Table 2.
Summary of classification systems for clinical activity and severity of thyroid eye disease.
| Grade | Criteria |
|---|---|
| NOSPECS a system | |
| 0 | No physical signs or symptoms |
| I | Only signs, no symptoms |
| II | Soft tissue involvement |
| II-0 | Absent |
| II-1 | Minimal |
| II-2 | Moderate |
| II-3 | Marked |
| III | Proptosis (>2 mm of normal upper limit) |
| III-1 | 3–4 mm |
| III-2 | 5–7 mm |
| III-3 | >8 mm |
| IV | Extraocular muscle involvement |
| IV-0 | Absent |
| IV-1 | Limitation of motion at extremes of gaze |
| IV-2 | Evident restriction of movements |
| IV-3 | Fixed globe(s) |
| V | Corneal involvement due to lagophthalmos |
| V-0 | Absent |
| V-1 | Stippling of cornea |
| V-2 | Ulceration |
| V-3 | Clouding, necrosis, and perforation |
| VI | Sight loss due to optic nerve involvement |
| VI-0 | Absent |
| VI-1 | Disc pallor or visual field defect Visual acuity 20/20 to 20/60 |
| VI-2 | Disc pallor or visual field defect Visual acuity 20/70 to 20/200 |
| VI-3 | Blindness Visual acuity less than 20/200 |
| Mild disease encompasses grades 1 to 3, whereas severe disease encompasses grades 4 to 6. | |
| RELIEF a system | |
| R | Resistance to retropulsion |
| E | Edema of conjunctiva and caruncle |
| L | Lacrimal gland enlargement |
| I | Edema of eyelids |
| F | Fullness of eyelids |
| Clinical activity score | |
| Initial evaluation scored out of 7 | |
| Spontaneous orbital pain | |
| Gaze-evoked orbital pain | |
| Lid edema | |
| Lid erythema | |
| Conjunctival erythema | |
| Chemosis | |
| Caruncle or plica inflammation | |
| Successive evaluations scored out of 10 | |
| Increase of >2 mm proptosis | |
| >8° decrease in ocular motility of one eye in any direction | |
| Decrease in visual acuity of 1 Snellen line | |
| A clinical assessment score (CAS) greater than 3 out of 7 or 4 out of 10 is considered to be an active disease. Each positive element is given one point. | |
| European Group of Graves’ Orbitopathy severity grading system | |
| Soft tissue assessment | |
| Eyelid swelling | |
| 1 | Absent |
| 2 | Mild: none of the features defining moderate or severe categories |
| 3 | Moderate: definite swelling, no lower eyelid festoons or angulation of the upper eyelid skin fold in downgaze |
| 4 | Severe: lower eyelid festoons or upper eyelid fold becomes rounded at 45° in downgaze |
| Eyelid erythema | |
| 1 | Absent |
| 2 | Present |
| Conjunctival erythema | |
| 1 | Absent |
| 2 | Mild: equivocal or minimal |
| 3 | Moderate: <50% |
| 4 | Severe: >50% |
| Conjunctival edema | |
| 1 | Absent |
| 2 | Present |
| Caruncle or plica semilunaris inflammation | |
| 1 | Absent |
| 2 | Present |
| Document | |
| Lid margin assessment at the mid-pupillary line | |
| Palpebral aperture (mm) | |
| Upper and lower lid retraction (mm) | |
| Levator function (mm) | |
| Lagophthalmos | |
| 1 | Absent |
| 2 | Present |
| Bell’s phenomenon | |
| 1 | Absent |
| 2 | Present |
| Proptosis assessment | |
| Hertel exophthalmometry to record intercanthal distance and amount of proptosis | |
| Ocular motility assessment | |
| Prism cover test | |
| Monocular ductions | |
| Head posture | |
| Torsion | |
| Binocular single vision | |
| Corneal integrity assessment | |
| Normal | |
| Punctate keratopathy | |
| Ulcer | |
| Perforation | |
| Optic nerve assessment | |
| Visual acuity | |
| Color vision | |
| Visual field analysis | |
| Optic disc assessment: normal, atrophied, glaucomatous, or edematous | |
| Afferent pupil defect | |
| 1 | Present |
| 2 | Absent |
| Mild disease encompasses 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 treatments; moderate-to-severe disease encompasses 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, inconsistent or consistent diplopia; sight-threatening disease encompasses patients with dysthyroid optic neuropathy and/or corneal breakdown. | |
| VISA a system | |
| 1 point | Vision |
| Visual acuity | |
| Pupillary reflex | |
| Color vision | |
| Visual fields | |
| Optic nerve examination | |
| Visual evoked potentials | |
| 10 points | Inflammation/congestion |
| Caruncular edema | |
| 0 | Absent |
| 1 | Present |
| Chemosis | |
| 0 | Absent |
| 1 | Conjunctiva lies behind the gray line of the lid |
| 2 | Conjunctiva extends anterior to the gray line of the lid |
| Conjunctival erythema | |
| 0 | Absent |
| 1 | Present |
| Lid redness | |
| 0 | Absent |
| 1 | Present |
| Lid edema | |
| 0 | Absent |
| 1 | Present, without redundant tissues |
| 2 | Present, with lower lid festoon and bulging palpebral skin |
| Retrobulbar ache at rest | |
| 0 | Absent |
| 1 | Present |
| Retrobulbar ache with gaze | |
| 0 | Absent |
| 1 | Present |
| Diurnal variation | |
| 0 | Absent |
| 1 | Present |
| 6 points | Strabismus/motility restriction |
| Diplopia | |
| 0 | Absent |
| 1 | Diplopia with horizontal or vertical gaze |
| 2 | Intermittent diplopia in primary gaze |
| 3 | Constant diplopia in primary gaze |
| Ocular restriction | |
| 0 | Duction > 45° |
| 1 | Duction 30–45° |
| 2 | Duction 15–30° |
| 3 | Duction < 15° |
| 3 points | Appearance/exposure |
| Appearance concerns (i.e., proptosis, lid retraction, and fat pockets) | |
| Symptoms from ocular exposure (i.e., foreign body sensation, photophobia, dryness, and secondary tearing) | |
| Measurements including eyelid retraction, scleral show, LPS function, lagophthalmos, and proptosis with Hertel exophthalmometer | |
| Signs of corneal exposure, including punctate epithelial erosions, ulcerations, corneal thinning, and corneal perforation | |
| The VISA classification system tool is useful for assessing disease progression or response to therapy, as well as disease activity or quiescence. | |
a Abbreviations: NOSPECS, No signs or symptoms, Only signs, no symptoms, Soft tissue involvement, Proptosis, Extraocular muscle involvement, Corneal involvement, Sight loss; RELIEF, Resistance to retropulsion, Edema of the conjunctiva and caruncle, Lacrimal gland enlargement, Injection of horizontal rectus muscle insertions, Edema of the eyelids, and Fullness of the eyelids; VISA, Vision, Inflammation, Strabismus, and Appearance.