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Capillary Leak Syndrome Manifesting With Lumbar Pa ...
Capillary Leak Syndrome Manifesting With Lumbar Pain and Renal Injury
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This case report describes a 66-year-old woman who presented with 2 weeks of severe lumbar back pain and was later found to have systemic capillary leak syndrome (SCLS), also known as Clarkson’s disease. SCLS is a very rare disorder, with fewer than 300 cases reported worldwide, caused by abnormal vascular permeability leading to leakage of fluid and proteins into the interstitial space. Its classic triad is hypotension, hemoconcentration, and hypoalbuminemia.<br /><br />The patient initially had back pain radiating to both thighs and only mild hypotension and tachycardia. Imaging showed degenerative lumbar spine disease, but her course rapidly worsened. By hospital day 2, she developed acute kidney injury that progressed despite aggressive hydration and albumin replacement. She later developed severe abdominal pain, worsening hypoalbuminemia, oliguria, generalized edema, metabolic acidosis, and fulminant liver failure. Blood and urine cultures were negative, helping exclude sepsis. She was also treated for possible adrenal insufficiency. As her condition deteriorated, she developed altered mental status and shortness of breath, requiring intubation. Despite multiple vasopressors, she progressed to hypoxic respiratory failure, refractory shock, multiorgan failure, and died 10 days after admission.<br /><br />The authors emphasize that diagnosis of SCLS is often delayed because it is rare and can mimic sepsis, anaphylaxis, or medication reactions. This case was unusual because the presenting symptom was severe lumbar pain, which had not previously been reported as a prodromal symptom of SCLS. The report highlights the importance of recognizing the triad of hypotension, hemoconcentration, and hypoalbuminemia early, as delayed diagnosis is associated with poor outcomes and high mortality.
Asset Subtitle
Tina J. Kana
Meta Tag
Author List
Ahmed Kawamj, Tina J. Kana, Vamsee Reddy, Zainab Shamim
Category
Clinical Vignettes
Concept
Systemic Capillary Leak Syndrome
Concept
Hypotension
Concept
Hemoconcentration
Concept
Hypoalbuminemia
Concept
Multiorgan failure
Distinguished
Non-Finalist
Presenter Organization
Lenox Hill Hospital
Presenting Author
Tina J. Kana
Track
Adult
Keywords
systemic capillary leak syndrome
Clarkson disease
hypotension
hemoconcentration
hypoalbuminemia
lumbar back pain
acute kidney injury
multiorgan failure
vascular permeability
septic shock mimic
Systemic Capillary Leak Syndrome
Hypotension
Hemoconcentration
Hypoalbuminemia
Multiorgan failure
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