CLINICAL ACTIVITY SCORE AS A GUIDE IN THE MANAGEMENT OF PATIENTS WITHGRAVES OPHTHALMOPATHY

OBJECTIVE Approximately 35% of patients with Graves' ophthalmopathy do not respond to immunosuppressive treatment, A possible explanation fo r this finding is that only patients with active ophthalmopathy respon d to immunosuppressive treatment, whereas patients with fibrotic end s tage disease do not, To distinguish between these two groups and to pr edict the outcome of immunosuppressive treatment, we developed a clini cal activity score (GAS) based on four of the five classical signs of inflammation and tested its efficacy in a double-blind, prospective st udy. DESIGN, PATIENTS AND MEASUREMENTS The CAS was determined by an op hthalmologist before, on the day of, and after the start of either ora l prednisone or retrobulbar irradiation in 43 patients with moderate t o severe Graves' ophthalmopathy. The therapeutic outcome was determine d by a second ophthalmologist unaware of the CAS stores given, Success of treatment was defined as an improvement in NOSPECS class or grade. RESULTS Responders (22) and non-responders (21) did not differ in age , sex, duration or severity of their Graves' ophthalmopathy, The pretr eatment GAS, however, was significantly higher in responders than in n on-responders, Twelve of 22 responders and three of 21 non-responders had a CAS greater than or equal to 4 (80% vs 36%; P < 0.01), Using thi s CAS cut-off point, the accuracy of CAS in predicting the therapeutic outcome was: specificity 86%, sensitivity 55%, positive predictive va lue 80%, negative predictive value 64%, Patients with a CAS greater th an or equal to 4 had a similar duration of Graves' ophthalmopathy as p atients with a CAS < 4. CONCLUSIONS The clinical activity score has a high predictive value for the outcome of immunosuppressive treatment i n Graves' ophthalmopathy. Disease activity, and not disease duration, is the prime determinant of therapeutic outcome.