Systemic Capillary Leak Syndrome Details

Expanded Description

Systemic Capillary Leak Syndrome (SCLS) is a rare acquired disorder characterized by acute and severe recurrent attacks associated with a rapid fall in blood pressure. Attacks often last several days and require emergency care. They are sometimes fatal. SCLS occurs most often in adults and the disease is very rare in children. SCLS is not hereditary.

Prevalence

100.0 ( Cases ) [Source]

Causes

Systemic Capillary Leak Syndrome is idiopathic, and thus at present there are no known causes. The literature reports exercise, upper respiratory infections and hormones as possible influencing factors. From personal experience, stress affects SCLS.

Disorder Symptoms

There are several symptoms that affect people with Systemic Capillary Leak Syndrome.

Name	Description
Swelling	swelling
Myalgia	Myalgia is muscle pain
Rhinorrhea	Rhinorrhea is a runny nose
Dizziness	Dizziness
Lightheadedness	Lightheadedness
Hypotension	Hypotension is abnormally low blood pressure
Hemoconcentration	Hemoconcentration is the decrease of the fluid content of the blood, with increased concentration of formed elements
Hypoalbunemia	Hypoalbunemia is low levels of protein in the blood
Nausea	Nausea
Excessive thirst	Excessive thirst
Generalized edema	Generalized edema
Decline in clinical picture	Clinical picture declines rapidly within hours
Cold limbs and sweating	Cold limbs and sweating
Rapid swelling and compartment syndrome	Rapid swelling of all limbs with development of compartment syndrome, especially during IV fluid administration
Decreased urine output	Decreased urine output
Acute attack	Acute attack sometimes preceded by runny nose, flu-like symptoms
Vomiting	Usually caused by severe nausea
Intestinal cramps	Cramps
Diarhea	Frequent loose stools

Diagnosis

Systemic Capillary Leak Syndrome is diagnosed based on symptoms and the majority occurrence of monoclonal gammopathy of unknown significance (MGUS) in patients.

Symptoms for diagnosis are hypotension, hemoconcentration, hypualbuminemia and presence of a paraprotein (MGUS)

Diagnostic Tests

• Elevated hemoglobin due to hemoconcentration (can be > 20 gm/dl)

• Decreased serum albumin

• Increase in creatine kinase (CPK) levels with marked edema and compartment syndrome

• Although creatinine may increase, overt renal failure is not common

• Chest X-ray may show fluid retention, but lungs are relatively spared except with overaggressive hydration

Name
Lab test for hemoconcentration

Disorder Treatments

• Continuous CVP monitoring, stat and serial lab work including CPK, lactate

• Judicious use of IVF boluses and drips to keep CVP 8-14

• Phenylephrine or Norepinephrine for hypotension, early institution

• Hydrocortisone 100 mg IV Q 8 hours

• Immediate Orthopedics consult and compartment pressure measurement

• Hematology consult for RBC exchange or plasmapheresis

• Venous Doppler for DVT, may need full anticoagulation

• Early, preventive limb fasciotomies if compartment pressures or CPK high

Treatment of a fully developed SCLS episode requires recognition that there are two phases of the acute attack. The first phase, which often lasts several days, is called the resuscitation phase aimed at controlling the capillary leak and maintaining blood pressure. In that phase an albumin and fluid leak from the capillaries into the tissue spaces causes swelling. This loss of fluid has similar effects on the circulation as dehydration, slowing the flow of oxygen carrying blood to tissues. The blood pressure falls and the red cells concentrate. Intravenous fluid replacement is usually required. In most cases intravenous fluids must be administered immediately and in high-volume in order to prevent excessive drops in blood pressure. Intravenous albumin and colloid may be used. Keeping up with the fluid loss is important because sustained low blood pressure can damage vital organs such as the kidneys.

The second phase of the treatment is sometimes called the recruitment phase as fluids and albumin are reabsorbed from the tissues. In this phase the capillary leak has abated and the main threat is fluid overload. Even though the blood pressure may still be low, it is important to avoid overly aggressive intravenous fluid administration causing massive swelling of the extremities requiring surgical decompression. In this procedure the skin of the arm or leg is incised to release the compressive pressure the retained fluid is having on blood flow to and from the extremities. Excessive intravenous fluids may also cause accumulation of fluid in the lungs and around other vital organs. Many of the deaths happen during this recruitment phase. The goal during the acute phase is NOT to attempt to maintain absolutely normal blood pressure or urine flow but to maintain the blood pressure at just sufficiently high enough levels to avoid permanent damage to vital organs yet spare the patient from the risks of excess fluid administration. Measurement of central venous or arterial pressure in an intensive care unit is often necessary to achieve this delicate balance. Diuretics may be required for excess fluid overload.

Glucocorticoids (steroids) are often used during the acute attack especially early in the recruitment phase in an attempt to reduce the capillary leak. Albumin and colloids administered with the intravenous fluids may have temporary benefit to increase blood flow to vital organs like the kidneys.

We don't have any treatments yet.

Prognosis

No prognosis information has been added yet

Tips for Living with the Disorder

Name

Description

Medical help

Find yourself a compassionate physician, preferably a specialist in internal medicine or hematology affiliated with a major university hospital, willing to do his/her homework on this rare disorder (namely, read the literature and follow the instructions), and willing to consult with the two SCLS experts available: in the United States, Dr. Philip R. Greipp, Professor of Medicine and Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, Minnesota, tel. 507 284-9094; in Europe, Dr. Zahir Amoura, Département de Médicine Interne, Hospitalier Pitié-Salpêtrière, Paris, tel. 0142 17 8003.