Weekly Blog - Whipple's disease with amyloidosis

Since it was mentioned I thought it would be worth a quick note on Whipple's disease:

See review NEJM here.

It is a disease mostly described in men (>85%)

The majority of patients have:
* Weight loss (>90%)
* Diarrhea (80%)
* Arthritis (70%)

Neurologic symptoms or the classic occular findings are seen in 33% and <10% respectively.

"Roughly 15% of patients with Whipple's disease do not have the classic signs and symptoms of the disease"

Diagnosis is made through:
* PAS staining of GI biopsies -- usually several are required
* PCR on tissue or blood/CSF can also be positive
* Electron microscopy can also be used

Treatment:
CEFTRIAXONE induction, TMP/SMX consolidation
Alternative: DOXYCYCLINE

Note that discussed patient also had evidence of a monoclonal light chain in the urine prompting suspicion of amyloidosis (not seen on biopsy of the gut, nor in the serum).

There are several case reports of Whipple's disease being associated with amyloidosis (particularly with renal involvement -- i.e. nephrotic syndrome; also presenting with wasting illness without diarrhea like that discussed)

This case has the full report if you are a McGillian -- here.

Also see here, here, here and here .  (many not available online)

Yes, the patient may not have Whipple's disease -- but it is curable and compatible with the illness (which primary amyloidosis, the alternative, is not in the specific patient's condition).