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Systemic Capillary Leak Syndrome (Clarkson Disease)

Recurrent Episodes of Hypovolemic Shock and Hemoconcentration

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is Systemic Capillary Leak Syndrome (Clarkson Disease)?

Systemic capillary leak syndrome (SCLS), also known as Clarkson disease, is a rare disorder characterized by recurrent episodes of severe plasma extravasation from the intravascular to the interstitial compartment.

Each episode comprises three phases: prodrome (flu-like symptoms), leak phase (hypovolemia, hemoconcentration, hypoalbuminemia), and recovery (massive fluid mobilization with risk of pulmonary edema).

Strongly associated with monoclonal gammopathy of undetermined significance (MGUS), most commonly IgG kappa, in 70–85% of adult cases.

Diagnosis based on the classic triad of hypotension, hemoconcentration (hematocrit elevation), and hypoalbuminemia in the absence of sepsis, anaphylaxis, or other secondary causes.

Symptoms

Prodromal symptoms: fatigue, myalgia, abdominal pain, nausea, lightheadedness, or upper respiratory infection 1–2 days before attack
Sudden severe hypotension with shock and end-organ hypoperfusion
Marked hemoconcentration with hematocrit often >55% and elevated total protein paradoxically
Generalized edema, anasarca, abdominal distension, and weight gain during recovery phase
Compartment syndromes (especially limb) requiring fasciotomy in severe leak
Pulmonary edema and respiratory failure during fluid mobilization phase
Acute kidney injury, rhabdomyolysis, and ischemic complications

Risk Factors

Monoclonal gammopathy of undetermined significance (MGUS), particularly IgG kappa
Adults aged 30–60 years (median onset 50 years)
No clear sex predominance
Viral infections often act as triggers
Stress, exertion, or menstrual cycle as reported triggers
Pediatric SCLS (rare) often without monoclonal gammopathy

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Acute hypotension, edema, and hemoconcentration of unknown cause
  • Recurrent unexplained shock-like episodes with full recovery between attacks
  • Known SCLS patient with prodromal symptoms (immediate emergency presentation)
  • Patient with MGUS developing episodic edema or hypotension
  • New severe edema or oliguria during a leak attack

Treatment Methods

01
Acute leak phase management: cautious crystalloid resuscitation guided by hemodynamics — over-resuscitation worsens recovery-phase pulmonary edema and compartment syndromes
02
Vasopressors (norepinephrine) and inotropes for refractory shock; ICU admission with invasive hemodynamic monitoring
03
Albumin or hyperoncotic colloids may be considered in selected cases but evidence is limited
04
Recovery-phase management: diuretics (furosemide) for fluid mobilization, careful monitoring for pulmonary edema and compartment syndromes
05
Prophylactic intravenous immunoglobulin (IVIG) 2 g/kg/month is the cornerstone preventive therapy and dramatically reduces attack frequency
06
Theophylline plus terbutaline historically used as prophylaxis; less effective than IVIG
07
Avoidance of triggers when identified; surveillance and management of underlying MGUS for progression to multiple myeloma
08
Patient education on early recognition of prodromal symptoms and access to emergency care plan with medical alert documentation

Which Department to Visit?

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