Systemic capillary leak syndrome - an autoimmune response resulting in drowning from within.

Habib R Khan, Saima Khan, Asha Srikanth, William H T Smith

Overview

A syndrome associated with leakage of intravascular fluid into third space with the most significant findings of recurrent pericardial effusion and pleural effusions without a specific cause.

Summary

The diagnosis of systemic capillary leak syndrome or Clarkson's disease is usually many months or years after original presentation. The patients go through multiple admissions with life-threatening presentations such as cardiac tamponade before the diagnosis is made. Timely treatment for this autoimmune condition can result in significant improvements in patient's quality of life, but also prevent hospitalisations and reduce admission and unnecessary investigation costs.

Author Comments

Dr Habib Khan, MD
Dr Habib Khan, MD
Imperial College London
Electrophysiology
United Kingdom
This case report presents a case of real-world experience of a patient with multiple emergency admissions due to cardiac tamponade. Repeat drainage of effusions failed and only upon the diagnosis, and treatment with steroids-immunotherapy was the patient able to improve and do well more than a year into follow up. Dr Habib Khan, MD

Resources

A case report of capillary leak syndrome with recurrent pericardial and pleural effusions
https://www.researchgate.net/publication/339314720_A_case_report_of_capillary_leak_syndrome_with_recurrent_pericardial_and_pleural_effusions
European Heart Journal - Case Reports
https://academic.oup.com/ehjcr/article/4/2/1/5739520

A case report of capillary leak syndrome with recurrent pericardial and pleural effusions.

Authors:
Dr Habib Khan, MD
Dr Habib Khan, MD
Imperial College London
Electrophysiology
United Kingdom

Eur Heart J Case Rep 2020 Apr 17;4(2):1-5. Epub 2020 Feb 17.

Trent Cardiac Centre, Nottingham University Hospitals, City Hospital Campus, Hucknall Road, Nottingham NG51PB, UK.

Background: Capillary leak syndrome (CLS) is a rare connective tissue disease, triggered by the leak of serous fluid into the interstitial spaces, characterized by a hallmark of oedema and effusions in confined spaces. The limiting factor in CLS management appears to be its diagnosis rather than treatment, which is usually to contain the disease progression rather than a cure.

Case Summary: We report a case of a 51-year-old woman with recurrent life-threatening presentations of pericardial effusions, pleural effusions, and generalized swelling of face and extremities. The only notable past medical history was of Type 1 diabetes. Numerous investigations did not lead to specific disease accounting for pericardial effusions and pleural effusions. Eventually, the diagnosis of CLS was made based on hypovolaemic shock, hypoalbuminaemia, and haemoconcentration without the presence of albuminuria. She was managed with steroids to reduce system inflammation and later with immunoglobulins and tumour necrosis factor to contain the disease process. Since her diagnosis and subsequent appropriate management, she has not had further admissions with cardiac tamponade 16 months of follow-up.

Discussion: The diagnosis of CLS is difficult to make unless there is a high degree of suspicion and until other causes have been ruled out. It remains a challenging condition to manage as the treatment options are limited and patients recurrently present with emergencies until the correct diagnosis is made and the optimal treatment is provided.

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Source
http://dx.doi.org/10.1093/ehjcr/ytaa013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7180544PMC
April 2020

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