WHIPPLE'S DISEASE: Roentgen Features and Differential Diagnosis

Abstract

Whipple's disease is a curious and rare condition with an unexplained predilection for middle-aged males. It is a systemic disease 23 in which nonspecific synovitis is common 5 and polyserositis occurs, but the major manifestations are intestinal. The fundamental pathologic lesion is infiltration of the affected organs by macrophages containing PAS-positive (periodic acid-Schiff stain) material. Recently bacterial infection by some species of Corynebacterium has been implicated in the pathogenesis, and the disease may be a peculiar reaction to this infection. 12 Formerly regarded as fatal, it now appears curable by antibiotic therapy. 20 From various sources we have collected 16 cases with roentgen studies. The purpose of this paper is primarily to describe the roentgen findings in this condition. The rarity of Whipple's disease is indicated by the fact that only three of the 16 cases are from a single institution (Yale-New Haven Hospital). The patients are all in the fourth to sixth decades of life, and only two of the 16 patients are female. SUMMARY Study of 16 cases of proven Whipple's disease has confirmed the invariable occurrence of a characteristic pattern on roentgen study of the small bowel. The diffuse thickening of the valvulae conniventes of the jejunum reflects the infiltration by PAS-positive macrophages. The roentgen differential diagnoses include amyloidosis, intestinal lymphangiectasia, histoplasmosis, some hypoproteinemic states, leukemic infiltration, diffuse lymphosarcoma, intestinal infiltrations associated with dysproteinemias, mastocytosis, and giardiasis. The characteristic clinical features of Whipple's disease resolves the differential diagnosis in most cases, but peroral intestinal biopsy is recommended to confirm the diagnosis.