intestinal lipodystrophy (Whipple's disease)
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Introduction
A rare disorder affecting middle-aged males.
Etiology
caused by gram-positive bacilli Tropheryma whipplei
Pathology
- small bowel biopsy shows periodic acid Schiff base (PAS) staining of bacilliform intracellular structures or inclusions within macrophages
- seen in abdominal & peripheral lymph nodes & other tissues, including central nervous system
- thought to represent partially degraded bacteria
- electron microscopy shows rod-shaped organisms in lamina propria of small intestine
Genetics
association with HLA-B27 in patients with back disease
Clinical manifestations
- arthritis, arthralgias
- occurs in 2/3 of patients
- generally precedes intestinal symptoms by years or months
- may subside with onset of intestinal symptoms
- involves predominantly peripheral joints
- arthritis is generally acute, transitory, migratory lasting only a few days & causing no permanent joint damage, but may be chronic
- long & irregular remissions common
- serositis
- diarrhea
- malabsorption
- weight loss
- skin hyperpigmentation
- lymphadenopathy
- splenomegaly
- fever in middle-aged men[3]
- central nervous system manifestations
- cardiac manifestation, no specifics[3]
- chronic cough (case report)[5]
Laboratory
- 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
- cell count & differential
- Tropheryma whipplei DNA
- polymerase chain reaction (PCR) to detect 16S ribosomal RNA of the organism
Diagnostic procedures
Radiology
- changes in sacroiliac joint may resemble ankylosing spondylitis
Management
- 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
- relapse on tetracycline requires retreatment with penicillin & streptomycin
- non-steroidal anti-inflammatory drugs (NSAIDs) may be useful in controlling joint symptoms
- glucocorticoids may be necessary in addition to antimicrobial agents in severely ill patients
More general terms
Additional terms
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
- ↑ 3.0 3.1 3.2 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, American College of Physicians, Philadelphia 1998, 2006
- ↑ 4.0 4.1 Geriatrics Review Syllabus, American Geriatrics Society, 5th edition, 2002-2004
- ↑ 5.0 5.1 Damaraju D et al A Surprising Cause of Chronic Cough. N Engl J Med 2015; 373:561-566. August 6, 2015 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/26244310 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMcps1303787
- ↑ 6.0 6.1 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 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/26840137 <Internet> 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
Patient information
intestinal lipodystrophy (Whipple's disease) patient information