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crinecerfontBlue Cross Blue Shield of Illinois

classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency

Initial criteria

  • The patient has a diagnosis of classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency confirmed by ONE of the following: positive infant screening with secondary tier 2 confirmatory testing OR elevated serum 17-hydroxyprogesterone level above ULN OR cosyntropin (ACTH) stimulation test OR genetic testing for mutation in the CYP21A2 gene consistent with CAH
  • If the patient has an FDA labeled indication, then ONE of the following: the patient’s age is within FDA labeling for the requested indication OR there is support for using the requested agent for the patient’s age for the requested indication
  • The patient is currently treated with glucocorticoid replacement therapy (e.g., hydrocortisone, prednisone, prednisolone, dexamethasone)
  • The patient will continue glucocorticoid replacement therapy in combination with the requested agent
  • The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist, geneticist), or the prescriber has consulted with such a specialist
  • The patient does NOT have any FDA labeled contraindications to the requested agent

Reauthorization criteria

  • The patient has been previously approved for the requested agent through the plan’s Prior Authorization process
  • The patient has had clinical benefit with the requested agent
  • The patient is currently treated with glucocorticoid replacement therapy
  • The patient will continue glucocorticoid replacement therapy in combination with the requested agent
  • The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist, geneticist), or has consulted with such a specialist
  • The patient does NOT have any FDA labeled contraindications to the requested agent

Approval duration

12 months