Insights into Current Management Strategies for Dysthyroid Optic Neuropathy: A Review

Figures & data

Table 1 Clinical Activity Score (CAS)

Initial exam (max score of 7 points)•

Ocular or retrobulbar pain

Pain with eye movement

Eyelid erythema

Eyelid swelling

Conjunctival chemosis

Conjunctival erythema

Swelling/erythema of caruncle

Subsequent exam, 1–3 months later (max score of 10 points, combining parameters above and below)•

≥2-mm increase in proptosis

Impaired ductions in any one direction >8 degrees

≥1 line decrease in Snellen visual acuity chart

Notes: Presence of each sign receives 1 point. A sum of the scores greater than 3/7 at the first exam or greater than 4/10 in subsequent examinations defines active ophthalmopathy. Reprinted with permission from Mourits MP, Prummel MF, Wiersinga WM, Koornneef L. Clinical activity score as a guide in the management of patients with Graves’ ophthalmopathy. Clin Endocrinol (Oxf). 1997;47(1):9–14. Blackwell Science Ltd, Oxford.⁴¹

Table 2 Classification of the Severity of Graves’ Orbitopathy (GO)

Classification	Features
Mild GO	Patients whose features of GO have only a minor impact on daily life that have insufficient impact to justify immunomodulation or surgical treatment. They usually have one or more of the following: ● minor lid retraction (<2 mm) ● mild soft-tissue involvement ● exophthalmos ● <3 mm above normal for race and gender ● no or intermittent diplopia and corneal exposure responsive to lubricants
Moderate-to-severe GO	Patients without sight-threatening GO whose eye disease has sufficient impact on daily life to justify the risks of immunosuppression (if active) or surgical intervention (if inactive). They usually have two or more of the following: ● lid retraction ≥2 mm ● moderate or severe soft-tissue involvement ● exophthalmos ≥3 mm above normal for race and gender ● inconstant or constant diplopia
Sight-threatening (very severe) GO	Patients with dysthyroid optic neuropathy and/or corneal breakdown

Notes: Reprinted with permission from Bartalena L, Baldeschi L, Boboridis K, et al. The 2016 European Thyroid Association/European Group on Graves’ orbitopathy guidelines for the management of Graves’ orbitopathy. Eur Thyroid J. 2016;5(1):9–26.⁸

Figure 1 MRI findings of GO with DON (T2-weighted and fat-suppressed images using short-tau inversion recovery (STIR) sequences). Right: extraocular muscle swelling in both orbital cavity/. Left: extraocular muscle swelling compressing the optic nerve around the common tendinous ring.

Figure 2 Algorithm for the management of sight-threatening Graves’ orbitopathy including DON. The first-line treatment for optic neuropathy is high-dose IV methylprednisolone or steroid pulse therapy (single doses of 500 to 1000 mg) for three consecutive days, or preferably and for safety reasons, every second day (alternate days) during the first week, which can be repeated for a second week. When the response is absent or poor, with deterioration in visual acuity or CFF, urgent orbital decompression surgery will be needed. Data from Bartalena L, Kahaly GJ, Baldeschi L, et al. The 2021 European Group on Graves’ orbitopathy (EUGOGO) clinical practice guidelines for the medical management of Graves’ orbitopathy. Eur J Endocrinol. 2021;185(4):G43–G67.¹⁹

Figure 3 Schema of this review: Summary.

Mourits MP, Prummel MF, Wiersinga WM, Koornneef L. Clinical activity score as a guide in the management of patients with Graves’ ophthalmopathy. Clin Endocrinol (Oxf). 1997;47(1):9–14. doi:10.1046/j.1365-2265.1997.2331047.x

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Bartalena L, Baldeschi L, Boboridis K, et al. The 2016 European Thyroid Association/European Group on Graves’ orbitopathy guidelines for the management of Graves’ orbitopathy. Eur Thyroid J. 2016;5(1):9–26. doi:10.1159/000443828

 PubMed Web of Science ®Google Scholar

Bartalena L, Kahaly GJ, Baldeschi L, et al. The 2021 European Group on Graves’ orbitopathy (EUGOGO) clinical practice guidelines for the medical management of Graves’ orbitopathy. Eur J Endocrinol. 2021;185(4):G43–g67. doi:10.1530/EJE-21-0479

 PubMed Web of Science ®Google Scholar