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Published byBeatrice Tyler Modified over 9 years ago
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Whipple´s Disease Manfred Zierhut Centre of Ophthalmology
Sebastian Thaler Manfred Zierhut Centre of Ophthalmology University of Tuebingen, Germany
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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
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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
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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
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First Presentation – Anterior Segment OD
Thaler et al. Int. J. of Infectious Diseases 2010
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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
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First Diagnosis suggestive for low grade endophthalmitis
removal of IOL in addition intravitreal antibiotics
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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
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Final Diagnosis Uveitis due to Whipple´s Disease based on clinical findings positive PCR from the vitreous
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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
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Treatment Begin January 2007
intravitreal Ceftriaxon for 2 weeks followed by oral Trimethoprim with Sulfamethoxazol for 1 year planned: secondary lens implantation
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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
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Final Follow Up – After 6 Months April 2007
re-biopsy from the small intestine: PCR negativ
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Whipple´s Disease – Clinical Symptoms Intestinal
diarrhea abdominal pain malabsoption, leading to anemia, hypoproteinemia and hypovitaminosis weight loss
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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%)
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Whipple´s Disease - Detection
PAS-staining PAS-positive SPC-Zellen: siccle particles containing cells culture: very difficult
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Differential Diagnosis
intraocular lymphoma ischemic ophthalmopathy low grade endophthalmitis sarcoidosis TB
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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
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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
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