Therapeutics (see also Chapter 19 on IBD)
The treatment of PLE is complicated by the variety of different underlying etiologies. Thus, the focus of treatment is to identify and correct or control (if possible) any underlying cause (e.g., IBD, lymphoma, gastric ulcers, giardiasis, histoplasmosis) and then to reduce the leakage of lymph and protein across the gut wall through non-specific but appropriate diet and/or drug therapy (Zoran 2014).
Treatment of specific diseases that may cause PLE, such as IBD, alimentary lymphoma, or infectious diseases like histoplasmosis are beyond the scope of this chapter and can be found elsewhere. The non-specific therapy of PLE has four main goals, namely provide adequate nutritional support, increase oncotic pressure, reduce inflammation associated with leakage of lymph and crypt lesions; and address or prevent complications, especially thromboembolic events (Dossin and Lavoue 2011; Zoran 2014).The two most important aspects in regard to nutritional support are the replenishment of the proteins lost to allow recovery of protein functions (e.g., albumin, clotting factors, anti-thrombin) and muscle mass and to provide enough energy in the diet while avoiding too much fat that the GI tract is unable to digest. A very detailed review of these aspects can be found elsewhere (Zoran 2014). The most efficient way to provide long-term oncotic support is to address and potentially cure the primary disease. In critical cases, hydroxyethyl starches are used at a maximal dosage rate of 20-30ml∕kg∕day (Dossin and Lavoue 2011). However, they only provide short-term support and may, at higher dosages, impair coagulation. Fresh frozen plasma may be used to increase the albumin and provide coagulation factors, and antithrombin. However, a large volume of plasma is required to significantly increase the albumin concentration. Coagulation should be monitored in patients with severe PLE because hypercoagulability and thrombosis have been reported. In these cases, fresh frozen plasma can be used to increase anti-thrombin and coagulation factors.