitial lung disease in systemic sclerosis (SSc), mixed connective tissue disease (MCTD) and anti-synthetase syndrome (SYN) The aim was the comparision of RTX effects on ILD in patients with SSc, MCTD and SYN.15 SYN, 6 MCTD and 23 SSc patients were enrolled. All underwent at baseline high-resolution computed tomography (HRCT) and lung function tests (LFTs) performed at 1 and 2 years of follow-up. The primary outcomes were the change in forced vital capacity (FVC) and the percentage of responders, defined by an increase in FVC≥10%.In SYN population, a trend toward FVC improvement from baseline to 2 years was observed (p=0,142). In SSc, a trend of improvement at 1 year (81.00% vs 89.00%,NS) and a stabilization at 2 years (74.50%,NS) were observed. In MCTD, FVC remained stable at 1 and 2 years (NS). In SYN patients, the percentage of responders for FVC was greater than in SSc (p=0.071) and MCTD (p=0.454). RTX showed a satisfactory safety profile in all patients. A trend of improvement in the SYN and a stabilization of ILD in SSc and MCTD were observed. Future trials, supported by the good safety profile, are warranted to determine the role of RTX in ILD treatment.

Clinical activities carried out during the stay Attendance of consultation of Prof. Y. Allanore and Dr. J Avouac. Daily attendance of One Day Hospital department (Cochin Hospital, Paris). Frequency in the laboratory for EPC quantification and participation in meetingsconductedin the departmentofRheumatology (Cochin Hospital, Paris)

Disclosure of Interest None declared

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