A 60 y old male presents for the third time with edema, hypoalbuminemia, lower extremity edema and hypotension. Per patient, the episodes are cyclical and come and go. You diagnose Systemic Capillary Leak Syndrome? In which phase do you see renal failure?
Shock Phase 44%
Leak Phase 32%
Post Leak Phase 24%
Systemic capillary leak syndrome is a extremely rare disease that leads to vascular collapse. A classic place where one can remember seeing this is post IL-2 Infusion for renal cell cancer patients. This vascular collapse leads to hemoconcentration and low albumin levels. So far around 150 cases have been reported. In this entity, its important to differentiate this disease from Nephrotic Syndrome, Protein losing enteropathy, Carcinoid, Mastocytosis, Pheochromocytoma, Sepsis and Toxic Shock syndrome in the right clinical context.
In terms of pathophysiology, there is some data suggesting that the damage is due to endothelial dysfunction. But there are association studies also suggesting that 80% or so of patients with this syndrome get MGUS or have MGUS as well and there has been association with plasma cell dyscrasias.
In terms of renal disease and acute kidney injury in these patients: although there can be renal insult possible at any phase during this disease, the most likely phase is Shock Phase, as most of you suggested in your answer.
Typically, in this syndrome, there is a prodrome of weakness that leads to severe pre syncope, hemoconcentration, cool skin and oliguria early on and then there is the SHOCK PHASE that is usually causing pre renal/ATN leading to renal injury, stroke or DVTs. Within hours, this can progress to LEAK PHASE where edema develops along with low albumin and all extremities and face swell up. This leads to compartment syndrome and Rhabdomyolysis as well( could lead to renal injury here as well). Within 3 days, you get the POST LEAK PHASE, where there is restoration of volume and diuresis-- sometimes leading to cardiopulmonary failure ( could have renal injury here from post diuresis as well and cardiac failure)
A rare disease but usually we see renal failure with it. Typically in the SHOCK phase but other phases possible depending on the severity of it
A nice review in Annals of Internal Medicine
Check it out