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intestinal lipodystrophy (Whipple's disease)
A rare disorder affecting middle-aged males.
Etiology: caused by gram-positive bacilli Tropheryma whipplei
Pathology:
1) small bowel biopsy shows periodic acid Schiff base (PAS) staining of bacilliform intracellular structures or inclusions within macrophages
a) seen in abdominal & peripheral lymph nodes & other tissues, including central nervous system
b) thought to represent partially degraded bacteria
2) electron microscopy shows rod-shaped organisms in lamina propria of small intestine
Genetics: association with HLA-B27 in patients with back disease
Clinical manifestations:
1) arthritis, arthralgias
a) occurs in 2/3 of patients
b) generally precedes intestinal symptoms by years or months
c) may subside with onset of intestinal symptoms
d) involves predominantly peripheral joints
1] knees & ankles most commonly affected
2] fingers, hips, shoulders & wrists less frequently affected
e) arthritis is generally acute, transitory, migratory lasting only a few days & causing no permanent joint damage, but may be chronic
f) long & irregular remissions common
2) serositis
3) diarrhea
4) malabsorption
5) weight loss
6) skin hyperpigmentation
7) lymphadenopathy
8) splenomegaly
9) fever in middle-aged men [3]
10) central nervous system manifestations
a) dementia
b) myoclonus
c) ophthalmoplegia
d) visual disturbances
e) seizures
f) coma
g) cerebellar ataxia [4]
11) cardiac manifestation, no specifics [3]
- pericardial effusion [6]
12) chronic cough (case report) [5]
Laboratory:
1) joint fluid aspiration
- cell count & differential
- WBC count 450-36,000/mm3
- differential 30-90% neutrophils
- monocytosis may be observed
- PAS-positive foamy macrophages may be observed
2) Tropheryma whipplei DNA
3) polymerase chain reaction (PCR) to detect 16S ribosomal RNA of the organism
Special laboratory:
- upper GI endoscopy with small intestine biopsy
Radiology:
- changes in sacroiliac joint may resemble ankylosing spondylitis
Management:
1) penicillin 1.2 million units plus streptomycin 1 g IV QD for 2 weeks, followed by tetracycline 1 g daily for one year
- Bactrim is alternative to tetracycline
2) relapse on tetracycline requires retreatment with penicillin & streptomycin
3) non-steroidal anti-inflammatory drugs (NSAIDs) may be useful in controlling joint symptoms
4) glucocorticoids may be necessary in addition to antimicrobial agents in severely ill patients
Related
Tropheryma whipplei
General
bacterial infection
intestinal disease
lipodystrophy; lipoatrophy
References
- Harrison's Principles of Internal Medicine, 11th ed.
Isselbacher et al (eds), McGraw-Hill Inc. NY, 1987, pg 1462
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 298
- Medical Knowledge Self Assessment Program (MKSAP) 11, 14,
American College of Physicians, Philadelphia 1998, 2006
- Geriatrics Review Syllabus, American Geriatrics Society,
5th edition, 2002-2004
- Damaraju D et al
A Surprising Cause of Chronic Cough.
N Engl J Med 2015; 373:561-566. August 6, 2015
PMID: 26244310
http://www.nejm.org/doi/full/10.1056/NEJMcps1303787
- Houston BA, Miller PE, Rooper LM, Scheel PJ Jr, Gelber AC
CLINICAL PROBLEM-SOLVING. From Dancing to Debilitated.
N Engl J Med. 2016 Feb 4;374(5):470-7
PMID: 26840137
http://www.nejm.org/doi/full/10.1056/NEJMcps1311794
- Whipple's Disease Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Whipples-Disease-Information-Page
- NIDDK: Whipple Disease
https://www.niddk.nih.gov/health-information/digestive-diseases/whipple-disease