ReviewsWhipple’s disease
Section snippets
Epidemiology
Current epidemiologic information about WD is still very limited. In this context, Dobbins3 published an outstanding clinical and epidemiologic analysis based on the largest number of cases (n = 696) collected from his own observation, data from cases published in the English language literature (case reports and small series), and a series of nonpublished patients. The substantial information provided by the review clearly showed that the disease predominantly occurs in men (86% of cases),
Clinical manifestations
The clinical features of WD are protean, involving mainly the gatrointestinal tract, but also producing manifestations in other systems according to their pathological compromise.14 Although the clinical severity is usually associated with a multiorgan involvement, there is general consensus that more organs are involved than is suggested by clinical symptoms. Table 1 summarizes the most common symptoms and signs at the time of diagnosis of patients. There is a triad characteristic of WD:
Pathogenesis
A recently identified infectious agent causes WD. There is some evidence suggesting that a series of immunologic and nonimmunologic deficiencies detected in patients seem also to be necessary. Malabsorption is often present in patients with intestinal involement and has been associated with lymphatic obstruction from lacteal compression in lamina propria and lymph nodes, and with suspected epithelial dysfunction.
Host immunologic and nonimmunologic defects
WD disease is a systemic disorder in which the possibility of a defective immune response has been raised.52 There are still key questions. Do these abnormalities precede the infection, or are they a consequence of the nutritional compromise? Are these immunologic defects nonspecific alterations, or are they specifically directed against the T. whipplei? Contradictory immunologic findings have been reported. A genetic predisposition was formerly suggested on the basis of the high prevalence of
Clinical suspicion of Whipple’s disease
In general, the onset of WD is insidious, and it often takes several years until overt disease is present and a definitive diagnosis can be made. Very often, polyarthralgias and low-grade persistent fever are the main or only symptoms detected during the prediagnostic period.3, 8 On the other hand, a characteristic of the natural history of the disease before treatment is a chronic relapsing course in a high proportion of patients. Even though most patients have severe clinical compromise,
Treatment
The therapeutic goal in all patients with WD is to eradicate the infection and to avoid relapses. Until the 1950s, the disease was incurable, and most patients died of the disease.8 Paulley34 was the first to report the successful treatment of WD with the use of chloramphenicol and opened a new era in the understanding of the pathogenesis and treatment of the disease. Since then, a variety of antibiotics and different schedules were successfully used. Thus, patients have shown rapid improvement
Conclusions
WD is an extremely interesting infectious systemic disorder. The increased attention to this condition in recent years has led to a greater understanding and awareness of the disease by the medical community. Classic patients have predominantly gastrointestinal manifestations. Extraintestinal manifestations are protean, and when not associated with intestinal symptoms, the diagnosis might be difficult. The diagnosis of WD often requires endoscopic biopsy of the duodenal mucosa showing the
References (80)
The PCR for Tropheryma whippelii
Lancet
(1999)- et al.
Whipple’s disease
Am J Med
(1955) - et al.
PCR-positive test for Tropheryma whippelii in patients without Whipple’s disease
Lancet
(1999) - et al.
Tropheryma whippelii in saliva of healthy people
Lancet
(1999) - et al.
Whipple’s disease
Lancet
(2003) - et al.
Joint changes in Whipple’s disease
Semin Arthritis Rheum
(1973) - et al.
Reversal of dementia associated with Whipple’s disease by trimethoprim-sulfamethoxazole, drugs that penetrate the blood-brain barrier
Gastroenterology
(1984) A case of Whipple’s disease (intestinal lipoditrophy)
Gastroenterology
(1952)- et al.
Phylogenia of the Whipple’s disease-associated bacterium
Lancet
(1991) - et al.
Heterogeneity among Whipple’s disease-associated bacteria
Lancet
(1994)