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Scleroderma Kidney (Renal) Involvement

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


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.

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. VeryWell.

Renal Involvement in Systemic Sclerosis: An Update. There is a strong association between renal involvement and patients' outcomes in systemic sclerosis. PubMed, Kidney Blood Press Res, 2020 Jun 10;1-17.

Getting lots of exercise tied to lower risk of kidney disease. People who get plenty of physical activity may be less likely than their more sedentary counterparts to develop chronic kidney disease, a recent study suggests. Reuters Health, 02/14/2020.

Kidney Stones Are More Beautiful Than You Might Think. Kidney stones are surprisingly dynamic, forming much like microscopic coral reefs, according to new research that could provide insights into how to better diagnose and treat the condition. New York Times, 09/19/2018.

Prognosis of Renal Involvement

Late Gadolinium Enhancement (LGE) in Cardiac Magnetic Resonance Imaging Is Associated with High Renal Resistive Index (RRI) in Patients with Systemic Sclerosis. Our study, showed RRI and LGE as markers of vascular and fibrotic damage. PubMed, Kidney Blood Press Res, 2020;45(2):350-356. (Also see Heart Complications)

Prognostic Factors of Renal Involvement in Systemic Sclerosis (SSc). In SSc patients, a periodic follow–up based on clinical and laboratory evaluation, colorDoppler ultrasound and, in some cases, renal biopsy is required to evaluate renal involvement. PubMed, Kidney Blood Press Res, 2018 May 10;43(3):682-689.

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.

Scleroderma Renal Crisis (SRC). There is an urgent need to improve early recognition and treatment, and to identify novel treatments to improve outcomes of SRC. PubMed, Presse Med, 2021 Feb 3;104063.

Case Report: Hypertensive Emergency in a Woman with Systemic Sclerosis. We present a clinical case of scleroderma renal crisis with a typical presentation of hypertensive emergency and acute kidney injury. PubMed, High Blood Press Cardiovasc Prev.

The incidence rate of pulmonary arterial hypertension (PAH) and scleroderma renal crisis (SRC) in systemic sclerosis patients with digital ulcers on endothelin antagonist receptors (ERAs) and phosphodiesterase-5 inhibitors (PDE5i). Treatment with ERAs and/or PDE5i appeared to delay the occurrence of SRC. PubMed, Rheumatology (Oxford), 2020 Aug 26;keaa401. (Also see Pulmonary Hypertension and Research on Digital Ulcers)

Classifications of scleroderma renal crisis (SRC) and reconsideration of its pathophysiology. We provide evidence suggesting that better categorization of patients presenting with certain clinical features of both narrowly defined-SRC and thrombocytic micro-angiopathy will improve treatment approaches. PubMed, Rheumatology (Oxford), 09/30/2019.

Systemic sclerosis (SSc) medications and risk of scleroderma renal crisis (SRC). Ace inhibitor (ACEi) use at SSC diagnosis was associated with an increased risk for SRC and so SSC patients that require ACEi should be more closely monitored for SRC. PubMed, BMC Nephrol, 2019 Jul 25;20(1):279.

Scleroderma renal crisis (SRC): risk factors for an increasingly rare organ complication. By far the highest risk for SRC was associated with the detection of anti-RNAP antibodies and proteinuria. PubMed, J Rheumatol, 04/01/2019.

Endothelial lesion and complement activation in patients with Scleroderma Renal Crisis (SRC). It is likely that we will be forced to redefine the SRC concept, focusing on the relationship between acute endothelial damage and complement activation. PubMed, J Bras Nefrol, 02/25/2019.

Risk Factors for Future Scleroderma Renal Crisis (SRC) at Systemic Sclerosis Diagnosis. These patients may benefit from close observation of blood pressure, proteinuria, and estimated glomerular filtration rate, for earlier SRC identification and intervention. PubMed, J Rheumatol, 07/15/2018.

Case Report: 13–year history of scleroderma complicated by renal crisis (SRC) and thrombotic microangiopathy (TMA) treated with plasma exchange (PE). PE should be considered as a treatment option for SRC complicated by TMA. PubMed, Nihon Rinsho Meneki Gakkai Kaishi. (Also see Therapeutic Plasma Exchange)

Case Report: Delayed onset of posterior reversible encephalopathy syndrome in a case of scleroderma renal crisis (SRC) with maintenance hemodialysis. This case demonstrates that microangiopathy and extra–renal manifestations can develop even in SRC patients with end–stage renal disease and that these manifestations can be successfully managed. PubMed, Medicine (Baltimore).

Scleroderma renal crisis (SRC) during intravenous cyclophosphamide pulse therapy for complicated interstitial lung disease (ILD) was successfully treated with angiotensin converting enzyme inhibitor and plasma exchange (PE). This study presents the possibility of favorable effects of PE for SSc–associated ILD and SRC. PubMed, Nagoya J Med Sci. (Also see Therapeutic Plasma Exchange and Treatments for Pulmonary Fibrosis)

(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. Springer Link. (Also see Mixed Connective Tissue Disease)

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. (Also see Medication Warnings, and Hypertension)

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. Rheumatol Int. (Also see Steroid Warnings for Scleroderma)

(Case Report) 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.

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

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)

ACE Inhibitors (Angiotensin-Converting Enzyme Inhibitors)

Scleroderma renal crisis (SRC). SRC still remains an important cause of morbidity and mortality in scleroderma. Since the advent of ACE inhibitors, the prognosis of SRC has improved substantially. PubMed, Semin Arthritis Rheum.

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.

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. Medscape.

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.

Personal 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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