Annals of Intensive Care (May 2025)
Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease)
- 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
Affiliations
- Nissim Grinberg
- Service de Médecine Intensive-Réanimation, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié–Salpêtrière
- Maddalena Alessandra Wu
- Division of Internal Medicine, Department of Biomedical and Clinical Sciences, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan
- Quentin Moyon
- Service de Médecine Intensive-Réanimation, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié–Salpêtrière
- Sybille Merceron
- Service de Médecine-Intensive Réanimation, Hôpital André Mignot
- Yannick Fedun
- Service de Réanimation Polyvalente, CH Bretagne Atlantique
- Marie Gousseff
- Service de Médecine Interne, CH Bretagne Atlantique
- Romain Sonneville
- Service de Médecine-Intensive Réanimation, CHU Bichat, AP-HP
- François Lhote
- Service de Médecine Interne, Hôpital Delafontaine
- Elie Azoulay
- Service de Médecine Intensive-Réanimation, AP-HP, Hôpital Saint-Louis
- Jean-Herlé Raphalen
- Service de Réanimation Polyvalente, AP-HP, Hôpital Necker
- David Saadoun
- Department of Internal Medicine and Clinical Immunology, Sorbonne Universités, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Centre National de Références Maladies Autoimmunes et Systémiques Rares, Centre National de Références Maladies Autoinflammatoires Rares et Amylose Inflammatoire, and INSERM, UMR S 959, Immunology- Immunopathology-Immunotherapy (I3)
- Ygal Benhamou
- Service de Médecine Interne, Normandie Université, UNIROUEN
- Jean-Paul Mira
- Service de Médecine Intensive-Réanimation, AP-HP, CHU Cochin
- Guillaume Dumas
- Service de Réanimation Médicale, CHU Grenoble Alpes
- Pierre Bay
- Service de Réanimation Médicale, CHU Henri-Mondor, AP-HP
- Jérôme Devaquet
- Service de Réanimation Polyvalente, CH Foch
- Laurent Argaud
- Service de Médecine Intensive-Réanimation, CHU Edouard-Herriot
- Marc Lambert
- Département de Médecine Interne et d’Immunologie Clinique, Univ. Lille, CHU Lille
- Avinash Aujayeb
- Northumbria Specialist Emergency Care Hospital
- Basile Henriot
- Service de Médecine Interne, GHT d’Armor
- Amandine Bichon
- Service de Médecine Intensive-Réanimation, Assistance Publique Hôpitaux de Marseille
- Thomas Bocar
- Service d’Accueil des Urgences, CHU Pontchaillou
- John Harty
- Southern Health and Social Care Trust
- Remo Melchio
- Divisione Di Medicina Interna, A.O. S. Croce E Carle
- Franck Leibinger
- Service de Réanimation Polyvalente, CH de Perpignan
- Laure Calvet
- Service de Médecine Intensive-Réanimation, CHU Gabriel-Montpied
- Tomas Urbina
- Service de Médecine Intensive-Réanimation, Hôpital Saint-Antoine, AP-HP
- Laurent Bodson
- Service de Réanimation, Clinique Saint Gatien Alliance
- Jean-Marie Tonnelier
- Service de Médecine Intensive-Réanimation, CHU de Brest
- Danielle Reuter
- Service de Médecine Intensive-Réanimation, Centre Hospitalier Sud Francilien
- Emmanuel Canet
- Service de Médecine Intensive-Réanimation, CHU de Nantes
- Gilles Blaison
- Service de Médecine Interne, Hôpital de Colmar
- Julien Maizel
- Service de Médecine Intensive-Réanimation, CHU d’Amiens
- Nicholas Sedillot
- Service de Médecine Intensive-Réanimation, CH de Bourg-en-Bresse
- Laurence Dangers
- Service de Médecine Intensive-Réanimation, CHU de La Réunion
- Vincent Eble
- Service de Médecine Interne, CH d’Evreux
- Franco Verlicchi
- Transfusion Medicine Faenza-Lugo, Transfusion Service Ravenna, AUSL Romagna
- Stanislas Faguer
- Département de Néphrologie et Transplantation D’organes, Centre de Référence des Maladies Rénales Rares, INSERM U1297 (I2MC, Équipe 12)
- Jonathan Montomoli
- Department of Intensive Care, Ospedale “Infermi”
- Geoffroy Dingemans
- Service de Médecine Intensive-Réanimation, CHU de Nîmes
- Marc Mikulski
- Service de Réanimation Polyvalente, CHT Gaston-Bourret
- Jonas Pochard
- Service de Réanimation Chirurgicale, Hôpital de Bicètre, AP-HP
- Fabrice Uhel
- Médecine Intensive Réanimation, AP-HP, Hôpital Louis Mourier, DMU ESPRIT
- Fleur Cohen-Aubart
- Division of Internal Medicine, Department of Biomedical and Clinical Sciences, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan
- Charles-Edouard Luyt
- Service de Médecine Intensive-Réanimation, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié–Salpêtrière
- Alexis Mathian
- Division of Internal Medicine, Department of Biomedical and Clinical Sciences, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan
- Alain Combes
- Service de Médecine Intensive-Réanimation, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié–Salpêtrière
- Riccardo Colombo
- Division of Anesthesiology and Intensive Care, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital-Polo Universitario-University of Milan
- Zahir Amoura
- Division of Internal Medicine, Department of Biomedical and Clinical Sciences, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan
- Marc Pineton de Chambrun
- Service de Médecine Intensive-Réanimation, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié–Salpêtrière
- the EurêClark Study Group
- DOI
- https://doi.org/10.1186/s13613-025-01483-7
- Journal volume & issue
-
Vol. 15,
no. 1
pp. 1 – 12
Abstract
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
Keywords
- Monoclonal gammopathy-associated capillary-leak syndrome
- Monoclonal gammopathy
- Clarkson disease
- SARS-CoV-2
- Intensive care unit
