The roentgen diagnosis of Whipple's disease (intestinal lipodystrophy), with emphasis on improvement following antibiotic therapy.

Abstract

Only in the past three or four years has it been recognized that Whipple's disease (intestinal lipodystrophy) can be successfully treated with long-term administration of antibiotics. From the time of Whipple's initial description in 1907 (17) until the past decade, the disease had been considered invariably fatal, although Paul-ley (11) as early as 1952 reported a case of Whipple's disease which responded to tetracycline therapy. There were also a number of reports of long-term remissions attributed to cortisone (15). It was not until 1960 (5), however, that the very impressive results of long-term antibiotic therapy were reported. Subsequent articles (1, 14) have substantiated this observation, and it is our opinion that no one should die of Whipple's disease if it is correctly diagnosed and treated. The major clinical manifestations of Whipple's disease are diarrhea, steatorrhea, arthralgias, increased skin pigmentation, lymph node enlargement, and serous effusions. Although these symptoms obviously represent multisystem involvement, the intestinal manifestations usually are predominant by the time the diagnosis is established. The diagnosis can be confirmed by intestinal mucosal or lymph node biopsy. The small intestinal villi are swollen, and the lamina propria is infiltrated with foamy macrophages. These macrophages contain particles which take the periodic acid-Schiff (PAS) stain. Similar macrophages are also encountered in lymph nodes and various organs throughout the body. The etiology of Whipple's disease has not been established. It is generally agreed, however, that the PAS-positive material seen in macrophages represents masses of bacteria which are either intact or in varying stages of digestion (3, 4, 7, 18). Serial biopsies have demonstrated that the bacil-liform bodies uniformly present in the lamina propria in untreated patients disappear when antibiotics are given (7). Several investigators have cultured similar Gram-positive anaerobic diphtheroids from lymph nodes removed under sterile conditions from patients with Whipple's disease (2, 12, 14). This histologic and bacterio-logic evidence suggests the possibility of a bacterial role in the pathogenesis of Whipple's disease and provides a plausible explanation for the efficacy of antibiotic therapy (3, 4, 18). At the present time, the therapeutic regimen employed at Duke Hospital includes ten to fourteen days of penicillin and streptomycin, followed by three to twelve months of continuous tetracycline therapy. The purpose of this paper is to report the roentgenographic findings in a series of 12 patients with proved Whipple's disease and to emphasize the improvement in the appearance of the small intestine following antibiotic therapy.