Purpose:
To report a case of severe, blinding thyroid-associated ophthalmopathy,refractory to treatment with both high dose steroids and bilateral,three-wall surgical orbital decompressions, that responded dramaticallyto Rituximab, an anti-CD20 monoclonal antibody.
Methods:
Case report.
Results:
We admitted a 46-year-old woman, a smoker with oral steroidintolerance, with a twelve month history of Graves Disease,from the Emergency Department with severe orbital congestionand bilateral loss of vision from corneal exposure and compressiveoptic neuropathy (Figure 1). When treated with high-dose intravenousmethylprednisolone, the congestion resolved and her vision improved.We did bilateral three-wall orbital decompressions, but whenthe methylprednisolone dose was tapered, the disease returnedin full force. The dose was increased, again with good diseasecontrol. We gave her two intravenous infusions of Rituximab500 mg one week apart. Her methylprednisolone was slowly taperedagain, and on no treatment except methimazole, she has had norecurrence of her orbital inflammation over 6 months, with comfortable,normal-looking eyes and normal visual acuity (Figure 2). Shehas had no other systemic complications from treatment.
Conclusions:
This patient with fulminant, blinding Graves disease had a dramaticand gratifying resolution of her orbital congestion when treatedwith Rituximab. How this anti-B lymphocyte drug successfullytreated this organ-specific autoimmune disease is not clear,but it was a safe, amazingly effective therapy in this patientand shows great promise in treating such patients with refractoryGraves disease.
Keywords: orbit • eye movements • optic nerve