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 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Scleroderma renal crisis (SRC) occurs in 5-10% of SSc patients, who may present with an abrupt onset of hypertension, acute renal failure, headaches, fevers, malaise, hypertensive retinopathy, encephalopathy and pulmonary oedema. Patients at greatest risk of developing SRC are those with diffuse cutaneous or rapidly progressive forms of SSc, and treatment with a recently commenced high dose of corticosteroid. Laboratory tests may demonstrate hypercreatinaemia, microangiopathic haemolytic anaemia (MAHA), thrombocytopaenia and hyperreninaemia. Renal crisis is also linked to a positive ANA speckled pattern, antibodies to RNA polymerase I and II, and an absence of anti-centromere antibodies. Early, aggressive treatment with angiotensin-converting enzyme inhibitors has improved prognosis in SRC, although 40% of the patients may require dialysis, and mortality at 5 yrs is 30-40%. Median time to recovery is 1 yr, and typically occurs within 3 yrs. Prognosis is worse for males, but may not be related to corticosteroid use, presence of MAHA or severity of renal pathology. Modification of endothelin over-activity, which is implicated in the pathogenesis of SRC, may offer a future therapeutic approach.
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PMID:Renal complications and scleroderma renal crisis. 1948 21

Scleroderma renal crisis (SRC) is an infrequent but serious complication of systemic sclerosis (SSc). It is associated with increased vascular permeability, activation of coagulation cascade, and renin secretion, which may lead to the acute renal failure typically associated with accelerated hypertension. The histologic picture of SRC is that of a thrombotic microangiopathy process with prominent small vessel involvement manifesting as myxoid intimal changes, thrombi, onion skin lesions, and/or fibrointimal sclerosis. Renal biopsies play an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in SSc patients, helping to predict the clinical outcome and optimizing patient management. Kidney transplantation may be the only treatment option available for a subset of SRC patients who develop end-stage renal failure despite aggressive angiotensin-converting enzyme inhibitor therapy. However, the posttransplant outcome for SSc patients is currently suboptimal compared to the general renal transplant population.
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PMID:Scleroderma renal crisis: a pathology perspective. 2098 12

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 is characterized by malignant hypertension and oligo-anuric acute renal failure. Scleroderma renal crisis occurs in 2 to 5% of patients with systemic sclerosis, particularly those with diffuse cutaneous systemic sclerosis in the first years of disease evolution. High-dose corticosteroid therapy (> 15 mg/d) is associated with an increased risk of scleroderma renal crisis. Patients present with prominent left heart failure and hypertensive encephalopathy. Renal failure can be associated with moderate proteinuria, without hematuria. Thrombotic microangiopathy is detected in 43% of the cases. Anti-RNA polymerase III antibodies are present in one third of patients with scleroderma renal crisis. In case of renal failure, iatrogenic or functional origin must be investigated, as well as crescentic glomerulonephritis associated with antineutrophil cytoplasm antibodies (ANCA) or thrombotic microangiopathy. Renal biopsy is not necessary to establish the diagnosis in typical forms of scleroderma renal crisis. However, it can help to evaluate the prognosis and it is recommended when clinical presentation of scleroderma renal crisis is unusual. The prognosis of scleroderma renal crisis dramatically improved with the use of angiotensin-converting enzyme (ACE) inhibitors. However, 5-year survival of patients who developed a scleroderma renal crisis is only 65%. The treatment relies on the early control of blood pressure with increasing doses of ACE inhibitors, in association with calcium channel blockers if necessary. In case of severe renal failure and/or hypertension, dialysis can help to quickly control the vascular overload and the blood pressure. Dialysis can be stopped in about half of cases. After 2 years on dialysis, eligible patients should be considered for renal transplantation. The prevention of scleroderma renal crisis lacks consensus. Corticosteroids and/or nephrotoxic drugs should be avoided in patients with diffuse cutaneous systemic sclerosis.
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PMID:[Renal involvement in patients with systemic sclerosis]. 2152 52

Scleroderma (systemic sclerosis) is a connective tissue disorder which involves skin, gastrointestinal tract, kidneys and lungs with considerable morbidity. Scleroderma renal crisis is a well-known severe form of renal involvement, characterised by the presence of high blood pressure with variable degrees of renal insufficiency. We report a case of scleroderma renal crisis diagnosed on the basis of history of scleroderma, high blood pressure and renal dysfunction. The biopsy confirmed the typical histopathological findings. We discuss herein the management of the case and review the literature.
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PMID:Scleroderma renal crises: case report and review of literature. 2390 21

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

Systemic sclerosis (SSc) is a rare autoimmune disorder that is typically divided into limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis. Scleroderma renal crisis (SRC) is a severe complication of SSc and typically presents with new-onset hypertension and a reduction in renal functioning. In patients presenting with typical features of SRC, treatment with an angiotensin-converting enzyme inhibitor along with dialysis as needed is typically initiated empirically. Renal biopsy is not recommended in patients with SSc presenting with typical features of SRC. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare co-occurrence with SSc, in around 2.5% to 9% of patients. AAV is an inflammatory condition that can result in renal failure due to mononuclear cell infiltration and destruction of blood vessels. Treatment of AAV is drastically different from SRC and typically consists of immunosuppressants and dialysis if needed. SRC and AAV can only reliably be distinguished by renal biopsy. We present a rare case of a 70-year-old female with limited cutaneous systemic sclerosis who presented to the emergency department with new-onset renal failure. Her serology was found to be positive for antinuclear antibodies and myeloperoxidase antibodies, resulting in a renal biopsy, which revealed an acute necrotizing vasculitis consistent with AAV. We suggest consideration of a renal biopsy in patients with SSc who present with new-onset renal failure, especially with nonresponse to SRC treatment or positive serology.
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PMID:ANCA-Associated Vasculitis Co-Occurrence With Systemic Sclerosis: A Case Report of a Rare Diagnostic Dilemma. 3008 57

Systemic sclerosis (SSc) is a rare autoimmune disease that causes fibrosis in the skin and subcutaneous tissue, involving other organs such as the heart, lungs, kidneys, and gastrointestinal tract. Additionally, it can cause pulmonary arterial hypertension. Scleroderma renal crisis (SRC) is one of the most dreadful complications of SSc. SRC is a medical emergency that can present as a clinical picture of hypertensive encephalopathy. The pathophysiology involves an abrupt onset of moderate to severe hypertension that ranges from days to weeks; it is associated with an increase in plasma renin activity and acute kidney injury. It is known that by introducing angiotensin-converting enzyme inhibitors, the mortality decreases significantly in SRC. The renal biopsy plays an important role on the diagnosis and opportune treatment. We present a clinical case of SRC with a typical presentation of hypertensive emergency and acute kidney injury.
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PMID:Hypertensive Emergency in a Woman with Systemic Sclerosis. 3300 10


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