How is Systemic Capillary Leak Syndrome diagnosed?

Systemic Capillary Leak Syndrome (SCLS) is a rare and potentially life-threatening condition characterized by recurrent episodes of severe hypotension, hypoalbuminemia, and hemoconcentration. Diagnosing SCLS can be challenging due to its rarity and similarity to other conditions. However, a combination of clinical evaluation, laboratory tests, and imaging studies can help in reaching a diagnosis.

Clinical evaluation: The first step in diagnosing SCLS involves a thorough assessment of the patient's medical history and physical examination. The doctor will look for symptoms such as sudden onset of edema, hypotension, and hemoconcentration during an acute episode. They will also inquire about any previous episodes and family history of similar symptoms.

Laboratory tests: Several laboratory tests can aid in the diagnosis of SCLS. These include:

• Complete blood count (CBC): This test helps identify hemoconcentration, which is a characteristic feature of SCLS. It may show an increased hematocrit level.


• Electrolyte panel: Abnormalities in electrolyte levels, such as low sodium and high creatinine, may be observed during an acute episode.


• Liver function tests: These tests can help assess liver involvement, as SCLS can cause liver dysfunction.


• Measurement of serum albumin: Hypoalbuminemia, or low levels of albumin in the blood, is a hallmark of SCLS.


• Measurement of serum protein: A decrease in total serum protein levels may be observed during an acute episode.


• Measurement of serum tryptase: Elevated levels of tryptase, a marker of mast cell activation, may be present in some cases.

Imaging studies: Imaging studies can provide additional information to support the diagnosis of SCLS. These may include:

• Chest X-ray: This can help rule out other causes of respiratory symptoms and identify potential complications like pleural effusion.


• Ultrasound: Abdominal ultrasound can reveal ascites (fluid accumulation in the abdomen) or hepatomegaly (enlarged liver).


• Magnetic resonance imaging (MRI) or computed tomography (CT) scan: These imaging techniques may be used to assess organ involvement and detect potential complications.

It is important to note that the diagnosis of SCLS is often made based on a combination of clinical findings and exclusion of other possible causes. Therefore, consultation with specialists, such as hematologists or immunologists, may be necessary to confirm the diagnosis and guide appropriate management.