[Systemic capillary leak syndrome: A case of severe presentation and treatment response]

An Sist Sanit Navar. 2025 Oct 16;48(3):e1141. doi: 10.23938/ASSN.1141.
[Article in Spanish]

Abstract

Systemic capillary leak syndrome is a rare disorder, first described in 1960, characterized by recurrent episodes of shock, hemoconcentration, and hypoalbuminemia. Diagnosis is primarily clinical, and management includes supportive care and immunoglobulin therapy. Given its potentially life-threatening course, early recognition and timely treatment are essential to improving outcomes. We report the case of a 48-year-old man with recurrent episodes of shock, hemoconcentration, and hypoalbuminemia, initially misdiagnosed as sepsis. He required intensive care management and underwent fasciotomies of all four extremities due to compartment syndrome. During the second hospitalization, he was correctly diagnosed with systemic capillary leak syndrome. Treatment with intravenous immunoglobulins, theophylline, and salbutamol was initiated, leading to a sustained favorable clinical response to date.

El síndrome de fuga capilar sistémica es una enfermedad rara, descrita por primera vez en 1960, caracterizada por episodios recurrentes de shock, hemoconcentración e hipoalbuminemia. Su diagnóstico es clínico y su manejo incluye medidas de soporte y tratamiento con inmunoglobulinas. Se trata de un cuadro potencialmente mortal, por lo que su diagnóstico precoz y un adecuado manejo resulta fundamental para mejorar el pronóstico.

Se presenta el caso de un varón de 48 años con episodios de shock, hemoconcentración e hipoalbuminemia, inicialmente sugestivo de sepsis. Requirió tratamiento intensivo y fasciotomías de las cuatro extremidades por síndrome compartimental, siendo diagnosticado de síndrome de fuga capilar sistémica tras un segundo ingreso. Se instauró tratamiento con inmunoglobulinas, teofilina y salbutamol con buena respuesta hasta la actualidad.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Capillary Leak Syndrome* / diagnosis
  • Capillary Leak Syndrome* / therapy
  • Humans
  • Male
  • Middle Aged
  • Severity of Illness Index
  • Treatment Outcome