Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036421 (PSS)
 10,989 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Systemic sclerosis is a connective tissue disease in which oxidative stress represents an important player among the complex pathogenetic mechanisms of the disease. Iloprost, an analogue of natural prostacyclin, is used in systemic sclerosis for the treatment of severe Raynaud's phenomenon and ischemic ulcers. There is a clear evidence that iloprost attenuates oxidative damage induced by ischemia-reperfusion phenomena. The aim of this study is to evaluate the effect of iloprost on oxidative status in ten patients with systemic sclerosis by measuring urinary levels of 8-isoprostaglandin-F(2alpha), a member of F(2)-isoprostanes. We found that systemic sclerosis patients cyclically treated with iloprost showed increased urinary level of 8-isoprostaglandin-F(2alpha )in comparison with healthy subjects; urinary 8-isoprostaglandin-F(2alpha) did not diminish soon after the iloprost infusion as well as 3, 15 and 30 days after the drug administration. Unlike experimental studies, in vivo the strong vasodilator effect of iloprost infusion did not reduce oxidative status.
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PMID:Iloprost infusion does not reduce oxidative stress in systemic sclerosis. 1770 20

Systemic sclerosis (SSc) is a multiorgan disease characterized by injury to vascular wall and extensive damage of the microvessels. The injury of the vascular wall is characterized by the formation of megacapillaries and avascular areas. The reduced capillary density leads to clinical manifestations such as digital ulcers. These lesions are extremely painful and lead to substantial functional disability. Management of digital ulcers includes non-pharmacologic and pharmacologic modalities. Despite the reduced blood flow and reduced partial oxygen pressure levels, there is paradoxically no evidence for a sufficient angiogenesis in the skin of patients with SSc. Angiogenesis is strongly disturbed in SSc, as demonstrated by Nailfold Video-Capillaroscopy changes, the damage of the vessels evolves progressively from early to late stages and is characterized by different morphological aspects. Almost all patients develop Raynaud's phenomenon which, together with structural vasculopathy, results in ulceration and critical digital ischemia. Many of the severe internal organ complications of SSc are vascular, including pulmonary arterial hypertension (PAH) and scleroderma renal crisis. Structural vascular damage occurs in many vascular beds and contribute to pulmonary, renal, cardiac and gastrointestinal complications. SSc has a high case-specific mortality due to organ-based complications including PAH, lung fibrosis, renal failure and involvement of the gastrointestinal tract.
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PMID:Vascular complications of scleroderma. 1785 42

Chronic ergotism is a rare cause of limb ischemia. In this case report, the authors present a 62-year-old woman with history of long-term use of ergotamine alkaloids for the treatment of menstrual pain, who developed a severe painful disease initially misdiagnosed as systemic sclerosis (scleroderma) for 3 decades. She presented with a combination of acral gangrene, foot ulcer, renal obstruction, mild pulmonary fibrosis, and reduced esophageal motility. Right-sided renal obstruction was evident. The condition was extremely painful and had led to muscular contractions and immobility, drug abuse, and anemia. After establishing the diagnosis of chronic gangrenous ergotism, changing drug therapy, mobilization, and treatment of chronic wounds, she showed a remarkable recovery. Eventually the foot ulcer was closed successfully using a mesh graft transplantation, and the patient was able to walk alone. Chronic ergotism is rare but has to be taken into account when presented with painful chronic digital and foot ulcers.
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PMID:Painful ANA-positive scleroderma-like disease with acral ulcerations: a case of chronic gangrenous ergotism. 1790 73

Autoimmune rheumatic diseases are considered to be influenced by both genetic and environmental factors. Tobacco smoking has been linked to the development of rheumatic diseases, namely systemic lupus erythematosus and rheumatoid arthritis, and has been shown to interact with genetic factors to create a significant combined risk of disease. Smoking also affects both the course and the outcome of rheumatic diseases. Smoking increases the risk of dermatologic features and nephritis in systemic lupus erythematosus, rheumatoid nodules and multiple joint involvement in rheumatoid arthritis and digital ischemia in systemic sclerosis, as well as further increasing the risk of accelerated atherosclerosis in these diseases. Smoking is known to modulate the immune system through many mechanisms, including the induction of the inflammatory response, immune suppression, alteration of cytokine balance, induction of apoptosis, and DNA damage that results in the formation of anti-DNA antibodies. No sole mechanism, however, has been linked to any of the autoimmune illnesses, which therefore complicates full comprehension of the 'smoking effect'. Further studies, perhaps using animal models, are needed to analyze the exact effect of smoking on each disease separately.
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PMID:Tobacco smoking and autoimmune rheumatic diseases. 1803 30

Catastrophic antiphospholipid syndrome (CAPS), described by Asherson in 1992, is a rare form of antiphospholipid syndrome resulting in multiorgan failure with a mortality rate of about 50%. The syndrome occurs in patients with either systemic lupus erythematosus and other rheumatic diseases (systemic sclerosis, rheumatoid arthritis, primary Sjogren syndrome) or alone. Whereas in "classic" antiphospholipid syndrome (APS), medium-large vessels are involved, a diffuse small vessel ischemia and thrombosis (microangiopathic disease) leading to a severe multiorgan dysfunction is predominant in CAPS. "Trigger" factors have been demonstrated in 45% of patients, but in the majority, they remain unknown. Not infrequently, CAPS arises in patients without any previous thrombotic history. The kidney is the organ most commonly affected, followed by the lung, the central nervous system, the heart and the skin. Disseminated intravascular coagulation occurs in approximately 13% of patients. The present study reports the clinical and serological features of 4 patients affected by this rare form of antiphospholipid syndrome. Nephrologists should be aware of the possibility of this syndrome as a cause of multiorgan failure since prompt recognition is essential for effective treatment.
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PMID:Catastrophic antiphospholipid syndrome: report of 4 cases. 1804 77

Systemic sclerosis (SSc) patients typically experience Raynaud phenomena that is often complicated by digital ischemic lesions, gangrene, and digital loss. Other causes of peripheral ischemia, such as atherosclerosis, cryoglobulinemia, antiphospholipid syndrome, myeloproliferative disorders, paraneoplastic syndromes, and hyperadrenergic endocrine conditions, may be masked in SSc patients. We present a woman with limited SSc who developed toe necrosis and acute coronary events as a complication of a previously undiagnosed pheochromocytoma.
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PMID:Toe necrosis and acute myocardial infarction precipitated by a pheochromocytoma in a patient with systemic sclerosis. 1817 42

Systemic sclerosis and its subtypes differ significantly from other diseases in rheumatology and clinical immunology, as the aberrant activation of the immune system does not result in an inflammation-driven destruction but in a progressive matrix synthesis, especially of the skin. Owing to the recently established networks in Germany (DNSS) as well as in Europe (EUSTAR), a detailed profile of the affected patients could be determined. No specific predictive markers for the disease exist in the early phases of the disease although a Raynaud phenomenon can be present up to ten years prior to the first organ manifestations. On the other hand, distinct clinical features and laboratory parameters can be independent predictive markers for the outcome of a given patient. Therefore, monitoring should be performed on a regular basis in the preclinical and early stages of the disease, as early diagnosis can reduce morbidity and mortality of the affected patients significantly, especially with regard to pulmonary hypertension, digital ischemia, reflux esophagitis and sclerodermal renal crisis.
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PMID:[Systemic sclerosis]. 1821 18

Systemic sclerosis (SSc, scleroderma) is an autoimmune disease characterized by excessive extracellular matrix deposition and vascular injury in the skin and internal organs. Although the pathogenesis remains unclear, Raynaud's phenomenon, a kind of ischemia-reperfusion, usually precedes the development of skin sclerosis. Therefore, it is possible that endothelial cell injury caused by recurring ischemia-reperfusion induces inflammatory cell infiltration and subsequent cytokine production, leading to the development of tissue fibrosis. During this process, chemokines likely have important roles via mediating chemotaxis and activation of leukocytes, result in the interaction between leukocytes and fibroblasts. While chemokine abnormalities of SSc have been reported in amounts of literatures, monocyte chemoattractant protein-1 (MCP-1/CCL2) and its receptor, CCR2, likely have the most critical role for the development of SSc. Here recent data will be reviewed on the potential role of chemokines and their receptors in SSc.
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PMID:The roles of chemokines in the development of systemic sclerosis. 1831 Oct 40

Vascular endothelial injury in systemic sclerosis (SSc) includes a spectrum of changes that involve predominantly the microcirculation and arterioles. The pathologic changes in the blood vessels adversely impact the physiology of many organ systems, with a reduction in the size of microvascular beds leading to decreased organ blood flow and ultimately to a state of chronic ischemia. Current hypotheses in SSc vascular disease suggest a possible chemical or infectious trigger.
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PMID:Vascular disease in scleroderma: mechanisms of vascular injury. 1832 32

The manifestations of peripheral vascular disease in patients who have systemic sclerosis (SSc) range from episodic Raynaud's phenomenon to irreversible tissue injury with ulceration and gangrene. Structural and functional changes may occur in the microvessels, digital arteries, and sometimes more proximal vessels. This article discusses the assessment of patients who have Raynaud's phenomenon in whom an underlying scleroderma-spectrum disorder is suspected and patients who have SSc with critical digital ischemia/ulceration. Different imaging techniques, including capillaroscopy and angiography, complement the history and examination, and developments in vascular imaging should facilitate future studies of pathogenesis and treatment response. New vasoactive treatments are currently being researched and older treatments revisited; therefore new approaches to therapy will likely be developed over the next 5 to 10 years.
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PMID:Diagnosis and management of scleroderma peripheral vascular disease. 1832 35


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