EP 85. Immunosuppression in Vogt-Koyanagi-Harada-syndrome

Abstract

Vogt–Koyanagi–Harada-syndrome is a rare autoimmune disease which affects organs containing large amounts of melanocytes (e.g. CNS, eyes, skin, inner ear). Relapsing uveitides and meningitides, sensorineural hearing loss and vitilligo/poliosis are typical symptomes. We describe the clinical course and difficulties of maintaining a long lasting and effective immunosuppression in Vogt–Koyanagi-syndrome. A 53-year-old female presented with relapsing uveitides and later on with visual loss and headaches. The neurological examination revealed neck stiffness and an organic brain syndrome containing severe content thought and formal thought disorders. Vitiligo was found in dermatological investigation. The cranial MRI showed a prominent contrast enhancement of the meninges. CSF-analysis revealed a pleocytosis of 200 cells/ μ l. Infectional serologies were negative. There was no serological evidence for collagenosis or malignant diseases. Due to the combination of uveitis, meningitis and vitiligo a Vogt–Koyanagi–Harada-syndrome was diagnosed. A four month lasting glucocorticoide therapy was without sufficient effect on uveitis and neuropsychological disorders. Periocular cortisone depots yielded a significant reduction of the visual loss. In the following months neither azathioprine nor methotrexate lead to decline of neuropsychological symptomes. Consequently we initiated cyclophosphamide therapy and gained complete regression of headaches and thought disorders. Unfortunately the patient was admitted with a further organic brain syndrome after six months under cyclophoshamide therapy. Due to limited therapeutical options we started intravenous immunoglobulin therapy which showed significant effectiveness. The following neurological examinations revealed a nearly complete regression of neuropsychological disorders. The case demonstrates that the rare Vogt–Koyanagi–Harada-syndrome should be considered as a differential diagnosis when the combination of uveitis, meningitis, neuropsychological disorders and vitiligo is present. Furthermore the clinical course described above revealed the difficulties of maintaining a stable and long lasting immunosuppression under glucocorticoide, azathioprine, methotrexate and cyclophophamide therapy. In our opinion after poor response of these substances an intravenous immunoglobuline therapy seems to be promising.