Teprotumumab for the Treatment of Active Thyroid Eye Disease

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Abstract

<p class="first" id="d5212336e231">Thyroid eye disease is a debilitating, disfiguring, and potentially blinding periocular condition for which no Food and Drug Administration-approved medical therapy is available. Strong evidence has implicated the insulin-like growth factor I receptor (IGF-IR) in the pathogenesis of this disease. </p>

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Most cited references 23

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Clinical activity score as a guide in the management of patients with Graves' ophthalmopathy.

L Koornneef, Wilmar M. Wiersinga, M Prummel … (1997)

Approximately 35% of patients with Graves' ophthalmopathy do not respond to immunosuppressive treatment. A possible explanation for this finding is that only patients with active ophthalmopathy respond to immunosuppressive treatment, whereas patients with fibrotic end stage disease do not. To distinguish between these two groups and to predict the outcome of immunosuppressive treatment, we developed a clinical activity score (CAS) based on four of the five classical signs of inflammation and tested its efficacy in a double-blind, prospective study. The CAS was determined by an opthalmologist before, on the day of, and after the start of either oral prednisone or retrobulbar irradiation in 43 patients with moderate to severe Graves' ophthalmopathy. The therapeutic outcome was determined by a second ophthalmologist unaware of the CAS stores given. Success of treatment was defined as an improvement in NOSPECS class or grade. Responders (22) and non-responders (21) did not differ in age, sex, duration or severity of their Graves' ophthalmopathy. The pretreatment CAS, however, was significantly higher in responders than in non-responders. Twelve of 22 responders and three of 21 non-responders had a CAS > or = 4 (55% vs 14%; P or = 4 had a similar duration of Graves' ophthalmopathy as patients with a CAS < 4. The clinical activity score has a high predictive value for the outcome of immunosuppressive treatment in Graves' ophthalmopathy. Disease activity, and not disease duration, is the prime determinant of therapeutic outcome.

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Clinical review: Intravenous glucocorticoids for Graves' orbitopathy: efficacy and morbidity.

S. Zang, K A Ponto, G J Kahaly (2011)

The administration of iv glucocorticoid pulses has been advocated as a treatment approach for patients with inflammatory and moderate to severe Graves' orbitopathy (GO). This review offers an update on this controversial regimen.

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Efficacy of B-cell targeted therapy with rituximab in patients with active moderate to severe Graves' orbitopathy: a randomized controlled study.

Mario Salvi, Guia Vannucchi, Nicola Currò … (2015)

Preliminary studies have shown that rituximab (RTX) is effective in the treatment of active Graves' orbitopathy (GO).

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Author and article information

Journal

Title: New England Journal of Medicine

Abbreviated Title: N Engl J Med

Publisher: Massachusetts Medical Society

ISSN (Print): 0028-4793

ISSN (Electronic): 1533-4406

Publication date Created: January 23 2020

Publication date (Print): January 23 2020

Volume: 382

Issue: 4

Pages: 341-352

Affiliations

[1 ]From Cedars–Sinai Medical Center, Los Angeles (R.S.D., A.P.); Johannes Gutenberg University Medical Center, Mainz (G.J.K.), and University Hospital Essen, Essen (A.E.) — both in Germany; Horizon Therapeutics, Lake Forest, IL (S.S., E.H.Z.T., R.P., J.W.S., T.V., R.J.H.); University of Tennessee Health Science Center, Memphis (J.C.F., B.T.F.); University of Pisa, Pisa (C.M., M.M., A.A.), and Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan (M.S.) — both in Italy; Oregon Health and Sciences...