Welcome to Study of the Week from Patient Worthy. In this segment, we select a study we posted about from the previous week that we think is of particular interest or importance and go more in-depth. In this story we will talk about the details of the study and explain why it’s important, who will be impacted, and more.

If you read our short form research stories and find yourself wanting to learn more, you’ve come to the right place.

This week’s study is…

Association of race and risk of future scleroderma renal crisis at systemic sclerosis diagnosis

We previously published about this research in a story titled “A Study Finds Scleroderma Renal Crisis is More Common Among Black People,” which can be found here. The study was originally published in the journal Arthritis Care & Research. You can view the abstract of the study here.

What Happened?

Scleroderma is a group of rare, autoimmune illnesses that can cause impacts throughout the body. Though limited to the skin in some cases, a possible complication of systemic scleroderma is scleroderma renal crisis, which is defined by sudden kidney failure, heart failure, and high blood pressure. Prior research has indicated that mortality and morbidity in scleroderma vary depending on race. This team of scientists sought to understand that variation more clearly using data from the United State Military Health System.

The researchers drew from data that included 294 patients with systemic scleroderma. 79 of this group identified as Black; the remainder were another race. The scientists then analyzed their medical records to see which patients developed renal crisis. They found that 13 Black patients experienced it while 16 of the other patients did. Overall, 16.5 of Black patients and 7.4 percent of the other patients developed scleroderma renal crisis.

The scientists also noticed that Black patients were more likely to be male and were younger than the other patients. When the team accounted for factors such as kidney function measures and age, the determined that Black patients were six times more likely to develop renal crisis. The researchers also determined some other factors that increased the risk of crisis regardless of race, such as proteinuria (high protein levels in the urine, a tell tale sign of kidney malfunction) and high blood pressure.

Among Black patients, risk factors for renal crisis included the presence of anti-RO antibodies; this was not a risk factor for the other patients, however. Risk factors for the non-Black patients included low platelet levels, old age, and the presence of cancer. Overall, the main takeaway was that Black patients, particularly those with anti-RO antibodies, are at the highest risk of scleroderma renal crisis.

About Scleroderma

Scleroderma, which is also referred to as systemic sclerosis, describes a group of autoimmune diseases that can cause system-wide effects in the most severe cases. The mechanism of this disease is believed to be an autoimmune response in which the immune system mistakenly attacks body tissue. Some factors that may contribute to triggering the autoimmune response include mutations of the HLA genes and exposure to certain materials, such as certain solvents, white spirits, ketones, and silica. Symptoms are broad ranging and systemic, including kidney failure, erectile dysfunction, fatigue, stroke, headaches, facial pain, congestive heart failure, skin abnormalities, high blood pressure, chest pain, indigestion, and many more. Treatments are varied and depend on the symptoms, but most patients take medications in an attempt to suppress the autoimmune response. In severe cases, life expectancy is around 11 years from onset. To learn more about scleroderma, click here.

Why Does it Matter?

The findings from this study indicate the first time that African ancestry has been identified an an independent risk factor for renal crisis in scleroderma. While past research has demonstrated differences in morbidity and mortality between racial groups, this data helps give further explanation for the disparities that have been identified. This is an important step, but additional research will be needed in order to understand the reason why Black patients are at greater risk:

“Black race was independently associated with a higher risk of future scleroderma renal crisis (SRC)… further studies are needed to elucidate the mechanisms that underlie this important association.”

It’s also important to note that this study identified unique risk factors among Black patients versus patients of other races for renal crisis. Knowing these factors will have concrete impacts on the effectiveness and timing of treatment for this rare disease.

“Based on our novel findings, Black SSc patients with anti-Ro antibody and proteinuria should be closely monitored for SRC. Identifying unique risk factors for future SRC development for Black and non-Black SSc patients would be important in earlier diagnosis, therapeutic intervention, and improvement in long-term clinical outcomes.”

Further research will definitely be necessary on this matter. The scientists emphasize that it is still to soon to draw cut and dry conclusions about cause and effect.