Which are the symptoms of Systemic Capillary Leak Syndrome?

Symptoms of Systemic Capillary Leak Syndrome

Systemic Capillary Leak Syndrome (SCLS), also known as Clarkson's disease or Idiopathic Systemic Capillary Leak Syndrome, is a rare and potentially life-threatening condition characterized by recurrent episodes of severe fluid and protein leakage from the blood vessels into surrounding tissues. This leakage leads to a sudden and significant drop in blood volume, resulting in a condition called hypovolemic shock.

1. Acute Onset: One of the key features of SCLS is the sudden and unpredictable onset of symptoms. Patients typically experience episodes of capillary leak that can last for a few hours to several days. The frequency and duration of these episodes can vary greatly between individuals.

2. Hypotension: During an episode, the leakage of fluid and proteins from the blood vessels causes a rapid decrease in blood volume. This leads to a drop in blood pressure, resulting in hypotension. Hypotension can cause symptoms such as dizziness, lightheadedness, fainting, and in severe cases, shock.

3. Edema: The leakage of fluid into the surrounding tissues causes swelling, known as edema. Edema can occur in various parts of the body, including the limbs, face, abdomen, and even the lungs. Swelling may be mild to severe, and it can be painful and restrict movement.

4. Hemoconcentration: As fluid leaks out of the blood vessels, the concentration of red blood cells and other blood components increases, leading to hemoconcentration. This can result in an elevated hematocrit level, indicating a higher proportion of red blood cells in the blood.

5. Hypoproteinemia: The loss of proteins, such as albumin, from the blood vessels during capillary leak episodes can lead to a decrease in the overall protein levels in the blood. This condition is known as hypoproteinemia. Low protein levels can cause fluid to accumulate in the tissues, contributing to edema.

6. Generalized Weakness and Fatigue: The combination of hypovolemia, hypotension, and hemoconcentration can result in generalized weakness and fatigue during and after capillary leak episodes. Patients may feel extremely tired and have difficulty performing daily activities.

7. Abdominal Pain: Some individuals with SCLS may experience abdominal pain during capillary leak episodes. This pain can be diffuse or localized and may be associated with bloating and discomfort.

8. Anasarca: In severe cases, SCLS can lead to a condition called anasarca, which is generalized edema involving the whole body. Anasarca can cause significant swelling, weight gain, and difficulty breathing.

9. Organ Dysfunction: Prolonged or recurrent episodes of capillary leak can potentially lead to organ dysfunction. This can manifest as impaired kidney function, liver abnormalities, respiratory distress, and cardiovascular complications.

10. Flu-like Symptoms: Prior to the onset of a capillary leak episode, some patients may experience flu-like symptoms such as malaise, muscle aches, headache, and low-grade fever. These symptoms can serve as warning signs for an impending episode.

It is important to note that the symptoms of SCLS can vary in severity and may not be present in every episode. Prompt recognition and medical intervention are crucial to manage the condition and prevent life-threatening complications.