Management of Thyroid Eye Disease
Diagnosis and assessment
Key Point 3.1
Key Point 3.2
Key Point 3.3
Key Point 4.1.1
Key Point 4.1.2
Key Point 4.2.1
Key Point 4.4.1
Key Point 4.4.2
Activity and severity definitions for patients with thyroid eye disease
- Activity
- Clinical activity score
- The 7-item CAS is shown hereunder. Each item scores 1 point if presenta
- Spontaneous retrobulbar pain
- Pain on attempted up or lateral gaze
- Redness of the eyelids
- Redness of the conjunctiva
- Swelling of the eyelids
- Inflammation of the caruncle and/or plica
- Conjunctival edema, also known as chemosis
- The 7-item CAS is shown hereunder. Each item scores 1 point if presenta
- Active TED
- A CAS ≥ 3/7 usually implies active TED. A history or documentation of progression of TED based on subjective or objective worsening of vision, soft tissue inflammation, motility, or proptosis is suggestive of active TED independently of the CAS
- Clinical activity score
- Severity
- Sight-threatening TED
- Patients with DON and/or corneal breakdown and/or globe subluxation (Fig. 2F)
- Moderate-to-severe TED
- Patients without sight-threatening disease whose eye disease has sufficient impact on daily life to justify the risks of medical or surgical intervention. Patients with moderate-to-severe TED usually have any one or more of the following: lid retraction ≥2 mm, moderate or severe soft tissue involvement, proptosis ≥3 mm above normal for race and sex, or diplopia (Gorman score 2–3).
- Sight-threatening TED
- Mild TED
- Patients whose features of TED have only a minor impact on daily life insufficient to justify immunosuppressive or surgical treatment. They usually have only one or more of the following: minor lid retraction (<2 mm), mild soft tissue involvement, proptosis <3 mm above normal for race and sex, transient or no diplopia, and corneal exposure responsive to lubricants.
aA 10-item CAS is also sometimes used and includes additional points for increase of at least 2 mm in proptosis, decrease of at least 8° in any duction, and decrease of visual acuity by two lines. A limitation of the 10-item CAS is that it requires an earlier assessment of the mentioned measures, which is usually unavailable on first consultation. See Bartalena et al.
Characteristics of high-risk thyroid eye disease patients
- Background
- Male sex
- Age >50 years
- Tobacco smoker
- History
- Unstable thyroid function
- Diabetes mellitus
- Radioiodine in the past 6 months
- Progressive symptoms and/or signs of TED
- Orbital aching
- Diplopia
- Examination
- Marked soft tissue inflammatory features
- Lagophthalmos (Fig. 2A)
- Impaired ocular motility, particularly elevation
The features outlined are associated with an increased probability of developing sight-threatening TED
Formal ophthalmic examination for thyroid eye disease based on vision, inflammation, strabismus, appearance
Primary indications for imaging in suspected or confirmed thyroid eye disease
- Exclusion of other diseases in atypical TED
- Euthyroid, without history of thyroid dysfunction
- Clinically unilateral or markedly asymmetric
- Absent upper lid retraction
- Upper lid ptosis
- Atypical strabismus
- Severe orbital pain
- Assessment in confirmed TED
- Sight-threatening TED
- Planning of orbital and in some cases strabismus surgery
Initial care and referral for specialty care
Key Point 5.1.1
Key Point 5.3.1
Key Point 5.4.1
Key Point 6.1.1
Key Point 6.2.1
TED Management Algorithm
Link to External ImageTherapy of moderate–severe TED
Key Point 7.1.1
Key Point 7.1.2
Key Point 7.1.1.1
Key Point 7.1.1.2
Key Point 7.1.1.3
Key Point 7.1.1.4
Key Point 7.1.2.1
Key Point 7.1.3.1
Key Point 7.1.4.1
Key Point 7.1.4.2
Key Point 7.1.6.1
Key Point 7.2.1
Key Point 7.2.2
Key Point 7.3.1.1
Key Point 7.3.1.2
Key Point 7.3.2.1
Key Point 7.3.3.2
Key Point 7.3.4.1
Therapy of sight-threatening TED
Key Point 8.1.1
Key Point 8.2.1
Key Point 8.3.1
Recommendation Grading
Abbreviations
- CT: Computed Tomography
- DON: Dysthyroid Optic Neuropathy
- IVGC: Intravenous Glucocorticoid
- IVMP: Intravenous Methylprednisolone
- QOL: Quality Of Life
- RT: Radiotherapy
- RTX: Rituximab
- TCZ: Tocilizumab
- TED: Thyroid Eye Disease
- TEP: Teprotumumab
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Overview
Title
Management of Thyroid Eye Disease
Authoring Organizations
American Thyroid Association
European Thyroid Association
Publication Month/Year
December 13, 2022
Last Updated Month/Year
April 1, 2024
Document Type
Consensus
Country of Publication
US
Document Objectives
Thyroid eye disease (TED) remains challenging for clinicians to evaluate and manage. Novel therapies have recently emerged, and their specific roles are still being determined. Most patients with TED develop eye manifestations while being treated for hyperthyroidism and under the care of endocrinologists. Endocrinologists, therefore, have a key role in diagnosis, initial management, and selection of patients who require referral to specialist care. Given that the need for guidance to endocrinologists charged with meeting the needs of patients with TED transcends national borders, and to maximize an international exchange of knowledge and practices, the American Thyroid Association and European Thyroid Association joined forces to produce this Consensus Statement.
Target Patient Population
Nonpregnant adults (age ≥ 18 years) with TED
Target Provider Population
Endocrinologists, ophthalmologists, and other clinicians caring for adult patients with thyroid eye disease
Inclusion Criteria
Male, Female, Adult, Older adult
Health Care Settings
Ambulatory
Intended Users
Nurse, nurse practitioner, optometrist, physician, physician assistant
Scope
Diagnosis, Assessment and screening, Treatment, Management
Diseases/Conditions (MeSH)
D013959 - Thyroid Diseases, D006111 - Graves Disease, D049970 - Graves Ophthalmopathy
Keywords
Graves' disease, hyperthyroidism, thyroid eye disease, TED
Source Citation
Burch HB, Perros P, Bednarczuk T, Cooper DS, Dolman PJ, Leung AM, Mombaerts I, Salvi M, Stan MN. Management of thyroid eye disease: a Consensus Statement by the American Thyroid Association and the European Thyroid Association. Eur Thyroid J. 2022 Dec 8;11(6):e220189. doi: 10.1530/ETJ-22-0189. PMID: 36479875; PMCID: PMC9727317.