Systemic Capillary Leak Syndrome Details

Expanded Description

Systemic Capillary Leak Syndrome (SCLS) is an exceedingly rare, life- and limb-threatening disorder characterized by acute and severe recurrent attacks featuring a rapid fall in blood pressure due to the temporary leak of plasma out of the blood circulatory system.

The episodes often last several days and require emergency and intensive care in a hospital setting. Identification and management of these episodes has improved in recent years and thus the survival rate has increased, but sometimes they still prove fatal depending on what medications are administered in what sequence and dosage, and depending on how the vital organs can cope (particularly the heart, lungs and kidney). SCLS is very rare in children and there is no evidence of it being hereditary.

Prevalence

1000.0 ( Cases ) [Source]

Causes

Systemic Capillary Leak Syndrome (SCLS) is idiopathic, and thus at present there are no known causes. Probably a mid-life gene mutation takes place that renders those affected vulnerable (possibly immune-deficient in some way) to these curiously self-reversing capillary leaks. The literature reports exercise, upper respiratory infections and hormones as possible influencing factors. Stress sometimes appears to trigger episodes of SCLS.

Disorder Symptoms

Many patients report having a runny nose, flu-like symptoms, gastro-intestinal disorders, or a general weakness or pain in their limbs, but others get no particular or consistent warning signs.

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
Hypoalbuminemia	Hypoalbuminemia 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
Fatigue	Extreme loss of energy

Diagnosis

Systemic Capillary Leak Syndrome is diagnosed based on symptoms and the presence of an unusual protein called a Monoclonal Gammopathy of Unknown Significance (MGUS) in most patients. Symptoms for diagnosis are hypotension, hemoconcentration, hypualbuminemia and the presence of the 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 as a result of overaggressive hydration.

Name
Lab test for hemoconcentration
Portable, instantaneous test for hemoconcentration

Disorder Treatments

• Methylprednisolone 125 mg IV STAT, repeated as needed.

• Judicious use of IVF boluses and drips to keep CVP above zero.

• Phenylephrine or Norepinephrine for hypotension, early institution.

• 50 ml of 25% albumin, repeated as needed.

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

• Immediate Orthopedics consult and compartment pressure measurement; early, preventive limb fasciotomies if compartment pressures or CPK high.

• Venous Doppler for DVT, may need full anticoagulation.

Treatment of a fully developed SCLS episode requires recognition that there are two phases. The first phase, which often lasts a couple of days, is called the resuscitation phase because the dual aim of ER/ICU treatment is to stop or control the capillary leak and to raise the patient’s blood pressure from near zero.

In this initial phase, an albumin and fluid leak from the capillaries into the tissue spaces causes swelling, especially into the extremities rather than the abdomen or organs (such as the lungs). The blood pressure falls and the red cells concentrate. This loss of fluid has similar effects on the circulation as dehydration, slowing both the flow of oxygen carrying blood to tissues and the output of urine.

Glucocorticoids (steroids like methylprednisolone) are recommended to reduce or stop the capillary leak, and albumin and colloids usually help to increase the remaining blood flow to vital organs like the kidneys. Keeping up with the fluid loss is important because sustained low blood pressure can damage vital organs such as the kidneys.

Even though blood pressure readings may reach and remain at very low levels, it is important to avoid overly aggressive intravenous fluid administration causing massive swelling of the extremities. The goal of saline and vasopressors administered should NOT be to restore a “normal” blood pressure (or urine flow), but to maintain it at a minimal level sufficient to avoid permanent damage to vital organs.

Measurement of central venous or arterial pressure in an ICU setting is often necessary to achieve this delicate balance. When too much fluid is administered, the result is excessive swelling, and the patient may well require surgical decompression of the limbs. In this procedure, known as a fasciotomy, the skin of the arms and/or legs is incised to release the compressive pressure the retained fluid is having on blood flow to and from the extremities.

The second phase of the treatment is known as the recruitment phase, when fluids and albumin are reabsorbed from the tissues during at least a couple of days. In this phase, the capillary leak has ended and the main threat is fluid overload. If intravenous fluids were given in excess, they usually cause an accumulation of fluid in the lungs and around other vital organs. Most of the patient deaths happen during this recruitment phase so it is important that diuretics be administered to help patients discharge all the fluid previously given — and to keep them from backing up, especially into the lungs.

Name
IVIG monthly infusions

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, email greipp.philip@mayo.edu; in Europe, Dr. Zahir Amoura, Département de Médicine Interne, Hospitalier Pitié-Salpêtrière, Paris, tel. 0142-17- 8001 or 8003, email zahir.amoura@psl.aphp.fr.