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212 |
16 Modern Treatment Concepts in Graves Disease |
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Table 16.3. Modified NOSPECS score for quantification of severity, maximal score of 14 |
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NOSPECS score |
0 |
1 |
2 |
3 |
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Lid retraction |
|
No |
Yes |
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16 |
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Soft tissue inflammationa |
0 |
1–4 |
5–8 |
>8 |
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Proptosis and or Site Di erence |
<17 mm |
17–18 mm |
19–22 mm |
>22 mm |
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<1 mm |
1–2 mm |
3–4 mm |
>4 mm |
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Extraocular muscle involvement |
No |
|
>20° upgaze |
≤20° upgaze |
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>35°abduction but not |
≤35°abduction |
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normal |
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Corneal defects |
No |
Yes |
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Optic nerve compression |
No |
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Yes |
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aUpper lid edema 0–2; Lower lid edema 0–2; conjunctival injection 1; conjunctival chemosis 1 |
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3.Mild GO: patients whose features of GO have only a minor impact on daily life, insu cient 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, exophthalmos <3 mm above normal for “race” and gender, transient or no diplopia, and corneal exposure responsive to lubricants.
Treatment decision can be made with the help of a detailed management plan (see Fig. 16.2)
the active phase and rehabilitative surgical treatments in the inactive phase of the disease.
■According to its grade,GO can be classified as mild, moderate to severe, and sight threatening. Mild GO permits a “wait and see” approach, moderate- to-severe GO requires immunosuppressive treatment in the active phase, and sight-threatening GO demands immediate treatment with i.v. steroids/ orbital decompression/treatment of ocular surface damage.
Summary for the clinician
■Graves’ Orbitopathy is part of an autoimmune systemic disease encompassing hyperthyroidism, orbitopathy, dermatopathy, and acropachy.
■TSHR receptor antibodies (TRAb) are indicators of the failed immune system and direct pathomechanism for hyperthyroidism. Their role in the pathogenesis of orbitopathy is less clear, though patients with high serum TRAb levels have a higher prevalence of GO and develop more severe disease stages. Orbital fibroblasts play a pivotal role in the pathologic changes in the orbit (release of chemokines,production of glycoseaminoglycanes/fibrosis, and di erentiation into adipose tissue).
■Assessment of activity (clinical activity score) and severity is necessary for disease management: immunomodulation is performed during
16.2Natural History
Control of thyroid function influences the course of GO (see Chap. 4). Patient with mild-to-moderate GO, monitored over 1 year without treatment, improved in 22%, showed minor improvement or no change in 42 and 22%, respectively, and deteriorated in 14% [22]. With or without treatment, there are often residual symptoms of GO in the form of lid retraction, proptosis, and muscle dysfunction. The outcome is significantly better in patients who have been diagnosed early and treatment started promptly.
Summary for the Clinician
■Spontaneous improvement of GO with restoration of euthyroidism occurs in more than 60% of the patients.