Activity and Severity of TED

The majority of Graves' patients have a mild and nonprogressive ocular involvement that does not require any specific or aggressive treatment, also because non-severe GO often tends to improve spontaneously. When evaluating a patient with TED, two basic questions have to be addressed. First, does the patient needs treatment for TED and, in a positive answer, which kind of treatment is indicated.

The decision of whether ophthalmopathy must be treated should rely on the assessment of two different parameters, the activity and severity of the disease. The activity of the disease is neither synonymous nor coincident with the severity of the disease. In other words, an individual patient may have severe ocular manifestations but the disease may be inactive (fig. 1). To assess the activity of ophthalmopathy, Mourits et al. [22] proposed a clinical activity score (CAS), which in its original formulation included 10 different items (table 1) mainly, but not solely, reflecting inflammatory changes: giving one point to each manifestation, a score is obtained, with a range from 0 (no activity) to 10 (highest activity). A slightly modified CAS which does not include some of the items originally proposed by Mourits et al. [22] was proposed by an ad hoc committee of the four thyroid societies as a tool to record ocular changes over time after treatment of ophthalmopathy [23] (table 1). Definition of severity of GO is somehow arbitrary (table 2). Undoubtedly, optic neuropathy which can be subclinical and heralded only by changes in the visual evoked potentials, depicts per se a situation that can be sight threatening, especially if it is associated

Fig. 1. Hypothetical relationship between disease activity and severity in the natural history of TED [60].

Table 1. Clinical activity score

Original formulation [22]

Revised formulation [23]

Painful, oppressive feeling on or behind the globe

Pain on attempted up, side, or down gaze

Redness of the eyelids

Diffuse redness of the conjuctiva

Chemosis

Swollen caruncle

Edema of the eyelids

Increase of 2 mm or more in proptosis in the last 1-3 months Decrease in visual acuity in the last 1-3 months

Decrease in eye movements of 5 degrees or more in the last 1-3 months spontaneous retrobulbar pain pain on eye movements eyelid erythema conjuctival injection chemosis swelling of the caruncle eyelid edema or fullness with an evident reduction of visual acuity. It has to be remembered that immunosuppression treatment is effective only in patients with active disease.

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