Tapering of corticosteroids key in autoimmune disease treatment
Case report: Boy with VKH developed Cushing's syndrome after long-term use
The case of a young adolescent boy illustrates that doctors should carefully taper corticosteroids and regularly monitor hormones in children with autoimmune Vogt-Koyanagi-Harada disease (VKH) to prevent Cushing’s syndrome and other serious complications, according to a study.
“Many pediatric patients with VKH and steroid-related complications are managed not only by pediatric endocrinologists but also by pediatric providers, including nurse practitioners,” researchers in Italy wrote. “This case highlights aspects relevant to a broad clinical audience.”
The boy’s case is described in “Secondary Adrenal Insufficiency and Iatrogenic Cushing’s Syndrome in a 13-Year-Old Male With Vogt-Koyanagi-Harada Disease: A Case Report,” which was published in the Journal of Pediatric Health Care.
Boy’s symptoms returned after prednisone was stopped
Cushing’s syndrome occurs due to high levels of cortisol in the body. An excess of cortisol can arise from problems in the body itself — most commonly a tumor, as in the case of Cushing’s disease. There are also external factors, typically as a side effect of medications such as corticosteroids, which work similarly to cortisol.
In this study, the researchers describe the case of a 13-year-old boy who developed Cushing’s syndrome and adrenal insufficiency due to long-term use of corticosteroids to treat VKH, a rare autoimmune disease where the body’s immune system mistakenly attacks pigmented cells in the eye, inner ear, and skin. Adrenal insufficiency occurs when the adrenal glands don’t make enough cortisol.
The boy first visited the hospital when he was almost 12 years old because of pain and redness in his right eye. He also reported sensitivity to light. Tests showed inflammation in both eyes, but no infection. He was started on oral prednisone, a corticosteroid, along with topical steroids on his eyes, which eased his symptoms.
After seven months, prednisone was stopped, and his symptoms returned. To reduce the need for high doses of prednisone, which can cause serious side effects, doctors added the immunosuppressant mycophenolate mofetil. Prednisone was then tapered and discontinued again after another seven months, when the boy had gained 15 kilograms (about 33 pounds) and developed “progressive cushingoid features,” including a fatty hump on his neck and reddish stretch marks on his skin. These features suggested that Cushing’s syndrome may have developed secondary to long-term use of corticosteroids.
We strongly advocate for multidisciplinary collaboration in managing complex VKH cases.
When the body detects excess cortisol, it may stop producing its own through negative feedback on the hypothalamic–pituitary–adrenal axis, which regulates the release of hormones between the brain and the adrenal glands. This can cause these glands, located on top of the kidneys, to lose function.
Indeed, when the doctors stopped prednisone, blood tests showed low cortisol, suggesting adrenal insufficiency.
When examined at the authors’ endocrinology clinic, the boy was shorter than expected for his age, was in the early puberty stage, and had central obesity, or excess accumulation of fat in the abdominal area. Further hormonal testing found that his blood cortisol levels had partially normalized.
However, two months after stopping prednisone, his VKH relapsed. He needed another course of prednisone and a higher dose of mycophenolate mofetil.
After months of careful monitoring, the doctors managed to stop corticosteroids completely. However, the boy’s face and body still showed some cushingoid features, and he “currently remains under combined rheumatologic, ophthalmologic, and endocrinologic management,” the researchers wrote.
They added that this case highlights the importance of individualized tapering of corticosteroids to prevent Cushing’s syndrome and adrenal insufficiency.
“We strongly advocate for multidisciplinary collaboration in managing complex VKH cases,” the researchers wrote.
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