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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2025-05-01
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| Online Access: | https://doi.org/10.1186/s13613-025-01483-7 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Nissim Grinberg |
| collection | DOAJ |
| description | 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 |
| format | Article |
| id | doaj-art-bf5c471f45bc414cb9c189aed5315c6d |
| institution | OA Journals |
| issn | 2110-5820 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | Annals of Intensive Care |
| spelling | doaj-art-bf5c471f45bc414cb9c189aed5315c6d2025-08-20T02:00:07ZengSpringerOpenAnnals of Intensive Care2110-58202025-05-0115111210.1186/s13613-025-01483-7Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease)Nissim Grinberg0Maddalena Alessandra Wu1Quentin Moyon2Sybille Merceron3Yannick Fedun4Marie Gousseff5Romain Sonneville6François Lhote7Elie Azoulay8Jean-Herlé Raphalen9David Saadoun10Ygal Benhamou11Jean-Paul Mira12Guillaume Dumas13Pierre Bay14Jérôme Devaquet15Laurent Argaud16Marc Lambert17Avinash Aujayeb18Basile Henriot19Amandine Bichon20Thomas Bocar21John Harty22Remo Melchio23Franck Leibinger24Laure Calvet25Tomas Urbina26Laurent Bodson27Jean-Marie Tonnelier28Danielle Reuter29Emmanuel Canet30Gilles Blaison31Julien Maizel32Nicholas Sedillot33Laurence Dangers34Vincent Eble35Franco Verlicchi36Stanislas Faguer37Jonathan Montomoli38Geoffroy Dingemans39Marc Mikulski40Jonas Pochard41Fabrice Uhel42Fleur Cohen-Aubart43Charles-Edouard Luyt44Alexis Mathian45Alain Combes46Riccardo Colombo47Zahir Amoura48Marc Pineton de Chambrun49the EurêClark Study GroupService de Médecine Intensive-Réanimation, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié–SalpêtrièreDivision of Internal Medicine, Department of Biomedical and Clinical Sciences, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of MilanService de Médecine Intensive-Réanimation, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié–SalpêtrièreService de Médecine-Intensive Réanimation, Hôpital André MignotService de Réanimation Polyvalente, CH Bretagne AtlantiqueService de Médecine Interne, CH Bretagne AtlantiqueService de Médecine-Intensive Réanimation, CHU Bichat, AP-HPService de Médecine Interne, Hôpital DelafontaineService de Médecine Intensive-Réanimation, AP-HP, Hôpital Saint-LouisService de Réanimation Polyvalente, AP-HP, Hôpital NeckerDepartment 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)Service de Médecine Interne, Normandie Université, UNIROUENService de Médecine Intensive-Réanimation, AP-HP, CHU CochinService de Réanimation Médicale, CHU Grenoble AlpesService de Réanimation Médicale, CHU Henri-Mondor, AP-HPService de Réanimation Polyvalente, CH FochService de Médecine Intensive-Réanimation, CHU Edouard-HerriotDépartement de Médecine Interne et d’Immunologie Clinique, Univ. Lille, CHU LilleNorthumbria Specialist Emergency Care HospitalService de Médecine Interne, GHT d’ArmorService de Médecine Intensive-Réanimation, Assistance Publique Hôpitaux de MarseilleService d’Accueil des Urgences, CHU PontchaillouSouthern Health and Social Care TrustDivisione Di Medicina Interna, A.O. S. Croce E CarleService de Réanimation Polyvalente, CH de PerpignanService de Médecine Intensive-Réanimation, CHU Gabriel-MontpiedService de Médecine Intensive-Réanimation, Hôpital Saint-Antoine, AP-HPService de Réanimation, Clinique Saint Gatien AllianceService de Médecine Intensive-Réanimation, CHU de BrestService de Médecine Intensive-Réanimation, Centre Hospitalier Sud FrancilienService de Médecine Intensive-Réanimation, CHU de NantesService de Médecine Interne, Hôpital de ColmarService de Médecine Intensive-Réanimation, CHU d’AmiensService de Médecine Intensive-Réanimation, CH de Bourg-en-BresseService de Médecine Intensive-Réanimation, CHU de La RéunionService de Médecine Interne, CH d’EvreuxTransfusion Medicine Faenza-Lugo, Transfusion Service Ravenna, AUSL RomagnaDépartement de Néphrologie et Transplantation D’organes, Centre de Référence des Maladies Rénales Rares, INSERM U1297 (I2MC, Équipe 12)Department of Intensive Care, Ospedale “Infermi”Service de Médecine Intensive-Réanimation, CHU de NîmesService de Réanimation Polyvalente, CHT Gaston-BourretService de Réanimation Chirurgicale, Hôpital de Bicètre, AP-HPMédecine Intensive Réanimation, AP-HP, Hôpital Louis Mourier, DMU ESPRITDivision of Internal Medicine, Department of Biomedical and Clinical Sciences, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of MilanService de Médecine Intensive-Réanimation, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié–SalpêtrièreDivision of Internal Medicine, Department of Biomedical and Clinical Sciences, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of MilanService de Médecine Intensive-Réanimation, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié–SalpêtrièreDivision of Anesthesiology and Intensive Care, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital-Polo Universitario-University of MilanDivision of Internal Medicine, Department of Biomedical and Clinical Sciences, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of MilanService de Médecine Intensive-Réanimation, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié–SalpêtrièreAbstract 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 abstracthttps://doi.org/10.1186/s13613-025-01483-7Monoclonal gammopathy-associated capillary-leak syndromeMonoclonal gammopathyClarkson diseaseSARS-CoV-2Intensive care unit |
| spellingShingle | 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 Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease) Annals of Intensive Care Monoclonal gammopathy-associated capillary-leak syndrome Monoclonal gammopathy Clarkson disease SARS-CoV-2 Intensive care unit |
| title | Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease) |
| title_full | Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease) |
| title_fullStr | Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease) |
| title_full_unstemmed | Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease) |
| title_short | Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease) |
| title_sort | characteristics of sars cov 2 associated severe episodes of monoclonal gammopathy associated capillary leak syndrome clarkson disease |
| topic | Monoclonal gammopathy-associated capillary-leak syndrome Monoclonal gammopathy Clarkson disease SARS-CoV-2 Intensive care unit |
| url | https://doi.org/10.1186/s13613-025-01483-7 |
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