1. Whipple´s Disease Manfred Zierhut Centre of Ophthalmology
Sebastian Thaler
Manfred Zierhut
Centre of Ophthalmology
University of Tuebingen, Germany

2. First Presentation – Ocular History November 2006
56 year old white German man
OU: persisting vitreous inflammation since 2 months
Complains: reddening, foreign body sensations, pressure feeling
treatment Prednisolone (10 mg)
topical corticosteroids 5x/day

3. First Presentation – Ocular History November 2006
„intermediate uveitis“ since 2/2003
diagnostic ppV
OD (2x) (12/03 and 4/04) no malignancy
OS (1x) (3/05)
last recurrence 2/06
improvement after 50 mg of prednisolone

4. First Presentation – Ocular History November 2006
last recurrences (2 and 6/06)
good response to systemic corticosteroids
recurrences after reduction

5. First Presentation – General History November 2006
arthritis – non-steroidal antiphlogistics
no other complains

6. First Presentation – Ocular Examination November 2006
VA: 0.1/0.5
IOP: 20/ 18 mmHg
OD: AC-cells 3+, snowflake-like particles in the AC, irishyperemia, pseudophacos, fundus without details
OS: AC cells 1+, cataract, fundus without signs of inflammation

7. First Presentation – Anterior SegmentOD OS

8. First Presentation – Anterior Segment OD
Thaler et al. Int. J. of Infectious Diseases 2010

9. First Presentation – Previous Investigations
CT-brain: unremarkable
chest X-ray: unremarkable
lab: ESR 30/60, Ig-A1 und A2 upper limit. TSH mildly elevated
serology: Lyme´s disease, syphilis, bartonella: all negativ
neurologically no signs of inflammation

10. First presentation – Diagnostic ppV
no detection of bacteria, no fungus
vitreal histology
purulent unspecific inflammation
no typical cells, no signs of malignancy
molecular biology: no signs of lymphoma

11. First Diagnosis suggestive for low grade endophthalmitis
removal of IOL
in addition intravitreal antibiotics

12. Follow Up – After 1 Month Re-ppV
no direct detection of bacteria or fungus
PCR: Tropheryma whipplei positiv .
Thaler et al. Int. J. of Infectious Diseases 2010

13. Final Diagnosis
Uveitis due to Whipple´s Disease
based on
clinical findings
positive PCR from the vitreous

14. Diagnostics – After 2 Months January 2007
biopsy of the small intestine
detection of Tropheryma whipplei
blood
spinal puncture
no detection of Tropheryma whipplei
endoscopy:
antrum of the stomach with spotted mucosal atrophy

15. Treatment Begin January 2007
intravitreal Ceftriaxon for 2 weeks
followed by
oral Trimethoprim with Sulfamethoxazol for 1 year
planned: secondary lens implantation

16. Final Follow Up – After 5 Months March 2007
VA OD: 0.2, OS: 0.67
no signs of intraocular inflammation
regular controls necessary because
recurrences 11 years after stop of antibiotic treatment reported

17. Final Follow Up – After 6 Months April 2007
re-biopsy from the small intestine:
PCR negativ

18. Whipple´s Disease – Clinical Symptoms Intestinal
diarrhea
abdominal pain
malabsoption, leading to anemia, hypoproteinemia and hypovitaminosis
weight loss

19. Whipple´s Disease – Clinical Symptoms Extraintestinal
arthralgia (often years before intestinal symptoms)
erythema nodosum
neurological symptomes (dementia, Parkinson´s disease, headach)
sec. ocular involvement: ophthalmoplegia, nystagmus
chronic cought (DD TB)
heart insufficiency, angina pectoris
rarely primary Uveitis (<3%)

20. Whipple´s Disease - Detection
PAS-staining
PAS-positive SPC-Zellen: siccle particles containing cells
culture: very difficult

21. Differential Diagnosis
intraocular lymphoma
ischemic ophthalmopathy
low grade endophthalmitis
sarcoidosis TB

22. Whipple´s Disease - Therapy
systemic antibiotics for 1 year
lethal if without treatment
in case of ocular involvement
Trimethoprim + Sulfamethoxazol
Doxycyclin + Rifampicin
Chloramphenicol
Ceftriaxon i.v. occ. for short time

23. Conclusion
rarely intermediate uveitis can be caused by Whipple´s Disease
detection of Tropheryma whipplei can be done from
vitreous, IOL and pars plana precipitates
blood
small intestine
therapy consists of antibiotics for 1 year