Abstract
Purpose: :
To determine the best long-term therapeutic strategy for the treatment of ocular Whipple disease (WD).
Methods: :
Medical records of patients with a diagnosis of WD were retrospectively reviewed. Included patients were diagnosed between 1993 and 2003 and followed until 2010. Diagnosis was based on cytology and molecular analysis (16S rRNA PCR on CSF, vitreous, duodenal or lymph node specimens). Treatment regimens included either oral trimetoprim sulfametoxazol (TMP-SMX) associated with rifampin, TMP-SMX alone, rifampin alone or tetracyclin alone. Treatment efficacy and tolerance were evaluated biologically and clinically.
Results: :
Eleven patients, (M/F:7/2) were included. Mean age at diagnosis was 63 years (range 51 - 73 y). Mean follow-up was 5 years (range: 6 months-13 years). All patients had PAS- positive macrophages, and 6 patients (54.5%) had a positive PCR for Tropheryma whipplei. Eight patients (72.7%) were treated with TMP-SMX, one patient (9.1%) received rifampin alone, one patient (9.1%) received TMP-SMX alone, and 1 patient received tetracycline alone. Patient treated with tetracycline alone relapsed and was successfully treated with TMP-SMX. Three patients were lost of follow-up (27.3%). Five patients (45.4%) developed minor side effects (cholestasis or GI distress). No major side effects (agranulocytosis, Stevens Johnson) were observed. Intraocular inflammation was controlled in all patients. One patient with Parinaud syndrome remained stable. No relapses were observed on TMP-SFM alone, TMP-SFM+Rifampin or Rifampin alone. At the end of the follow-up period, one patient was off treatment, and seven patients were still on TMP-SFM.
Conclusions: :
Antibiotherapy in WD must include antibiotics that cross the cell membrane and the blood-brain barrier. Tetracyclin is not an efficient treatment for ocular WD. TMP-SMX associated with rifampin seems to be an efficient treatment for the management of ocular WD. Antibiotic treatment must be prolonged for a minimum of 1 year but in most patients, we found that long-term low-dose antibiotherapy over a year was mandatory to prevent relapses with neurological involvement and death.
Keywords: uveitis-clinical/animal model • bacterial disease • inflammation