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Updated: Jun 21, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Idiopathic capillary leak syndrome].
1Service de médecine interne, Centre national de références maladies rares, hôpital de la Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France.
La Revue De Medecine Interne
|July 21, 2009
Summary
Idiopathic capillary leak syndrome (ICLS) is a rare condition with a poor prognosis. Intravenous immunoglobulins show promise in reducing ICLS attack frequency and severity, offering new hope for patients.
Area of Science:
- Rare diseases
- Hematology
- Critical care medicine
Background:
- Idiopathic capillary leak syndrome (ICLS) is a rare, poorly understood condition.
- Approximately 100 cases reported since 1960; affects middle-aged adults.
- ICLS has a poor prognosis with a 10-year mortality rate of 34%.
Purpose of the Study:
- To improve understanding of ICLS natural course and therapeutic challenges.
- To evaluate the effectiveness of prophylactic treatments for ICLS.
- To emphasize the importance of patient registries for ICLS research.
Main Methods:
- Analysis of data from a French ICLS patient register initiated in 1997.
- Review of diagnostic criteria including hypotension, hemoconcentration, and hypoalbuminemia.
- Assessment of treatment outcomes, including symptomatic management and prophylactic measures.
Main Results:
- Monoclonal gammopathy present in approximately 80% of ICLS patients.
- Severe crises and ICU complications contribute to 80% of ICLS mortality.
- Early data suggest intravenous immunoglobulins are more effective than beta-2 stimulants for ICLS prophylaxis.
Conclusions:
- ICLS management requires cautious fluid infusion and patient education.
- Intravenous immunoglobulins demonstrate superior efficacy in reducing ICLS attack frequency and severity.
- Patient registries are crucial for advancing ICLS etiology and treatment knowledge.
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