SHM Converge Scientific Abstract ePoster Gallery-A Leaky Case of Syncope

Pdf Summary

This case describes a 76-year-old man with known systemic capillary leak syndrome (SCLS), monoclonal gammopathy of undetermined significance (MGUS), and other chronic conditions who presented after syncope and a fall with profound hypotension. Initial workup showed low albumin, elevated lactate, and later hemoconcentration with a hemoglobin peak of 18.5 g/dL, while imaging and cortisol testing were unrevealing. Although urinalysis and culture indicated a multidrug-resistant E. coli urinary tract infection, his clinical pattern was most consistent with an acute SCLS attack.<br /><br />SCLS, also called Clarkson’s disease, is a rare disorder characterized by the triad of hypotension, hemoconcentration, and hypoalbuminemia due to sudden plasma leakage into the extravascular space. It is a diagnosis of exclusion, so other causes of shock or edema such as sepsis, heart failure, anaphylaxis, and hereditary angioedema must be ruled out. MGUS is a supportive finding often associated with SCLS.<br /><br />The patient improved after aggressive IV crystalloid resuscitation, IV albumin, and IV dexamethasone. He was then treated with terbutaline, theophylline, cetirizine, and continued fluids in the ICU. After cultures returned, he received ertapenem, transitioned to oral antibiotics, and was given IVIG before discharge. His blood pressure, hemoglobin, and albumin normalized.<br /><br />Key management points include treating acute attacks with IV fluids and corticosteroids, and preventing recurrences with monthly IVIG. Theophylline and terbutaline may also be used prophylactically because they increase intracellular cyclic AMP. Recognizing SCLS early is essential because delayed diagnosis can lead to serious complications such as multiorgan dysfunction, compartment syndrome, and thrombosis.

Asset Subtitle

Evan Raff

Meta Tag

Author List Evan Raff, Hannah Pollock, Meg Johnson, Rachel Lile-King

Category Clinical Vignettes

Concept Systemic Capillary Leak Syndrome

Concept Hypotension

Concept Hemoconcentration

Concept Fluid Resuscitation

Concept Hypoalbuminemia

Distinguished Non-Finalist

Presenter Organization University of North Carolina School of Medicine

Presenting Author Evan Raff

Track Adult

Keywords

systemic capillary leak syndrome

Clarkson disease

hypotension

hemoconcentration

hypoalbuminemia

monoclonal gammopathy

IVIG

dexamethasone

terbutaline

theophylline

Systemic Capillary Leak Syndrome

Hypotension

Hemoconcentration

Fluid Resuscitation

Hypoalbuminemia