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Whipple´s Disease Sebastian Thaler Manfred Zierhut Centre of Ophthalmology University of Tuebingen, Germany 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 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 First Presentation – General History November 2006 arthritis – non-steroidal antiphlogistics no other complains 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 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 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