| Kidney (Renal) Involvement (Main Menu) |
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| Scleroderma Kidney (Renal) Involvement |
| This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer. |
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| Overview of Kidney (Renal) Involvement |
| Renal disease in systemic sclerosis with normal serum Creatinine. Patients with systemic sclerosis should be screened for renal involvement irrespective of disease severity or duration. Reem H. A. Mohamed Clinical Rheumatology. February 22, 2010. |
| 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. |
| Glomerular Filtration Rate (GFR). Glomerular filtration rate is the best test to measure your level of kidney function and determine your stage of kidney disease. Your doctor can calculate it from the results of your blood creatinine test, your age, race, gender and other factors. 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. |
| Corticosteroids strongly increase the short-term risk of developing scleroderma renal crisis! |
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. |
| 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. |
| Antiphospholipid antibodies and kidney involvement in patients with systemic sclerosis (SSc). Antiphospholipid (aPL) antibodies are often detected in systemic autoimmune diseases. Positivity for IgG aCL and IgG a-B2GPI in patients with SSc without secondary antiphospholipid syndrome seems to be connected with decrease of glomerular filtration. Ewa Wielosz Clinical Rheumatology 13 May 2009. (Also see: Antibodies) |
| 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. |
| 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) |
| 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. |
| 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. |
| 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. |
| Renal Failure Prevention and Treatments |
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| Corticosteroids strongly increase the short-term risk of developing scleroderma renal crisis! |
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) |
| 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. |
| 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) |
| 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. |
| Iloprost |
| Iloprost for the treatment of systemic sclerosis. Intravenous iloprost improves kidney vasospasm in patients with scleroderma. PubMed, 2008 May. |
| 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 |
| Donating Kidney Doesn't Shorten Lifespan. A study of over 80,000 American live kidney donors found they were likely to live just as long as people who have two healthy kidneys and that the procedure carries very little medical risk. Catharine Paddock, PhD . Medical News Today. 03/11/10. |
| 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) |
| 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. eMedicine. |
| 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) |
| 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) |
| 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. |
| 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... |
| Debs B: Diffuse Systemic Sclerosis (England) This seems to be a long list of ailments that I have been coping with for years, so the addition of diffuse systemic sclerosis did not really panic me... |
| 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... |
| Karligash: Systemic Scleroderma (Republic of Kazakhstan) Young, beautiful, full of hope and expectations for my life, for happiness and love — that was me, 19 years of age... |
| (Russian) Карлыгаш: системная склеродермия (Республика Казахстан) Молодая,красивая,полная ожидания от жизни счастья,любви такая я была в 19 лет... |
| Katia C: Sister of a Scleroderma Patient (Italy) She is in the acute phase of the illness, and she has a hardened skin, third degree, that has affected her hands, arms, chest and legs... |
| (Italiano) Katia: Fenomeno di Raynaud con Sclerodermia Diffusa (Italia) In conseguenza ha una crisi renale sclerodermica che gli ha alzato la presione e gli ha provocato una ischemia... |
| 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... |
| Maria T: Daughter of Deceased Scleroderma Patient (Italy) He, upon looking at my mom's face and hands diagnosed her with sclerodermia, in fact he took care of her until the end... |
| (Italiano) Maria T: Figlia di una Paziente Defunta di Sclerodemia (Italia) Lui, come vide a mia madre disse "tu hai la sclerodermia", guardandole le mani e la faccia... |
| 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... |
