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[Whipple's disease with reactive (AA) amyloidosis]
P A Schmid1, H R Burger, R P Linke
1Medizinische Klinik, Stadtspital Waid, Zürich.
Deutsche Medizinische Wochenschrift (1946)
|August 20, 1993
Summary
Whipple's disease, a rare bacterial infection, can lead to reactive amyloidosis (AA), causing systemic complications. This case highlights the critical link between Whipple's disease and AA amyloidosis, particularly cardiac involvement.
Area of Science:
- Gastroenterology
- Rheumatology
- Pathology
Background:
- Whipple's disease is a rare systemic infectious disease caused by Tropheryma whipplei.
- It can present with diverse symptoms, often mimicking other conditions like inflammatory arthritis or malignancy.
- Systemic amyloidosis is a known, albeit uncommon, complication of chronic inflammatory conditions.
Observation:
- A 59-year-old man presented with elevated erythrocyte sedimentation rate, arthralgia, weight loss, and gastrointestinal issues.
- Initial suspicion of lymphoma due to lymphadenopathy was disproven by biopsy showing macrophages with PAS-positive inclusions, indicative of Whipple's disease.
- The patient experienced sudden cardiac death on the day of diagnosis.
Findings:
- Necropsy revealed generalized amyloidosis with significant cardiac involvement.
- Immunohistochemical analysis confirmed the amyloid deposits as reactive AA amyloidosis.
- Other forms of amyloidosis were excluded, establishing a direct association with Whipple's disease.
Implications:
- This case underscores the potential for Whipple's disease to trigger reactive AA amyloidosis.
- It emphasizes the importance of considering Whipple's disease in patients with unexplained systemic inflammation and gastrointestinal symptoms.
- The findings highlight the severe systemic consequences, including cardiac complications, that can arise from undiagnosed or untreated Whipple's disease and subsequent amyloidosis.