Osteitis Fibrosa Cystica

Summary about Disease


Osteitis Fibrosa Cystica (OFC), also known as brown tumor, is a skeletal disorder resulting from excessive parathyroid hormone (PTH), which typically occurs in hyperparathyroidism. The excess PTH causes bone resorption, leading to replacement of bone tissue with fibrous tissue and cysts. This weakens the bones and can cause pain, fractures, and deformities.

Symptoms


Symptoms can vary in severity and may include:

Bone pain

Bone fractures (especially in the long bones, ribs, and vertebrae)

Bone deformities

Muscle weakness

Fatigue

Joint pain

Kidney stones

Nausea, vomiting, and constipation (due to hypercalcemia)

Cognitive changes (confusion, memory problems) in severe cases

Causes


The primary cause of osteitis fibrosa cystica is hyperparathyroidism, which can be:

Primary hyperparathyroidism: Usually caused by a benign tumor (adenoma) on one of the parathyroid glands.

Secondary hyperparathyroidism: Occurs in response to another condition, such as chronic kidney disease, that causes low calcium levels in the blood. The parathyroid glands overproduce PTH to compensate.

Tertiary hyperparathyroidism: Occurs after long-standing secondary hyperparathyroidism, where the parathyroid glands become autonomous and continue to overproduce PTH even after the underlying cause is corrected.

Medicine Used


Treatment focuses on addressing the underlying hyperparathyroidism. Medications may include:

Calcimimetics (e.g., Cinacalcet): Used to lower PTH levels by increasing the sensitivity of the calcium-sensing receptor on the parathyroid gland. Primarily used in secondary hyperparathyroidism associated with chronic kidney disease.

Vitamin D supplements: Used if vitamin D deficiency is contributing to the secondary hyperparathyroidism.

Bisphosphonates: Can help to strengthen bones and reduce the risk of fractures.

Surgery (Parathyroidectomy): Surgical removal of the overactive parathyroid gland(s) is the definitive treatment for primary hyperparathyroidism.

Is Communicable


No, osteitis fibrosa cystica is not a communicable disease. It is not contagious and cannot be spread from person to person.

Precautions


Precautions are aimed at preventing complications and managing the underlying condition:

Maintain adequate calcium and vitamin D intake: As directed by a healthcare professional.

Avoid activities that could lead to bone fractures: Due to weakened bones.

Regular monitoring of calcium and PTH levels: To ensure treatment is effective.

Manage underlying conditions: Such as chronic kidney disease, which can contribute to secondary hyperparathyroidism.

How long does an outbreak last?


Osteitis fibrosa cystica is not an outbreak, but a chronic condition. Once the underlying hyperparathyroidism is treated, the skeletal lesions can heal, but this can take months to years. Symptom duration depends on the severity of the disease and how quickly the hyperparathyroidism is addressed.

How is it diagnosed?


Diagnosis typically involves:

Blood tests: To measure calcium, PTH, phosphorus, and vitamin D levels. Elevated PTH and calcium levels are suggestive of hyperparathyroidism.

Urine tests: To assess calcium excretion and kidney function.

Imaging studies:

X-rays: To visualize bone lesions (brown tumors).

Bone densitometry (DEXA scan): To assess bone mineral density and detect osteoporosis.

Parathyroid scan (Sestamibi scan): To locate overactive parathyroid glands.

Bone biopsy: Rarely needed, but may be performed to confirm the diagnosis if imaging is inconclusive.

Timeline of Symptoms


The timeline of symptoms can vary greatly depending on the severity and progression of the underlying hyperparathyroidism:

Early stages: May be asymptomatic or have mild, nonspecific symptoms like fatigue or bone pain.

Progressive stages: As PTH levels remain elevated, symptoms become more pronounced, including increased bone pain, fractures, muscle weakness, and kidney stones.

Advanced stages: Severe bone deformities, significant pain, and neurological complications can develop.

Post-treatment: After treatment of hyperparathyroidism, bone lesions can heal over months to years, and symptoms gradually improve.

Important Considerations


Early diagnosis and treatment of hyperparathyroidism are crucial to prevent or minimize the skeletal complications of osteitis fibrosa cystica.

Regular monitoring of calcium and PTH levels is essential to ensure treatment effectiveness.

Patients with OFC are at increased risk of fractures, so fall prevention strategies are important.

Treatment of underlying conditions, such as chronic kidney disease, is essential in managing secondary hyperparathyroidism.

Surgical intervention (parathyroidectomy) is often the most effective treatment for primary hyperparathyroidism.