Low CAS “Reversible” Thyroid Eye Disease
Author: Shirin Hamed Azzam
Base Hospital / Institution: Tzafon Medical Center
ePoster presentation
Abstract ID: 25-186
Purpose
We aimed to characterize the clinical presentation, imaging findings, and treatment outcomes of “Reversible” thyroid eye disease (TED) phenotypes presenting with “white” eyes or clinical activity score (CAS).
Methods
We conducted a retrospective review of patients diagnosed with low CAS TED who were treated to modify their natural history, between January 2020- January 2024. Data collected included demographics, clinical and phenotype features, treatment response and the prognosis.
Results
30 TED patients were included, all with low CAS. Three broad phenotypes were identified: White apex (n=14), cicatricial active (n=13) and late muscle-predominant TED (n=3).
White apex phenotype is characterized by low CAS scores and development of optic neuropathy. These were further sub-classified into 3 further distinct sub-types. Patients showed poor response to immunosuppressive therapy but improved following apical orbital decompression.
Cicatricial active phenotype showed significant motility restriction or lid displacement presenting early. Imaging revealed predominantly single muscle enlargement with evidence of high signal with STIR-type sequence MRI. They were clinically resistant to steroid therapy and responded better to second line therapy.
Late muscle-predominant TED treated with Teprotumumab showed reduction in proptosis and muscle volume.
Conclusion
TED exhibits significant heterogeneity in both clinical presentation and response to treatment. There are different drivers for disease including inflammation, mechanical and immunomodulation. The white apex, cicatricial active and late muscle-predominant phenotypes may be misleading to clinicians, as they often present with low CAS despite “active” disease. These phenotypes demonstrate distinct clinical behavior and varying responses to medical and surgical therapies. Recognizing and differentiating these subtypes is essential to ensure individualized and effective management strategies for patients with TED, especially those conventionally deemed to be “inactive”.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Jimmy | Uddin | Moorfields Eye Hospital |
