Protein-Losing Enteropathy Medication

Updated: Aug 30, 2023
  • Author: Naeem Aslam, MD; Chief Editor: Burt Cagir, MD, FACS  more...
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Medication

Medication Summary

Octreotide has limited benefit in treating patients with Ménétrier disease, but a therapeutic trial may be worthwhile. [40] A monoclonal antibody against the epidermal growth factor receptor has been shown to be effective in treating Ménétrier disease. External elastic support is helpful in reducing peripheral edema.

Prednisone may be used in patients with total villous atrophy that is unresponsive to gluten restriction. Prednisone rapidly reverses the symptoms and signs of eosinophilic gastroenteritis and returns the serum albumin to the reference range. Medium-chain triglycerides are not helpful in hereditary intestinal lymphangiectasia. Specific treatment of infectious enteritides is indicated when present. 

Pozelimab, a monoclonal antibody targeting C5, has been approved for treatment of CD55 deficiency with hyperactivation of complement, angiopathic thrombosis, and severe protein-losing enteropathy (CHAPLE) syndrome in patients aged 1 year and older. [36]  

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Corticosteroids

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

Prednisone (Orasone, Sterapred)

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and by suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocyte and antibody production.

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Complement Inhibitors

Class Summary

In protein-losing enteropathy related to CHAPLE disease, pozelimab, a monoclonal antibody targeting C5, has been shown to normalize serum albumin and serum IgG concentrations as well as reduce hospitalizations and albumin infusions. [37]

Pozelimab (Veopoz)

Indicated for treatment of CD55 deficiency with hyperactivation of complement, angiopathic thrombosis, and severe protein-losing enteropathy (CHAPLE) syndrome in patients aged 1 year and older. Pozelimab is an immunoglobulin antibody directed against terminal complement protein C5. The role of C5 is to inhibit terminal complement activation by blocking cleavage of C5 into C5a (anaphylatoxin) and C5b. This action blocks formation of the membrane-attack complex (C5b-C9), a structure mediating cell lysis.

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