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Query: UMLS:C0036421 (PSS)
 10,989 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Gliddon et al. conducted a randomized, double-blind, multicenter, placebo-controlled study to evaluate the efficacy of the angiotensin-converting enzyme inhibitor quinapril for the management of vascular damage in systemic sclerosis (SSc). The trial comprised 213 patients with limited cutaneous SSc or Raynaud's phenomenon (mean age 54 years, 182 females) who were randomly assigned to receive 80 mg/day, or the maximum tolerated dose, of quinapril (n = 105) or placebo (n = 108) for 2-3 years. Patients were assessed every 3 months. The number of new ischemic digital ulcers was recorded as the primary end point, while the frequency of Raynaud's phenomenon episodes, skin score, health status, pulmonary artery pressure and treatments for ischemia were also monitored as secondary end points. There were no detectable differences between patients treated with quinapril and those receiving placebo; however, although no severe adverse effects were observed, patients taking quinapril experienced significantly more adverse effects.
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PMID:Lack of efficacy of quinapril on vascular damage in limited cutaneous systemic sclerosis. 1846 Oct 63

Scleroderma (systemic sclerosis) is a disease of unknown origins that involves tissue ischemia and fibrosis in the skin and internal organs such as the lungs. The tissue ischemia is due to a lack of functional blood vessels and an inability to form new blood vessels. Bone marrow--derived circulating endothelial progenitor cells play a key role in blood vessel repair and neovascularization. Scleroderma patients appear to have defects in the number and function of circulating endothelial progenitor cells. Scleroderma patients also develop fibrotic lesions, possibly as the result of tissue ischemia. Fibroblast-like cells called fibrocytes that differentiate from a different pool of bone marrow-derived circulating progenitor cells seem to be involved in this process. Manipulating the production, function, and differentiation of circulating progenitor cells represents an exciting new possibility for treating scleroderma.
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PMID:Circulating progenitor cells and scleroderma. 1863 25

Microvascular abnormalities and fibrosis are important targets of therapy in systemic sclerosis (scleroderma). Calcium channel blockers, ACE inhibitors, sartans, phosphodiesterase-5 inhibitors and serotonin re-uptake blockers are used for Raynaud's phenomenon. Intravenous prostanoids (alprostadil, iloprost, epoprostenol, treprostinil) and endothelin receptor antagonists (bosentan, sitaxsentan, ambrisentan) show efficacy in treatment of pulmonary hypertension and distal ischemia. Successful treatment of digital ulcers secondary to systemic sclerosis was possible with sildenafil and bosentan. A platelet gel is currently in clinical trials for scleroderma-related digital ulcers. Several drugs, which directly reduce excessive production of collagens and other connective tissue proteins have been applied in systemic sclerosis. These include interferon gamma, d-penicillamine, kolchichicine, calcitriol, and imanitib. However, so far, strategies to control fibrosis by directly reducing excessive connective tissue production have been disappointing in controlled studies.
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PMID:[Current treatment of systemic sclerosis. Part II. Vascular and antifibrotic treatment]. 1894 47

Involvement of the large arteries in patients with systemic sclerosis (SSc) has been only rarely reported. We describe the case of a 56-year-old woman with early diffuse cutaneous SSc who presented with acute unilateral digital ischemia. Evaluation identified thrombosis at the site of a previous subclavian-to-common carotid artery bypass, rather than Raynaud's phenomenon, as the cause of ischemia. Areview of the literature indicates that large artery involvement, although a relatively uncommon clinical manifestation, can be frequently detected in the lower limbs as well as the ulnar arteries in patients with SSc.
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PMID:Large artery disease in scleroderma: case report and review of the literature. 1907 71

Raynaud's phenomenon is a frequent reason for seeking of medical attention, since it affects 3-5% of the population. It is characterized by sudden, transient and recurrent episodes of pallor and/or digital cyanosis, after exposure to cold or stressful situations. No known underlying illness is identified in over 80% of cases and consequently these cases are classified as primary Raynaud's phenomenon. Connective tissue diseases, particularly systemic sclerosis, are the main causes of the phenomenon. Once a complete clinical and physical evaluation rule out other causes, a nailfold capillaroscopy and antinuclear antibodies determination are the most useful adjunctive tests. Mild Raynaud's phenomenon can be managed almost exclusively with conservative non-pharmacological lifestyle modifications. However, if a patient develops a severe vascular condition a suitable vasodilator treatment is needed. When critical digital ischemia develops, intravenous treatment with prostaglandin analogues and surgery may be useful.
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PMID:[Raynaud's phenomenon]. 2002 66

Ischemia modified albumin (IMA) is a new biological marker for early identification of chest pain and ruling out myocardial infarction among patients with acute syndromes submitting to emergency department. Recently IMA has been investigated in the light of other cardiac markers (cTnT, CK-MBmas, NT-proBNP) in various states of ischemia (acute coronary syndromes, after percutaneous coronary intervention, in coronary vasospasm). Ischemia modified albumin levels were elevated in these states what suggests myocardial ischemia. However decrease in IMA concentration after exercise-induced skeletal muscle ischemia still remains unclear. Increased IMA concentration in patients with acute ischemic stroke and exposed to trauma limits its ability for detection myocardial ischemia. Specificity of IMA measurement is limited also in patients with peripheral vascular disease, systemic sclerosis, diabetes, end stage renal disease, pulmonary embolism and other pathological states with accompanying oxidative stress.
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PMID:[Ischemia modified albumin--specific marker in cardiological diagnostics?]. 1932 66

Systemic sclerosis (SSc) is an autoimmune systemic disease characterized by small vessel involvement that leads to tissue ischemia and fibroblast stimulation resulting in accumulation of collagen (fibrosis) in the skin and internal organs. Lipomembranous panniculitis is a peculiar type of fat necrosis and has been reported with clinical conditions, commonly with peripheral vascular diseases. We describe a case of a 43-year-old woman with SSc manifestations, who presented with black scaly skin plaques, associated with thickening of the subcutaneous fat tissue, on the lateral surface of her thighs, her calves, gluteal area and lower abdomen. Biopsy revealed lipomembranous panniculitis. Lipomembranous changes have been seen in connective tissue disorders such as lupus profundus, morphea, systemic sclerosis and panniculitis associated with dermatomyositis, but rarely in thighs, calves, gluteal area and lower abdomen.
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PMID:Panniculitis-an unusual cutaneous manifestation of systemic sclerosis. 1961 30

Vasculopathy in patients with connective tissue diseases (CTDs), including systemic sclerosis (SSc) and systemic lupus erythematosus (SLE), is a serious complication that mainly affects small arteries and capillaries, reduces the blood flow and causes progressive tissue ischemia. Recently, CTD patients have been reported to have abnormalities in circulating endothelial progenitor cells (EPCs); these abnormalities are believed to contribute to the pathophysiology of vasculopathy and to the premature and accelerated development of atherosclerosis in CTD patients. Furthermore, we are currently conducting a clinical pilot study to determine the efficacy of implanting autologous mononuclear cells obtained from the bone marrow and peripheral blood into the ischemic digits or limbs of CTD patients. In this review, we discuss the role of EPCs in the process of neovascularization and in the pathophysiology of CTDs, and we describe a clinical pilot study on the use of autologous cell therapy for treating ischemic digits in patients with CTDs.
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PMID:Therapeutic neovascularization by the implantation of autologous mononuclear cells in patients with connective tissue diseases. 1968 48

Pneumatosis intestinalis (PI) is an uncommon condition characterized by the presence of gas within the wall of the gastrointestinal tract. PI is a physical or imaging finding that is the result of an underlying pathological process or a disease. This finding may present in diverse conditions, such as obstructive pulmonary disease and intestinal disease, including obstruction, inflammation, or ischemia. PI has also been reported in different autoimmune conditions, especially in systemic sclerosis and rarely with systemic lupus erythematosus. In this report we present PI occurring in three patients with different autoimmune conditions and review the literature.
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PMID:Pneumatosis intestinalis presenting in autoimmune diseases: a report of three patients. 1975 51

Systemic sclerosis (SSc, scleroderma) is a chronic, multisystem connective tissue disorder affecting the skin and various internal organs. Although the disease is characterized by a triad of widespread microangiopathy, fibrosis and autoimmunity, increasing evidence indicates that vascular damage is a primary event in the pathogenesis of SSc. The progressive vascular injury includes persistent endothelial cell activation/damage and apoptosis, intimal thickening, delamination, vessel narrowing and obliteration. These profound vascular changes lead to vascular tone dysfunction and reduced capillary blood flow, with consequent tissue ischemia and severe clinical manifestations, such as digital ulceration or amputation, pulmonary arterial hypertension and scleroderma renal crisis. The resulting tissue hypoxia induces complex cellular and molecular mechanisms in the attempt to recover endothelial cell function and tissue perfusion. Nevertheless, in SSc patients there is no evidence of significant angiogenesis and the disease evolves towards chronic tissue ischemia, with progressive and irreversible structural changes in multiple vascular beds culminating in the loss of capillaries. A severe imbalance between pro-angiogenic and angiostatic factors may also lead to impaired angiogenic response during SSc. Besides insufficient angiogenesis, defective vasculogenesis with altered numbers and functional defects of bone marrow-derived endothelial progenitor cells may contribute to the vascular pathogenesis of SSc. The purpose of this article is to review the contribution of recent studies to the understanding of the complex mechanisms of impaired vascular repair in SSc. Indeed, understanding the pathophysiology of SSc-associated vascular disease may be the key in dissecting the disease pathogenesis and developing novel therapies. Either angiogenic or vasculogenic mechanisms may potentially become in the future the target of therapeutic strategies to promote capillary regeneration in SSc.
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PMID:Mechanisms in the loss of capillaries in systemic sclerosis: angiogenesis versus vasculogenesis. 2013 9


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