Whipple's disease.Gastroenterol Clin Biol. 2007 Aug-Sep;31(8-9 Pt 1):729-39.

For many years, Whipple's disease was considered a rare, mainly intestinal disease causing malabsorption. At present, however, it appears to be multivisceral mainly occuring in subjects with specific and subtle cell-mediated immunity defects. Until recently, diagnosis and follow-up of treatment efficacy depended on PAS positive macrophage inclusions in duodenal biopsies. New diagnostic methods based on PCR gene amplification and immunohistochemistry are now available by DNA sequencing and culture, respectively, of the causal bacteria, which was recently renamed Tropheryma whipplei. Although results are still empirical, and the first randomized study is in progress, an evolution in the choice and duration of antibiotic treatment of this normally fatal disease has led to a marked reduction in clinical relapses, especially for neurological manifestations. The present review shows how recent medical advances have completely transformed the understanding of a disease first described a century ago.

Whipple's disease: current problems.Orv Hetil. 2007 Jul 1;148(26):1225-30.

Whipple's disease is a rare multisystemic infectious disease of bacterial origin characterized by variable clinical manifestations, and an insidious and chronic relapsing course. Untreated disease can be even fatal. The presence of the characteristic (though not specific) triad of weight loss, chronic diarrhea and arthralgias may raise its suspicion. When chronic intermittent fever and lymphadenopathy are associated, the suspicion is substantial. Recognition of the causative agent, Tropheryma whippelii with unique characteristics was essential. Despite the presumed ubiquitous presence of the bacteria the disease probably occurs only in cases of immunological host susceptibility. Presence of the bacteria living and multiplying especially in macrophages has suggested alterations of the mononuclear-phagocytic system. (Whipple's disease is commonly mentioned as a macrophage disorder.) Clinical manifestations are quite diverse. While it has traditionally been regarded as a gastrointestinal disease, currently is considered to be a systemic disorder. In cases of suspected infection the approach of first choice is upper gastrointestinal endoscopy. Small, whitish-yellow diffusely distributed plaques alternating with an erythematous, erosive, friable mucosa in the postbulbar region of the duodenum or in the jejunum can appear. Histological samples indicate tissue infiltration of macrophages with intracellular bacterial invasion. The hallmark of Whipple's disease is the presence of PAS positive macrophages in the lamina propria of duodenal biopsy specimens, still the diagnosis needs to be confirmed with the detection of bacteria by PCR. The selection of antibiotics and duration of treatment still remains largely empiric.

Whipple's disease. Description of a case and survey of the literature. Minerva Gastroenterol Dietol. 2000 Jun;46(2):105-12.

A case of Whipple's disease (WD) personally observed is described. A 28-year-old male was admitted to hospital for evaluation of weakness, intermittent fever and weight loss arisen since a month. On clinical investigation, he complained of vomit and diarrhea since three months. He had neither familial and personal past history of gastrointestinal diseases, nor any other important diseases. He denied use of drugs. Physical examination was negative. Laboratory findings showed anemia, low blood lymphocytes, low serum iron and total iron binding capacity, low total serum protein and low serum albumin and high level of ESR. Stool were negative for parasites and occult blood. Cultures of blood and urine were negative. Stool fat assay was > 7 g/24 h and D-xylose test showed a two-hour serum concentration < 25 mg/dl. Abdominal TC showed lumbo-aortic and mesenteric enlarged lymph nodes. An upper video endoscopy showed a duodenal lymphangectasia. Histological examination showed villar atrophy with massive infiltration of large PAS-positive diastase-resistant foamy cells. Ziehl-Nielsen staining was negative. WD was diagnosed and patient underwent therapy based on cotrimoxazole. This report emphasizes the difficulty to diagnose WD correctly, because of its rareness and clinical polymorphism. Recently, studies have identified a bacillus, Tropheryma whippelii, associated with WD, so that, in the next future, the diagnosis of WD will be faster and more accurate. Finally, it is important to administer antibiotics which can cross the blood brain barrier for at least one year, in order to prevent neurological relapse, often lethal.