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Kidney (Renal) Involvement Kidney (Renal) Dialysis and Transplant

Scleroderma Kidney (Renal) Involvement

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
Scleroderma Renal Crisis
Treatments
Hypertension
Hypertension Treatments
Rapidly Progressive Glomerulonephritis
Support
Personal Stories

Overview of Kidney (Renal) Involvement

All patients with systemic sclerosis (scleroderma) should be regularly screened for kidney involvement, even if they have short duration of mild disease and normal serum creatinine.
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.
Kidney Pain. Pain caused by the kidneys is typically felt in the flank area, which is in the back, just at the lower edge of the ribs on either side of the spine. Pain caused by the kidneys tends to be sharp and severe, to and occur in waves. It is uncommon for the kidneys to cause dull, aching pain. People sometimes equate back pain with kidney pain, but aching pain in the back—especially the lower back or the middle of the back—is usually due to muscles, ligaments, or even vertebrae and disks in the spinal column. Net Wellness.
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.

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.
Case Report: Scleroderma renal crisis in a newly diagnosed mixed connective tissue disease (MCTD) resulting in dialysis-dependent chronic kidney disease despite angiotensin-converting enzyme inhibition. A case of MCTD consisting of scleroderma, Sjögren's syndrome and polymyositis complicated by scleroderma renal crisis (SRC) but with negative anti-nuclear antibody (ANA), anti-Scl 70 and anti-centromere antibodies. Springer Link, May 2013;58:153 (Also see: Mixed Connective Tissue Disease)
Renal involvement in autoimmune connective tissue diseases. Patients with scleroderma renal crisis (SRC) show a significant benefit of early angiotensin-converting-enzyme (ACE) inhibitor use in particular and strict blood pressure control in general. PubMed, BMC Med, 2013 Apr 4;11:95.
Cautious ACE Inhibitor Use Needed in Scleroderma. This study suggests that ACE inhibitors should be used with great caution in patients with scleroderma, particularly early in disease when the risk of renal crisis is greatest. MedPageToday. 11/11/12. (Also see: Medication Warnings, and Hypertension)
Treatment of Systemic Sclerosis Complications: What to Use When First-Line Treatment Fails—A Consensus of Systemic Sclerosis Experts. Symptoms treatment recommendations by over 100 scleroderma experts, for scleroderma renal crisis (SRC), digital ulcers (DU), pulmonary hypertension (PAH/PH), reflux, skin involvement, and arthritis. Seminars in Arthritis and Rheumatism Volume 42, Issue 1 , Pages 42-55, August 2012. (Also see: Digital Ulcers, Pulmonary Hypertension, Reflux, Skin Involvement, Skeletal Involvement, Scleroderma Treatments, and Dr. Janet Pope)
Corticosteroids and the risk of scleroderma renal crisis: a systematic review. Great caution must continue to be exerted when initiating such therapy, especially in high doses and in the early diffuse subset of SSc patients. Trang G. Rheumatol Int. 2010 Dec 4. (Also see: Steroids)
The Scleroderma Kidney: Progress in Risk Factors, Therapy, and Prevention. Scleroderma Renal Crisis (SRC) occurs in 2% to 5% of patients with systemic sclerosis (SSc), particularly those with diffuse cutaneous SSc in the first years of disease evolution. Several retrospective studies have found high-dose corticosteroid therapy to be associated with increased risk of SRC, and anti-RNA-polymerase III antibodies have been detected in one third of patients with SRC. Bussone G., (PubMed) Curr Rheumatol Rep. 2010 Nov 9.
Scleroderma Renal Crisis: A Pathology Perspective (2010). Scleroderma renal crisis (SRC) is an infrequent but serious complication of systemic sclerosis (SSc). Renal biopsies play an important role in confirming the clinical diagnosis. Ibrahim Batal. International Journal of Rheumatology. 2010. ID 543704. (Also see: Kidney Involvement)
Scleroderma renal crisis. Renal involvement is common in systemic sclerosis, with most patients manifesting only mild renal dysfunction. By comparison, severe and life-threatening renal disease, called scleroderma renal crisis, develops in approximately 10 to 20 percent of patients with the diffuse cutaneous form of systemic sclerosis and much less frequently in limited cutaneous systemic sclerosis. John Varga. UpToDate. May 2010.
Association of RNA Polymerase III Antibodies with Scleroderma Renal Crisis (SRC). The authors report that the majority of the SRC patients had anti-topoisomerase I (anti-topo I) antibody (30/46, 65%), whereas a minority of patients (7/46, 15%) had anti-RNA polymerase III (RNAP). Binh Nguyen, MD. Journal of Rheumatology. Vol. 27. No. 5. May 2010. (Also see: Antibodies)
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

Corticosteroids strongly increase the short-term risk of developing scleroderma renal crisis!
ACE Inhibitors
Kidney (Renal) Dialysis
Kidney (Renal) Transplant
End-stage kidney disease due to scleroderma—outcomes in 127 consecutive ANZDATA. registry cases. Scleroderma is an uncommon cause of end-stage kidney disease (ESKD) which carries significant morbidity and mortality risks. Five year deceased donor and live donor renal allograft survival rates of recipients with scleroderma were 53 and 100%, respectively. Brian Siva, Nephrol. Dial. Transplant (2011) February 28, 2011.
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. Clinical Rheumatology. (Also see: Pulmonary Hypertension)
Prognosis of scleroderma renal crisis: a long-term observational study. Short-term prognosis of SRC has improved, but long-term prognosis remains disappointing. Plasma exchange (PEx) in addition to ACE inhibitors seems to be a therapeutic option in patients with SRC who develop micro-angiopathy or are intolerant to high doses of ACE inhibitors. Nephrol. Dial. Transplant. 2012.
ACE Inhibitors (Angiotensin-Converting Enzyme Inhibitors)
Scleroderma Renal Crisis (SRC): A Rare but Severe Complication of Systemic Sclerosis. The prognosis of SRC has dramatically improved with the introduction of angiotensin-converting enzyme inhibitors (ACEi). The treatment of SRC relies on aggressive control of blood pressure with ACEi, if needed in combination with other types of antihypertensive drugs. PubMed, Clin Rev Allergy Immunol. 2009 Dec 15.
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.

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.

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

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, nineteen 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...
 
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