Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0028961 (oliguria)
 1,847 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Scleroderma renal crisis (SRC) is a rare, potentially life-threatening complication that affects 2-15% of patients with systemic sclerosis (SSc, also known as scleroderma). SRC typically presents in patients with early, rapidly progressive, diffuse cutaneous SSc within the first 3-5 years after the onset of a non-Raynaud sign or symptom. SRC is characterized by an acute, usually symptomatic increase in blood pressure, a rise in serum creatinine levels, oliguria and thrombotic microangiopathy in about 50% of patients. The prognosis of SRC substantially improved in the 1980s with the introduction of angiotensin-converting-enzyme inhibitors for rapid blood pressure control, with additional antihypertensive agents as required. However, the survival of patients with SRC can still be improved. Current patient survival is 70-82% at 1 year, but decreases to 50-60% at 5 years despite dialysis support. Patients with SRC who show no signs of renal functional recovery despite timely blood pressure control are candidates for transplantation. In this Review, we discuss progress made in the identification and proactive management of patients at risk of SRC and make recommendations aimed at optimizing management for those who progress to chronic kidney failure.
 ...
PMID:Scleroderma renal crisis and renal involvement in systemic sclerosis. 2929 73