A Lesser‐Known Cause of Recurrent Hypovolemic Shock

1Cleveland Clinic Foundation, Cleveland, OH
2Cleveland Clinic Foundation. Cleveland, OH
3Cleveland Clinic Foundation, Cleveland, OH
4Cleveland Clinic Foundation, Cleveland, OH

Meeting: Hospital Medicine 2010, April 8-11, Washington, D.C.

Abstract number: 209

Case Presentation:

A 41‐year‐old woman was admitted for evaluation of recurrent diarrhea and lightheadedness. She described recurrent episodes over the preceding year of nausea and diarrhea, followed by worsening dizziness. She also had bilateral hip and lower‐extremity cramping and at times progressed to syncope. She had had multiple ICU admissions for hypovolemic shock (once requiring dialysis for ATN). Interestingly, all episodes had resolved promptly with aggressive IV hydration. She would leave the hospital hypervolemic after 10–12 Lof IV fluids but would return in a few weeks with extreme polyuria and a 10‐pound weight loss. Her past history included breast cancer treated with surgery and chemotherapy and MGUS. She was afebrile with a BP of 87/53 and pulse of 93. She had a normal cardiopulmonary examination but bilateral pitting edema of all extremities. Admission H/H was 17.8/51% (baseline at previous discharge: 9.5/21%) but without leukocytosis, renal failure, or liver enzyme elevation. Her hemoconcentration improved in 1 day with IV fluids, but the albumin decreased to 2.2 g/dL. She previously had a negative infectious, cardiac (echo, tilt table, Holler monitoring), neurologic (MRI brain, carotid US), and neoplastic (CT chest and abdomen and paraneoplastic panel) workup. During the current admission, C1 esterase deficiency, reactive mastocytosis, and carcinoid syndrome were ruled out. Intense polyuria prompted a water deprivation test to rule out Dl and an ACTH stimulation test, both negative. The clinical history was suggestive of massive fluid shifts out of the intravascular space, and a literature review revealed an association of MGUS with systemic capillary leak syndrome (SCLS). She fit the clinical picture and in view of the exlensive negative workup was started on theophylline and lerbutaline with a reduction in duration and seventy of a subsequent episode.

Discussion:

SCLS (Clarkson disease) is a rare disease characterized by the triad of hypotension, hemoconcentralion, and hypoalbuminemia associated with monoclonal gammopathy (without evidence of MM). The exact pathogenesis is unknown, but vascular permeability appears to be altered because of complement, interleukin, or monoclonal protein mediation. In the capillary leak phase, fluid and macromolecules, including albumin, extravasate out into the extracellular tissues. This is followed by a recruitment phase, where the fluid is reabsorbed with the potential of fluid ovedoad and pulmonary edema. This condition has been historically associated with a poor outcome, likely because of lack of awareness. Recognizing SCLS early can abort or reduce attack severity, thus reducing resource utilization and patient morbidity.

Conclusions:

Systemic capillary leak syndrome is a rare but potentially fatal disease associated with MGUS. Prompt recognition of this diagnosis by hospitalists can improve patient morbidity and reduce critical care resource utilization

Author Disclosure:

M. C. Alraies, none; B. Harte, none; C. Whinney, none: M. Patel, none.

To cite this abstract:

Alraies M, Patel M, Harte B, Whinney C. A Lesser‐Known Cause of Recurrent Hypovolemic Shock. Abstract published at Hospital Medicine 2010, April 8-11, Washington, D.C. Abstract 209. Journal of Hospital Medicine. 2010; 5 (suppl 1). https://www.shmabstracts.com/abstract/a-lesserknown-cause-of-recurrent-hypovolemic-shock/. Accessed October 18, 2019.

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