Diagnostic and prognostic value of cerebrospinal fluid analysis in Vogt-Koyanagi-Harada syndrome

Abstract

Purpose: To evaluate the diagnostic and prognostic values of lumbar puncture in Vogt-Koyanagi-Harada (VKH) syndrome.

Methods: Medical records of patients seen in a single tertiary center between 1995 and 2013, with confirmed VKH syndrome according to the Revised Diagnostic Criterias, were retrospectively reviewed. Clinical stage of the disease, cerebrospinal fluid (CSF) analysis, therapeutic strategy, and prognosis were compared.

Results: Medical records of 68 patients with a confirmed diagnosis of VKH disease were reviewed. Among them, 29 patients (42%) had a lumbar puncture (LP). 21 LP (72.4%) were performed at the early stage of the disease, and 11 LP (37.9%) were performed at the chronic stage of the disease. LP was abnormal in 66.7% of cases during the acute phase and in 18% of cases during the chronic phase. Pleiocytosis was present in 16 patients (55%). An average of 62.3 lymphocytes /mm3 was observed when LP was performed during the acute phase and 9.5 lymphocytes / mm3 when performed during the chronic phase ( p = 0,03) of the disease. In two cases, the patient met the diagnostic criteria because of the LP findings. Review of CSF analysis performed during the acute phase of the disease revaled that the patients ultimately dependant on low doses of steroids (<5mg/d) had more lymphocytes (93,8 lymphocytes\mm3) into the CSF than the patients ultimately dependant on high doses of steroids (>5mg/d) ( 29,3 lymphocytes\mm3) (p=0.03). These patients were also more likely to require immunosupressive drugs, in adjubction to corticosteroids.

Conclusions: The number of lymphocytes in the CSF at the diagnostic phase seems to be one of the prognostic factors for the outcome of VKH. Patients with a low pleiocytosis in the initial lumbar puncture should be closely monitored and may require a more aggressive treatment. Those conclusions must be confirmed with a prospective study.

Keywords: 557 inflammation • 462 clinical (human) or epidemiologic studies: outcomes/complications • 432 autoimmune disease