WHIPPLE'S DISEASE

WHIPPLE'S DISEASE

Gram +ve organism : Trophreyma whipplei
                                           - Actinobacteria
                                           - slow doubling times
                                           - can't be cultured


  • most common in males
  • age group - 50 - 60yrs
  • distal small intestine more affected than proximal small intestine
  • whipple's disease is chronic multisystem disease - GIT  +  CNS  +  CVS  +  JOINTS
  • HLA DR B1 13, HLA DQ B1 06
SYMPTOMS:

 GIT:
  •   Diarrhoea/weight loss
  •   anemia
  •   foul smelling stools
  •   abdomen distension + ascites
  •   abdominal lymphadenopathy
CNS:
  • Progressive dementia (m/c neurological manifest)
  • progressive supranuclear opthalmoplegia
  • opsclonus - myoclonus syndrome
  • myorrhythmia

            Neurological relapse - Bad prognosis

  
cvs: endocarditis
JOINTS: Arthritis

other: Hyperpigmentation , uveitis



diagnosis:
  • biopsy of upper small intestine : PAS +ve macrophages in lamina propria containing organism
  • PCR - Polymerase chain reaction               


treatment:
Induction Rx ; PENICILLIN   OR   CEFTRIAXONE         +     STREPTOMYCIN (14 DAYS)


Maintenance Rx : COTRIMOXAZOLE                                                                                     
                                                                                                                                         

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