Idiopathic systemic capillary leak syndrome (Clarkson disease)

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Presentation transcript:

Idiopathic systemic capillary leak syndrome (Clarkson disease) Kirk M. Druey, MD, Samir M. Parikh, MD Journal of Allergy and Clinical Immunology Volume 140, Issue 3, Pages 663-670 (September 2017) DOI: 10.1016/j.jaci.2016.10.042 Copyright © 2016 Terms and Conditions

Fig 1 Cardiovascular magnetic resonance imaging suggests myocardial vascular leak in patients with SCLS. A, Precontrast and postcontrast myocardial T1 maps in a patient with SCLS. Regions of interest drawn within the septum and blood pool are used to calculate the ECV. B, Midventricular short-axis ECV map in the same patient. The color scale for displaying ECV values was chosen so that green colors represent means ± 3 SDs of normal myocardium from age-matched control subjects. C, Cardiovascular magnetic resonance imaging demonstrates increased myocardial ECV in patients with active VEH compared with the asymptomatic group. *P = .003, Mann-Whitney test. Adapted from Ertel et al56 and Kellman et al.57 Journal of Allergy and Clinical Immunology 2017 140, 663-670DOI: (10.1016/j.jaci.2016.10.042) Copyright © 2016 Terms and Conditions

Fig 2 Sera from patients with acute SCLS induce junctional destabilization and contraction of ECs. Application of same-subject sera from patients with SCLS from a nonsymptomatic period (Basal, left) versus that obtained during an acute attack (Episodic, right) to primary human dermal microvascular ECs demonstrates disruption of junctional VE-cadherin (green), increased actin stress fibers (red), and development of gaps between adjacent cells (white arrows). DAPI, 4′-6-diamidino-2-phenylindole dihydrochloride. Journal of Allergy and Clinical Immunology 2017 140, 663-670DOI: (10.1016/j.jaci.2016.10.042) Copyright © 2016 Terms and Conditions

Fig 3 The Angpt-Tie2 pathway mediates endothelial barrier function in response to inflammatory stressors. A, Schematic of the Tie2-dependent barrier defense pathway in endothelium. B and C, Vascular permeability measurements from lungs of mice treated with LPS with and without Angpt-1, demonstrating enhanced susceptibility to vascular leakage arising from incomplete loss of expression of either Tie2 (receptor for Angpt-1; Fig 3, B) or p47Phox (Fig 3, C). Results are normalized to respective genetic controls to illuminate differences in responses to LPS. ****P < .0001, 2-way ANOVA. Adapted from Ghosh et al,64 Mammoto et al,65 and Ghosh et al.66 Journal of Allergy and Clinical Immunology 2017 140, 663-670DOI: (10.1016/j.jaci.2016.10.042) Copyright © 2016 Terms and Conditions

Fig 4 RNA sequencing of BOECs from patients with SCLS implicates persistent promigratory and proinflammatory responses. Ingenuity pathway analysis of RNA sequencing results from 6 BOECs from patients with SCLS versus 6 BOECs from control subjects organized by using the Benjamini-Hochberg (B-H)–corrected P value. Dash, P = .05. The highlighted box indicates categories with particular functional relevance to SCLS. Journal of Allergy and Clinical Immunology 2017 140, 663-670DOI: (10.1016/j.jaci.2016.10.042) Copyright © 2016 Terms and Conditions

Fig 5 Proposed treatment algorithms for active SCLS (left) and prophylactic management (right). DVT, Deep venous thrombosis; MODS, multiorgan dysfunction syndrome; SCIG, subcutaneous immunoglobulin. Journal of Allergy and Clinical Immunology 2017 140, 663-670DOI: (10.1016/j.jaci.2016.10.042) Copyright © 2016 Terms and Conditions