Revista da Sociedade Portuguesa de Endocrinologia Diabetes e Metabolismo

Online first

Revista Portuguesa de Endocrinologia, Diabetes e Metabolismo - Online first: 2018-07-08
Original article

Spontaneous vertebral fractures in a young patient with ACTH-independent hypercortisolism

Pereira B

Abstract

Osteoporosis at a young age should prompt clinicians to search for secondary causes, namely endogenous Cushing’s syndrome. This very rare disease leads to high levels of glucocorticoids and prone patients to fractures, mainly in the vertebrae. Herein we present a young patient with osteoporosis and spontaneous vertebral fractures due to ACTH-independent Cushing’s syndrome. A 46 year-old male with a 2-year history of osteoporosis presented with extremely painful thoracic hyperkyphosis and florid features of Cushing’s syndrome, confirmed biochemically and by imaging as ACTH-independent hypercortisolism. Dual-energy X-ray absorptiometry revealed total Z-scores in the lumbar spine and femoral of -1.9 SD and -2.3 SD, respectively. Spinal magnetic resonance imaging revealed multiple vertebral fractures with focal thoracic medullar edema. Surgical excision of an adrenal adenoma and medical therapy with biphosfonates led to remission of all Cushing’s associated comorbidities and to a remarkable improvement in dual-energy X-ray absorptiometry, mainly in the lumbar spine (total Z-score 0.4 SD). Endogenous Cushing’s syndrome should always be thought in the context of severe osteoporosis at a young age. The delivery of the best therapy for osteoporosis in this context depends on its recognition.

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