Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome

Lifermann, François; Chambrun, Marc Pineton de; Gousseff, Marie; Mauhin, Wladimir; Hot, A.; Argaud, Laurent; Hernu, Romain; Salle, Sylvie de la; Lega, Jean‐Christophe; Ledochowski, Stanislas; Lambert, Marc; Moreau, Anne‐Sophie; Rivière, Sophie; Dossier, Antoine; Papo, Thomas; Sonneville, Romain; Ruivard, M.; Lhote, F.; Verdière, Bruno; Blaison, G.; Merceron, Sybille; Zappella, Nathalie; Alric, Laurent; Agard, C.; Landais, Mickaël; Limal, Nicolas; Contou, Damien; Saadoun, David; Demoule, Alexandre; Similowski, Thomas; Graveleau, J.; Soubrier, Martin; Souweine, Bertrand; Haroche, Julien; Boileau, J.; Lucchini-Lecomte, Marie-Josée; Lecomte, Bernard; Hanslik, Thomas; Vieillard‐Baron, Antoine; Christides, C.; Bosseray, A.; Terzi, Nicolas; Lévesque, H.; Bulte, Caroline; Talasczka, Aline; Hachulla, É.; Decaux, Olivier; Ibouanga, Florent; Arnulf, Bertrand; Groh, Matthieu; Azoulay, Élie; Benedit, Marcel; Viallard, Jean‐François; Tieulié, N.; Maalouf, Assaad; Moulin, B.; Cohen‐Aubart, Fleur; Lovey, Pierre‐Yves; Friolet, Raymond; Moal, S. Le; Bibes, B.; Pha, M.; Rivard, Georges‐Étienne; Rondeau, Éric; Malizia, Giuseppe; Debourdeau, Philippe; Puidupin, M.; Abgueguen, P.; Beloncle, François; Devaquet, Jérôme; Presne, Claire; Liferman, François; Mazou, Jean-Marc; Andrieu, Maude; Paulus, S.; Fédun, Yannick; Mira, Jean‐Paul; Raphalen, Jean-Herlé; Abad, O. Len; Devilliers, H.; Rogers, Alister; Godmer, Pascal; Luyt, Charles‐Édouard; Combes, Alain; Hié, M.; Mathian, Alexis; Hatron, Pierre‐Yves; Ninet, J.; Amoura, Zahir

doi:10.1016/j.amjmed.2017.05.023

Cited by 56 publications

(63 citation statements)

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“…[3][4][5] However, the clinical picture and frequency of myocardial dysfunction in such patients is unknown. Using the European Clarkson (EurêClark) registry, 6 we evaluated the frequency of myocardial dysfunction in patients with severe attacks of SCLS admitted in intensive care unit from May 1992 to February 2016. 7 [17.9%]; P 5 .044), and chest pain (6 [54.5%] vs 3 [10.7%]; P 5 .008).…”

Section: Myocardial Dysfunction Is Frequent In Systemic Capillary-leamentioning

confidence: 99%

“…Transient hypertrophic cardiomyopathy has been described in neonates, 6 but it is uncommon in adults and is generally progressive and irreversible. In an isolated case report of an adult with transient hypertrophic cardiomyopathy, Cahill et al 7 postulated that myocarditis-induced myocardial edema could explain this phenotype, which might have been detectable by magnetic resonance imaging (MRI).…”

Section: Replymentioning

confidence: 99%

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Myocardial dysfunction is frequent in systemic capillary-leak syndrome (Clarkson disease) severe episodes

Chambrun

Mathian²,

Luyt

et al. 2018

Journal of Allergy and Clinical Immunology

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Section: Myocardial Dysfunction Is Frequent In Systemic Capillary-leamentioning

confidence: 99%

Section: Replymentioning

confidence: 99%

Myocardial dysfunction is frequent in systemic capillary-leak syndrome (Clarkson disease) severe episodes

Chambrun

Mathian²,

Luyt

et al. 2018

Journal of Allergy and Clinical Immunology

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“…Clinical observation and monitoring for the early signs and symptoms of an acute attack of SCLS, and treatment in a highly equipped intensive care unit (ICU) are all important measures to improve the chances of patient survival. A monoclonal gammopathy, typically IgG kappa, has been described in up to 90% of patients with acute SCLS [ 3 , 4 ]. From the published literature, most cases of SCLS present in middle-aged adults, and SCLS is equally reported in both sexes [ 5 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

“…Although a flu-like prodrome has been reported in up to 88% of cases of SCLS, specific infectious or other triggers for attacks can only be identified in approximately 60% of cases [ 3 , 5 ]. Common complications of acute attacks of SCLS include acute kidney failure (89%), muscle compartmental syndromes with rhabdomyolysis (43%), thromboses, pulmonary edema, and painful peripheral neuropathies.…”

Section: Introductionmentioning

confidence: 99%

Raised Serum Levels of Syndecan-1 (CD138), in a Case of Acute Idiopathic Systemic Capillary Leak Syndrome (SCLS) (Clarkson’s Disease)

et al. 2018

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Patient: Female, 49Final Diagnosis: Systemic capillary leak syndrome (SCLS)Symptoms: HypotensionMedication: —Clinical Procedure: NoneSpecialty: AllergologyObjective:Rare diseaseBackground:Systemic capillary leak syndrome (SCLS) (Clarkson’s disease) is a rare disorder of unknown etiology, characterized by transient episodes of hypotension, and the microvascular leak of fluids into the peripheral tissues, resulting in edema. Between 80–90% of patients with SCLS have a concomitant monoclonal gammopathy. Although translational in vitro studies have implicated vascular endothelial barrier dysfunction in the etiology of SCLS, the etiology and disease associations in clinical cases remain unknown.Case Report:We report a case of SCLS in a 49-year-old woman who initially presented with an upper respiratory tract infection, which was complicated by edema and compartment syndromes in the extremities that required fasciotomies. Serum levels of the cell surface heparan sulfate proteoglycan, syndecan-1 (CD138), a measure of endothelial surface glycocalyx (ESG) damage, were measured by enzyme-linked immunoassay (ELISA), peaked at up to 500 ng/mL (reference range, 50–100 ng/mL) and normalized on disease remission.Conclusions:This case report supports the view that damage to the microvascular endothelium, has a role in the pathogenesis of acute SCLS. This case also indicated that monitoring serum levels of syndecan-1 (CD138) might be used to monitor the progression and resolution of episodes of SCLS.

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“…For some infectious diseases, IgM shows an acute increase in early stages of infection, usually around 1 week, and can be used as a biomarker for early laboratory diagnosis [ 7 , 8 ]. The specific IgG shows an increase about four weeks after infection and lasts for the long term, years sometimes, and could be used as a biomarker for the evaluation of recovery [ 9 , 10 ]. In addition, the Igs are always involved in hypersensitivity of the body [ 11 , 12 ].…”

Section: Introductionmentioning

confidence: 99%

Construction and Potential Applications of Biosensors for Proteins in Clinical Laboratory Diagnosis

Jiang

2017

Sensors

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Biosensors for proteins have shown attractive advantages compared to traditional techniques in clinical laboratory diagnosis. In virtue of modern fabrication modes and detection techniques, various immunosensing platforms have been reported on basis of the specific recognition between antigen-antibody pairs. In addition to profit from the development of nanotechnology and molecular biology, diverse fabrication and signal amplification strategies have been designed for detection of protein antigens, which has led to great achievements in fast quantitative and simultaneous testing with extremely high sensitivity and specificity. Besides antigens, determination of antibodies also possesses great significance for clinical laboratory diagnosis. In this review, we will categorize recent immunosensors for proteins by different detection techniques. The basic conception of detection techniques, sensing mechanisms, and the relevant signal amplification strategies are introduced. Since antibodies and antigens have an equal position to each other in immunosensing, all biosensing strategies for antigens can be extended to antibodies under appropriate optimizations. Biosensors for antibodies are summarized, focusing on potential applications in clinical laboratory diagnosis, such as a series of biomarkers for infectious diseases and autoimmune diseases, and an evaluation of vaccine immunity. The excellent performances of these biosensors provide a prospective space for future antibody-detection-based disease serodiagnosis.

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Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome

Abstract: We describe the largest cohort to date of patients with well-defined monoclonal gammopathy-associated systemic capillary-leak syndrome. Preventive treatment with IVIg was the strongest factor associated with survival, suggesting the use of IVIg as the first line in prevention therapy.

Cited by 56 publications

References 26 publications

Myocardial dysfunction is frequent in systemic capillary-leak syndrome (Clarkson disease) severe episodes

Myocardial dysfunction is frequent in systemic capillary-leak syndrome (Clarkson disease) severe episodes

Raised Serum Levels of Syndecan-1 (CD138), in a Case of Acute Idiopathic Systemic Capillary Leak Syndrome (SCLS) (Clarkson’s Disease)

Construction and Potential Applications of Biosensors for Proteins in Clinical Laboratory Diagnosis

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