2014
DOI: 10.1016/j.lpm.2014.08.001
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Pathophysiology of systemic sclerosis: State of the art in 2014

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Cited by 56 publications
(39 citation statements)
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“…We have already suggested that it can be explained by the diverse and sometimes overlapping mechanisms of PH in the setting of SSc. Indeed, SSc combines an extensive vasculopathy, inflammation, autoimmunity and fibrogenesis leading to widespread fibrosis and vascular manifestations [17]. As a consequence, PH can therefore be explained by a pulmonary arterial vasculopathy (group 1), lung fibrosis (group 3) or heart involvement (group 2) as well as by PVOD-like lesions (group 1′).…”
Section: Clinical Phenotypes Of Pulmonary Hypertensionmentioning
confidence: 99%
“…We have already suggested that it can be explained by the diverse and sometimes overlapping mechanisms of PH in the setting of SSc. Indeed, SSc combines an extensive vasculopathy, inflammation, autoimmunity and fibrogenesis leading to widespread fibrosis and vascular manifestations [17]. As a consequence, PH can therefore be explained by a pulmonary arterial vasculopathy (group 1), lung fibrosis (group 3) or heart involvement (group 2) as well as by PVOD-like lesions (group 1′).…”
Section: Clinical Phenotypes Of Pulmonary Hypertensionmentioning
confidence: 99%
“…Upon the degree of skin involvement, extended and limited SSc are differentiated. The extensive skin damage, associated with a degree of visceral organs involvement and the presence of heart, lung or renal disease, can increase the 5 year mortality rate up to 40-50% [52][53][54][55][56].…”
Section: Hct For Systemic Sclerosismentioning
confidence: 99%
“…8 Although the pathophysiology of SSc is undoubtedly complex and remains incompletely understood, progresses have been made in elucidating at least some of the multiple mechanisms which are likely to contribute to the vascular and fibrotic alterations. 92 Most research on the changes in vascular and fibrotic features in SSc has focused on the MSCs with conflicting results. 93 BM-MSCs from patients with SSc are similar to those from healthy donors in terms of their phenotype and capacity to differentiate into adipogenic and osteogenic lineages, 94 showed an upregulation of pericytespecific markers and a decreased proliferation capacity.…”
Section: Focus On Adscs Application For Wound Healing In Systemic Sclmentioning
confidence: 99%