| Kidney (Renal) Involvement (Main Menu) |
|
| Scleroderma Kidney (Renal) Involvement |
| This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer. |
|
| Overview of Kidney (Renal) Involvement |
| Kidney Failure. The kidneys' main function is to eliminate excess fluid and waste material from your blood. When your kidneys lose their filtering ability, dangerous levels of fluid and waste accumulate in your body— a condition known as kidney (renal) failure. Mayo Clinic. |
| Kidney Failure.When both of your kidneys fail, your body holds fluid. Your blood pressure rises. Harmful wastes build up in your body. You develop fatigue, nausea, and loss of appetite. MedicineNet.com. |
| Pain in Kidney or Urine Diseases. First, most of the important causes of kidney failure do not cause any pain in the kidneys. Secondly, not all pains that come from the areas of the kidneys mean that there is a problem with the kidneys. When assessing someone with pain, a doctor has to be careful and may need to perform at lot of tests which can take some time. Even after tests, a doctor may not be absolutely sure of the cause of pain. National Kidney Foundation. |
| Autoimmune Kidney Disease Kidney disease is a sneaky thing. There may be no symptoms at all for a long time while it is developing. This means that unfortunately, many people never realize they have a kidney problem until a symptom such as back pain or blood in the urine appears. About.com. |
| Renal manifestations in rheumatic diseases. The differential diagnosis of renal abnormalities in a rheumatic patient should include drug induced renal impairment as well as infection. PubMed Internist (Berl). 2007 Aug;48(8):779-85. |
| Changes in causes of death in systemic sclerosis, 1972- 2002. Survival of scleroderma has changed since the treatment of renal crisis became possible. The change in pattern of scleroderma-related mortality over the past 30 years implicates the lung (both pulmonary hypertension and pulmonary fibrosis) as the primary causes of scleroderma related deaths today. PubMed. Ann Rheum Dis. 2007 Feb 28. (Also see: SSc: Prognosis and Mortality and Pulmonary Involvement) |
| Scleroderma Renal Crisis |
| The systemic forms of scleroderma can cause renal (kidney) problems. |
| Sudden onset of high blood pressure and kidney failure is called scleroderma renal crisis. This occurs in about 20% of people with diffuse scleroderma and can also occur with limited scleroderma and CREST Syndrome. It can result in kidney failure within days to weeks. |
| Kidney involvement is the most serious and life threatening complication, so close monitoring of kidney function is necessary. Reduction in kidney function is often accompanied by high blood pressure. Current drugs of choice to treat kidney disease associated with scleroderma are ACE inhibitors such as Captopril or enalapril. Other drugs to reduce blood pressure have also been used with some success. |
 Calculators for Health Care Professionals: Glomerular filtration rate (GFR). GFR is the best overall index of kidney function. Normal GFR varies according to age, sex, and body size, and declines with age. Patients, if you know your Creatinine level, you can use this easy online calculator to determine your kidney status. National Kidney Foundation. |
| BUN (Blood Urea Nitrogen) Test University of Michigan Health System |
| Blood (Serum) Creatinine Test University of Michigan Health System |
| Diagnosis, management and prevention of scleroderma renal disease. Renal involvement remains a major complication of scleroderma. Long-term outcome after renal crisis remains poor despite the use of angiotensin-converting enzyme inhibitors. Penn H. (PubMed) Curr Opin Rheumatol. 2008 Nov;20(6):692-6. |
| Serologic Profile And Mortality Rates Of Scleroderma Renal Crisis (SRC) In Italy. SRC is a rare manifestation of systemic scleroderma in Italy but it is still associated with a severe prognosis. The autoimmune profile in SRC does not seem to be specifically associated with anti-RNAP I-III in our series, while there is a relative increase of anti-topo I positive patients. V. Codullo. FRI0256. EULAR 2008. |
| Scleroderma renal crisis: patient characteristics and long-term outcomes. Poor renal outcome was associated with lower blood pressure at presentation, and with higher age in those requiring dialysis. Steroid use, microangiopathic haemolytic anaemia, and antibody profile were not related to renal outcome. PubMed Quarterly Journal of Medicine. 2007 Aug;100(8):485-94. |
| Scleroderma renal crisis. 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. PubMed. Presse Med. 2006 Dec;35(12 Pt 2):1966-74. |
| A case of renal crisis in a Korean scleroderma patient with anti-RNA polymerase I and III antibodies. Sudden hypertension, oliguria, and pulmonary edema were features of her renal crisis. Subsequent renal biopsy findings showed severe fibrinoid necrosis with luminal obliteration in interlobar arteries and arterioles consistent with SSc renal crisis. PubMed. J Korean Med Sci. 2006 Dec;21(6):1121-3. (Also see: Antibodies) |
| Scleroderma Renal Crisis (SRC): Presentation, Outcome And Risk Factors Based On A Retrospective Multicenter Study Of 50 Patients. Our study suggests that, in spite of wide use of ACE inhibitors, SRC remains associated with severe morbidity and mortality and underlines that corticosteroids might strongly increase the short-term risk of SRC. Luis Teixeira. 1872/544 ACR 2006. |
| Scleroderma renal crisis in a patient with anticentromere antibody-positive limited cutaneous systemic sclerosis. This case, scleroderma renal crisis with detection of anticentromere antibody, is thought to be extremely uncommon. PubMed. Mod Rheumatol. 2006;16(5):309-11. |
| Renal manifestations of systemic autoimmune disease : diagnosis and therapy. Renal involvement is relatively common in certain systemic autoimmune diseases, but can be clinically silent. Scleroderma renal crises are managed by blood pressure control using angiotensin-converting enzyme inhibitors and other agents as required. PubMed. Nephrol Ther. 2006 Jul;2(3):140-151. (Also see: Lupus and Vasculitis) |
| Antineutrophil Cytoplasmic Antibody-Positive Crescentic Glomerulonephritis in Scleroderma - A Different Kind of Renal Crisis. The presence of antineutrophil cytoplasmic antibodies (ANCA) and anti-MPO defines a subset of patients with SSc who are susceptible to crescentic glomerulonephritis. These patients may present in a manner identical to scleroderma renal crisis, yet treatment requirements differ significantly. PubMed. J Rheumatol. 2006 Jul 1. (Also see: Antibodies) |
| A case of early onset normotensive scleroderma renal crisis (SRC) in a patient with diffuse cutaneous systemic sclerosis. 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. PubMed. South Med J. 2006 Aug;99(8):870-2. |
| Development of Polymyalgia Rheumatica in Patients with Scleroderma. Systemic sclerosis (SSc) and polymyalgia rheumatica (PMR) are uncommon but not rare rheumatic diseases. The various musculoskeletal symptoms that accompany SSc can make the recognition of PMR challenging in these patients. J Rheumatol 2006 June;33:1206. Letters. (Also see: Shared Autoimmunity) |
| Corticosteroids strongly increase the short-term risk of developing scleroderma renal crisis! |
Scleroderma Renal Crisis (SRC): Retrospective Multicenter Analysis of 50 Patients. Despite extensive ACEI (angiotensin-convertase inhibitors) use, scleroderma renal crisis (SRC) remains associated with severe morbidity and mortality, and that corticosteroids (CS) might strongly increase the short-term risk of developing scleroderma renal crisis. These findings further support the crucial preventive role of avoiding corticosteroids in patients at risk for scleroderma renal crisis , as well as the persistent need for effective adjunctive or alternative agents to manage SRC. L. Teixeira. FRI0145 EULAR 2005. (Also see: Medications) |
| Kidney crisis in systemic sclerosis. This occurs in the group of patients with rapid and aggressive course of the disease, often after several years of the ailment and with the diffuse form. Scleroderma renal crisis is frequently characterized by malignant hypertension, renal insufficiency, and less often by the symptoms of microangiopathic hemolytic anemia. PubMed. Rocz Akad Med Bialymst. 2005;50 Suppl 1:294-6. (Also see: Diffuse Scleroderma) |
| Low Frequency and Poor Outcome of Scleroderma Renal Crisis in the Follow-Up of 306 Patients with Systemic Sclerosis (SSc). Despite the low frequency of scleroderma renal crisis (SRC) in the present study, renal involvement still has a bad prognosis, associated to male sex and diffuse SSc in the first 5 years of the disease. P. D. Sampaio-Barros. FRI0126 EULAR 2005. |
| Scleroderma associated with ANCA-associated vasculitis. Scleroderma and ANCA-associated vasculitides (AAV), such as microscopic polyangiitis, are distinct disease entities, but are rarely known to coexist with each other. When scleroderma patients exhibit atypical features such as normotensive renal failure with signs of active inflammation, the possibility of AAV should always be considered. PubMed. Rheumatol Int. 2005 Jul 13;:1-4. (Also see: Vasculitis) |
| Kidney disease other than renal crisis in patients with diffuse scleroderma. Patients with diffuse scleroderma but without renal crisis rarely have significant increases in serum creatinine or proteinuria that cannot be explained by other etiologies. These patients should be carefully evaluated for non-scleroderma causes of kidney disease. J Rheumatol. 2005 Apr;32(4):649-55. (Also see: Diffuse Scleroderma) |
| Thrombotic thrombocytopenic purpura (TTP) in the setting of systemic sclerosis (SSc). The diagnosis of TTP, and the distinction from scleroderma renal crisis (SRC), may be challenging. However, the correct diagnosis is critical because treatment differs substantially for each of these life-threatening conditions. PubMed. Semin Arthritis Rheum. 2005 Feb;34(4):683-8. (Also see: Associated Conditions) |
| Renal manifestations of systemic autoimmune disease: diagnosis and therapy. Renal involvement is relatively common in certain systemic autoimmune diseases, but can be clinically silent. Scleroderma renal crises are managed by blood pressure control using angiotensin-converting enzyme inhibitors and other agents as required. Dialysis and transplantation can be successful in these conditions. PubMed. Best Pract Res Clin Rheumatol. 2004 Jun;18(3):411-27. (Also see: Dialysis) |
| Changes in Causes of Death in Systemic Sclerosis Over the Past 30 Years. Throughout the past 30 years, the frequency of deaths from renal crisis (RC) has dramatically decreased and at the same time the frequency of pulmonary fibrosis (PF) increased. Over the past 10 years, SSc patients have had improved survival, longer disease duration at the time they die and are less likely to die from scleroderma related complications. Pulmonary hypertension and pulmonary fibrosis now account for 50% of SSc related deaths and 25% of all causes of death in SSc patients. Virginia Steen. 1052/432. ACR 2004. (Also see: Pulmonary Fibrosis, Pulmonary Hypertension, and Types of Scleroderma: Prognosis and Mortality) |
| Tubular and Glomerular Proteinuria as Markers of Occult Renal Involvement in Systemic Sclerosis. Tubular and/or glomerular proteinuria were identified in over 40% of our patients and may occur with or without renal impairment. Screening using serum creatinine alone will underestimate the prevalence and severity of renal failure in SSc. Edward J. Kingdon. ACR Conference Oct. 2003. |
| Corticosteroid-induced scleroderma renal crisis The use of high dose corticosteroids in patients with early diffuse scleroderma should be strongly discouraged, and intensive monitoring for SRC is recommended if low dose corticosteroids are required. Med J Aust 2002 Oct 21;177(8):459. |
| An unusual cause of acute renal failure in systemic sclerosis. The patient's renal failure improved on an oxalate free diet. PubMed. Ann Rheum Dis. 2003 Dec;62(12):1133-4. |
| Assessment of kidney involvement. Its detection requires the assessment of a few core set variables: arterial blood pressure, serum creatinine, and urinalysis. In clinical investigations sysemic sclerosis patients developing arterial hypertension after the disease onset (new onset hypertension) without scleroderma renal crisis (SRC) should also be reported. PubMed. Clin Exp Rheumatol. 2003;21(3 Suppl 29):S29-31. |
| ANCA-related crescentic glomerulonephritis in a patient with scleroderma without marked dermatological change and malignant hypertension. Methylprednisolone pulse therapy followed by oral prednisolone effectively suppressed renal failure and lowered the MPO-ANCA titer. We believe this is a rare case of ANCA-related renal failure in a patient with scleroderma without marked dermatological change. PubMed. Intern Med. 2004 Jun;43(6):496-502. |
| Renal Complications of Systemic Sclerosis. Recent figures of prevalence (Royal Free Rheumatology Database 1990-2000) of clinically significant renal disease attributable to SSc for our own unit suggest a frequency of 12.4 % in diffuse cutaneous SSc (dcSSc) and 1.6 % in the limited cutaneous subset (lcSSc), and an overall rate of 5.4% in all SSc cases. C. Denton. SP0014 EULAR 2003. |
| Scleroderma renal crisis (SRC): poor outcome despite aggressive antihypertensive treatment. SRC is a rare event occurring in a small proportion of patients with diffuse scleroderma. After onset, the outcome of SRC was poor despite aggressive antihypertensive treatment. It is hypothesized that prophylactic ACE inhibition in susceptible patients might prevent or ameliorate this complication. PubMed. Intern Med J 2003 May;33(5-6):216-20. |
| A 60 year old male patient having systemic scleroderma and normotensive scleroderma renal crisis. Before admission he had been treated with high doses of corticosteroids. We believe corticosteroids led to the worsening of renal damage with renal failure, microangiopathic hemolytic anemia without high blood pressure. PubMed. Medicina (B Aires) 2003;63(1):49-50. (Also see: Anemia) |
| Scleroderma renal crisis sine scleroderma during pregnancy. Renal biopsy showed features compatible with scleroderma renal crisis but typical cutaneous changes were only evident two months after the renal episode. PubMed. Scand J Rheumatol 2003;32(1):55-7. |
| Calculated glomerular filtration rate is a useful screening tool to identify scleroderma patients with renal impairment. Serum creatinine is a poor marker of renal function in SSc patients. Calculating GFR from demographic and serum variables is a simple technique to identify SSc patients who have abnormal renal function. PubMed. Rheumatology (Oxford) 2003 Jan;42(1):26-33. |
| Kidney Impairment Common In Systemic Sclerosis Despite Lack Of Clinical Signs Ann Rheum Dis 2002 Aug;61(8):682-6. Doctor's Guide 8-2-02. |
| Pulmonary-renal vasculitic disorders: differential diagnosis and management. Pulmonary-renal syndrome is not a single entity and is caused by a variety of conditions, including Goodpastures syndrome associated with autoantibodies to the glomerular and alveolar basement membranes, various forms of primary systemic vasculitis associated with serum positivity for antineutrophil cytoplasmic antibodies (ANCA), cryoglobulinemia, systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, environmental factors, and drugs. PubMed. Curr Rheumatol Rep 2003 Apr;5(2):107-15 (Also see: Pulmonary Involvement) |
| Scleroderma renal crisis in pregnancy associated with massive proteinuria. Scleroderma renal crisis is a well-recognized complication of systemic sclerosis (SSc) usually occurring early in the course of the disease in patients with diffuse skin involvement. PubMed. Clin Exp Rheumatol 2003 Jan-Feb;21(1):114-6. |
| Scleromyxoedema-like changes in four renal dialysis patients. We describe four renal dialysis patients from our hospital who, over a 6-month period, developed erythematous, thickened, indurated dermal plaques. The clinical features most resembled scleromyxoedema. We believe this to be a new and distinct phenomenon. PubMed. Br J Dermatol 2003 Mar;148(3):563-8 (Also see: Diseases Similar to Scleroderma: Scleromyxedema) |
| Systemic sclerosis is not associated with clinical or ambulatory blood pressure. Raynaud's phenomenon is often the first symptom and occurs eventually in more than 95% of patients with systemic sclerosis (SSc). Systemic sclerosis is not associated with clinical blood pressure or the parameter of 24 h blood pressure monitoring. PubMed. Clin Exp Rheumatol 2003 Mar-Apr;21(2): 199-204 (Also see: Cardiac Involvement) |
| Anti-neutrophil cytoplasmic autoantibody-associated rapid progressive glomerulonephritis complicated with both limited and diffuse scleroderma. Our cases demonstrated that MPO-ANCA associated glomerulonephritis could be associated with limited scleroderma as well as systemic scleroderma. PubMed. Nihon Rinsho Meneki Gakkai Kaishi 2002 Dec;25(6):473-9 (Also see: Limited Scleroderma and Diffuse Scleroderma) |
| Renal Failure Prevention and Treatments |
|
| Pulmonary-renal syndrome (PRS) in systemic sclerosis: a report of three cases and review of the literature. Clinical courses of the patients with PRS with thrombotic microangiopathy suggest that high-dose corticosteroid therapy is a trigger of diffuse alveolar hemorrhage in patients with diffuse SSc with signs of thrombotic microangiopathy. PubMed. Mod Rheumatol. 2007;17(1):37-44. (Also see: Pulmonary Involvement, Medications, and Clinical Trials: Negative Results) |
| Corticosteroids strongly increase the short-term risk of developing scleroderma renal crisis! |
Scleroderma Renal Crisis (SRC): Retrospective Multicenter Analysis of 50 Patients. Despite extensive ACEI (angiotensin-convertase inhibitors) use, scleroderma renal crisis (SRC) remains associated with severe morbidity and mortality, and that corticosteroids (CS) might strongly increase the short-term risk of developing scleroderma renal crisis. These findings further support the crucial preventive role of avoiding corticosteroids in patients at risk for scleroderma renal crisis , as well as the persistent need for effective adjunctive or alternative agents to manage SRC. L. Teixeira. FRI0145 EULAR 2005. (Also see: Medications) |
| Bier's white spots associated with scleroderma renal crisis. We report the case of a 47-year-old woman with a 2-year history of systemic scleroderma, who developed Bier's white spots associated with a vascular and renal crisis. Interestingly, these spots appeared with the crisis, and disappeared with the treatment and resolution of the crisis. PubMed. Clin Exp Dermatol. 2006 Nov 27. |
| Predictors and risk factors for recurrent scleroderma renal crisis in the kidney allograft: case report and review of the literature. The risk for allograft scleroderma renal crisis (SRC) recurrence appears to correlate with early native renal function loss following the onset of SRC. Recurrent SRC in the allograft may be heralded by multiple clinical markers known to be predictive of severe scleroderma, including progression of diffuse skin thickening, new-onset anemia and cardiac complications. PubMed. Am J Transplant. 2005 Oct;5(10):2565-9. |
| Nephrogenic fibrosing dermopathy, a new mimicker of systemic sclerosis. This is a unique fibrosing disorder recently identified to occur exclusively among patients with renal disease. The cutaneous findings are similar to those of systemic sclerosis, but it is important to differentiate between these two disorders because of significant prognostic and therapeutic implications. PubMed. Am J Med Sci. 2005 Oct;330(4):192-4. (Also see: Diseases Similar to Scleroderma: Nephrogenic Fibrosing Dermopathy) |
| Treatment of secondary pulmonary hypertension with bosentan and its pharmacokinetic monitoring in ESRD. Bosentan for the treatment of secondary PH seems to be safe as well as effective in end-stage renal disease (ESRD) patients and no adjustment of the bosentan dosing regimen appears necessary. PubMed. Am J Kidney Dis. 2004 May;43(5):923-6. (Also see: Pulmonary Hypertension: Bostentan/Tracleer) |
| ACE Inhibitors (Angiotensin-Converting Enzyme Inhibitors) |
| Scleroderma renal crisis. Renal crisis occurs in patients who have systemic sclerosis with rapidly progressive diffuse cutaneous thickening early in their disease. Prompt diagnosis and early, aggressive initiation of therapy with ACE inhibitors will result in the most optimal outcome. PubMed. Rheum Dis Clin North Am. 2003 May;29(2):315-33. |
| Resolution of hypertensive encephalopathy and scleroderma renal crisis with an angiotensin receptor blocker. There may be a role for angiotensin II type-1 receptor blockers in the setting of scleroderma renal crisis with central nervous system involvement. PubMed. J Clin Rheumatol. 2005 Aug;11(4):205-8. |
| Long-Term Outcomes of Scleroderma Renal Crisis. Renal crisis can be effectively managed when hypertension is aggressively controlled with ACE inhibitors. Patients should continue taking ACE inhibitors even after beginning dialysis in hopes of discontinuing dialysis. 17 October 2000 Volume 133 Number 8 Annals of Internal Medicine |
| Iloprost |
| Iloprost Prevents Chronic Renal Vascular Functional Damage in Systemic Sclerosis (SSc). Renal involvement in SSc is often subclinical. Intravenous treatment with Iloprost may be useful to prevent the progression of chronic renal damage in systemic sclerosis. R. Livi. FRI0094 EULAR 2003. |
| Kidney (Renal) Dialysis |
| Kidney (Renal) Dialysis When the kidneys fail, dialysis of some form must be instituted to take over the work of the failed kidneys. There are two types of kidney dialysis: hemodialysis and peritoneal. ISN. |
| Kidney (Renal) Transplant |
| Kidney transplantation is a surgical procedure that places a healthy, donated kidney into the patient with kidney failure. The donated kidney can perform all the functions that the patient’s damaged kidneys are unable to do. Emory Transplant Center |
| Hypertension (High Blood Pressure) |
| Everyone with Diffuse Systemic Scleroderma should be measuring their blood pressure at home every day or two. If its elevated over a certain point (to be determined by your doctor) you should call your doctor immediately. CREST patients should measure their blood pressure at least once a month. |
| Lightheadedness and Hypertension Contrary to popular belief, high blood pressure (hypertension) itself does not cause lightheadedness. Hypertensive patients may experience lightheadedness, however, when their blood pressure drops for any reason. MedicineNet. |
| Hypertension Treatments |
| Ace Inhibitors are often used to control high blood pressure in Scleroderma. Calcium Channel Blockers are often used for Raynaud's or Hypertension in Scleroderma. |
| Scientific Evidence Lacking For Patients Choosing Among Treatments For Narrowed Kidney Arteries. Increasing numbers of patients with narrowed kidney arteries are undergoing vessel-widening angioplasty and placement of a tubular stent, but the latest scientific evidence does not show a clear advantage of that treatment over prescription drug therapy. Medical News Today. 10/26/06. |
| ACE Inhibitors May Boost Birth Defects. The FDA is encouraging women who are taking ACE inhibitors to reconsider the use of those drugs before or during pregnancy. A study published in The New England Journal of Medicine shows a higher rate of birth defects in babies born to women who filled prescriptions for ACE inhibitors in the first trimester of pregnancy. WebMD. 06/08/06. (Also see: Scleroderma and Pregnancy) |
| New trends in the treatment of scleroderma renal crisis. "Angiotensin converting enzyme inhibitors are the cornerstone in the treatment of patients with scleroderma renal crisis. Statins are used in the prevention of primary and secondary cardiovascular events." Nephron 2002 Nov;92(3):716-8 Department of Internal Medicine 'E', Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel. PubMed |
| Rapidly progressive glomerulonephritis |
| Rapidly Progressive Glomerulonephritis. Also called Crescentic Glomerulonephritis. Rapidly progressive glomerulonephritis is a form of kidney disease that causes damage to the small structures (glomeruli) inside the kidneys that help filter waste and fluids from blood to form urine. The disease leads to a rapid loss of kidney function. Medline Plus. |
| Fever of unknown origin secondary to type I crescentic glomerulonephritis (CGN) and anti-SCl 70 antibodies without clinical manifestations of systemic sclerosis. Anti-Scl 70 antibodies are highly specific for scleroderma and are seldom present in other diseases. As far as we are aware there are no published cases of the association of type 1 CGN with anti-Scl 70 antibodies. Vega Stieb J. (PubMed) Clin Exp Nephrol. 2008 Oct;12(5):388-92. (Also see: Antibodies) |
| Patient Organizations |
| American Association of Kidney Patients, a full-service patient organization. |
| National Kidney Foundation Inc. provides direct financial assistance to needy kidney transplant patients. Services include transportation, medication, food, lodging, and educational materials. The Foundation also supports community projects and promotes organ donation in Nevada. NKFI |
| Research Articles |
| Scleroderma with crescentic glomerulonephritis: a case report. Scleroderma is a complex disease, and the best characterized renal involvement in scleroderma is scleroderma renal crisis. However, other renal pathologies can occur in scleroderma. Arunachalam Ramaswami. 7th Space Interactive. 05/13/08. |
| Breakdown Of Kidney's Ability To Clean Its Own Filters Likely Causes Disease. The kidney actively cleans its most selective filter to keep it from clogging with blood proteins. Breakdown of this self-cleaning feature can make kidneys more vulnerable to dysfunction and disease. Science Daily. 02/03/08. |
| Antibodies to RNA Polymerase III in Systemic Sclerosis Detected by ELISA. Anti-RNAP-III autoantibodies were found in nearly 20% of SSc patients but in less than 1% of controls, thus detection of this antibody is a useful marker to help diagnose SSc. As well, this antibody has prognostic utility, since it is associated with scleroderma renal crisis and the diffuse cutaneous form of SSc. J Rheumatol 2007 July;34:1528-34. (Also see: Antibodies and Diffuse Scleroderma) |
| Prevalence of renal involvement in Indian patients with systemic sclerosis. SRC (scleroderma renal crisis) is very rare in Indian patients with SSc. However, non-renal crisis abnormalities appear to be as common in Indian patients as compared to the western literature. PubMed. Indian J Med Sci. 2007 Feb;61(2):91-6. (Also see: Causes of Scleroderma: Ethnicity, Race, and Geographical Regions) |
| Mortality and risk factors of scleroderma renal crisis: a French retrospective study in 50 patients. SRC remains associated with severe morbidity and mortality. CS (corticosteroid exposure) might increase the risk of developing SRC. Further studies are needed to confirm these results. Ann Rheum Dis. Published Online First: 8 June 2007. doi:10.1136/ard.2006.066985. |
| Unilateral limited scleroderma-like changes following formation of an arteriovenous fistula. The arteriovenous fistula for haemodialysis had been formed on the same arm 2 years previously. Tissue hypoxia is believed to be a contributing factor in the pathogenesis of scleroderma, and this case demonstrates scleroderma-like changes in the setting of limb ischaemia. PubMed. Australas J Dermatol. 2007 Feb;48(1):37-9. (Also see: Diseases Similar to Scleroderma: Scleroderma-Like) |
| Mixed connective tissue disease (MCTD): a case with scleroderma renal crisis following abortion. Immune response to U1-ribonucleoprotein is the defining serological feature of MCTD. We report a case of MCTD with pulmonary involvement that developed scleroderma renal crisis after an abortion. PubMed. Clin Rheumatol. 2006 Nov 21. (Also see: MCTD and Pulmonary Involvement) |
| Alteration of Renal Perfusion in Normotensive Patients with Scleroderma: A Duplex Ultrasonography Study. The elevated doppler indices of renal vascular resistance in normotensive patients with PSS suggest an altered renal perfusion in the absence of obvious renal involvement. S. M. Weiner. SAT0261 EULAR 2006. |
| Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis (NFD/NSF) in 2 patients with end-stage renal disease on hemodialysis. NFD/NSF can pose many diagnostic challenges. A team of experienced rheumatologists, nephrologists, dermatologists, and dermatopathologists is essential in identifying this disorder. PubMed. J Clin Rheumatol. 2006 Jun;12(3):134-6. (Also see: Diseases Similar to Scleroderma) |
| Nephrogenic systemic fibrosis (NSF): an emerging threat among renal patients. NSF is a scleroderma-like skin disease observed in patients with renal insufficiency. Recent observations indicate that specific cells normally involved in wound repair and tissue remodeling may be aberrantly recruited to the skin and soft tissues of NSF patients. PubMed. Semin Dial. 2006 May-Jun;19(3):191-4. (Also see: Diseases Similar to Scleroderma |
| Changes in Renal Perfusion in Patients with Systemic Sclerosis. Contrast enhanced ultrasound is suitable to detect differences in renal perfusion in patients with connective tissue diseases compared to healthy controls, especially in Systemic Scleroderma (SSc). This finding is interesting regarding the fact that most of the SSc patients had no clinically evident renal involvement. Stefan Kleinert. 1579/355. ACR 2005. |
| Serum xylosyltransferase I activity, the new biochemical fibrosis marker, is not affected by renal insufficiency. Serum xylosyltransferase I (XT-I) is a marker for the determination of tissue remodeling in systemic sclerosis. Serum XT-I activity is applicable as a fibrosis marker independent from renal function. PubMed. Clin Biochem. 2005 May;38(5):486-488. (Also see: Skin Fibrosis) |
| The Influence of Renal Disease and Race on In-Hospital Mortality Among Patients with Systemic Sclerosis. During hospitalizations for SSc patients, renal disease was associated with an almost two-fold risk of in-hospital mortality. Black patients and other non-white patients also appear to experience an elevated risk of death during their hospital stays. Paul J. Nietert. 1050/430. ACR 2004. (Also see: Genetics and Scleroderma) |
| A case of chronic renal insufficiency due to vascular injury of systemic sclerosis. The renal biopsy specimens showed an arteriosclerotic lesion with intimal thickening and luminal narrowing and ischemic glomerular changes. PubMed. Nippon Jinzo Gakkai Shi. 2004 Oct;46(7):709-14. |
| Prevention of progressive fibrosis in chronic renal diseases: Antifibrotic agents. The inhibition of pathological ECM (extracellular matrix) accumulation and the modulation of fibrotic mechanisms with these new antifibrotic agents is an achievable goal and could confer further benefits beyond the current therapies used in the treatment of chronic renal diseases. PubMed. J Nephrol. 2004 Jul-Aug;17(4):496-503. |
| The pathogenesis of fibrosis and renal disease in scleroderma: recent insights from glomerulosclerosis. Although treatment for acute scleroderma renal crisis may arrest the rapid progression of renal disease, many patients develop persistent renal dysfunction. PubMed. Curr Rheumatol Rep. 2004 Apr;6(2):141-8. |
| Scleroderma renal crisis: new insights and developments. Early recognition and treatment with angiotensin-converting enzyme inhibitors are important in the effective management of SRC, though given the continuing morbidity and mortality caused by SRC, they are clearly not sufficient. PubMed. Curr Rheumatol Rep. 2004 Apr;6(2):129-36. |
| Nephrogenic fibrosing dermopathy: the first 6 years. Nephrogenic fibrosing dermopathy (NFD) is a newly recognized scleroderma-like fibrosing skin condition. It develops in patients with renal insufficiency. Recent studies have contributed to a clearer definition of the clinical spectrum, epidemiology, and pathogenesis of NFD. PubMed. Curr Opin Rheumatol. 2003 Nov;15(6):785-90. (Also see: Diseases Similar to Scleroderma) |
| Historical and Clinical Overview of Recombinant Human Erythropoietin. In addition to renal disease, recombinant human erythropoietin (RHuEPO) is used to treat anaemia associated with other conditions requiring blood product support, including premature birth, malignancy, bone marrow/stem cell transplantation, surgery, HIV, rheumatological disorders, haemolysis, and anaemia in the critically ill. Doctor's Guide. 08/19/2003. Postgrad Med J 2003 Jul;79:933:367-376. (Also see: Dialysis) |
| Immunotherapy for autoimmune and inflammatory renal diseases. Immune-mediated renal disease (IMRD) accounts for 20 - 30% of the cases of end stage renal failure. There is a clear need for better-targeted, more effective and less toxic therapy. PubMed. Expert Opin Biol Ther. 2003 Jun;3(3):487-500. |
| Nephrogenic fibrosing dermopathy: an unusual skin condition associated with kidney disease. We report the case of a patient who, while on long-term hemodialysis (HD), developed nephrogenic fibrosing dermopathy, a newly described sclerosing skin disorder. PubMed. Semin Dial 2003 May-Jun;16(3):276-80 (Also see: Diseases Similar to Scleroderma) |
| Factors predicting development of renal involvement in progressive systemic sclerosis. PubMed. |
| Scleroderma renal crisis complicated by hemolytic uremic syndrome in a case of elderly onset systemic sclerosis. PubMed. |
| Media Stories |
| Kidney Disease Basics That Could Save Your Life. The kidneys are fist-sized organs that remove waste and extra fluid from the body, stimulate bone to make red blood cells and help regulate blood pressure and strengthen bones. Diseased kidneys can compromise many bodily systems and cause anemia, malnutrition, heart disease and ultimately death. The Washington Post. 08/23/05. |
| Patient & Caregiver Stories |
| Anastasia: Surviving Granddaughter of a Diffuse Scleroderma Patient Traveling to a different hospital finally we heard scleroderma... |
| Annette G: MCTD, Systemic Scleroderma, Chronic Kidney Disease, CFS, Fibromyalgia, Trigeminal Neuralgia, TMJ People think that because I am a nurse I have all the answers. Well guess what? I don't... |
| Archie Bailey: Scleroderma: Sue's Story Time has passed since Sue's death. Only those who have experienced the grieving process can understand... |
| Betty M: CREST Syndrome I was in so much pain for so long that I tried to take my own life. This only ended me up in a mental institution where I was given shock treatments that wiped out my whole memory... |
| Beverly: CREST Scleroderma I was overtired and needed naps in the afternoon, pain was slowly getting the better of me and I finally went to the doctor... |
| Buggzy: Autoimmune Hepatitis, Fibromyalgia, Kidney Cancer I am really desperate to get some answers or to hear from others in the same boat as me... |
| Corey C: Surviving Daughter of Scleroderma Patient It was a very long process and scary because she really did not know what to expect next... |
| Craig R: CREST Scleroderma I had my first scleroderma symptoms at age twenty-two, which included severe Raynaud's with some ulceration and tissue loss... |
| Iris: Family History of Scleroderma Is there anyone else who feels that their family has a history of scleroderma? |
| Jaci: MCTD and Autoimmune Hepatitis Out of fear of going back to the doctors, I tried just to live with it, whatever it was. What I found strange was that nobody seemed to noticed how terrible I was feeling, not even the doctors... |
| Jackie: Scleroderma, CREST, and Pulmonary Hypertension We were identical twins, and she died in 1988 from kidney failure... |
| Judy Dee: Diffuse Scleroderma My skin began to harden and turn a shade of brown... |
| (Update) Judith R. Thompson Devlin: Diffuse with CREST Last week I saw a nephrologist due to abnormal kidney results. This was disconcerting. My doctor has suggested vascular surgery on them... |
| Kathy GR: Scleroderma Patient The doctor who had been doing the nerve blocks had told me I had scleroderma, but never mentioned that I needed to see a rheumatologist... |
| Keith: Surviving Son of Scleroderma Patient In the fall of 1982 my mom was diagnosed with this dreadful disease and it caused kidney failure... |
| Linda W: Surviving Daughter of Scleroderma Patient All she wanted was for others to learn of this disease and know her story. She requested this about two weeks before she died... |
| Lori: Surviving Daughter of Father who had Scleroderma We held his hands and told him how much we loved him and watched him take his last breath at 5:50 p.m. on September 6, 2002... |
| Mendyon: Mother-in-Law of CREST Patient Unfortunately, as I sit here writing this
letter
my husband and I are faced with some grim circumstances. She is currently losing
her battle to CREST syndrome... |
| Michela P: Swelling in Hands and Feet I do not feel a lot of pain, but I feel like my skin is burning on my hands... |
| Nickole: Surviving Daughter of Diffuse Scleroderma Mother I am not sure how long my mother had scleroderma, but I do know that the doctors did not know much about it at the time... |
| Papa V: Lupus, Antiphospholipid Syndrome, Myasthenia Gravis, and Scleroderma About thirty years passed, and then—BAM !—I got a heart blockage, stroke, liver biopsy due to elevated enzymes, skin rash, high blood pressure, very high ANA.. |
| Robin H: End-Stage Renal Disease (ESRD)/Transplant Recipient It has been almost two years now since my kidney transplant. I am doing better than ever... |
| Rosemary F: Surviving Daughter of Diffuse Scleroderma Patient She tried to explain it, but it was hard for me to comprehend the disease's symptoms. Mom said that it was the same thing that her oldest sister died from... |
| Sandy: Diffuse Scleroderma After years of complaining about various symptoms to doctors, I was diagnosed with Diffuse Scleroderma in 1993... |
| Sherrill: Diffuse Systemic Scleroderma (New Zealand) I could no longer walk, due to knee contractures. My hands resembled claws. I had elbow contractures and neck contractures, and could not straighten my arms... |
| Susan H: Eosinophilic Fasciitis While I was in the hospital and starting dialysis, my right arm became hard as a rock and swollen... |
| Susie: CREST Syndrome I have had CREST syndrome for the past eight years. My specialist is Dr. Frederick Wigley, from Johns Hopkins... |
