Renal complications and scleroderma renal crisis.

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.

[1]  L. Mouthon,et al.  Mortality and risk factors of scleroderma renal crisis: a French retrospective study of 50 patients , 2007, Annals of the rheumatic diseases.

[2]  C. Denton,et al.  Scleroderma renal crisis: patient characteristics and long-term outcomes. , 2007, QJM : monthly journal of the Association of Physicians.

[3]  T. Medsger,et al.  Changes in causes of death in systemic sclerosis, 1972–2002 , 2007, Annals of the rheumatic diseases.

[4]  L. Mouthon,et al.  Crise rénale sclérodermique , 2006 .

[5]  K. Welsh,et al.  Endothelin axis polymorphisms in patients with scleroderma. , 2006, Arthritis and rheumatism.

[6]  G. V. van Montfrans,et al.  Microangiopathic Hemolysis and Renal Failure in Malignant Hypertension , 2005, Hypertension.

[7]  C. Denton,et al.  Scleroderma--clinical and pathological advances. , 2004, Best practice & research. Clinical rheumatology.

[8]  K. Sharma,et al.  The pathogenesis of fibrosis and renal disease in scleroderma: Recent insights from glomerulosclerosis , 2004, Current rheumatology reports.

[9]  A. Fukamizu,et al.  The endothelin receptor antagonist ameliorates the hypertensive phenotypes of transgenic hypertensive mice with renin-angiotensin genes and discloses roles of organ specific activation of endothelin system in transgenic mice. , 2004, Life sciences.

[10]  J. Walker,et al.  Scleroderma renal crisis: poor outcome despite aggressive antihypertensive treatment , 2003, Internal medicine journal.

[11]  C. Black,et al.  Calculated glomerular filtration rate is a useful screening tool to identify scleroderma patients with renal impairment. , 2003, Rheumatology.

[12]  Richard W. Martin,et al.  Predictors and outcomes of scleroderma renal crisis: the high-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial. , 2002, Arthritis and rheumatism.

[13]  C. Denton,et al.  Anti-fibrillarin antibodies in systemic sclerosis. , 2001, Rheumatology.

[14]  R. Poupon,et al.  The endothelin receptor antagonist bosentan modifies canalicular bile secretion , 2001 .

[15]  T. Medsger,et al.  Long-Term Outcomes of Scleroderma Renal Crisis , 2000, Annals of Internal Medicine.

[16]  R. Kasukawa,et al.  Immunohistological Study of Endothelin-1 and Endothelin-A and B Receptors in Two Patients with Scleroderma Renal Crisis , 1999, Clinical Rheumatology.

[17]  T. Medsger,et al.  Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. , 1998, Arthritis and rheumatism.

[18]  C. Denton,et al.  Anti-RNA polymerases and other autoantibody specificities in systemic sclerosis. , 1998, British journal of rheumatology.

[19]  A. Silman,et al.  Survival following the onset of scleroderma: results from a retrospective inception cohort study of the UK patient population. , 1996, British journal of rheumatology.

[20]  C. Ferri,et al.  Circulating endothelin-1 levels in systemic sclerosis subsets--a marker of fibrosis or vascular dysfunction? , 1996, The Journal of rheumatology.

[21]  V. Steen.  Scleroderma renal crisis. , 2003, Rheumatic diseases clinics of North America.

[22]  C. Black,et al.  Circulating endothelin-1 levels in systemic sclerosis subsets--a marker of fibrosis or vascular dysfunction? , 1994, The Journal of rheumatology.

[23]  V. Steen,et al.  Renal Involvement in Systemic Sclerosis , 2019, New Insights into Systemic Sclerosis [Working Title].

[24]  V. Steen.  Treatment of systemic sclerosis. , 1991, Current opinion in rheumatology.

[25]  J. Costantino,et al.  Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. , 1990, Annals of internal medicine.

[26]  T. Medsger,et al.  Normotensive renal failure in systemic sclerosis. , 1989, Arthritis and rheumatism.

[27]  T. Medsger,et al.  Factors Predicting Development of Renal Involvement in Progressive Systemic Sclerosis , 1984 .

[28]  T. Medsger,et al.  Factors predicting development of renal involvement in progressive systemic sclerosis. , 1984, The American journal of medicine.

[29]  P. Cannon,et al.  THE RELATIONSHIP OF HYPERTENSION AND RENAL FAILURE IN SCLERODERMA (PROGRESSIVE SYSTEMIC SCLEROSIS) TO STRUCTURAL AND FUNCTIONAL ABNORMALITIES OF THE RENAL CORTICAL CIRCULATION , 1974, Medicine.

[30]  A. Masi,et al.  Survival with systemic sclerosis (scleroderma). A life-table analysis of clinical and demographic factors in 309 patients. , 1971, Annals of internal medicine.

[31]  G. Rodnan,et al.  An historical account of the study of progressive systemic sclerosis (diffuse scleroderma). , 1962, Annals of internal medicine.