Bone tissue death successfully treated in man, 71, with Cushing’s
Timely treatment of avascular necrosis can prevent long-term disability
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A 71-year-old man with Cushing’s disease developed avascular necrosis in the hips and shoulders, a condition in which bone tissue dies due to lack of blood supply, a U.S. case study reports.
His Cushing’s symptoms, including avascular necrosis in the hips, were successfully managed with surgery to remove the disease-causing pituitary tumor, radiotherapy, and Isturisa (osilodrostat). He later developed avascular necrosis in the shoulders, which was effectively treated with local corticosteroid injections.
“Screening for [avascular necrosis] in patients with [Cushing’s] should be considered to enable timely intervention and prevent long-term complications, particularly in patients with hip or shoulder pain and severe hypercortisolism,” researchers wrote. “Early detection and intervention … are crucial to prevent irreversible joint damage and disability.”
His case was described in the study “Avascular Necrosis in Patients With Cushing Syndrome,” which was published in JCEM Case Reports.
Cushing’s disease is one of the most common forms of Cushing’s, an umbrella term for conditions characterized by elevated levels of the hormone cortisol in the body (hypercortisolism). Cushing’s disease is caused by tumors in the brain’s pituitary gland that trigger the excessive production of the adrenocorticotropic hormone (ACTH), which stimulates the adrenal glands to produce cortisol.
Avascular necrosis less common Cushing’s symptom
Common Cushing’s symptoms include weight gain and fat buildup, skin changes, muscle weakness, and mental health issues. A less frequent symptom is avascular necrosis, which tends to affect bones in the hips and shoulders, causing pain and tenderness in affected joints.
In the report, researchers described the case of a man with Cushing’s disease who had avascular necrosis in both hips. He was initially diagnosed with new-onset high blood pressure during his annual primary care visit and started on blood pressure-lowering medications.
The medications failed to control his blood pressure, and over the course of two months, he developed generalized swelling, shortness of breath, eyelid droopiness, and double vision. He also reported irritability, mood swings, increased appetite, weight gain, low sex drive, and hip pain.
Physical examination revealed the man had facial plethora (puffiness and redness), fat accumulation between the shoulder blades, muscle weakness, and hip tenderness. Laboratory testing confirmed a diagnosis of ACTH-dependent Cushing’s, with elevated levels of ACTH and 24-hour urine free cortisol. He also had diabetes and low levels of testosterone.
Imaging tests revealed the presence of a tumor in the pituitary gland. Additionally, the patient had avascular necrosis in both thigh bones.
He started treatment to prevent infections and underwent transsphenoidal surgery, a minimally invasive surgical procedure to remove the pituitary tumor through the nose. Analysis of the removed tissue confirmed the tumor was ACTH-positive.
Early detection and intervention … are crucial to prevent irreversible joint damage and disability.
Due to persistent hypercortisolism and residual tumor, he received targeted radiotherapy and Isturisa at a dose of 2 mg twice a day. He also received hydrocortisone when he started showing signs of low cortisol, and the dosage of Isturisa was decreased. Both therapies were stopped after his cortisol levels normalized.
Clinically, his high blood pressure and diabetes resolved, he lost weight, and his vision issues and hip pain improved without restrictions in mobility.
However, one year after surgery, the patient started having pain in both shoulders, consistent with avascular necrosis detected by MRI. He received methylprednisolone injections, which eased the pain and improved his joint mobility.
“We aim to highlight this presenting feature [of avascular necrosis] to heighten awareness for screening for this progressive condition, which can potentially lead to joint damage, loss of mobility, and long-term disability,” the researchers wrote. “Early diagnosis relies on MRI or CT imaging.”