Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease)

Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease)

Abstract Background Monoclonal gammopathy-associated capillary leak syndrome (MG-CLS) is a rare condition characterized by recurrent episodes of hypovolemic shock caused by a sudden increase in capillary permeability. The COVID-19 pandemic has been associated with a rise in MG-CLS episodes and incre...

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Main Authors: Nissim Grinberg, Maddalena Alessandra Wu, Quentin Moyon, Sybille Merceron, Yannick Fedun, Marie Gousseff, Romain Sonneville, François Lhote, Elie Azoulay, Jean-Herlé Raphalen, David Saadoun, Ygal Benhamou, Jean-Paul Mira, Guillaume Dumas, Pierre Bay, Jérôme Devaquet, Laurent Argaud, Marc Lambert, Avinash Aujayeb, Basile Henriot, Amandine Bichon, Thomas Bocar, John Harty, Remo Melchio, Franck Leibinger, Laure Calvet, Tomas Urbina, Laurent Bodson, Jean-Marie Tonnelier, Danielle Reuter, Emmanuel Canet, Gilles Blaison, Julien Maizel, Nicholas Sedillot, Laurence Dangers, Vincent Eble, Franco Verlicchi, Stanislas Faguer, Jonathan Montomoli, Geoffroy Dingemans, Marc Mikulski, Jonas Pochard, Fabrice Uhel, Fleur Cohen-Aubart, Charles-Edouard Luyt, Alexis Mathian, Alain Combes, Riccardo Colombo, Zahir Amoura, Marc Pineton de Chambrun, the EurêClark Study Group
Format: Article
Language:English
Published: SpringerOpen 2025-05-01
Series:Annals of Intensive Care
Subjects:
Monoclonal gammopathy-associated capillary-leak syndrome
Monoclonal gammopathy
Clarkson disease
SARS-CoV-2
Intensive care unit
Online Access:https://doi.org/10.1186/s13613-025-01483-7
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Summary:Abstract Background Monoclonal gammopathy-associated capillary leak syndrome (MG-CLS) is a rare condition characterized by recurrent episodes of hypovolemic shock caused by a sudden increase in capillary permeability. The COVID-19 pandemic has been associated with a rise in MG-CLS episodes and increased mortality. We aimed to explore the association between MG-CLS and SARS-CoV-2 infection. We conducted a multicenter retrospective observational study involving MG-CLS patients who were admitted to the intensive care unit (ICU). The primary endpoint was 28-day mortality according to whether SARS-CoV-2 was identified as a trigger. Results The study included 84 patients (44% women) with a median age of 55 years [IQR 46–62], accounting for 127 ICU admissions. Most patients (88%) had monoclonal gammopathy, predominantly with an IgG heavy chain (98%). A trigger was identified in 63% of cases, primarily suspected or confirmed viral infections, including 26 episodes of SARS-CoV-2 infection. Within 28 days of ICU admission, 32% of patients died. Episodes triggered by SARS-CoV-2 were associated with a higher need for mechanical ventilation (69% vs. 38%, p = 0.004), renal replacement therapy (54% vs. 31%, p = 0.03), and increased 28-day mortality (42% vs. 17%, p = 0.005). Multivariable analysis revealed that SARS-CoV-2 infection was independently associated with 28-day mortality (OR 4.67 [1.08–20.1], p = 0.04). The use of intravenous immunoglobulins did not improve 28-day survival. Conclusion In this large cohort of MG-CLS episodes requiring ICU admission, SARS-CoV-2as a trigger was associated with significantly higher 28-day mortality compared to other triggers. Further research is essential to elucidate the specific mechanisms by which SARS-CoV-2 impacts MG-CLS patients. Graphical abstract
ISSN:2110-5820

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