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CONSTRICTIVE PERICARDITIS AND PROTEIN LOSING ENTEROPATHY

AN IMITATOR OF INTESTINAL LYMPHANGIECTASIA

DAVID A. KUMPE M.D.1, RICHARD B. JAFFE M.D.2, THOMAS A. WALDMANN M.D.3, and MEREDITH A. WEINSTEIN M.D.4

1 University Hospital, Baltimore, Maryland.
2 University of Utah Medical Center, Salt Lake City, Utah.
3 Head, Immunophysiology Section, Metabolism Branch, National Cancer Institute, N.I.H.
4 Cleveland Clinic, Cleveland, Ohio.

1. Constrictive pericarditis with secondary protein losing enteropathy can mimic intestinal lymphangiectasia exactly on both small bowel barium examination and small bowel biopsy.

2. Clinically, there is less ascites relative to the degree of peripheral edema than in uncomplicated constrictive pericarditis. The pattern of lower extremity edema is symmetric, unlike the asymmetric distribution more characteristic of intestinal lymphangiectasia.

3. Lymphocytopenia is present due to loss of lymphocytes into the gut. This produces anergy, which can mask an underlying tuberculous etiology of the constrictive pericarditis.

4. Constrictive pericarditis can be a subtle clinical entity. It should be carefully excluded in all cases where gastrointestinal roentgenographic studies and intestinal biopsy indicate intestinal lymphangiectasia.

5. Following surgical correction of the constrictive pericarditis, the clinical syndrome and its consequences can disappear. The abnormal small bowel roentgenographic pattern can revert completely to normal, as will small bowel biopsy.


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