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. 2020 Feb 17;4(2):1-5.
doi: 10.1093/ehjcr/ytaa013. eCollection 2020 Apr.

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

Habib R Khan  1   2   3 , Saima Khan  1 , Asha Srikanth  1 , William H T Smith  1
Affiliations

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

Habib R Khan et al. Eur Heart J Case Rep. .

Abstract

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.

Keywords: Case report; Clarkson’s disease; Pericardial effusion; Pleural effusion; Systemic capillary leak syndrome.

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Figures

Figure 1
Figure 1
Chest X-ray in AP showing bilateral pleural effusions.
Figure 2
Figure 2
Parasternal short axis (A) and subcostal view (B) showing features of cardiac tamponade during early diastolic phase. Pericardial effusion measuring 2.9 cm (1), collapsed right ventricle (2), compressed right atrium (3), left ventricle (4), and left atrium (LA).
None
See this image and copyright information in PMC

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