Blood Platelets and Systemic Sclerosis

نویسندگان

  • Sébastien Lepreux
  • Anne Solanilla
  • Julien Villeneuve
  • Joël Constans
  • Alexis Desmoulière
  • Jean Ripoche
چکیده

Systemic sclerosis (SSc) is characterized by a progressive fibrosis of the perivascular and interstitial connective tissues which can involve the skin, heart, lungs, kidneys, and the gastrointestinal tract. SSc is an uncommon, debilitating condition, associated to a vital risk linked to visceral extensions and has a high case-fatality rate among connectivitis. SSc begins in the vast majority of cases with a Raynaud’s phenomenon, may have a limited or diffuse skin extension, and is often associated to arterial occlusions, digital ulcerations or necrosis. SSc clinical manifestations are heterogeneous and classifications distinguish limited to diffuse disease, depending on the distribution of the skin lesions and organ involvement [1-3]. There is today no curative treatment. Disease susceptibility differs according to sex, age and race, there is a notable familial clustering, and SSc incidence may be rising [4, 5]. The clinical management of the patients still remains a difficult challenge and the pharmacopeia offers limited choices to the clinician to bring relief to patients. Pulmonary, renal and myocardial complications have benefited from the introduction of angiotensin converting enzyme inhibitors, calcium pump inhibitors, prostacyclin analogs and endothelin antagonists. Based on recent pathophysiological insights, a number of novel agents are being developed [6-8].

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تاریخ انتشار 2012