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Whipple’s Disease

Title

Ñ    Whipple’s Disease

Definition

Ñ    A rare multisystem disease

Caused by:

Ñ    Trophyrema whippellii

Aetiology

Infection by:

Ñ    Trophyrema whippellii

Pathogenesis

Ñ    Small intestinal villi shortened and contain macrophages which are PAS positive (periodic acid-Schiff)

Ñ    Similar macrophages are found in the lymphatics, lymph nodes, joints, brain

Ñ    Inflammation essentially absent

Clinical Features

History

Age

Ñ    Usually middle aged

Sex

Ñ     Males > Females

E&M

Ñ    Weight loss

Ñ    Fever

RS

Ñ    Cough

Ñ    Pleuritic chest pain

GIT

Ñ    Diarrhoea

Ñ    Steatorrhoea

Ñ    Abdominal pain

CNS

Ñ    Insomnia, hyperphagia, polydypsia:

Hypothalamic syndrome

LMS

Ñ    Arthritis

Examination

E&M

Ñ    Loss of weight

Ñ    Fever

IS

Ñ    Clubbing

CVS

Ñ    Murmur

Ñ    Displaced apex beat

Ñ    Pericardial rub

RS

Ñ    Crepitations

Ñ    Pleural rub

GIT

Ñ    Chylous or serous ascites

HS

Ñ    Lymphadenopathy

CNS

Ñ    Photophobia

Ñ    Altered mental status

LMS

Ñ    Peripheral joint swelling

Ñ    Joint crepitations

Complications

CVS

Ñ    Endocarditis

Ñ    Cardiomyopathy

Ñ    Cardiac conduction defects

Ñ    Coronary arteritis

Ñ    Pericarditis

RS

Ñ    Pleurisy

Ñ    Pulmonary infiltrates

CNS

Ñ    Uveitis

Ñ    Scotoma

Ñ    Papilloedema

Ñ    Ophthalmoplegia

Ñ    Meningitis

LMS

Ñ    Migratory, non-deforming, seronegative arthritis predominantly affecting the peripheral joints

Investigations

Fluids

Haematology

Ñ    Anaemia

Imaging

Contrast Studies

Ñ    Small bowel radiology:

Thickened and oedematous mucosa

Endoscopy

Gastroscopy

Ñ    Small bowel biopsy

Ñ    Large, foamy PAS positive macrophages in the lamina propria

Ñ    Overlying villi distorted

Management

Cure

Drugs

Ñ    Penicillin and streptomycin for 2 weeks followed by:

Ñ    Tetracycline for 1 year

Prognosis

Ñ    Untreated it is eventually fatal

Ñ    5% relapse rate

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