Microvascular damage in systemic sclerosis: detection and monitoring with biomarkers.
ABSTRACT Vascular disease is universal in patients with systemic sclerosis (SSc), but there is a wide variability in its severity. It is clear that there is an early insult to the microvasculature, followed-up by on-going chronic process. This results in profound vascular damage in a subset of patients who develop severe events such as digital loss and pulmonary arterial hypertension. Although there is abundant evidence of vascular perturbation from studies of peripheral blood in SSc, there are few data about the ability to use these biomarkers to predict vascular outcomes. This paper examines the possibility of using circulating biomarkers to assess vascular disease activity and to predict severe vascular events among patients with SSc.
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ABSTRACT: Objective. Multisite photoplethysmography (PPG) cardiovascular assessments can evaluate endothelial, peripheral autonomic and arterial dysfunction. The aim of this pilot study was to investigate the potential clinical utility of the technology in assessing patients with SSc and primary RP (PRP). Methods. Multisite PPG pulse measurements, a reference ankle brachial pressure index (ABPI) and a full clinical assessment were undertaken for three subject groups: SSc, PRP and controls. Endothelial and autonomic function and arterial disease measures were obtained using pulse wave analysis. Results. Nineteen SSc, 19 PRP and 23 control subjects were assessed and compared. Endothelial function was significantly impaired in SSc (P < 0.02), but with no difference between controls and PRP. Receiver operating characteristic-based classification accuracy was 81% (sensitivity 90%, specificity 74%) for separating SSc from controls and 82% (sensitivity 84%, specificity 79%) for separating SSc from PRP. SSc patients with digital ulcers had significantly lower endothelial function compared with those without ulcers (P < 0.05). Autonomic dysfunction was suggested in both SSc and PRP and was most exaggerated in patients with diffuse SSc. All groups had overall normal ABPI and arterial stiffness timing measures. Bilateral timing differences at the toes, which represents peripheral occlusive arterial disease, did show increased asymmetry in SSc (P < 0.02). Conclusion. Multisite PPG pulse technology showed potential diagnostic ability. By using measures of endothelial function, it differentiated SSc from control and PRP subjects with an accuracy of at least 81%. Objective pulse-derived measures of autonomic function and arterial disease in SSc have also been reported in this pilot study.Rheumatology (Oxford, England) 05/2014; 53(10). DOI:10.1093/rheumatology/keu196 · 4.44 Impact Factor
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ABSTRACT: OBJECTIVES: To describe three patients with systemic sclerosis (SSc) who developed acute unilateral blindness in the absence of any common etiologic factor for blindness. In one patient, the affected eye required enucleation and was examined histopathologically. METHODS: Following identification of the first patient with retinal artery occlusion at the Scleroderma Center of Thomas Jefferson University, every patient evaluated at the Center from May 2001 to December 2010 was prospectively assessed for the development of acute unilateral blindness. Two additional cases were identified. Here, we describe the clinical features, laboratory and ancillary examinations of the three patients with SSc who developed acute unilateral blindness and present the histopathological examination of one eye enucleated from one of the patients. RESULTS: Clinical and angiographic studies were consistent with acute retinal artery occlusion. The histopathological studies showed severe retinal ischemic atrophy and concentric narrowing and fibrosis of small retinal vessels. CONCLUSIONS: These findings suggest that acute retinal artery occlusion in these patients is a manifestation of the fibroproliferative vasculopathy characteristic of SSc.Seminars in arthritis and rheumatism 02/2013; 43(2). DOI:10.1016/j.semarthrit.2012.12.025 · 3.63 Impact Factor
Article: Etiopatogenia. Nuevos conceptos[Show abstract] [Hide abstract]
ABSTRACT: Systemic sclerosis is a complex, progressive autoimmune disease. The origin is, so far, unknown and it is characterized by immunological and endothelial damage followed by fibrosis. Interaction between the host genetic backgrounds with environmental factors is thought to turn out an abnormal immune response characterized by clonal expansion of Th2 repertoire, upregulation of pro-fibrotic cytokines and dysregulated B cells. Specific autoantibodies profiles are associated with clinical subtypes of the diseases. Endothelial activation is an early feature with damage of the vasocontrictive and vasodilation response. Finally, persistent tissue ischemia and abnormal immune response produce myofibroblast proliferation andoverproduction of extracellular matrix proteins and fibrosis.Reumatología Clínica 11/2006; 2. DOI:10.1016/S1699-258X(06)73100-X