What Is Scleroderma Renal Crisis?

A Potentially Life-Threatening Complication of Scleroderma

Table of Contents
View All
Table of Contents

One of the most serious potential complications from the autoimmune disease scleroderma (also called “systemic sclerosis”) is renal crisis. When this happens, your blood pressure rises sharply, and your kidneys begin working poorly. Because of this, you may need temporary or even permanent dialysis. 

Scleroderma renal crisis is thought to happen in somewhere between 2% and 15% of people with scleroderma.

I'm experiencing headaches so my blood pressure might be high

laflor/ E+/ Getty Images

Symptoms

People with scleroderma renal crisis may have symptoms because their blood pressure has increased sharply and swiftly. For example, these might include:

  • Headache
  • Dizziness
  • Nausea 
  • Shortness of breath
  • Confusion
  • Seizures
  • Fatigue
  • Visual disturbances

However, not all people with scleroderma renal crisis will have any of these because elevated blood pressure doesn’t always cause symptoms. Blood pressure is often greater than 150/90 (mm Hg) or at least 30 above a person’s baseline blood pressure. 

Other times, a person might have symptoms related to kidney damage. For example, they might not be urinating as much as they would normally. 

If you are someone with scleroderma, it’s important to know about these potential symptoms of possible scleroderma renal crisis, like headache and dizziness. If you experience symptoms like this, it’s important to see a health professional right away.

Ideally, keep a device that you can use to check your blood pressure at home. That way, if you feel strange, you can check your blood pressure yourself. If it’s significantly elevated, definitely seek immediate medical attention. 

Cause

Scleroderma is an autoimmune disease that leads to skin thickening. Scleroderma can also lead to inflammation in various parts of the body.

This can lead to the formation of scar-like tissue that can prevent these internal organs from working well. For example, it can lead to another serious complication of scleroderma from scar tissue buildup in the lungs.

Inflammation and scar tissue can affect the blood vessels in the kidneys. In some cases, this causes the blood pressure to increase relatively quickly. The kidney itself may not be getting enough blood flow. This can lead to scleroderma renal crisis—a sudden increase in blood pressure that also damages the kidneys. 

Who Is at Highest Risk of Scleroderma Renal Crisis?

Researchers don’t fully understand why a minority of people with scleroderma eventually develop renal crisis. But certain factors put you more at risk:

  • Relatively recent scleroderma symptoms: The risk is highest in the first four years after symptoms begin.
  • Scleroderma skin symptoms that are rapidly getting worse
  • Diffuse cutaneous version of scleroderma: People who have the limited cutaneous form of the disease are much less likely to have a scleroderma renal crisis.
  • Scleroderma symptoms that include arthritis
  • Scleroderma that requires treatment with glucocorticoids like prednisone
  • Scleroderma that is positive for certain antibodies on blood tests (like anti-RNA polymerase III antibodies)
  • Other health features, such as anemia or heart issues

Blood Pressure Monitoring if You Are High-Risk

If you have a high risk of scleroderma renal crisis because of these factors, you should regularly monitor your blood pressure at home. Let your healthcare provider know right away if your blood pressure is significantly higher than normal. 

Besides doing regular blood pressure checks, people at higher risk of scleroderma renal crisis also need periodic tests to check if their kidneys are doing well. This may include blood tests like creatinine and tests on your urine. 

You can’t do anything about these risk factors for scleroderma renal crisis. However, there is one risk factor over which you have some control—dehydration. So, it’s probably a good idea for people with scleroderma to drink enough to stay hydrated. Also, avoid situations where dehydration might become an issue (e.g., a sauna).

Diagnosis

The person’s medical history and physical exam provide the starting clues to diagnosis. The healthcare provider will ask about your recent symptoms and your medical conditions and check you out physically. This will include basic assessments like your blood pressure.

It’s especially important that you tell your healthcare provider that you have been diagnosed with scleroderma. You can even bring up the possibility of scleroderma renal crisis if you are concerned about increased blood pressure. (Although specialists will be very familiar with this scenario, it might not leap to mind for more general physicians, like ER doctors.)

It’s a good idea to keep a record of your baseline blood pressure so you can provide this as part of your medical history. This can give important context for your current blood pressure reading.

Most people with scleroderma renal crisis have very high blood pressure. However, not everyone with scleroderma renal crisis has a very elevated blood pressure when compared with the general population.

For example, this might not be the case if your blood pressure generally runs lower than average. But if your blood pressure increased 30 mm Hg or more from your normal pressure, scleroderma renal crisis is a strong possibility.

Lab Tests

Basic lab tests can also be helpful in diagnosis. For example, basic blood tests for creatinine are usually increased in people with scleroderma renal crisis. This indicates kidney damage. 

Some people with scleroderma renal crisis will have signs of anemia, such as might be seen on a complete blood count (CBC). Specifically, they might have a type of anemia characterized as microangiopathic hemolytic anemia.

Individuals may also show thrombocytopenia (decreased number of blood platelets). Other blood tests may help rule out other possible causes for your symptoms as well.

Tests on your urine can also give some clues. For example, people with scleroderma renal crisis often have protein in their urine, which is abnormal. The urine also may have a tiny amount of blood in it as well. 

For diagnosis, it’s important to rule out other potential causes. For example, some types of vasculitis (inflammation of the blood vessels) can have some similarities. If a diagnosis is in question, sometimes more in-depth testing might be needed, like a kidney biopsy.

Diagnosing Scleroderma Renal Crisis and Scleroderma

Often, a person experiencing a scleroderma renal crisis will already know that they have scleroderma. However, a scleroderma renal crisis is sometimes the first major sign that a person has scleroderma. Before this, they might have had only subtle symptoms or even no symptoms at all.

If this is the case, diagnosing scleroderma renal crisis can be more challenging because the clinician isn’t thinking of scleroderma right away. Scleroderma is a rare disease, and other causes of increased blood pressure are more common.

Symptoms like hard, thickened skin or changes in skin color can be a clue that scleroderma is a possibility. Other symptoms might be present, like constipation, fatigue, and shortness of breath.

Other parts of the medical history and physical exam can point toward scleroderma. For example, people with scleroderma often have previously experienced Raynaud’s phenomenon, in which their fingers get very painful and pale in response to cold or stress.

Being positive for certain autoimmune antibodies can also make scleroderma more likely. Rarely, a skin biopsy can be helpful in diagnosis. Unfortunately, there isn’t one single test that proves a person has scleroderma. A healthcare provider must assess the individual’s full clinical picture.  

It’s vital that scleroderma renal crisis be diagnosed as quickly as possible. That’s because you’ll have a much better chance of restoring your kidney function if you are treated with the correct medication right away.

Treatment

Drugs called ACE inhibitors are the key initial treatment for scleroderma renal crisis. Two types commonly used in this situation are Capoten (captopril) and Vasotec (enalapril). It’s essential that an ACE inhibitor drug be started quickly.

ACE inhibitors are commonly used to lower blood pressure, even in people who don’t have scleroderma. However, they have other beneficial effects on the kidney that help restore kidney function in scleroderma renal crisis. (Unfortunately, taking ACE inhibitors long-term to prevent scleroderma renal crisis doesn’t seem to work.)

Sometimes other blood pressure medications are given in addition to ACE inhibitors. This might be done if the person is already receiving high doses of ACE inhibitors, but their blood pressure is still very high.

For example, depending on the situation, this might include a calcium channel blocker drug like Norvasc (amlodipine). Or a diuretic might be used. 

However, it’s very important that ACE inhibitors be used as the initial drug to help lower blood pressure. This is different from a very elevated blood pressure that can arise from other causes, which is part of the reason it is so important that it be correctly diagnosed.

Other newer types of drugs are sometimes used to treat scleroderma renal crisis if it still isn’t under control. Endothelin-1 receptor antagonists such as Tracleer (bosentan) are sometimes used, as is a drug called Soliris (eculizumab). However, their effectiveness is not as well established.

Some people with scleroderma renal crisis are very ill and require treatment in the intensive care unit. 

Dialysis

Many people with scleroderma renal crisis also need to be put on dialysis because their kidneys are working so poorly. Dialysis does the work that your kidneys normally do, filtering out wastes and balancing electrolytes in your body. Nearly half of people who experience scleroderma renal crisis will need to be put on dialysis.

The good news is that not everyone who starts dialysis for scleroderma renal crisis will need to be on it permanently. Often, a person’s kidneys will recover well enough to come off dialysis treatment eventually, perhaps within a couple of weeks.  

Unfortunately, that isn’t the case for everyone with scleroderma renal crisis, especially if they weren’t correctly diagnosed and treated properly. In this case, you might need to be on some type of dialysis long-term.

If your kidneys never recover, you might be able to receive a kidney transplant. In this case, you’d need to go on lifelong medications to prevent organ rejection. 

Prognosis

Scleroderma renal crisis is a very serious, potentially life-threatening condition. Particularly before we learned about the benefit of ACE-inhibitor drugs, scleroderma renal crisis was incredibly difficult to treat. At that time, most people died from it, unless they could get an emergency kidney transplantation. 

Although the prognosis of scleroderma renal crisis is now much better, we still need better treatments for it. Around 20% of people who develop scleroderma renal crisis die within a year, often from complications of the disease. And even more people never get enough recovery of their kidneys to get off dialysis.

A Word From Verywell

Most people with scleroderma will never experience a renal crisis. Still, if you have scleroderma, it can be scary to learn that this is a possibility. But you can learn about your specific level of risk and take steps to monitor your health. Through education, you’ll give yourself the best chance of having less severe issues from scleroderma crisis. 

Was this page helpful?
6 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  1. Soukup T, Toms J, Oreska S, et al. Renal involvement in systemic sclerosis: New insights into systemic sclerosis. July 9. 2019. IntechOpen. doi:10.5772/intechopen.87187

  2. Mouthon L, Bussone G, Berezné A, Noël LH, Guillevin L. Scleroderma renal crisis. J Rheumatol. 2014;41(6):1040-8. doi:10.3899/jrheum.131210

  3. Chrabaszcz M, Małyszko J, Sikora M, et al. Renal involvement in systemic sclerosis: An update. Kidney Blood Press Res. 2020;45(4):532-548. doi:10.1159/000507886

  4. Zanatta E, Codullo V, Allanore Y. Scleroderma renal crisis: Case reports and update on critical issues. Eur J Rheumatol. 2020 Nov 19. doi:10.5152/eurjrheum.2020.20048

  5. Saketkoo LA, Magnus JH, Doyle MK. The primary care physician in the early diagnosis of systemic sclerosis: the cornerstone of recognition and hopeAm J Med Sci. 2014;347(1):54-63. doi:10.1097/MAJ.0b013e3182a55d24

  6. Hickman RJ. The American College of Rheumatology. The history of ACE inhibitors in scleroderma renal crisis. The Rheumatologist. February 26, 2021.