Definition

Primary capillary leak syndrome is rare, but causes episodes of severe swelling with fluid retention, especially around the muscles of the arms and legs. Also, symptoms from the lungs, heart bag (pericardial fluid), kidneys and nervous system may occur. Symptoms often develop over 1-3 days.

• The disease occurs in its own disease (primarily capillary leak syndrome) or related (secondary) to other conditions, including Systemic connective tissue diseases:

Disease Causes

The disease mechanism is that fluid from the blood (plasma) penetrates through the vein walls and into the connective tissue. Thus swelling with fluid accumulation occurs.

• The condition may occur in severe course of Systemic connective tissue diseases: that Myositis (including at dermatomyositis in children), blood poisoning (Sepsis), Macrophage activation syndrome (MAS), Poisoning, Snakebite, Cancer with more.

Wrong diagnosis (Differential diagnoses)

• Allergic reaction
• Eosinophilic myositis
• Hypokomplementary vasculitis / urcticular vasculitis
• Lipedema (fat accumulation)
• Lymphoedema
• Kidney failure (Acute)
• RS3PE
• Early systemic sclerosis (diffuse form)

Literature

• Druey KM, 2017

• Bindevevssykdommer.no
• Vaskulitt.no