Whipple's Disease: Clinical, Biochemical, and Histopathologic Features and Assessment of Treatment in 29 Patients

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Whipple's disease is a chronic systemic illness, the optimal treatment of which remains poorly defined. In our analysis of a 30-year, 29-patient experience with Whipple's disease at the Mayo Clinic, the frequent initial manifestations of diarrhea, weight loss, arthritis, and lymphadenopathy correlated with findings reported previously by other investigators. Antibiotic therapy yielded rapid symptomatic and biochemical improvement, and histologic changes in the small bowel occurred subsequently. Despite antimicrobial therapy, relapses in patients with Whipple's disease are common, and the central nervous system is considered the most serious site of involvement for recurrence. Administration of an antibiotic agent that is able to cross the blood-brain barrier may be more important in preventing relapse than prolonged duration of initial antimicrobial therapy.

Section snippets

METHODS

With use of the Mayo Clinic computerized medical record retrieval system, we identified patients with a diagnosis of Whipple's disease during the 30-year period from 1954 to 1984. For confirmation of the diagnosis, small bowel and lymph node biopsy specimens were reviewed by a single pathologist. The histologic criteria for inclusion in the study were the presence of macrophages that contained periodic acid-Schiff (PAS) granules (resistant to diastase) in the lamina propria of the small

Patient Characteristics.

At the time of diagnosis, all patients had histologic evidence of Whipple's disease—26 on jejunal biopsy specimens and 3 on lymph nodes. On review at the time of this study, four specimens were not available and three were interpreted as being inadequate.

The overall mean age of the 29 patients (23 men and 6 women) at the time of diagnosis was 54.0 years (range, 34 to 70 years). The mean age of the male and female patients, respectively, was 53.9 and 54.2 years.

At the time of initial

Patient Characteristics.

The epidemiologic findings in this group of patients with Whipple's disease were consistent with those of other investigators. Seventy-nine percent of our study group were male patients, similar to the reported frequency of 80 to 90%.5, 6, 7, 8, 9 The mean age of 54.0 years at the time of diagnosis was slightly older than the fifth decade reported by others.5, 7 Our youngest patient was 34 years of age at the time of diagnosis, and the oldest patient was 70 years old. Extremities of age

CONCLUSION

During the 30-year period from 1954 to 1984, 29 patients were diagnosed as having Whipple's disease at our institution. A review of this study group showed that the common clinical manifestations of weight loss, arthritis, lymphadenopathy, and diarrhea corresponded to the findings reported by other investigators. We found a rapid symptomatic and biochemical improvement in patients who received antibiotic agents. Normalization of the histologic features on repeat biopsy lagged behind the

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    Current address: McFarland Clinic, Ames, Iowa.

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    Copyright © 1988 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.