Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

during surgery, febrile illness, or other stress

1st line – stress-dose glucocorticoid

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ACUTE
|
during surgery, febrile illness, or other stress
1st line – 

stress-dose glucocorticoid

During periods of stress (e.g., surgery, febrile illness, shock), all patients require increased amounts of glucocorticoid.[14] Affected patients should carry medical information regarding emergency corticosteroid dosing (medical alert bracelet or necklace).

Stress doses of glucocorticoids should continue around the clock until symptoms resolve. Typically, two to three times the normal dose is administered orally, or by intramuscular injection when oral intake is not tolerated. Higher doses may be required during surgical procedures.

The glucocorticoid of choice is hydrocortisone. However, prednisone may also be used. Intramuscular hydrocortisone can be given in case of vomiting or impending adrenal crisis.

Stress doses of glucocorticoids vary depending on the glucocorticoid dose the patient is currently on, the type of stress, and the clinical situation. Consult your local protocols for further information on stress doses of glucocorticoids.

ONGOING

classical CAH form

1st line – glucocorticoid

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ONGOING
|
classical CAH form
|
infant or child
1st line – 

glucocorticoid

Glucocorticoid treatment is effective and prevents further virilization. Patients with classical congenital adrenal hyperplasia require lifelong administration of glucocorticoids.

Titration of the dose should aim to maintain androstenedione levels at age- and sex-appropriate levels, while this should not be the case for 17-hydroxyprogesterone (17-OHP) levels. Oversuppression, including normalization of 17-OHP should be avoided because it can lead to growth suppression and iatrogenic Cushing syndrome.

Hydrocortisone is the treatment of choice in children. Its short half life minimizes the adverse effects of glucocorticoids, particularly growth suppression.[14]​ In children, growth velocity, bone age advancement and puberty should be monitored closely. Adults should be monitored for reproductive function and chronic complications.

Primary options

hydrocortisone: 10-15 mg/square meter of body surface area/day orally given in 3 divided doses; adjust dose according to disease severity and treatment response under specialist guidance

Plus – mineralocorticoid + sodium chloride

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ONGOING
|
classical CAH form
|
infant or child
Plus – 

mineralocorticoid + sodium chloride

Treatment recommended for ALL patients in selected patient group

Neonates and infants with the most severe form of classical salt-wasting congenital adrenal hyperplasia (CAH) present with severe dehydration due to a salt-wasting crisis. They require rapid evaluation and treatment with both a glucocorticoid and a mineralocorticoid, alongside appropriate fluid and sodium chloride replacement.

Mineralocorticoid therapy is recommended for all patients with classical CAH in infancy.

Sodium chloride is given to infants in order to achieve adequate sodium repletion and normalization of plasma renin activity. Added to formula or foods, it may not be necessary after infancy; the amount of mineralocorticoid required daily may likewise decrease with age.

Primary options

fludrocortisone: 0.05 to 0.3 mg/day orally

OR

sodium chloride: 1-3 g/day orally

1st line – glucocorticoid

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ONGOING
|
classical CAH form
|
adult with full linear height reached
1st line – 

glucocorticoid

Glucocorticoid treatment is effective in treatment of congenital adrenal hyperplasia (CAH). Patients with classical CAH require lifelong administration of glucocorticoids.

The dose should be titrated with an aim of maintaining androgen levels at age- and sex-appropriate levels, and 17-hydroxyprogesterone levels of 200 to 1000 nanograms/dL. Oversuppression should be avoided because it can lead to iatrogenic Cushing syndrome.

Primary options

hydrocortisone: 10-15 mg/square meter of body surface area/day orally given in 3 divided doses; adjust dose according to disease severity and treatment response under specialist guidance

OR

prednisone: 2-5 mg/square meter of body surface area/day orally given in 2-3 divided doses; adjust dose under specialist guidance

Plus – mineralocorticoid

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ONGOING
|
classical CAH form
|
adult with full linear height reached
Plus – 

mineralocorticoid

Treatment recommended for ALL patients in selected patient group

Most adults with classical salt-wasting congenital adrenal hyperplasia and without hypertension benefit from continued fludrocortisone treatment.[14] The need for ongoing replacement in those without true salt-wasting should be assessed at the transition from pediatric to adult care.

Primary options

fludrocortisone: 0.1 to 0.2 mg/day orally; or 0.1 mg orally three times weekly

nonclassical form

1st line – glucocorticoid

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ONGOING
|
nonclassical form
|
child with accelerated bone age or adolescent with overt virilization
1st line – 

glucocorticoid

Glucocorticoid treatment is not recommended for asymptomatic people with nonclassical congenital adrenal hyperplasia.[14] Glucocorticoid treatment is recommended when premature bone maturation is likely to affect a child’s final adult height. If the child has early-onset pubarche without advanced bone age, careful monitoring is often appropriate. The risks and benefits of glucocorticoid replacement should be discussed with the patient and their carers.[14] Irregular menses and acne in adolescents usually respond to glucocorticoid treatment within 3 months.[14] 

Titration of the dose should be aimed at maintaining androstenedione levels, while this should not be the case for 17-hydroxyprogesterone (17-OHP) levels. Oversuppression, including normalization of 17-OHP should be avoided because it can lead to growth suppression and iatrogenic Cushing syndrome.

Hydrocortisone is the treatment of choice in children.

Treatment may be tapered and discontinued once the child has reached near-adult height.[14]​ In children, growth velocity, bone age advancement and puberty should be monitored closely.

Primary options

hydrocortisone: 10-15 mg/square meter of body surface area/day orally given in 3 divided doses; adjust dose according to disease severity and treatment response under specialist guidance

1st line – glucocorticoid

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ONGOING
|
nonclassical form
|
adult female with hyperandrogenism or infertility
1st line – 

glucocorticoid

Glucocorticoid treatment is not recommended for asymptomatic people with nonclassical congenital adrenal hyperplasia.[14] Titration of the dose should aim to maintain androstenedione levels at age- and sex-appropriate levels, while this should not be the case for 17-hydroxyprogesterone (17-OHP) levels. Oversuppression, including normalization of 17-OHP should be avoided because it can lead to iatrogenic Cushing syndrome. Adults should be monitored for reproductive function and chronic complications.

Primary options

prednisone: 2-5 mg/square meter of body surface area/day orally given in 2-3 divided doses; adjust dose under specialist guidance

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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