Publications by authors named "Marc Pineton De Chambrun"

52 Publications

Prone-Positioning for Severe Acute Respiratory Distress Syndrome Requiring Extracorporeal Membrane Oxygenation.

Crit Care Med 2021 Jul 14. Epub 2021 Jul 14.

Service de Médecine Intensive-Réanimation, Institut de Cardiologie, APHP, Sorbonne Université Hôpital Pitié-Salpêtrière, Paris, France. SAMOVAR, Telecom SudParis, Institut Polytechnique de Paris, Evry, France. Service de Radiologie, APHP, Université de Paris, Hôpital Bichat-Claude-Bernard, Paris, France. Université de Picardie Jules Verne, Amiens, France. Sorbonne Université, INSERM, UMRS_1166-ICAN, Institute of Cardiometabolism and Nutrition, Paris, France. Service de Chirurgie Cardiaque, Institut de Cardiologie, APHP, Sorbonne Université Hôpital Pitié-Salpêtrière, Paris, France.

Objectives: To determine the characteristics and outcomes of patients prone-positioned during extracorporeal membrane oxygenation for severe acute respiratory distress syndrome and lung CT pattern associated with improved respiratory system static compliance after that intervention.

Design: Retrospective, single-center study over 8 years.

Settings: Twenty-six bed ICU in a tertiary center.

Measurements And Main Results: A propensity score-matched analysis compared patients with prone-positioning during extracorporeal membrane oxygenation and those without. An increase of the static compliance greater than or equal to 3 mL/cm H2O after 16 hours of prone-positioning defined prone-positioning responders. The primary outcome was the time to successful extracorporeal membrane oxygenation weaning within 90 days of postextracorporeal membrane oxygenation start, with death as a competing risk. Among 298 venovenous extracorporeal membrane oxygenation-treated adults with severe acute respiratory distress syndrome, 64 were prone-positioning extracorporeal membrane oxygenation. Although both propensity score-matched groups had similar extracorporeal membrane oxygenation durations, prone-positioning extracorporeal membrane oxygenation patients' 90-day probability of being weaned-off extracorporeal membrane oxygenation and alive was higher (0.75 vs 0.54, p = 0.03; subdistribution hazard ratio [95% CI], 1.54 [1.05-2.58]) and 90-day mortality was lower (20% vs 42%, p < 0.01) than that for no prone-positioning extracorporeal membrane oxygenation patients. Extracorporeal membrane oxygenation-related complications were comparable for the two groups. Patients without improved static compliance had higher percentages of nonaerated or poorly aerated ventral and medial-ventral lung regions (p = 0.047).

Conclusions: Prone-positioning during venovenous extracorporeal membrane oxygenation was safe and effective and was associated with a higher probability of surviving and being weaned-off extracorporeal membrane oxygenation at 90 days. Patients with greater normally aerated lung tissue in the ventral and medial-ventral regions on quantitative lung CT-scan performed before prone-positioning are more likely to improve their static compliance after that procedure during extracorporeal membrane oxygenation.
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http://dx.doi.org/10.1097/CCM.0000000000005145DOI Listing
July 2021

Extracorporeal Membrane Oxygenation Induces Early Alterations in Coagulation and Fibrinolysis Profiles in COVID-19 Patients with Acute Respiratory Distress Syndrome.

Thromb Haemost 2021 Jun 15. Epub 2021 Jun 15.

Sorbonne Université, INSERM UMRS_1166, Institute of Cardiometabolism And Nutrition, Paris, France.

Hemostatic changes induced by extracorporeal membrane oxygenation (ECMO) support have been yet poorly documented in coronavirus-19 (COVID-19) patients who have a baseline complex hypercoagulable state. In this prospective monocentric study of patients with severe acute respiratory distress syndrome (ARDS) rescued by ECMO, we performed longitudinal measurements of coagulation and fibrinolysis markers throughout the course of ECMO support in 20 COVID-19 and 10 non-COVID-19 patients. Blood was sampled before and then 24 hours, 7, and 14 days after ECMO implantation. Clinical outcomes were prospectively assessed until discharge from the intensive care unit or death. The median age of participants was 47 (35-56) years, with a median body mass index of 30 (27-35) kg/m, and a Sepsis-related Organ Failure Assessment score of 12 (8-16). Baseline levels of von Willebrand factor, fibrinogen, factor VIII, prothrombin F1 + 2, thrombin-antithrombin, D-dimer, and plasminogen activator inhibitor-1 (PAI-1) were elevated in both COVID-19 and non-COVID-19 ARDS patients, indicating that endothelial activation, endogenous thrombin generation, and fibrinolysis shutdown occur in all ARDS patients before ECMO implantation. From baseline to day 7, thrombin generation (prothrombin F1 + 2,  < 0.01) and fibrin formation markers (fibrin monomers,  < 0.001) significantly increased, further resulting in significant decreases in platelet count ( < 0.0001) and fibrinogen level ( < 0.001). PAI-1 levels significantly decreased from baseline to day 7 ( < 0.0001) in all ARDS patients. These changes were more marked in COVID-19 patients, resulting in 14 nonfatal and 3 fatal bleeding. Additional studies are warranted to determine whether monitoring of thrombin generation and fibrinolysis markers might help to early predict bleeding complications in COVID-19 patients supported by ECMO.
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http://dx.doi.org/10.1055/a-1529-2257DOI Listing
June 2021

Recurrent cardiac arrest due to eosinophilia-related coronary vasospasm successfully treated by benralizumab.

J Allergy Clin Immunol Pract 2021 May 18. Epub 2021 May 18.

Department of Cardiology, Centre Hospitalier de Versailles, Le Chesnay-Rocquencourt, France.

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http://dx.doi.org/10.1016/j.jaip.2021.04.067DOI Listing
May 2021

Arrhythmia-induced cardiomyopathy: A potentially reversible cause of refractory cardiogenic shock requiring venoarterial extracorporeal membrane oxygenation.

Heart Rhythm 2021 Jul 12;18(7):1106-1112. Epub 2021 Mar 12.

Service de Médecine Intensive Réanimation, Institute of Cardiology, Pierre et Marie Curie Sorbonne Université, APHP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France; UPMC Université Paris 06, INSERM, UMRS_1166-ICAN, Institute of Cardiometabolism and Nutrition, Paris, France.

Background: The most severe form of arrhythmia-induced cardiomyopathy in adults- refractory cardiogenic shock requiring mechanical circulatory support-has rarely been reported.

Objective: The purpose of this study was to describe the management of critically ill patients admitted for acute, nonischemic, or worsening of previously known cardiac dysfunction and recent-onset supraventricular arrhythmia who developed refractory cardiogenic shock requiring venoarterial extracorporeal membrane oxygenation (VA-ECMO).

Methods: This study is a retrospective analysis of prospectively collected data.

Results: Between 2004 and 2018, 35 patients received VA-ECMO for acute, nonischemic cardiogenic shock and recent supraventricular arrhythmia (77% atrial fibrillation [AF]). Cardiogenic shock was the first disease manifestation in 21 patients (60%). Characteristics at ECMO implantation [median (interquartile range)] were Sequential Organ Failure Assessment score 10 (7-13); inotrope score 29 (11-80); left ventricular ejection (LVEF) fraction 10% (10%-15%); and lactate level 8 (4-11) mmol/L. For 12 patients, amiodarone and/or electric cardioversion successfully reduced arrhythmia, improved LVEF, and enabled weaning off VA-ECMO; 11 had long-term survival without transplantation or long-term assist device. Eight patients experiencing arrhythmia-reduction failure underwent ablation procedures (7 atrioventricular node [AVN] with pacing, 1 atrial tachycardia) and were weaned off VA-ECMO; 7 survived. Of the remaining 15 patients without arrhythmia reduction or ablation, only the 6 bridged to heart transplantation or left ventricular (LV) assist device survived.

Conclusion: Arrhythmia-induced cardiomyopathy, mainly AF-related, is an underrecognized cause of refractory cardiogenic shock and should be considered in patients with nonischemic cardiogenic shock and recent-onset supraventricular arrhythmia. VA-ECMO support allowed safe arrhythmia reduction or rate control by AVN ablation while awaiting recovery, even among those with severe LV dilation.
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http://dx.doi.org/10.1016/j.hrthm.2021.03.014DOI Listing
July 2021

Outcomes of severe systemic rheumatic disease patients requiring extracorporeal membrane oxygenation.

Ann Intensive Care 2021 Feb 9;11(1):29. Epub 2021 Feb 9.

Service de Médecine Intensive-Réanimation, Hôpital La Pitié-Salpêtrière, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France.

Background: Systemic rheumatic diseases (SRDs) are a group of inflammatory disorders that can require intensive care unit (ICU) admission because of multiorgan involvement with end-organ failure(s). Critically ill SRD patients requiring extracorporeal membrane oxygenation (ECMO) were studied to gain insight into their characteristics and outcomes.

Methods: This French monocenter, retrospective study included all SRD patients requiring venovenous (VV)- or venoarterial (VA)-ECMO admitted to a 26-bed ECMO-dedicated ICU from January 2006 to February 2020. The primary endpoint was in-hospital mortality.

Results: Ninety patients (male/female ratio: 0.5; mean age at admission: 41.6 ± 15.2 years) admitted to the ICU received VA/VV-ECMO, respectively, for an SRD-related flare (n = 69, n = 38/31) or infection (n = 21, n = 10/11). SRD was diagnosed in-ICU for 31 (34.4%) patients. In-ICU and in-hospital mortality rates were 48.9 and 51.1%, respectively. Nine patients were bridged to cardiac (n = 5) or lung transplantation (n = 4), or left ventricular assist device (n = 2). The Cox multivariable model retained the following independent predictors of in-hospital mortality: in-ICU SRD diagnosis, day-0 Simplified Acute Physiology Score (SAPS) II score ≥ 70 and arterial lactate ≥ 7.5 mmol/L for VA-ECMO-treated patients; diagnosis other than vasculitis, day-0 SAPS II score ≥ 70, ventilator-associated pneumonia and arterial lactate ≥ 7.5 mmol/L for VV-ECMO-treated patients.

Conclusions: ECMO support is a relevant rescue technique for critically ill SRD patients, with 49% survival at hospital discharge. Vasculitis was independently associated with favorable outcomes of VV-ECMO-treated patients. Further studies are needed to specify the role of ECMO for SRD patients.
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http://dx.doi.org/10.1186/s13613-021-00819-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7871308PMC
February 2021

Venous or arterial thromboses after venoarterial extracorporeal membrane oxygenation support: Frequency and risk factors.

J Heart Lung Transplant 2021 Apr 30;40(4):307-315. Epub 2020 Dec 30.

Service de Médecine Intensive Réanimation; INSERM, UMRS_1166-ICAN, Institute of Cardiometabolism and Nutrition, Sorbonne Université, Paris, France. Electronic address:

Background: Although venous thrombosis after venovenous-extracorporeal membrane oxygenation (ECMO) is well described, vascular complications occurring after venoarterial ECMO (VA-ECMO) removal have not yet been thoroughly described. Our aim was to evaluate the frequency of vascular (arterial and venous) complications after VA-ECMO removal and try to identify the risk factors associated with them.

Methods: Retrospective analysis of data prospectively collected in 2 intensive care units was performed. Consecutive patients successfully weaned off VA-ECMO during year 1 were screened for cannula-associated deep vein thrombosis (CaDVT) or arterial complications (arterial thrombosis/stenosis) using Doppler ultrasonography.

Results: From November 2018 to November 2019, a total of 107 patients with a median (interquartile range [IQR]) age of 54 (42-63) years and a median (IQR) ECMO support duration of 8 (2-5) days were successfully weaned off VA-ECMO and included. CaDVT occurred in 44 patients (41%), and arterial complications occurred in 15 (14%) (9 acute leg ischemia, 1 arteriovenous femoral fistula, and 5 late femoral stenosis). Multivariable analysis retained longer duration of ECMO support (odds ratio [OR]: 1.12 per day; 95% CI: 1.02-1.22) and infection occurring on ECMO (OR: 3.03; 95% CI: 1.14-8.03) as independent risk factors for CaDVT, whereas older age (OR: 0.97 per year; 95% CI: 0.94-0.99) and previous anti-coagulation use (OR: 0.21; 95% CI: 0.06-0.68) were protective factors for CaDVT. No risk factors for arterial complications were identified.

Conclusions: In patients requiring VA-ECMO support, vascular complications occurred frequently after its removal, especially CaDVT. Arterial complications, either early leg ischemia or late arterial stenosis, were observed less often. Strategies aimed at preventing CaDVT after VA-ECMO remain to be determined.
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http://dx.doi.org/10.1016/j.healun.2020.12.007DOI Listing
April 2021

Clarkson's Disease Episode or Secondary Systemic Capillary Leak-Syndrome: That Is the Question!

Chest 2021 01;159(1):441

Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Hôpital de la Pitié-Salpêtrière, Service de Médecine Interne 2, Centre de Référence National Lupus et SAPL et Autres Maladies Auto-immunes et Systémiques Rares, Paris, France.

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http://dx.doi.org/10.1016/j.chest.2020.07.084DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7831696PMC
January 2021

Ventilator-associated pneumonia in patients with SARS-CoV-2-associated acute respiratory distress syndrome requiring ECMO: a retrospective cohort study.

Ann Intensive Care 2020 Nov 23;10(1):158. Epub 2020 Nov 23.

Service de Médecine Intensive Réanimation, Institut de Cardiologie, ICAN, Assistance Publique-Hôpitaux de Paris (APHP), Sorbonne-Université, Groupe Hospitalier Pitié-Salpêtrière, 47-83, Boulevard de L'Hôpital, 75651, Paris Cedex 13, France.

Background: The data on incidence, clinical presentation, and outcomes of ventilator-associated pneumonia (VAP) in patients with severe coronavirus disease 2019 (COVID-19) pneumonia requiring mechanical ventilation (MV) are limited. We performed this retrospective cohort study to assess frequency, clinical characteristics, responsible pathogens, and outcomes of VAP in patients COVID-19 pneumonia requiring MV between March 12th and April 24th, 2020 (all had RT-PCR-confirmed SARS-CoV-2 infection). Patients with COVID-19-associated acute respiratory distress syndrome (ARDS) requiring ECMO were compared with an historical cohort of 45 patients with severe influenza-associated ARDS requiring ECMO admitted to the same ICU during the preceding three winter seasons.

Results: Among 50 consecutive patients with Covid-19-associated ARDS requiring ECMO included [median (IQR) age 48 (42-56) years; 72% male], 43 (86%) developed VAP [median (IQR) MV duration before the first episode, 10 (8-16) days]. VAP-causative pathogens were predominantly Enterobacteriaceae (70%), particularly inducible AmpC-cephalosporinase producers (40%), followed by Pseudomonas aeruginosa (37%). VAP recurred in 34 (79%) patients and 17 (34%) died. Most recurrences were relapses (i.e., infection with the same pathogen), with a high percentage occurring on adequate antimicrobial treatment. Estimated cumulative incidence of VAP, taking into account death and extubation as competing events, was significantly higher in Covid-19 patients than in influenza patients (p = 0.002). Despite a high P. aeruginosa-VAP rate in patients with influenza-associated ARDS (54%), the pulmonary infection recurrence rate was significantly lower than in Covid-19 patients. Overall mortality was similar for the two groups.

Conclusions: Patients with severe Covid-19-associated ARDS requiring ECMO had a very high late-onset VAP rate. Inducible AmpC-cephalosporinase-producing Enterobacteriaceae and Pseudomonas aeruginosa frequently caused VAP, with multiple recurrences and difficulties eradicating the pathogen from the lung.
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http://dx.doi.org/10.1186/s13613-020-00775-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7682692PMC
November 2020

Letter to the Editor. Brain biopsy in children and adults with neurological diseases of unknown etiology: two sides of the same coin?

J Neurosurg Pediatr 2020 Oct 23:1-3. Epub 2020 Oct 23.

1AP-HP, La Pitié-Salpêtrière - Charles Foix University Hospital, Paris, France.

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http://dx.doi.org/10.3171/2020.7.PEDS20619DOI Listing
October 2020

Systemic Inflammatory Response Syndrome Is a Major Contributor to COVID-19-Associated Coagulopathy: Insights From a Prospective, Single-Center Cohort Study.

Circulation 2020 08 17;142(6):611-614. Epub 2020 Jun 17.

Medical Intensive Care Unit (P.M., G.H., J.C., C.D., M.P.D.C., A.N., N.B., M.S., C.E.L., A.C.), Department of Hematology (M.L., I.M.-T., C.F.), and Cardiothoracic Surgery Department (G.L.), Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, France. Sorbonne Université, INSERM UMRS_1166, Institute of Cardiometabolism and Nutrition, Paris, France (G.H., G.L., M.S., C.E.L., A.C., C.F.).

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http://dx.doi.org/10.1161/CIRCULATIONAHA.120.048925DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7418760PMC
August 2020

Encephalitis of Unknown Etiology? Not Until the Results of a Brain Biopsy!

Clin Infect Dis 2021 05;72(9):e432

Department of Internal Medicine and Clinical Immunology, AP-HP, La Pitié-Salpêtrière, Charles Foix University Hospital, Paris, France.

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http://dx.doi.org/10.1093/cid/ciaa1093DOI Listing
May 2021

Tofacitinib in antisynthetase syndrome-related rapidly progressive interstitial lung disease.

Rheumatology (Oxford) 2020 Dec;59(12):e142-e143

Université Paris 13, Assistance Publique Hôpitaux de Paris, Hôpital Avicenne, Service de Pneumologie, Bobigny, France.

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http://dx.doi.org/10.1093/rheumatology/keaa323DOI Listing
December 2020

Response to: 'Presence of anti-phospholipid antibodies in COVID-19: a case series study' by Amezcua-Guerra .

Ann Rheum Dis 2020 Aug 4. Epub 2020 Aug 4.

Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Groupement Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Paris, France.

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http://dx.doi.org/10.1136/annrheumdis-2020-218145DOI Listing
August 2020

Neurological diseases of unknown etiology: Brain-biopsy diagnostic yields and safety.

Eur J Intern Med 2020 10 10;80:78-85. Epub 2020 Jul 10.

Sorbonne Université, UPMC Univ. Paris 06, F-75005, Paris, France; AP-HP, Hôpitaux Universitaires La Pitié-Salpêtrière - Charles Foix, Institut E3M, Service de Médecine Interne 2, Centre de Référence National Lupus Systémique, Syndrome des Anticorps Anti-Phospholipides et Autres Maladies Auto-Immunes Systémiques Rares, F-75013, Paris, France; AP-HP, Hôpitaux Universitaires La Pitié-Salpêtrière - Charles Foix, Institut de Cardiométabolisme et Nutrition (ICAN), Service de Médecine Intensive-Réanimation, F-75013, Paris, France.

Background: For nonneoplastic neurological diseases, no recommendation exists regarding the place or appropriate timing of brain biopsy. The aim of this study was to evaluate the diagnostic yield and safety of brain biopsies from patients with neurological diseases of unknown etiology.

Methods: We performed a retrospective cohort study from January 1, 2008 to December 31, 2018. We analyzed 1847 brain-biopsied patients, including 178 biopsies indicated for neurological diseases of unknown etiology. Specific histological and final diagnosis rates, positive diagnosis-associated factors, complication rate and complication-associated factors were assessed.

Results: Specific histological diagnosis and final diagnosis rates were 71.3% and 83.1%, respectively, leading to therapeutic management change(s) for 75.3% of patients. Brain- biopsy-related mortality and permanent neurological morbidity occurred in 1.1% and 0.6% of the patients, respectively. The multivariable logistic-regression model retained (odds ratio [95% CI] only immunodepression (2.2 [1.1-4.7]; P=.04) as being independently associated with specific histological diagnosis, while supratentorial biopsy-targeted lesions (4.1 [1.1-15.2]; P=.04) were independently associated with a final diagnosis. Biopsies obtained from comatose patients were less contributive to the diagnosis (0.2 [0.05-0.7]; P=.01). Prebiopsy platelet count <100 G/L (28.5 [1.8-447]; P=.02), hydrocephalus (6.3 [1.2-15.3]; P=.02) and targeted lesions <1 cm (4.3 [1.2-15.3]; P=.03) were independently associated with brain biopsy-related complications.

Conclusion: For highly selected patients with neurological diseases of unknown etiology, brain biopsy has a high diagnostic yield and low frequency of severe complications. We advocate that this procedure be considered early in the diagnosis algorithm of these patients.
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http://dx.doi.org/10.1016/j.ejim.2020.05.029DOI Listing
October 2020

Response.

Chest 2020 07 2;158(1):429-430. Epub 2020 Jul 2.

Sorbonne Université, Assistance Publique-Hôpitaux de Paris (APHP), Hôpital La Pitié-Salpêtrière, Institut E3M, Service de Médecine Interne 2, Centre de Référence National Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et Autres Maladies Auto-Immunes Systémiques Rares, Paris, France.

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http://dx.doi.org/10.1016/j.chest.2020.01.019DOI Listing
July 2020

The place of extracorporeal life support in cardiogenic shock.

Curr Opin Crit Care 2020 08;26(4):424-431

Service de Médecine Intensive-Réanimation, Institut de Cardiologie, APHP Hôpital Pitié-Salpêtrière.

Purpose Of Review: Temporary circulatory support (TCS) devices are increasingly used as a salvage therapy for patients with refractory cardiogenic shock. The exact place of the different TCS devices in the management of cardiogenic shock patients remains unclear and intensely debated. This article provides an overview on new cardiogenic shock classification, currently available devices, place of TCS in the management of cardiogenic shock patients, and discusses the results of recent case series and trials in this setting.

Recent Finding: A new classification system for cardiogenic shock has recently been proposed to homogenize definitions of cardiogenic shock and appropriately differentiate patient subsets in clinical trials and registries. Although the routine use of intraaortic balloon pump is no more recommended, other TCS are increasingly used and investigated but many advantages favor the use of venoarterial extracorporeal membrane oxygenation (VA-ECMO) as the first-line TCS.

Summary: TCS devices have become the cornerstone of the management of patients with refractory cardiogenic shock. VA-ECMO has emerged as the first-line support system in this setting, with a growing number of accepted indications. Large adequately powered randomized controlled trials are now underway and should help to determine the respective place of different TCS devices in strategies to treat cardiogenic shock patients.
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http://dx.doi.org/10.1097/MCC.0000000000000747DOI Listing
August 2020

Chronic obstructive pulmonary disease associated with hypocomplementemic urticarial vasculitis.

J Allergy Clin Immunol Pract 2020 10 3;8(9):3222-3224.e1. Epub 2020 Jun 3.

Department of Internal Medicine, Cochin Hospital, Paris, France; National Referral Centre for Systemic and Autoimmune Diseases, Cochin Hospital, Paris, France; Paris Descartes University, Université de Paris, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jaip.2020.05.031DOI Listing
October 2020

SARS-CoV-2 Induces Acute and Refractory Relapse of Systemic Capillary Leak Syndrome (Clarkson's Disease).

Am J Med 2020 11 13;133(11):e663-e664. Epub 2020 May 13.

Sorbonne Université, APHP, Institut E3M, Service de Médecine Interne 2, Centre de Référence National Lupus Systémique et SAPL et Autres Maladies Auto-immunes et Systémiques Rares, Hôpital de la Pitié-Salpêtrière, Paris, France.

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http://dx.doi.org/10.1016/j.amjmed.2020.03.057DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7217792PMC
November 2020

One-Year Outcome of Critically Ill Patients With Systemic Rheumatic Disease: A Multicenter Cohort Study.

Chest 2020 09 11;158(3):1017-1026. Epub 2020 Apr 11.

Intensive Care Medicine Department, Lapeyronie Hospital, Montpellier, France; PhyMedExp, INSERM, CNRS, Montpellier, France.

Background: Critically ill patients with systemic rheumatic disease (SRD) have benefited from better provision of rheumatic and critical care in recent years. Recent comprehensive data regarding in-hospital mortality rates and, most importantly, long-term outcomes are scarce.

Research Question: The aim of this study was to assess short and long-term outcome of patients with SRD who were admitted to the ICU.

Study Design And Methods: All records of patients with SRD who were admitted to ICU between 2006 and 2016 were reviewed. In-hospital and one-year mortality rates were assessed, and predictive factors of death were identified.

Results: A total of 525 patients with SRD were included. Causes of admission were most frequently shock (40.8%) and acute respiratory failure (31.8%). Main diagnoses were infection (39%) and SRD flare-up (35%). In-hospital and one-year mortality rates were 30.5% and 37.7%, respectively. Predictive factors that were associated with in-hospital and one-year mortalities were, respectively, age, prior corticosteroid therapy, simplified acute physiology score II ≥50, need for invasive mechanical ventilation, or need for renal replacement therapy. Knaus scale C or D and prior conventional disease modifying antirheumatic drug therapy was associated independently with death one-year after ICU admission.

Interpretation: Critically ill patients with SRD had a fair outcome after an ICU stay. Increased age, prior corticosteroid therapy, and severity of critical illness were associated significantly with short- and long-term mortality rates. The one-year mortality rate was also associated with prior health status and conventional disease modifying antirheumatic drug therapy.
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http://dx.doi.org/10.1016/j.chest.2020.03.050DOI Listing
September 2020

Systemic chloroquine intoxication: a hint from the peripheral blood smear.

Am J Hematol 2020 07 3;95(7):873-875. Epub 2020 Apr 3.

Sorbonne Université, Assistance Publique-Hôpitaux de Paris (APHP), Institut E3M, Hôpital La Pitié-Salpêtrière, service de médecine interne 2, Paris, France.

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http://dx.doi.org/10.1002/ajh.25788DOI Listing
July 2020

What's new in cardiogenic shock?

Intensive Care Med 2020 05 26;46(5):1016-1019. Epub 2020 Feb 26.

Service de médecine intensive-réanimation, Institut de Cardiologie, APHP Hôpital Pitié-Salpêtrière, 75013, Paris, France.

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http://dx.doi.org/10.1007/s00134-020-05973-zDOI Listing
May 2020

Withdrawal of low-dose prednisone in SLE patients with a clinically quiescent disease for more than 1 year: a randomised clinical trial.

Ann Rheum Dis 2020 03 18;79(3):339-346. Epub 2019 Dec 18.

Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Groupement Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Paris, France

Objectives: To compare the efficacy to prevent flares of maintenance versus withdrawal of 5 mg/day prednisone in systemic lupus erythematosus (SLE) patients with clinically quiescent disease.

Methods: A monocentric, 12-month, superiority, open-label, randomised (1:1) controlled trial was conducted with 61 patients continuing 5 mg/day prednisone and 63 stopping it. Eligibility criteria were SLE patients who, during the year preceding the inclusion, had a clinically inactive disease and a stable SLE treatment including 5 mg/day prednisone. The primary endpoint was the proportion of patient experiencing a flare defined with the SELENA-SLEDAI flare index (SFI) at 52 weeks. Secondary endpoints included time to flare, flare severity according to SFI and British Isles Lupus Assessment Group (BILAG) index and increase in the Systemic Lupus International Collaborating Clinics (SLICC) damage index (SDI).

Results: Proportion of patients experiencing a flare was significantly lower in the maintenance group as compared with the withdrawal group (4 patients vs 17; RR 0.2 (95% CI 0.1 to 0.7), p=0.003). Maintenance of 5 mg prednisone was superior with respect to time to first flare (HR 0.2; 95% CI 0.1 to 0.6, p=0.002), occurrence of mild/moderate flares using the SFI (3 patients vs 12; RR 0.2 (95% CI 0.1 to 0.8), p=0.012) and occurrence of moderate/severe flares using the BILAG index (1 patient vs 8; RR 0.1 (95% CI 0.1 to 0.9), p=0.013). SDI increase and adverse events were similar in the two treatment groups. Subgroup analyses of the primary endpoint by predefined baseline characteristics did not show evidence of a different clinical response.

Conclusion: Maintenance of long term 5 mg prednisone in SLE patients with inactive disease prevents relapse.

Trial Registration Number: NCT02558517; Results.
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http://dx.doi.org/10.1136/annrheumdis-2019-216303DOI Listing
March 2020

Severe Viral Myopericarditis With Autoantibodies Directed Against RNA Polymerase III.

Ann Intern Med 2020 04 17;172(7):502-504. Epub 2019 Dec 17.

Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Hôpital La Pitié-Salpêtrière, Institut E3M, Service de Médecine Interne 2, Centre de Référence National Lupus Systémique et SAPL et Autres Maladies Auto-immunes, Paris, France (A.M., F.C., Z.A.).

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http://dx.doi.org/10.7326/M19-3074DOI Listing
April 2020

In-Hospital Mortality-Associated Factors in Patients With Thrombotic Antiphospholipid Syndrome Requiring ICU Admission.

Chest 2020 05 26;157(5):1158-1166. Epub 2019 Nov 26.

Sorbonne Université, Assistance Publique-Hôpitaux de Paris (APHP), Hôpital La Pitié-Salpêtrière, Institut E3M, Service de Médecine Interne 2, Centre de Référence National Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et Autres Maladies Auto-Immunes Systémiques Rares, Paris, France.

Background: The antiphospholipid syndrome (APS) is a systemic autoimmune disease defined by thrombotic events that can require ICU admission because of organ dysfunction related to macrovascular and/or microvascular thrombosis. Critically ill patients with thrombosis and APS were studied to gain insight into their prognoses and in-hospital mortality-associated factors.

Methods: This French national, multicenter, retrospective study included all patients with APS and any new thrombotic manifestations admitted to 24 ICUs (January 2000-September 2018).

Results: During the study period, 134 patients (male/female ratio, 0.4) with 152 APS episodes were admitted to the ICU (mean age at admission, 46.0 ± 15.1 years). In-hospital mortality of their 134 last episodes was 35 of 134 (26.1%). The Cox multivariable model retained certain factors (hazard ratio [95% CI]: age ≥ 40 years, 11.4 [3.1-41.5], P < .0001; mechanical ventilation, 11.0 [3.3-37], P < .0001; renal replacement therapy, 2.9 [1.3-6.3], P = .007; and in-ICU anticoagulation, 0.1 [0.03-0.3], P < .0001) as independently associated with in-hospital mortality. For the subgroup of definite/probable catastrophic APS, the Cox bivariable model (including the Simplified Acute Physiology Score II score) retained double therapy (corticosteroids + anticoagulant, 0.2 [0.07-0.6]; P = .005) but not triple therapy (corticosteroids + anticoagulant + IV immunoglobulins or plasmapheresis: hazard ratio, 0.3 [0.1-1.1]; P = .07) as independently associated with in-hospital mortality.

Conclusions: In-ICU anticoagulation was the only APS-specific treatment independently associated with survival for all patients. Double therapy was independently associated with better survival of patients with definite/probable catastrophic APS. In these patients, further studies are needed to determine the role of triple therapy.
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http://dx.doi.org/10.1016/j.chest.2019.11.010DOI Listing
May 2020

The eclipse sign as a radiological presentation of neurosarcoidosis.

Int J Neurosci 2020 May 1;130(5):435-437. Epub 2019 Dec 1.

Service de Médecine Interne 2, Institut e3m, Hôpital de la Pitié-Salpêtrière, Centre National de Référence Maladies Systémiques Rares, Lupus, Syndrome des anticorps antiphospholipides, Sorbonne Université, Assistance Publique Hôpitaux de Paris, Paris, France.

Neurosarcoidosis is a rare inflammatory neurological condition. We describe a challenging radiological presentation of neurosarcoidosis. The eclipse sign refers to hypo T1-weighted parenchymal or leptomeningeal images surrounded by circular gadolinium enhancement. The eclipse sign was identified in 3 out of 46 patients with histologically-proven neurosarcoidosis. The eclipse sign may correspond to necrotizing parenchymal or leptomeningeal granuloma. This sign expands the spectrum of radiological presentations of neurosarcoidosis.
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http://dx.doi.org/10.1080/00207454.2019.1688807DOI Listing
May 2020

Ultrasensitive serum interferon-α quantification during SLE remission identifies patients at risk for relapse.

Ann Rheum Dis 2019 12 30;78(12):1669-1676. Epub 2019 Sep 30.

Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Groupement Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Paris, France.

Objectives: Maintenance of remission has become central in the management of systemic lupus erythematosus (SLE). The importance of interferon-alpha (IFN-α) in the pathogenesis of SLE notwithstanding, its expression in remission has been poorly studied as yet. To study its expression in remission and its prognostic value in the prediction of a disease relapse, serum IFN-α levels were determined using an ultrasensitive single-molecule array digital immunoassay which enables the measurement of cytokines at physiological concentrations.

Methods: A total of 254 SLE patients in remission, according to the Definition of Remission in SLE classification, were included in the study. Serum IFN-α concentrations were determined at baseline and patients were followed up for 1 year. Lupus flares were defined according to the Safety of Estrogens in Lupus Erythematosus: National Assessment version of the Systemic Lupus Erythematosus Disease Activity Index Flare Index, whereas the Kaplan-Meier analysis and Cox regression analysis were used to estimate the time to relapse and to identify baseline factors associated with time to relapse, respectively.

Results: Of all patients in remission, 26% displayed abnormally high IFN-α serum levels that were associated with the presence of antibodies specific for ribonucleoprotein (RNP), double stranded (ds)DNA and Ro/SSA60, as well as young age. Importantly, elevated-baseline IFN-α serum levels and remission duration were associated in an independent fashion, with shorter time to relapse, while low serum levels of complement component 3 and anti-dsDNA Abs were not.

Conclusion: Direct serum IFN-α assessment with highly sensitive digital immunoassay permits clinicians to identify a subgroup of SLE patients, clinically in remission, but at higher risk of relapse.
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http://dx.doi.org/10.1136/annrheumdis-2019-215571DOI Listing
December 2019