title: Ocular Whipple's disease: therapeutic strategy and long-term follow-up.
abstract: OBJECTIVE
  To characterize the clinical features of ocular Whipple's disease (WD) and determine the long-term prognosis after antibiotic treatment.
  
  
  METHODS
  Retrospective case series.
  
  
  METHODS
  Medical records of patients referred between January 1993 and December 2010 were reviewed for chronic corticosteroid-resistant uveitis or neuro-ophthalmologic findings consistent with WD. Eleven patients (male/female = 9/2) were included in this study.
  
  
  METHODS
  Diagnosis was based on cytologic examination and molecular analysis of samples (cerebrospinal fluid, vitreous, duodenum, or any involved lymph node). It was based on cytology before the routine use of polymerase chain reaction (PCR) and on both cytology and molecular biology for more recent patients. Long-term antibiotic therapy included oral trimethoprim-sulfamethoxazole (TMP-SMX) and rifampin, TMP-SMX alone, rifampin alone, or tetracycline alone.
  
  
  METHODS
  (1) Demographic and clinical characteristics of patients with positive PCR for Tropheryma whipplei or periodic acid-Schiff-positive macrophages in the vitreous and (2) long-term prognosis after antibiotic treatment.
  
  
  RESULTS
  Mean age at diagnosis was 63 years (range, 51-73 years). Average time between the onset of the disease and diagnosis was 2 years (range, 1 month to 11 years). Mean follow-up was 7.2 years (range, 0.25-18 years). Ophthalmologic findings consisted of chronic uveitis (9 patients), isolated bilateral optic disc swelling (1 patient), and Parinaud syndrome (1 patient). All patients had PAS-positive macrophages, and 6 patients had a positive PCR for T. whipplei. Nine patients were treated with TMP-SMX and rifampin. One patient treated with only tetracycline relapsed and was successfully treated with TMP-SMX. No major side effects were reported. Intraocular inflammation and neurologic manifestations were controlled in all cases. At the end of follow-up, 2 patients were off treatment, 2 patients had a neurologic relapse after treatment interruption, and 5 patients were still taking TMP-SMX. One patient was taking only rifampin. Two patients were lost to follow-up.
  
  
  CONCLUSIONS
  Ocular WD seems to be a neurologic manifestation of WD. Trimethoprim-SMX with rifampin is an efficient treatment, and prolonging treatment for at least 1 year is recommended. Long-term low-dose antibiotic therapy may reduce the rate of relapse, neurologic involvement, and death.
authors.affiliation: Department of Ophthalmology, Pitié-Salpêtrière Hospital, Paris, France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Touitou
authors.forename: Florence
authors.initials: F
authors.lastname: Fenollar
authors.forename: Nathalie
authors.initials: N
authors.lastname: Cassoux
authors.forename: Helene
authors.initials: H
authors.lastname: Merle-Beral
authors.forename: Phuc
authors.initials: P
authors.lastname: LeHoang
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Michel
authors.initials: M
authors.lastname: Drancourt
authors.forename: Bahram
authors.initials: B
authors.lastname: Bodaghi
mesh_terms: D000368:Aged; D000890:Anti-Infective Agents / Q000627:therapeutic use; D002555:Cerebrospinal Fluid / Q000382:microbiology; D004351:Drug Resistance; D004359:Drug Therapy, Combination; D004791:Enzyme Inhibitors / Q000627:therapeutic use; D015818:Eye Infections, Bacterial / Q000175:diagnosis* / Q000188:drug therapy* / Q000382:microbiology; D005260:Female; D005451:Fluorescein Angiography; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008198:Lymph Nodes / Q000382:microbiology; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D011379:Prognosis; D012329:RNA, Bacterial / Q000235:genetics; D012336:RNA, Ribosomal, 16S / Q000235:genetics; D012189:Retrospective Studies; D012293:Rifampin / Q000627:therapeutic use; D013752:Tetracycline / Q000627:therapeutic use; D015662:Trimethoprim, Sulfamethoxazole Drug Combination / Q000627:therapeutic use; D054851:Tropheryma / Q000235:genetics / Q000302:isolation & purification*; D014605:Uveitis / Q000188:drug therapy; D014822:Vitreous Body / Q000382:microbiology; D008061:Whipple Disease / Q000175:diagnosis* / Q000188:drug therapy* / Q000382:microbiology
chemical_list: D000890:Anti-Infective Agents; D004791:Enzyme Inhibitors; D005938:Glucocorticoids; D012329:RNA, Bacterial; D012336:RNA, Ribosomal, 16S; D015662:Trimethoprim, Sulfamethoxazole Drug Combination; D013752:Tetracycline; D012293:Rifampin