Nine patients with severe
Graves' ophthalmopathy were treated by intensive
plasma exchange, followed by immunosuppression. Severity of ocular involvement and response to
therapy were evaluated by numerical scoring (ophthalmopathy index) and clinically. Serum
thyroid stimulating immunoglobulins (TSI) and urinary excretion of
glycosaminoglycans (GAG) were measured immediately before and immediately after
plasmapheresis.
Plasma exchange was rapidly accompanied by marked clinical improvement in 8/9 patients. The most marked effects were on soft tissue involvement,
proptosis, intraocular pressure, and visual acuity. The ophthalmopathy index decreased from 9.7 +/- 4.1 to 5.7 +/- 2.2 (p less than 0.001) after
plasmapheresis. Serum TSI levels were initially elevated in 6 patients and remained positive in 3 patients
after treatment. Urinary GAG excretion was initially 2- to 12-fold normal levels and was decreased by 60%. After
plasmapheresis, patients received immunosuppressive drugs for 3-6 months. The follow-up period, after withdrawal of drugs, ranged from 5 to 38 months with a median of 17 months. The ocular condition remained stable in 6 patients. Three patients had a relapse 1 year after
plasmapheresis: they were treated a second time by
plasma exchange with subsequent improvement. In conclusion, intensive
plasma exchange provided prompt and effective improvement in patients with severe progressive
Graves' ophthalmopathy. This therapeutic procedure, followed by immunosuppression, gave long lasting results. Relapses were responsive to
plasmapheresis therapy. The data suggest that
plasma exchange may represent the best primary treatment for severe progressive
Graves' ophthalmopathy.