Intestinal Lymphangiectasia

Updated: Dec 19, 2014
  • Author: Hisham Nazer, MB, BCh, FRCP, , DTM&H; Chief Editor: Julian Katz, MD  more...
  • Print
Overview

Background

Traditionally, protein-losing gastroenteropathies have been classified into 3 groups (depending on the mechanism of their etiology) that include the following: (1) those causing mucosal damage leading to increased permeability to protein (usually not involving mucosal ulcerations), (2) those with mucosal erosions and/or ulcerations, and (3) those in which protein loss is secondary to mechanical lymphatic obstruction.

While a more detailed discussion on Protein-Losing Enteropathy is presented in another article, this article specifically addresses intestinal lymphangiectasia.

Next:

Pathophysiology

Intestinal lymphangiectasia is a disease characterized by hypoproteinemia, edema, and lymphocytopenia, resulting from dilatation of intestinal lymphatics and loss of lymph fluid into the gastrointestinal (GI) tract. [1] This leads to immunologic abnormalities, including hypogammaglobulinemia, anergy, and impaired allograft rejection. In addition to the loss of other serum components (eg, lipids), iron and certain trace metals may also be affected. [2]

Previous
Next:

Epidemiology

Frequency

United States

Frequency in the United States and internationally is unknown.

Mortality/Morbidity

Morbidity is related to the pathophysiology of this disease. Edema and diarrhea are predominant clinical features; however, the following negative sequelae are also observed:

Race

No racial predilection exists.

Sex

The male-to-female ratio is 3:2.

Age

Intestinal lymphangiectasia can be primary (ie, congenital), in which case it affects children and young adults (mean age of onset, 11 y). The diagnosis in these cases often occurs during the first decade of life, with the first manifestations being persistent diarrhea and peripheral edema. This condition can also be secondary to other disease states, thus affecting older adults. [3] In a series from Japan, the average age at onset was 22.9 years.

Previous