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At a glance

This is a medical condition acquired from an infectious agent called Tropheryma whipplei, which is closely related to the actinomycetes. It results in a Malabsorption Syndrome with involvement of small intestine, joints, skin, lungs, central nervous system, and cardiovascular system. Other features include weight loss, diarrhea, joint pain, and arthritis, but the presentation can be highly variable and approximately 15% of patients do not have these classic signs and symptoms. The onset of the disease can be seen at all ages.

N.B.: Do not confuse with Whipple Triad which consists of: 1) symptoms of spontaneous hypoglycemia; 2) blood glucose level <50 mg/dl; and 3) relief after ingestion of glucose. Classically, it suggests the presence of an insulinoma.

Synonyms

Idiopathic Steatorrhea Syndrome; Intestinal Lipodystrophy Syndrome; Intestinal Lipogranulomatosis Syndrome; Lipodystrophia Intestinalis Syndrome; Lipophagic Intestinal Granulomatosis Syndrome; Mesenteric Lymphadenitis Syndrome; Nontropical Sprue; Steatorrhea Arthropericarditica Syndrome.

History

This disease was first described in 1907 by George Hoyt Whipple (1878-1976). Whipple described the disease in 1907 in a paper in the now-defunct Bulletin of Johns Hopkins Hospital. The patient was a 35-year-old medical missionary. Although it was originally described as a gastrointestinal disorder because of the malabsorption associated with this condition, it can also affect any part of the body, particularly the heart, brain, joints, skin, lungs, and the eyes. In 1934 George Hoyt Whipple, George Richards Minot (1885-1950) and William Parry Murphy (1892-1987) received the Nobel Prize in Physiology and Medicine “for their discoveries concerning liver therapy in cases of anemia.”

Incidence

The exact incidence remains unknown. However, it is suspected that the number of new cases per year is approximately 1 per million in the general population. Almost 90% of the affected patients are males. The disease is usually diagnosed around 50 years of age. (median 49 years). It is reported that the age of diagnosis has been rising since the 1960s.

Genetic inheritance

There is no genetic involvement.

Pathophysiology

Invasion of affected tissue by Tropheryma whipplei results in local inflammation and organ dysfunction, as well as generalized systemic symptoms. Appears to be associated with the human leukocyte antigen B27 (HLA-B27) haplotype.

Diagnosis

Clinically evocated by arthralgias, arthritis, fever, and diarrhea. Biopsy of duodenum or proximal jejunum reveals infiltration of lamina propria by “foamy” macrophages with granules, which stain positively with the periodic acid-Schiff (PAS) technique. The macrophages contain gram-positive acid-fast negative bacilli—Tropheryma whipplei.

Clinical aspects

Can occur at any age (reports range from 3 months to 82 years) but is more common after the fourth decade. More than 70% of patients are male. Although the primary organ system involved is the gastrointestinal (GI) tract, systemic symptoms and those related to involvement of other organ systems frequently precede those attributable to the GI tract. Clinical ...

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