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Whipple´s Disease
Sebastian Thaler
Manfred Zierhut
Centre of Ophthalmology
University of Tuebingen,
Germany
First Presentation – Ocular History
November 2006
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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
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
First Presentation – Ocular History
November 2006
last recurrences (2 and 6/06)
 good response to systemic corticosteroids
 recurrences after reduction
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First Presentation – General History
November 2006
arthritis – non-steroidal antiphlogistics
 no other complains
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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
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First Presentation – Anterior Segment
OD
OS
First Presentation – Anterior Segment
OD
Thaler et al. Int. J. of Infectious Diseases 2010
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
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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
First Diagnosis
 suggestive for low grade
endophthalmitis
 removal of IOL
 in addition intravitreal antibiotics
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
Final Diagnosis
 Uveitis due to Whipple´s Disease
 based on
 clinical findings
positive PCR from the vitreous
Diagnostics – After 2 Months
January 2007
 biopsy of the small intestine
 detection of Tropheryma whipplei
 blood
 detection of Tropheryma whipplei
 spinal puncture
 no detection of Tropheryma whipplei
 endoscopy:
 antrum of the stomach with spotted mucosal atrophy
Treatment
Begin January 2007
 intravitreal Ceftriaxon for 2 weeks
followed by
 oral Trimethoprim with
Sulfamethoxazol for 1 year
 planned: secondary lens implantation
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
Final Follow Up – After 6 Months
April 2007
 re-biopsy from the small intestine:
 PCR negativ
Whipple´s Disease – Clinical Symptoms
Intestinal
 diarrhea
 abdominal pain
 malabsoption, leading to anemia,
hypoproteinemia and hypovitaminosis
 weight loss
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%)
Whipple´s Disease - Detection
 PAS-staining
 PAS-positive SPC-Zellen:
siccle particles containing cells
 culture: very difficult
Differential Diagnosis
 intraocular lymphoma
 ischemic ophthalmopathy
 low grade endophthalmitis
 sarcoidosis
 TB
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
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