Rare Diseases Symptoms Automatic Extraction

[Recurrent unexplained lipothymia: do not forget the capillary leak syndrome].

[systemic capillary leak syndrome]

Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent spontaneous episodes of hypovolaemic shock due to marked plasma shifts from the intravascular to the extravascular space. It presents as the characteristic triad of hypotension, haemoconcentration and hypoalbuminemia.We describe a patient with SCLS with recurrent lipothymia who presented first with delayed oedema that was thought to be due to orlistat treatment. On the second episode the patient was seen with a pulmonary hypertension when plasma came back into vessels. On the third time the characteristic triad led to the diagnosis of SCLS.SCLS should be considered in the differential diagnosis of recurrent hypovolemic shock without identifiable cause. Nevertheless, symptoms may be restricted to sole lipothymia or transient oedema or delayed hypoalbuminemia rendering the diagnosis difficult.

Diseases presenting "pulmonary hypertension" symptom

  • acute rheumatic fever
  • alpha-thalassemia
  • cohen syndrome
  • congenital diaphragmatic hernia
  • heparin-induced thrombocytopenia
  • homocystinuria without methylmalonic aciduria
  • lymphangioleiomyomatosis
  • malignant atrophic papulosis
  • systemic capillary leak syndrome
  • waldenström macroglobulinemia

This symptom has already been validated