# A rare cause of shock in cases of refractory hypotension, hypoproteinaemia and haemoconcentration

## TL;DR

A woman experienced frequent episodes of severe hypotension and related symptoms, later diagnosed with a rare condition called idiopathic systemic capillary leak syndrome.

## Contribution

The paper presents a rare case of ISCLS with an unusually high frequency of episodes and identifies multiple triggers.

## Key findings

- The patient had 34 ISCLS episodes over nearly 6 years, an unusually high frequency.
- Triggers included infections like COVID-19 and influenza, as well as vaccinations.
- Intravenous immunoglobulins were effective for both preventing and treating ISCLS episodes.

## Abstract

This case report describes a woman in her mid-40s of European ancestry presenting with recurrent profound hypotension, hypoproteinaemia and haemoconcentration. She first presented following administration of a Japanese encephalitis vaccine. The initial differential diagnoses included sepsis or an anaphylactic reaction to the vaccine. No evidence of allergy or infection was found during extensive investigation including a lumbar puncture. Following a second similar presentation, she was diagnosed with idiopathic systemic capillary leak syndrome (ISCLS). Her presentation was highly atypical due to the high frequency of episodes encountered (34 over a near 6-year period). A variety of triggers for episodes were identified including COVID-19 infections, influenza infection and vaccinations. We discuss her clinical presentation, investigations, ISCLS episode triggers and management. This highlights the benefits of intravenous immunoglobulins (IVIg), both for prophylaxis and for the acute treatment of ISCLS episodes.