Whipple's disease. Roentgenoloic findings, including those of an eight-year remission.

Abstract

Whipple's disease is a systemic condition characterized by diarrhea, intestinal malabsorption, abdominal pain, lymphadenopathy, and a polyarthritis, which may antedate the other symptoms by several years. In the first forty-two years after Whipple's original description of this disease in 1907 (10), only ten references to it could be found in the English literature, but more recently it has attracted a great deal of attention, and in the past decade it has been the subject of over fifty articles. Very little, however, has been written about the detailed radiologic features (3, 6, 8). The present report is concerned with 3 cases of Whipple's disease, with special attention to the radiologic findings. One of these cases, originally published by Jones, Benson, and Roque (5), is of particular interest because of reversion of a grossly abnormal small-bowel roentgen picture to normal following administration of steroids and ACTH. The eight-year remission in this instance is the longest yet recorded. Only 2 other reports could be found in the literature with roentgenograms showing return of the small-bowel picture to normal following therapy (4, 9). Case Reports Case I: M. H., a 49-year-old female, was admitted to the hospital Sept. 19, 1951, with a probable diagnosis of sprue. Two years previously she had experienced a typical attack of rheumatoid arthritis. There was a history of intermittent epigastric pain for twenty years. This had become worse in the year before admission and had not improved following cholecystectomy. In the past four months the stools had become loose and foamy; lower abdominal pain, abdominal distention, and deepening pigmentation had developed, and there had been a weight loss of 25 pounds. On admission, the patient was emaciated, with a striking yellowish-brown pigmentation of the skin, most marked in the palmar creases. The hemoglobin was 9.0 grams and the red cells were microcytic. The serum calcium was 7.8 mg. per 100 ml., the albumin 3.1 grams per 100 ml., the globulin 1.8 grams per 100 ml., and the alkaline phosphatase 9.6 Bodansky units. The stool contained 23 grams of fat and 2.7 grams of nitrogen per twenty-four hours excretion. A gastrointestinal series (Fig. 1) showed the small bowel to be grossly abnormal, with marked thickening of the mucosal folds and minimal segmentation and flocculation. Because of the severity of the symptoms, the patient was started on ACTH on Sept. 27, and within three days there was a marked improvement in appetite, gain of weight, and decrease in the number of stools. Ten days after the beginning of therapy the fecal fat had fallen to 15.8 grams and the fecal nitrogen to 1.9 grams a day. The serum calcium had risen to 9.6 mg. per 100 mI. On Oct. 7, the daily medication was changed to 100 mg. of cortisone orally. On Oct. 16, three weeks after steroids were first administered, another small-bowel study was done (Fig. 2).