Introduction

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Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disorder characterized by episodes of severe hypotension, hypoalbuminemia, and hemoconcentration [1,2]. During "attacks" of ISCLS, profound derangement of the vascular endothelium results in leakage of plasma and proteins into the interstitial compartment [1,2]. Episodes vary in severity and frequency and may be fatal. ISCLS was first described by Clarkson in 1960 and is variably referred to as Clarkson's disease or syndrome.
Epidemiology
Approximately 150 cases of ISCLS have been reported worldwide [3,4]. These have been diagnosed primarily in middle aged adults, although cases in children as young as five months old have been reported [5-8]. There is no apparent gender predilection.
Pathogenesis
The vascular endothelium is a semi-permeable barrier that controls the passage of fluid and macromolecules between the intravascular and interstitial spaces. Dysfunction of this barrier leads to leakage, with loss of intravascular fluid and protein into the interstitial compartment. The resulting intravascular fluid depletion can cause hypotension and impair the delivery of oxygen to the tissues. The capillary leak is often severe and results in significant hypotension and shock. Cells and platelets are generally retained within the vasculature, resulting in elevations in white cell, red blood cell, and platelet counts.
There are many causes of capillary leak, which can be categorized as follows: Increased hydrostatic pressure within the capillaries can force fluid and protein through the endothelial barrier and into the interstitium. This is the mechanism of capillary leak in heart failure, renal failure, hepatic venous obstruction (eg, cirrhosis), and lower extremity deep vein thrombosis. Decreased capillary oncotic pressure may fail to retain fluid within the vascular space. This is the mechanism of capillary leak in conditions characterized by albumin loss (e.g., nephrotic syndrome, protein losing enteropathy) or decreased albumin synthesis (e.g., liver disease). Increased capillary permeability allows fluid and protein to readily pass through the endothelial barrier and into the interstitium. This is the mechanism of capillary leak in many medical conditions, including sepsis, the systemic inflammatory response syndrome, acute pancreatitis, anaphylaxis, snake bites [9,10], and certain infectious syndromes (eg, Dengue hemorrhagic fever, brucellosis [11], hantavirus cardiopulmonary syndrome [12]).
Objectives:
1) To confirm the relation of Dengue fever with Idiopathic systemic capillary leak syndrome (ISCLS).
2) To assess the role of ultrasonography in early detection of Idiopathic systemic capillary leak syndrome (ISCLS) in Patients of Dengue fever during recent epidemic spread in the city of Lahore, Pakistan.
3) To correlate the incidence of Idiopathic systemic capillary leak syndrome (ISCLS) with number of attacks of Dengue fever.

Materials and methods

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One hundred patients of Dengue fever were included in this study performed at the radiology department of Nawaz Sharif social security hospital (NSSSH) Multan Road, Lahore, Pakistan, from 15th April 2011 to 14th September 2011. The total number of 100 confirmed cases of dengue fever patients (by clinical and laboratory findings) from 16-50 years of age, including 62 male and 38 female patients were referred to the radiology department for ultrasonographic assessment.
69 (51- male, 18-female) patients had second or third attack of dengue in last two years (called Group A) whereas 31 (11-male, 20-female) were those having first attack of dengue fever (Group B). All of them were scanned by all three investigators using Toshiba Power vision, Xerio and Mindray DC7. The transducer used was convex 3.5 to 5 M.Hz frequencies.

Results

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The usual findings on ultrasound were Hepatosplcenomegaly, Liver inflammation/edema, Peri-cholecystic fluid/congestion (not edema of wall), abdomino-pelvic Ascities/pelvic ascities, unilateral/bilateral pleural effusion. 67/69 (97%) of group A had multiple findings of Idiopathic systemic capillary leak syndrome (ISCLS) whereas 09/31 (29%) of group B had same findings but of lesser intensity.

Discussions and Conclusion

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This study confirms the relation of Dengue fever with Idiopathic systemic capillary leak syndrome (ISCLS).As the sonographic findings strongly proved the relation.
The role of ultrasonography is vital and essential diagnostic tool in early detection of Idiopathic systemic capillary leak syndrome (ISCLS) in Patients of Dengue fever during recent epidemic spread in the city of Lahore, Pakistan, as those who were diagnosed to have some fluid in serous cavities with dengue fever were a contraindication for IV fluid, so as to maintain electrolyte and albumin levels in the body.
There is correlation between the incidence of Idiopathic systemic capillary leak syndrome (ISCLS)with number of attacks of Dengue fever as in our study 97% of those having more than 2 attacks of dengue developed ISCLS. The percentage of male patient was more than females and most of them were from younger age group. The relation between excessive IV fluids given to dengue patients was also noted.

Two patients with systemic capillary leak syndrome (SCLS) were followed up clinically for 6 months. Muscle biopsy was performed .In both cases, muscle capillary basement membranes were extremely thick--more than 15 times thicker than normal. Capillary basement membrane enlargement appears to be a permanent lesion, probably limited to muscle vessels. This finding has not been previously reported in SCLS, and would appear to indicate a relationship with the pathogenesis and severity of the crisis.

Conclusion

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This study confirms that there is relation of Dengue fever with Idiopathic systemic capillary leak syndrome (ISCLS).Ultrasound in early detection of Idiopathic systemic capillary leak syndrome (ISCLS) in Patients of Dengue fever during recent epidemic spread in the city of Lahore, Pakistan. There is correlation between Idiopathic systemic capillary leak syndrome (ISCLS) with number of attacks of Dengue fever.

References

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Source(s) of Funding

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Competing Interests

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