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Graves Orbitopathy — Teprotumumab Therapy

IGF-1 receptor inhibitor monoclonal antibody approved for active moderate-to-severe thyroid eye disease that reduces proptosis, diplopia, and inflammation by blocking the IGF-1R/TSH-R cross-signaling axis on orbital fibroblasts.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Endokrinoloji department. Book Appointment →

What is Graves Orbitopathy — Teprotumumab Therapy?

Graves orbitopathy (thyroid eye disease, TED) is the orbital manifestation of Graves disease driven by autoantibodies activating TSH receptor and IGF-1 receptor on orbital fibroblasts, causing adipogenesis, hyaluronan deposition, and extraocular muscle enlargement.

Teprotumumab is a fully human IgG1 monoclonal antibody that selectively blocks IGF-1 receptor and indirectly attenuates TSH-R signaling, decreasing fibroblast activation, hyaluronan production, and orbital tissue expansion.

Phase 2 (HZNP-001) and phase 3 (OPTIC) trials showed proptosis response (≥2 mm reduction) in 71–83% versus 9–10% placebo, with sustained benefit for many patients up to 51 weeks; dosing is 10 mg/kg first infusion then 20 mg/kg every 3 weeks for 8 total infusions.

Symptoms

Proptosis (eye protrusion) measured by Hertel exophthalmometer with progressive worsening
Diplopia in primary or eccentric gaze due to extraocular muscle restriction (commonly inferior and medial rectus)
Eyelid retraction, lid lag, conjunctival injection, chemosis, periorbital edema
Pain or pressure behind the eye, exposure keratopathy from incomplete lid closure
Decreased visual acuity, color vision loss, optic neuropathy in severe sight-threatening disease
Active inflammatory phase (Clinical Activity Score ≥3) appropriate for teprotumumab; burnt-out chronic disease less responsive

Risk Factors

Active Graves disease, especially with high TSH receptor antibody titers
Smoking — strong dose-dependent risk factor for severity and recurrence
Female sex (more common) but male and elderly with worse prognosis
Radioactive iodine therapy without prophylactic glucocorticoids
Selenium deficiency, uncontrolled hyperthyroidism or hypothyroidism after treatment
Family history of autoimmune thyroid disease

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Active TED with proptosis ≥3 mm above normal, diplopia, or CAS ≥3 — orbital specialist for teprotumumab eligibility
  • Optic neuropathy signs (decreased acuity, dyschromatopsia, RAPD) — emergency referral for IV pulse steroids and decompression
  • Diabetes mellitus or pre-existing inflammatory bowel disease — pre-treatment optimization due to teprotumumab risks
  • Hearing change, tinnitus, or muffled hearing during teprotumumab — hold infusion and audiology evaluation
  • Pregnancy or planning pregnancy — teprotumumab contraindicated, contraception during and 6 months after

Treatment Methods

01
Teprotumumab infusion 10 mg/kg first dose then 20 mg/kg every 3 weeks for 8 doses; pre-medicate per protocol; observe for infusion reaction
02
Concurrent smoking cessation and stable euthyroidism (with antithyroid drug or levothyroxine) before and during therapy
03
Selenium 200 mcg daily for 6 months in mild active disease, often combined or sequential to teprotumumab
04
Monitor for hyperglycemia (capillary glucose pre-infusion 1–2, then before each infusion), hearing changes (audiometry baseline, mid, end), inflammatory bowel disease flare, infusion reactions, and amenorrhea
05
Adjuvant or alternative options: IV methylprednisolone (12 weekly doses), orbital decompression for sight-threatening disease, mycophenolate, rituximab, tocilizumab; eyelid and strabismus surgery in inactive phase

Which Department to Visit?

You can visit our Endokrinoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.