Sarcoidosis’ return after transplant spotlights need for better protocols

Woman dies of infection while under various immunosuppressive treatments

Lindsey Shapiro, PhD avatar

by Lindsey Shapiro, PhD |

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A woman who underwent a heart transplant for cardiac sarcoidosis later showed signs of sarcoidosis in the brain, a condition known as neurosarcoidosis, highlighting the need for better protocols to monitor and treat sarcoidosis patients after a transplant, scientists report.

Immunosuppressive treatments helped to ease the woman’s neurological symptoms, but she later developed a severe infection and died.

“Balancing adequate immunosuppression with infection risk post-transplant is further complicated in instances of sarcoidosis recurrence,” the scientists wrote. “There is a definite need for additional research on optimal monitoring, immunosuppressive protocols, and recurrence management for patients undergoing heart transplant for [cardiac sarcoidosis].”

The report detailing her case, “New-Onset Neurosarcoidosis Following Heart Transplant for Cardiac Sarcoidosis,” was published in JACC: Case Reports.

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Pondering the patchwork nature of sarcoidosis diagnosis and treatment

Woman had no signs of sarcoidosis outside the heart at time of transplant

In sarcoidosis, the immune system becomes overactive and small clumps of inflammatory cells called granulomas build in various tissues and disrupt their function. The disease can affect virtually any organ in the body, so it can manifest in different forms over time.

While sarcoidosis can happen as isolated episodes that resolve on their own, it also can lead to chronic problems with granuloma formulation that substantially affect organs. Disease treatments targeting one organ system do not necessarily prevent a recurrence in another part of the body.

Scientists at University of Chicago Medicine reported the case of a 63-year-old woman who underwent a heart transplant due to complications from cardiac sarcoidosis, and later developed symptoms of neurosarcoidosis.

The woman had been experiencing heart failure secondary to cardiac sarcoidosis, but she had no signs of disease manifestations elsewhere. Despite treatment, her health continued to decline and ultimately warranted a heart transplant.

After the transplant surgery, signs of immune activity that could signal organ rejection were evident, and the woman’s dose of mycophenolate mofetil (MMF), an immunosuppressant, was increased.

Seven months later, she was admitted to the hospital with severe diarrhea and a diminishing tolerance for oral foods, as well as neurological symptoms including headache, vision changes, and cold intolerance.

Blood tests showed signs of certain hormonal imbalances that could be driven by changes in the brain. A subsequent MRI showed abnormalities in the hypothalamus, a brain structure involved in producing hormones that regulate a variety of the body’s automatic functions.

Neurological symptoms led to suspicion of neurosarcoidosis

No signs of cancer or clinically significant infections were seen that could explain her neurological symptoms, and test findings were not thought to be consistent with a neurodegenerative disease like multiple sclerosis. A short course of intravenous steroids seemed to ease her symptoms.

Given that a tissue biopsy of her diseased heart confirmed sarcoidosis, “she fulfilled diagnostic criteria for presumed neurosarcoidosis,” the scientists wrote.

When a colon biopsy showed the likely cause of her gastrointestinal symptoms to be toxicity due to MMF, that treatment was discontinued and different immunosuppressant medications were given to prevent organ rejection.

A course of oral steroids also was initiated to treat her neurosarcoidosis, but its symptoms returned.

She then started on Remicade (infliximab), an immune-suppressing therapy approved for certain inflammatory diseases and used to treat sarcoidosis in patients who fail to respond to other medications. Remicade’s label, however, carries a boxed warning that its use raises the risk of serious infections.

Her condition improved and the woman was discharged from the hospital. But one week after a second Remicade infusion, she was readmitted for septic shock — a very severe and life-threatening reaction to an infection — secondary to a bacterial infection spreading from the bile ducts.

Despite intervention, she went into multiorgan failure and died. Her family did not want an autopsy, so a recurrence of sarcoidosis in the brain or heart was not confirmed.

Protocols lacking for monitoring, managing sarcoidosis patients after transplant

The scientists noted that there are no established protocols for monitoring cardiac sarcoidosis patients after a transplant, or for managing their immunosuppressive treatment regimens.

Such treatment is a delicate balance, as immune suppression must be enough to prevent sarcoidosis from recurring, but an overly suppressed immune system can lead to life-threatening infections.

More study, the scientists noted, is needed to understand whether whole-body imaging for sarcoidosis prior to a heart transplant might improve patient outcomes. Moreover, established protocols for post-transplant sarcoidosis monitoring are lacking.

“Further study is needed to understand if whole-body PET [scans] before [a heart transplant for cardiac sarcoidosis] would change management or outcomes,” the scientists wrote.

“Optimal methods of surveillance for post-transplant recrudescence of sarcoidosis have not been established but may include serial imaging,” they added.