Pediatric Graves Disease Guidelines

Updated: Oct 05, 2021
  • Author: Lynne Lipton Levitsky, MD; Chief Editor: Sasigarn A Bowden, MD  more...
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Guidelines Summary

Management of neonates born to mothers with Graves disease

A study by van der Kaay et al established a literature-based management algorithm for neonates born to mothers with Graves’ disease. The following eight suggestions were included [19, 20] :

1. Evaluate initial risk assessment on maternal thyroid stimulating hormone (TSH) receptor antibodies. If levels are negative, no need for follow-up; if positive, or not available, newborns should be considered "at risk" for hypothyroidism.

2. Newborns with negative TSH-receptor antibodies can be discharged from follow-up so test cord blood for TSH-receptor antibodies as soon as possible.

3. Measurement of cord fT4 and TSH levels is not indicated.

4. Evaluate fT4 and TSH levels at day 3 to 5, repeat at day 10 to 14, and follow clinically until 2 to 3 months.

5. The testing schedule is the same for neonates born to mother with treated or untreated Graves’ disease.

6. Methimazole (MMI) is the treatment of choice when warranted. Beta blockers can be added for sympathetic hyperactivity. Patients with refractory symptoms can also be given potassium iodide along with MMI. It is not clear whether asymptomatic infants with biochemical hyperthyroidism should be treated. It is not known whether asymptomatic infants with biochemical hyperthyroidism need to be treated.

7. Until they are stable, assess the MMI-treated newborn weekly and then every one to two weeks, with a decrease of medications as tolerated. The length of MMI treatment is generally one to two months.

8. Be mindful that central or primary hypothyroidism can develop in these newborns.

Medical management of Graves orbitopathy

Clinical practice guidelines for the medical management of Graves orbitopathy by the European Group on Graves' Orbitopathy were published in August 2021 in the European Journal of Endocrinology. [21]

Give oral prednisone/prednisolone prophylaxis to patients who have been treated with radioactive iodine (RAI) and who are at risk for progression or de novo development of Graves orbitopathy. These include smokers and those with severe/unstable hyperthyroidism or high serum thyrotropin receptor antibody (TSHR-Ab). Use the following regimen:

  • High risk: 0.3–0.5 mg/kg/body weight as starting dose, tapered, and withdrawn after 3 months
  • Low risk: 0.1–0.2 mg/kg/body weight, tapered, and withdrawn after 6 weeks

Patients with longstanding and stably inactive GO can receive RAI without prednisone/prednisolone cover if risk factors for GO progression (particularly smoking and high serum TSHR-Ab titers) are absent. Avoid uncontrolled post-RAI hypothyroidism.

All patients with GO should receive extensive local treatment with artificial tears at all times during the course of their disease unless corneal exposure requires higher protection than ophthalmic gels or ointment, especially at nighttime.

Treat mild GO with local treatments and general measures to control risk factors; give a 6-month selenium supplementation to patients with mild and active GO of recent onset, because it improves eye manifestations and quality of life, and usually prevents GO progression to more severe forms.

Do not exceed a cumulative dose of 8 g of IV glucocorticoids for each cycle; do not give IV glucocorticoids to patients with GO who have evidence of recent viral hepatitis, significant hepatic dysfunction, severe cardiovascular morbidity, or uncontrolled hypertension; diabetes should be well controlled before starting treatment. The authors strongly recommend only applying such treatment in centers with experience managing potentially serious adverse events.

Give an intermediate dose of IV glucocorticoids (ie, a starting dose of methylprednisolone 0.5 g IV once weekly for 6 weeks, followed by 0.25 g once weekly for 6 weeks, with a cumulative dose of 4.5 g) in most cases of moderate-to-severe and active GO.

Reserve high-dose treatment (ie, a starting dose of methylprednisolone 0.75 g IV once weekly for 6 weeks, followed by 0.5 g once weekly for 6 weeks, with a cumulative dose of 7.5 g) for the more severe cases (constant/inconstant diplopia, severe proptosis, severe soft-tissue pathology or involvement) within the moderate-to-severe and active GO spectrum.

Intravenous methylprednisolone in combination with oral mycophenolate sodium (or mofetil) represents the first-line treatment for moderate-to-severe and active GO.

Monotherapy with methylprednisolone at the highest cumulative dose (7.5 g per cycle) represents an additional valid first-line treatment for those with more severe forms of moderate-to-severe and active GO, including constant/inconstant diplopia, severe inflammatory signs, and exophthalmos >25 mm.

Severe corneal exposure should be urgently treated medically or by means of progressively more invasive surgeries to avoid progression to corneal breakdown, the latter of which requires immediate surgery.

Sight-threatening GO is an emergency; treatment is an absolute priority; treat hyperthyroidism with antithyroid drugs until treatment of GO is completed.