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Graves' Orbitopathy


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      The disadvantages of the CAS relate to 2 aspects. Firstly, all features are given equal weighting, and it is not clear whether this is appropriate. Secondly, it is a poor tool for monitoring change as it employs a binary score, whereby improvement of any feature does not alter the score unless it completely resolves [4].

      A small minority of patients appear to show no signs of active disease but subsequently either change spontaneously or respond to immunomodulation. Identifying such patients is of course difficult; however, they may have orbital pain or gaze-related pain suggesting active disease, and/or describe worsening of severity features usually implying active disease.

      In this situation management will depend on the presenting features and their severity. Unless there is clear evidence to the contrary, all sight-threatening features should be assumed to be of recent onset, implying active disease and a need for urgent intervention. At the other end of the spectrum, patients who present with only eyelid retraction and mild exophthalmos do not require any urgent intervention and can safely be sequentially assessed until their disease phase is apparent and any necessary therapy then offered.

ClassGrade
0No physical signs or symptoms
IOnly signs
IISoft-tissue involvement
aAbsent
bMinimal
cModerate*
dMarked*
IIIExophthalmos (Proptosis)*
aAbsent
bMinimal
cModerate
dMarked
IVExtraocular muscle involvement*
aAbsent
bLimitation of motion in extremes of gaze
cEvident restriction of motion
dFixation of a globe or globes
VCorneal involvement
aAbsent
bStippling of the cornea
cUlceration
dClouding, necrosis, perforation
VISight loss (due to optic nerve compression)*
aAbsent
bVisual acuity 0.63 – 0.5
cVisual acuity 0.4 – 0.1
dVisual acuity <0.1 to no light perception
Note that grades within class II, class III, and class IV are largely undefined. Severity should be scored by the method given in the section “How Reproducible Are These Assessments?”. The severity signs marked with an asterisk are also used to assess activity, namely class IIc and IId, or a defined deterioration in class III, IV, or VI.

      At present we do not know the value of assessing all patients using these additional methods as no data relate to patients with all grades of severity. Of course all patients in routine clinical practice can have disease duration and soft-tissue evaluation without any additional cost or facilities. At present the place for the other methods described will depend partly on their availability and cost, and partly on the presenting features of an individual patient. There is no proven necessity for additional