Systemic scleroderma is an autoimmune disease of unknown cause, characterized by the formation of scar tissue in multiple organs — from the skin to, possibly, the heart, lungs, gastrointestinal tract , and the kidneys.

When the kidneys are affected, as they are in 5 to 10 percent of scleroderma patients, it can lead to a serious complication known as a renal crisis. In a crisis, the kidneys — which besides cleansing the blood work to regulate blood pressure — cause a rise in a person’s blood pressure because of scleroderma-related changes to blood vessels within the kidneys that deprives these organs of blood and oxygen. If not treated, or if treatment is ineffective, kidney failure could occur and dialysis, followed by a kidney transplant, likely will be necessary.

About kidney transplantation

A kidney transplant involves placing a single, healthy donor kidney in the abdomen. Usually, the damaged kidneys are not removed, because they usually are still functioning to a degree, if not sufficiently. A third kidney is transplanted and connected to blood vessels and the bladder.

Donor kidneys may be from a living donor (usually a friend or relative of the patient) or a recently deceased person whose kidneys were healthy. In either case, a close blood type and tissue match between donor and patient are required.

Abdominal surgery, under general anesthesia, is required to place the donor’s kidney and connect the blood vessels and ureter (the tube connecting the kidney and the bladder that carries urine).

The surgery typically takes about four hours, and most patients are out of bed in a day or two. Kidney transplant patients can usually go home after a week, but it may take two to four weeks for the new kidney to be fully functional, and patients may still need to be on dialysis during that time.

There are many risks associated with a kidney transplant, including blood clots, bleeding, infections, failure of the donated kidney, and rejection of the kidney. The prognosis for systemic scleroderma patients with a kidney transplant is good, and it is very unlikely that renal crisis will reoccur in the transplanted kidney. The possibility, however, exists.

Outlook for scleroderma patients

A 2017 published study in France assessed 34 scleroderma patients with renal crisis who received 36 kidney transplants between 1987 and 2013. Survival was found to be 100 percent the first year post-transplant, and 83 percent at five years. Complications, however, were recorded: heart symptoms worsened in 45 percent of these transplant patients, and gastrointestinal symptoms worsened in 26 percent. Three also had a reoccurrence of scleroderma-related renal crisis following the transplant.

A U.S. study that followed 258 patients with scleroderma renal crisis who had undergone a transplant or were on dialysis, found that kidney transplants significantly improved patient survival at one and three years post-transplant. Interestingly, the study also reported four patients who received a new kidney showed a decrease in skin symptoms post-transplant, indicating that a kidney transplant (or the combination of the transplant and immune-suppressing medications) may reduce the systemic symptoms of scleroderma.

Life after a kidney transplant

A kidney transplant is not a cure for kidney failure, and the damaged kidneys are usually not removed unless a pressing reason exists to do so. It  typically takes time for the donated kidney to function as needed, and it may never work as well as the original kidneys before they were damaged.

Patients are advised to modify their diet and lifestyle to minimize the strain on the new kidney, such as following a heart-healthy diet and engaging in a light-to-moderate exercise routine.

Patients will also have to take immune-suppressing medications for the rest of their lives to help prevent their body from rejecting the transplanted kidney.

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Scleroderma News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.