Which are the causes of Systemic Capillary Leak Syndrome?

Systemic Capillary Leak Syndrome (SCLS) is a rare and potentially life-threatening condition characterized by recurrent episodes of severe hypotension, hypoalbuminemia, and hemoconcentration. The exact cause of SCLS is not fully understood, but several factors have been identified as potential triggers for this syndrome.

1. Idiopathic: In many cases, the cause of SCLS is unknown, and it is referred to as idiopathic SCLS. This means that the syndrome occurs spontaneously without any identifiable underlying cause.

2. Infections: Certain infections, such as viral or bacterial infections, have been associated with the development of SCLS. It is believed that the infection triggers an immune response that leads to increased capillary permeability and fluid leakage.

3. Allergic reactions: Some individuals may develop SCLS as a result of severe allergic reactions, such as anaphylaxis. The release of inflammatory mediators during an allergic reaction can disrupt the integrity of the capillary walls, leading to fluid leakage.

4. Medications: Certain medications have been implicated in the development of SCLS. These include interleukin-2, which is used in cancer immunotherapy, and some antibiotics. It is thought that these medications directly affect the endothelial cells lining the capillaries, causing increased permeability.

5. Autoimmune disorders: SCLS has been reported in association with autoimmune disorders, such as systemic lupus erythematosus and rheumatoid arthritis. The immune dysregulation in these conditions may contribute to the development of SCLS.

6. Genetic predisposition: Although rare, there have been reports of familial cases of SCLS, suggesting a genetic component to the syndrome. However, the specific genes involved have not yet been identified.

7. Other factors: Other potential triggers for SCLS include physical exertion, emotional stress, and exposure to extreme temperatures. These factors may induce a systemic inflammatory response, leading to capillary leakage.

It is important to note that while these factors may contribute to the development of SCLS, the exact mechanisms underlying the syndrome are still not fully understood. Further research is needed to elucidate the pathophysiology of SCLS and identify effective treatment strategies.