Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Scleroderma renal crisis (SRC) is defined as sudden development of accelerated hypertension, hyperreninemia, and acute renal failure in a patient with progressive systemic sclerosis (PSS). Although the diagnosis of PSS is generally straightforward because of dermal fibrosis, we report 2 patients who had PSS with SRC without the characteristic fibrotic skin changes of scleroderma. PSS should be considered in the differential diagnosis of unexplained acute renal failure and accelerated hypertension even though the cutaneous fibrotic manifestations of the disease may be absent.
 ...
PMID:Acute renal failure without fibrotic skin changes in progressive systemic sclerosis. 334 51

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.
 ...
PMID:Scleroderma renal crisis is still a life-threatening syndrome. 887 81

Scleroderma renal crisis is a clinical syndrome of systemic sclerosis that is classically characterized by accelerated hypertension, rapidly progressive renal failure, and hyperreninemia. It has been reported that cocaine may initiate scleroderma in an already susceptible individual or unmask it at an earlier age in subclinical disease. We describe a 46-year-old male patient with a history of heavy cocaine abuse that unmasked scleroderma and precipitated scleroderma renal crisis.
 ...
PMID:Cocaine-induced scleroderma and scleroderma renal crisis. 978 94

Scleroderma renal crisis is characterized by intimal thickening of the afferent glomerular arterioles resulting in hypertension and fibrinoid necrosis of the capillary tuff. We report a 67-year-old man with long-standing systemic sclerosis who developed normotensive progressive renal failure, proteinuria, and a nephritic urinary sediment with serum myeloperoxidase-antineutrophil cytoplasmatic antibodies (MPO-ANCA). Renal biopsy showed pauci-immune crescentic glomerulonephritis but none of the typical vascular changes of scleroderma renal crisis. Because comparable cases have recently been reported from Japan, normotensive MPO-ANCA-positive crescentic glomerulonephritis may form an entity of progressive renal failure in scleroderma.
 ...
PMID:MPO-ANCA-Positive crescentic glomerulonephritis: a distinct entity of scleroderma renal disease? 1019 34

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.
 ...
PMID:[Renal involvement in scleroderma]. 1253 68

Scleroderma renal crisis (SRC) represents the classic manifestation of kidney involvement in SSc. It particularly occurs in patients with early, rapidly progressive, diffuse skin involvement. Its detection requires the assessment of a few core set variables: arterial blood pressure, serum creatinine, and urinalysis. In clinical investigations SSc patients developing arterial hypertension after the disease onset (new onset hypertension) without SRC should also be reported.
 ...
PMID:Assessment of kidney involvement. 1288 19

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.
 ...
PMID:Targeted therapy for systemic sclerosis. 1643 41

Scleroderma renal crisis (SRC) has classically been defined as a new onset of accelerated arterial hypertension associated with a rapid increase in serum creatinine concentration and/or microangiopathic hemolytic anemia. SRC occurs in approximately 20% of patients with systemic sclerosis who have diffuse cutaneous manifestations. In addition, 10% of reported cases of SRC with diffuse cutaneous involvement have normal blood pressures; in the majority of these cases, SRC occurs after treatment with corticosteroids. We describe a patient who presented with an early onset SRC in the setting of diffuse cutaneous systemic sclerosis in evolution without prior accelerated arterial hypertension or corticosteroid use.
 ...
PMID:A case of early onset normotensive scleroderma renal crisis in a patient with diffuse cutaneous systemic sclerosis. 1692 70

Scleroderma renal crisis (SRC) occurs in patients with systemic sclerosis (SSc) and is defined by otherwise unexplained rapidly progressive renal insufficiency associated with oliguria or rapidly progressive arterial hypertension or both. SRC is a rare and severe complication of SSc, most often encountered during the first 4 years of disease, almost only in patients with diffuse SSc. Factors predicting SRC were identified, including high-dose corticosteroid administration. Use of angiotensin-converting enzyme inhibitors (ACEI) has dramatically impressed the prognosis of SRC, but it mortality rate is still high. Treatment aims at normalizing blood pressure as soon as possible. ACEI should always be used, and additional anti-hypertensive agents, including calcium channel blockers and alpha- and beta-blockers, may be useful. Renal replacement therapy may be needed, but often (for almost half of patients) only temporarily.
 ...
PMID:[Scleroderma renal crisis]. 1715 23

Scleroderma renal crisis (SRC) is a complication of systemic sclerosis characterized by the sudden onset of accelerated arterial hypertension, followed by progressive renal failure. Rarely, patients with SRC may be normotensive on presentation. These patients have poorer prognosis and higher mortality rates than those with hypertensive SRC. This is partly explained by the insidious course of normotensive SRC leading to delayed diagnosis and treatment. Normotensive patients also seem to be less responsive to current treatment modalities. Since available data on etiology, pathogenesis, and risk factors of the disease are inadequate, no effective therapy has been established to date. We report a patient with diffuse cutaneous scleroderma who developed SRC during his hospitalization. The patient remained normotensive and had an insidious course until oliguria and signs of hypervolemia occurred. Etiology, pathogenesis, risk factors, diagnosis, treatment modalities and prognosis of normotensive SRC are also discussed through previously published reports.
 ...
PMID:A "silent" course of normotensive scleroderma renal crisis: case report and review of the literature. 1904 56


1 2 Next >>