Medication Summary
No maintenance medications for primary intestinal lymphangiectasia are indicated, other than the use of octreotide.
Patients with secondary intestinal lymphangiectasia should continue the maintenance medications of their primary underlying disease.
Two case reports documented the use of octreotide to control symptoms in refractory cases. In the first report, octreotide improved symptoms, findings on scintigraphy and endoscopy, and histology of the duodenum in a patient with intestinal lymphangiectasia. [28] The second report showed that octreotide at 200 mcg twice daily resulted in reduction in enteric protein loss from 16% to 4.1% in 5 days, and albumin infusions, which were necessary to maintain an acceptable level, were eliminated in a single patient with intestinal lymphangiectasia. [29]
Additional cases have been reported with the successful use of octreotide, including the long-acting formulation (LAR). [19, 30]
Somatostatin Analogs
Class Summary
Somatostatin analogs are used to inhibit effects of gastrointestinal (GI) hormones.
Octreotide (Sandostatin)
Octreotide (Sandostatin) functions similarly to the hormone somatostatin. It is a highly potent inhibitor of various hormones, including growth hormone, glucagon, and insulin. Octreotide significantly reduces splanchnic blood flow and suppresses LH response to GnRH. It decrease intestinal absorption of fats. Moreover, it exerts a strong suppressive effect on GI hormones, including gastrin, motilin, secretin, and pancreatic polypeptide. Due to its ability to suppress GI hormone activity, octreotide is employed in the treatment of various GI disorders, such as VIPoma and carcinoid tumors.
mTOR inhibitors
Class Summary
Mammalian Target of Rapamycin (mTOR) inhibitors are a class of medications that target the mTOR signaling pathway, which plays an important role in regulating cell growth, proliferation, and survival.
Sirolimus
Within context of primary intestinal lymphangiectasia, sirolimus affects lymphatic endothelial cells by altering mTOR signaling, suppressing lymphatic sprouting and proliferation, and inducing apoptosis.
Antifibrinolytic Agents
Class Summary
Antihemophilic agents like tranexamic acid function by competitively inhibiting the activation of plasminogen to plasmin, thereby reducing or diminishing bleeding. Elevated fibrinolytic activity, believed to contribute to intestinal protein loss, is considered a factor in primary intestinal lymphangiectasia.
Tranexamic acid (Cyklokapron)
Tranexamic acid (Cyklokapron) is an antifibrinolytic agent. It works by inhibiting plasminogen activators, thereby blocking the breakdown of blood clots.
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Intestinal villi of normal height with dilated lymphatics as usually seen on histology of villi in intestinal lymphagiectasia.
