Introduction
Whipple's disease is a rare multi-system disorder caused by Tropheryma whippelii infection. It is more common in those who are HLA-B27 positive and in middle-aged men.
Epidemiology
- Incidence: 0.01 cases per 100,000 person-years
- Peak incidence: 40-50 years
- Sex ratio: 1:1
| Condition | Relative incidence |
|---|---|
| Coeliac disease | 10000.00 |
| Whipple's disease | 1 |
| <1 | 1-5 | 6+ | 16+ | 30+ | 40+ | 50+ | 60+ | 70+ | 80+ |
Clinical features
Features
- malabsorption: diarrhoea, weight loss
- large-joint arthralgia
- lymphadenopathy
- skin: hyperpigmentation and photosensitivity
- pleurisy, pericarditis
- neurological symptoms (rare): ophthalmoplegia, dementia, seizures, ataxia, myoclonus
Investigations
Investigation
- jejunal biopsy shows deposition of macrophages containing Periodic acid-Schiff (PAS) granules
Management
Management
- guidelines vary: oral co-trimoxazole for a year is thought to have the lowest relapse rate, sometimes preceded by a course of IV penicillin