Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.4.15.1 (ACE)
 18,300 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Scleroderma renal crisis (SRC) was known as a rare and catastrophic syndrome responsible for acute renal failure (ARF) in a context of widespread microvascular disease occurring in progressive systemic sclerosis (PSS). Following pathogenetic hypoteses, angiotensin converting enzyme (ACE) inhibitors, plasma infusions (PI), and plasma-exchange (PE) have been employed in SRC with favorable results. Our purpose was to verify whether these therapies have consistently changed the fatal prognosis of SRC, even in our experience. In the last 10 years, SRC was diagnosed in eight patients (all eight with histologic data). The first five cases were treated with steroids, antihypertensive-cocktail, and PI: all five died, two within 48 hours, three after 10, 15, and 300 days, respectively. Three other patients were treated with ACE inhibitors, PI, and PE: all three died after 1, 9, and 12 months of HD. Clinical-histological correlations showed a strong relationship between the extent of glomerular involvement and the degree of renal failure, while arterial lesions seem to be more related to the past history of PSS, independently from the previous existence of hypertension. We conclude that "true" SRC diagnosed by restrictive criteria is still a rare life-threatening syndrome, and, unfortunately, no clear predictive biochemical or clinical signs could be identified; vascular renal involvement correlates to the duration of PSS independently of previous clinical evidence of renal failure or hypertension; a glomerular pattern similar to that reported for hemolytic-uremic syndrome (HUS)/thrombotic thrombocytopenic purpura (TTP) syndrome is directly related to the degree of acute renal involvement; SRC may occur even in the absence of hypertension, mainly if cardiomyopathy is present: in our experience. ACE inhibitors and plasma therapies have changed the short-time prognosis of SRC, but they may be unable to provide recovery from dialysis and do not avoid further evolution of extrarenal PSS exiting in late death.
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PMID:Scleroderma renal crisis is still a life-threatening syndrome. 887 81

Guidelines for the conduct of clinical trials in progressive systemic sclerosis have been recommended to determine drug efficacy better. To date, the results of disease-modifying drugs in scleroderma have been disappointing. The treatment of esophagitis has been revolutionized by omeprazole. Raynaud's phenomenon can be treated with calcium channel blockers and iloprost. Scleroderma renal crisis can be treated with aggressive blood pressure control using angiotensin converting enzyme inhibitors. The best treatment for rapidly progressive scleroderma lung is still unknown. Future treatments in scleroderma should be tested with the use of recommended guidelines.
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PMID:Treatment of systemic sclerosis. 892 2

Scleroderma renal crisis occurs most often during the first years of the disease, in patients with systemic sclerosis and evolving cutaneous lesions. Clinically, it is responsible for severe hypertension, sometimes associated with cardiac failure or neurological symptoms. Laboratory tests disclose rapidly progressive renal failure, and often signs of thrombotic microangiopathy. If performed (which is rarely the case), renal biopsy shows scleroderma-induced chronic vascular lesions, but also vascular lesions that are secondary to malignant hypertension. The cornerstone of treatment is blood pressure control using angiotensin converting enzyme inhibitors, often in association with other antihypertensive agents. It has to be started as early as possible, in order to optimise vital and renal prognosis.
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PMID:[Renal involvement in scleroderma]. 1253 68

Systemic sclerosis is a multisystem disease whose therapy is focused at pathogenic pathways causing variable types of damage in the individual organs. There are 3 major pathways that cause organ damage in scleroderma. First, t-cells, cytokines and inflammation are prominent very early in the disease. Early alveolitis which occurs before interestial fibrosis in the lungs is the best example of inflammation. Second, endothelial cell damage causes severe thickening of vessels and two of the most deadly complications in scleroderma, pulmonary arterial hypertension and renal crisis. Scleroderma renal crisis is now very treatable with angiotensin converting enzyme inhibitors. There are now treatments for pulmonary arterial hypertension which should improve outcome in these patients as well. Third, fibroblasts lead to severe cutaneous fibrosis or skin thickening that is the hallmark of the disease. No treatment is available but we are hopeful that new antagonists to the cytokine, TGF beta, will prove helpful. B cells and autoantibodies are not involved in the pathogenesis of the disease, but there are scleroderma specific antibodies that help in defining patient subsets. All of these factors are influenced by unknown inciting agents and the permissive genetic background.
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PMID:Targeted therapy for systemic sclerosis. 1643 41

Scleroderma renal crisis (SRC) is a rarebut severe complication of systemic sclerosis. SRC usually occurs in the presence of high blood pressure but may be seen in patients with normal blood pressure. Normotensive SRC cases have a poorer prognosis and failure to recognize this condition can result in a fatal outcome, depriving the patient of the potential benefits of ACE inhibitors. We present a case of diffuse cutaneous systemic sclerosis with early onset normotensive SRC and oliguric acute renal failure.
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PMID:Normotensive scleroderma renal crisis in a patient with progressive systemic sclerosis: case report and review of literature. 2147 77

Scleroderma renal crisis (SRC) is defined as the new onset of accelerated arterial hypertension and /or rapidly progressive oliguric renal failure during the course of systemic sclerosis. It is a rare but life-threatening complication. This formerly serious complication has got a considerable brighter outlook since the introduction of angiotensin converting enzyme inhibitors (ACE) however the mortality is still remaining high. We report two cases of SRC which to our knowledge are the firsts described in Dakar. They were two women aged 45 and 32 years, one of them was previously following for systemic sclerosis. Both of them had malignant hypertension associated with rapidly progressive renal failure, the other was put under corticosteroid therapy four months before SRC occurrence. The histological and laboratory finding showed thrombotic microangiopathy. The height blood pressure returned to normal value after treatment with ACE inhibitors. The final outcome was undesirable with the death of one after two months due to the hemodialysis discontinuation and persistence of renal failure in the other.
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PMID:Scleroderma renal crisis in tropical region: two senegalese cases. 2640 82

Systemic sclerosis (SSc) is a multiorgan disorder involving the skin, heart, lungs, kidneys, and intestines. Progressive interstitial lung disease (ILD) is a serious complication in SSc patients, and cyclophosphamide (CYC) is the only recommended therapy for this condition;(1)) however, its clinical effectiveness is not sufficient. Scleroderma renal crisis (SRC) is a rare complication, characterized by acute renal failure and progressive hypertension. Angiotensin-converting-enzyme inhibitor (ACE-i) is a widely accepted therapy for SRC. We report an SSc patient with SRC and progressive ILD who underwent treatment with CYC and successful treatment with ACE-i and plasma exchange (PE). SRC and ILD are significant contributors to morbidity and mortality among SSc patients, and the therapy for these disorders is of great interest to rheumatologists. This study presents the possibility of favorable effects of PE for SSc-associated ILD and SRC.
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PMID:Scleroderma renal crisis during intravenous cyclophosphamide pulse therapy for complicated interstitial lung disease was successfully treated with angiotensin converting enzyme inhibitor and plasma exchange. 2757 17

Scleroderma renal crisis (SRC) is an uncommon but still life-threatening manifestation of systemic sclerosis (SSc). The incidence of SRC has decreased in the last few decades, probably due to a widespread use of vasodilators in SSc patients. It is well-recognized that exposure to different drugs can trigger SRC (corticosteroids, cyclosporine) or might prevent its occurrence (iloprost, calcium channel blockers). The prognosis of this life-threatening manifestation has not substantially improved since 1980s, when ACE-inhibitors were introduced in its treatment. ACE-inhibitors remain the mainstay in the therapy of SRC due to their efficacy in controlling malignant hypertension; indeed, the prognosis largely depends on the rapid improvement of the ongoing renal ischemia. Calcium-channel blockers and in third line diuretics and alpha-blockers should be used as additional therapy if blood pressure control remains suboptimal despite maximum tolerated doses of ACE-inhibitors. Given the growing evidence on the role of complement activation and endothelin-1 in the pathogenesis of SRC, recent case-series and case reports have suggested the use of C5-inhibitors and endothelin receptor antagonists in the therapy of SRC, mainly in the refractory cases. Plasma-exchange seems to give some benefits in patients with SRC and microangiopathy or intolerant to ACE-inhibitors. Renal transplantation is the last treatment option and its outcome is similar to that reported in other connective tissue disorders, with a 5-year patient survival rate of about 82%. In this review we summarize the current knowledge in the treatment of SRC.
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PMID:Therapy of scleroderma renal crisis: State of the art. 3000 60