Publications by authors named "Hervé Devilliers"

71 Publications

Correspondence on 'Factors associated with COVID-19-related death in people with rheumatic diseases: results from the COVID-19 Global Rheumatology Alliance physician-reported registry'.

Ann Rheum Dis 2021 Feb 18. Epub 2021 Feb 18.

Internal Medicine and Systemic Diseases, Hôpital François Mitterrand, CHU de Dijon, Dijon, France.

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http://dx.doi.org/10.1136/annrheumdis-2021-220037DOI Listing
February 2021

IgA dominates the early neutralizing antibody response to SARS-CoV-2.

Sci Transl Med 2021 01 7;13(577). Epub 2020 Dec 7.

Sorbonne Université, Inserm, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), 91 boulevard de l'Hôpital, 75013 Paris, France.

Humoral immune responses are typically characterized by primary IgM antibody responses followed by secondary antibody responses associated with immune memory and composed of IgG, IgA, and IgE. Here, we measured acute humoral responses to SARS-CoV-2, including the frequency of antibody-secreting cells and the presence of SARS-CoV-2-specific neutralizing antibodies in the serum, saliva, and bronchoalveolar fluid of 159 patients with COVID-19. Early SARS-CoV-2-specific humoral responses were dominated by IgA antibodies. Peripheral expansion of IgA plasmablasts with mucosal homing potential was detected shortly after the onset of symptoms and peaked during the third week of the disease. The virus-specific antibody responses included IgG, IgM, and IgA, but IgA contributed to virus neutralization to a greater extent compared with IgG. Specific IgA serum concentrations decreased notably 1 month after the onset of symptoms, but neutralizing IgA remained detectable in saliva for a longer time (days 49 to 73 post-symptoms). These results represent a critical observation given the emerging information as to the types of antibodies associated with optimal protection against reinfection and whether vaccine regimens should consider targeting a potent but potentially short-lived IgA response.
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http://dx.doi.org/10.1126/scitranslmed.abd2223DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7857408PMC
January 2021

Diagnostic performance of sacroiliac and spinal MRI for the diagnosis of non-radiographic axial spondyloarthritis in patients with inflammatory back pain.

Joint Bone Spine 2021 Mar 10;88(2):105106. Epub 2020 Nov 10.

Rheumatology department, CHU Dijon-Burgundy, Dijon, France; Inserm EFS BFC, UMR1098, university of Bourgogne-Franche Comté, Dijon, France. Electronic address:

Objective: The lack of specificity of the ASAS MRI criteria for non-radiographic axial spondylarthritis (NR-axSpA) justifies the evaluation of the discriminatory capacity of other MRI abnormalities in the sacroiliac joints and dorsolumbar spine.

Methods: In patients hospitalized for inflammatory lumbar back pain, the diagnostic performance (sensitivity, specificity, positive likelihood ratio (PLR)) of MRI abnormalities was calculated using the rheumatologist expert opinion as a reference: (i) sacroiliac joints: Bone marrow edema (BME) (number and location), extended edema>1cm (deep lesion), fatty metaplasia (number), erosion (number and location), backfill. (ii) Dorsolumbar spine: BME (number and location), fatty metaplasia (number), posterior segment involvement.

Results: In this prospective cohort, 40 NR-axSpA cases and 79 other diagnoses were included. The presence of at least 3 inflammatory signals in the sacroiliac joints (PLR: 25.67 [95% CI: 3.48-48.9]), the presence of at least one sacroiliac erosion (PLR: 12.80 [3.04-54]), the combination of an inflammatory signal and sacroiliac erosion (PLR: 11.85 [2.79-50]), the combination of deep lesion and fatty metaplasia (PLR: 15.80 [2.05-121.9]) or erosion (PLR: 11.86 [1.47-95.01]) had the best diagnostic performance. The combination of spinal and sacroiliac MRI criteria significantly increased diagnostic performance for the diagnosis of NR-axSpA.

Conclusion: When NR-axSpA is suspected, in addition to the presence and number of inflammatory lesions, MRI interpretation should include the location and the extent of the sacroiliac lesions, the presence of erosion or fatty metaplasia, and anterior involvement of the lumbar spine.
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http://dx.doi.org/10.1016/j.jbspin.2020.105106DOI Listing
March 2021

Clinical phenotypes of extrapulmonary sarcoidosis: an analysis of a French, multi-ethnic, multicentre cohort.

Eur Respir J 2021 Apr 1;57(4). Epub 2021 Apr 1.

Service de Médecine Interne 2, Centre National de Référence Maladies Systémiques Rares, Lupus et Syndrome des Anticorps Antiphospholipides, Centre National de Référence Histiocytoses, Sorbonne Université, Assistance Publique Hôpitaux de Paris, Hôpital de la Pitié-Salpêtrière, Paris, France

Sarcoidosis is a rare disease of unknown cause with wide heterogeneity in clinical features and outcomes. We aimed to explore sarcoidosis phenotypes and their clinical relevance with particular attention to extrapulmonary subgroups.The Epidemiology of Sarcoidosis (EpiSarc) study is a French retrospective multicentre study. Sarcoidosis patients were identified through national hospitalisation records using appropriate codes from 11 hospital centres between 2013 and 2016 according to a standardised protocol. Medical charts were reviewed. The phenotypes of sarcoidosis were defined using a hierarchical cluster analysis.A total of 1237 patients were included (562 men and 675 women). The mean age at sarcoidosis diagnosis was 43.5±13 years. Hierarchical cluster analysis identified five distinct phenotypes according to organ involvement and disease type and symptoms: 1) erythema nodosum, joint involvement and hilar lymph nodes (n=180); 2) eye, neurological, digestive and kidney involvement (n=137); 3) pulmonary involvement with fibrosis and heart involvement (n=630); 4) lupus pernio and a high percentage of severe involvement (n=41); and 5) hepatosplenic, peripheral lymph node and bone involvement (n=249). Phenotype 1 was associated with being European/Caucasian and female and with non-manual work, phenotype 2 with being European/Caucasian, and phenotypes 3 and 5 with being non-European/Caucasian. The labour worker proportion was significantly lower in phenotype 5 than in the other phenotypes.This multicentre study confirms the existence of distinct phenotypes of sarcoidosis, with a non-random distribution of organ involvement. These phenotypes differ according to sex, geographical origin and socioprofessional category.
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http://dx.doi.org/10.1183/13993003.01160-2020DOI Listing
April 2021

Use of Physician Global Assessment in systemic lupus erythematosus: a systematic review of its psychometric properties.

Rheumatology (Oxford) 2020 12;59(12):3622-3632

Service de rhumatologie, Hôpitaux Universitaires de Strasbourg, Université de Strasbourg, Strasbourg, France.

Objective: The Physician Global Assessment (PGA) is a visual analogue score that reflects the clinician's judgement of overall SLE disease activity. The aim of this systematic literature review is to describe and analyse the psychometric properties of the PGA.

Methods: This systematic literature review was conducted by two independent reviewers in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. All articles published through 1 July 2019 in PubMed were screened, with no limitation on year of publication, language or patients' age. Psychometric properties data were analysed according to the OMERACT Filter methodology version 2.1.

Results: The literature search identified 91 studies. Face validity was reported in all the articles retrieved in which the PGA was used alone or as part of composite indices (Systemic Responder Index, Safety of Estrogen in Lupus Erythematosus National Assessment Flare Index, Lupus Low Disease Activity State, Definitions of Remission in Systemic Lupus Erythematosus criteria). Content validity was reported in 89 studies. Construct validity was demonstrated by a good correlation (r ≥ 0.50) between the PGA with the SLEDAI (12 studies), SLAM (4 studies), LAI, BILAG and ECLAM (2 studies each). Criterion validity was assessed exploring the PGA correlation with quality of life measurements, biomarker levels and treatment changes in 28 studies, while no study has evaluated correlation with damage. A good responsiveness for PGA was shown in eight studies. A high variability in scales was found, causing a wide range of reliability (intraclass correlation coefficient 0.67-0.98).

Conclusion: PGA is a valid, responsive and feasible instrument, though its reliability was impacted by the scale adopted, suggesting the major need for standardization of its scoring.
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http://dx.doi.org/10.1093/rheumatology/keaa383DOI Listing
December 2020

Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: Study of 121 cases.

Neurology 2020 07 2;95(1):e70-e78. Epub 2020 Jun 2.

From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France.

Objectives: The predominance of extramuscular manifestations (e.g., skin rash, arthralgia, interstitial lung disease [ILD]) as well as the low frequency of muscle signs in anti-melanoma differentiation-associated gene 5 antibody-positive (anti-MDA5+) dermatomyositis caused us to question the term myositis-specific antibody for the anti-MDA5 antibody, as well as the homogeneity of the disease.

Methods: To characterize the anti-MDA5+ phenotype, an unsupervised analysis was performed on anti-MDA5+ patients (n = 83/121) and compared to a group of patients with myositis without anti-MDA5 antibody (anti-MDA5-; n = 190/201) based on selected variables, collected retrospectively, without any missing data.

Results: Within anti-MDA5+ patients (n = 83), 3 subgroups were identified. One group (18.1%) corresponded to patients with a rapidly progressive ILD (93.3%; < 0.0001 across all) and a very high mortality rate. The second subgroup (55.4%) corresponded to patients with pure dermato-rheumatologic symptoms (arthralgia; 82.6%; < 0.01) and a good prognosis. The third corresponded to patients, mainly male (72.7%; < 0.0001), with severe skin vasculopathy, frequent signs of myositis (proximal weakness: 68.2%; < 0.0001), and an intermediate prognosis. Raynaud phenomenon, arthralgia/arthritis, and sex permit the cluster appurtenance (83.3% correct estimation). Nevertheless, an unsupervised analysis confirmed that anti-MDA5 antibody delineates an independent group of patients (e.g., dermatomyositis skin rash, skin ulcers, calcinosis, mechanic's hands, ILD, arthralgia/arthritis, and high mortality rate) distinct from anti-MDA5- patients with myositis.

Conclusion: Anti-MDA5+ patients have a systemic syndrome distinct from other patients with myositis. Three subgroups with different prognosis exist.
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http://dx.doi.org/10.1212/WNL.0000000000009727DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7371381PMC
July 2020

High Risk of Sustained Ventricular Arrhythmia Recurrence After Acute Myocarditis.

J Clin Med 2020 Mar 20;9(3). Epub 2020 Mar 20.

Cardiology Department, Dijon Bourgogne University Hospital, 21000 Dijon, France.

Acute myocarditis is associated with cardiac arrhythmia in 25% of cases; a third of these arrhythmias are ventricular tachycardia (VT) or ventricular fibrillation (VF). The implantation of a cardiac defibrillator (ICD) following sustained ventricular arrhythmia remains controversial in these patients. We sought to assess the risk of major arrhythmic ventricular events (MAEs) over time in patients implanted with an ICD following sustained VT/VF in the acute phase of myocarditis compared to those implanted for VT/VF occurring on myocarditis sequelae. Our retrospective observational study included patients implanted with an ICD following VT/VF during acute myocarditis or VT/VF on myocarditis sequelae, from 2007 to 2017, in 15 French university hospitals. Over a median follow-up period of 3 years, MAE occurred in 11 (39%) patients of the acute myocarditis group and 24 (60%) patients of the myocarditis sequelae group. Kaplan-Meier MAE rate estimates at one and three years of follow-up were 19% and 45% in the acute group, and 43% and 64% in the sequelae group. Patients who experienced sustained ventricular arrhythmias during acute myocarditis had a very high risk of VT/VF recurrence during follow-up. These results show that the risk of MAE recurrence remains high after resolution of the acute episode.
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http://dx.doi.org/10.3390/jcm9030848DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7141537PMC
March 2020

Social deprivation among socio-economic contrasted french areas: Using item response theory analysis to assess differential item functioning of the EPICES questionnaire in stroke patients.

PLoS One 2020 2;15(4):e0230661. Epub 2020 Apr 2.

Clinical Epidemiology Unit, INSERM, CIC 1432, University Hospital of Dijon, Epidemiology and infection control unit, Bourgogne-Franche-Comté University, Dijon, France.

Background: Multiple approaches have been proposed to measure low socio-economic status. In France the concept of precariousness, akin to social deprivation, was developed and is widely used. EPICES is a short questionnaire that was developed to measure this concept. This study aimed to evaluate Differential Item Functioning (DIF) in the EPICES questionnaire between contrasted areas: mainland France, French West Indies (FWI) and French Guiana (FG).

Methods: The population was taken from the INDIA study, which aimed to evaluate the impact of social inequalities on stroke characteristics and prognosis. Eligible people were patients referred to neurology or emergency departments for a suspicion of stroke. We assessed the DIF using hybrid ordinal logistic regression method, derived from item response theory.

Results: We analysed 1 553 stroke patients, including 768 from FWI (49.5%), 289 from FG (18.6%) and 496 from mainland (31.9%). We identified five items with a moderate to large DIF in area comparisons: "meeting with a social worker", "complementary health insurance", "home-owning", "financial difficulties" and "sport activities". Correlation between EPICES score and the latent variable was strong (r = 0.84).

Conclusion: This is the first attempt to assess the DIF of the EPICES score between different French populations. We found several items with DIF, which can be explained by individual interpretation or local context. However, the DIFs did not lead to a large difference between the latent variable and the EPICES score, which indicates that it can be used to assess precariousness and social deprivation between contrasted areas.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0230661PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7117693PMC
July 2020

Criteria deemed important by the relatives for designating a reference person for patients hospitalized in ICU.

J Crit Care 2020 06 26;57:191-196. Epub 2020 Feb 26.

Department of Intensive Care, Centre Hospitalier de Dieppe, France; Espace de Réflexion Ethique de Normandie, University Hospital Caen, France. Electronic address:

Purpose: We investigated the criteria that patients' relatives deem important for choosing, among themselves, the person best qualified to interact with the caregiving staff.

Methods: Exploratory, observational, prospective, multicentre study between 1st March and 31st October 2018 in 2 intensive care units (ICUs). A 12-item questionnaire was completed anonymously by family members of patients hospitalized in the ICU 3 and 5 days after the patient's admission. Relatives were eligible if they understood French and if no surrogate had been appointed by the patient prior to ICU admission. More than one relative per patient could participate.

Results: In total, 87 relatives of 73 patients completed the questionnaire, average age of relatives was 58 ± 15 years, 46% were the spouse, 30% were children/grandchildren. Items classed as being the most important attributes for a reference person were: good knowledge of the patient's wishes and values; an emotional attachment to the patient; being a family member; and having an adequate understanding of the clinical status and clinical history.

Conclusion: This study identifies the attributes considered by relatives to be most important for designating, among themselves, a reference person for a patient hospitalized in the ICU.
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http://dx.doi.org/10.1016/j.jcrc.2020.02.017DOI Listing
June 2020

Influence of deprivation on initial severity and prognosis of patients admitted to the ICU: the prospective, multicentre, observational IVOIRE cohort study.

Ann Intensive Care 2020 Feb 11;10(1):20. Epub 2020 Feb 11.

INSERM, CIC 1432, Module Epidémiologie Clinique, Dijon, France.

Background: The influence of socioeconomic status on patient outcomes is unclear. We assessed the impact of socioeconomic deprivation on severity of illness at intensive care unit (ICU) admission, and on the risk of death at 3 months after ICU admission.

Methods: The IVOIRE study was a prospective, observational, multicentre cohort study in the ICU of 8 participating hospitals in France, including patients aged ≥ 18 years admitted to the ICU and receiving at least one life support therapy for organ failure. The primary outcomes were severity at admission (assessed by SAPSII score), and mortality at 3 months. Socioeconomic data were obtained from interviews with patients or family. Deprivation was assessed using the EPICES score.

Results: Among 1294 patents included between 2013 and 2016, 629 (48.6%) were classed as deprived and differed significantly from non-deprived subjects in terms of sociodemographic characteristics and pre-existing conditions. The mean SAPS II score at admission was 50.1 ± 19.4 in deprived patients and 52.3 ± 17.3 in non-deprived patients, with no significant difference by multivariable analysis (β = - 1.85 [95% CI - 3.86; + 0.16, p = 0.072]). The proportion of death was 31.1% at 3 months, without significant differences between deprived and non-deprived patients, even after adjustment for confounders.

Conclusions: Deprivation is frequent in patients admitted to the ICU and is not associated with disease severity at admission, or with mortality at 3 months between deprived and non-deprived patients. Trial registration The IVOIRE cohort is registered with ClinicalTrials.gov under the identifier NCT01907581, registration date 17/7/2013.
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http://dx.doi.org/10.1186/s13613-020-0637-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7013026PMC
February 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

Anti-MDA5 juvenile idiopathic inflammatory myopathy: a specific subgroup defined by differentially enhanced interferon-α signalling.

Rheumatology (Oxford) 2020 08;59(8):1927-1937

Paediatric Hematology-Immunology and Rheumatology Department, Reference center for Rheumatic, AutoImmune and Systemic diseases in children (RAISE), Hôpital Necker-Enfants Malades, AP-HP, Paris.

Objectives: JDM and juvenile overlap myositis represent heterogeneous subtypes of juvenile idiopathic inflammatory myopathy (JIIM). Chronic evolution can occur in up to 60% of cases, and morbidity/mortality is substantial. We aimed to describe the clinical, biological, histological and type I IFN status in JIIM associated with anti-melanoma differentiation-associated protein 5 (anti-MDA5) autoantibodies at presentation (group 1) in comparison with other JIIM (group 2).

Methods: This was a retrospective and prospective study of patients with JIIM ascertained from three French paediatric rheumatology reference centres between 2013 and 2019. Muscle biopsies were reviewed. Type I interferon pathway activity was assessed by dosage of IFNα serum protein and the expression of IFN-stimulated genes.

Results: Sixty-four patients were included, 13 in group 1 (54% JDM and 46% juvenile overlap myositis) and 51 in group 2 (76% JDM and 24% juvenile overlap myositis). Group 1 patients demonstrated more arthritis, skin ulcerations, lupus features and interstitial lung disease, and a milder muscular involvement. Serum IFNα levels were higher in group 1 than 2, and decreased after treatment or improvement in both groups. Outcome was similar in both groups. Unconventional treatment (more than two lines) was required in order to achieve remission, especially when skin ulceration was reported.

Conclusion: This study indicates a higher frequency of arthritis, skin ulcerations and interstitial lung disease, but milder muscular involvement, in JIIM with positive anti-MDA5 autoantibodies compared with other JIIM. Our data support an important role of systemic IFNα in disease pathology, particularly in the anti-MDA5 auto-antibody-positive subgroup. In severe and refractory forms of JIIM, IFNα may represent a therapeutic target.
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http://dx.doi.org/10.1093/rheumatology/kez525DOI Listing
August 2020

Subretinal Drusenoid Deposits in the Elderly in a Population-Based Study (the Montrachet Study).

Invest Ophthalmol Vis Sci 2019 11;60(14):4838-4848

Department of Ophthalmology, University Hospital, Dijon, France.

Purpose: The aim of this study was to investigate the prevalence of subretinal drusenoid deposits (SDD) and to identify associated factors in an elderly population.

Methods: The participants of the population-based Montrachet study underwent an exhaustive ophthalmologic examination, including color fundus photography and macular spectral domain-optical coherence tomography (SD-OCT), coupled with infrared reflectance imaging. The presence of SDD and other age-related macular degeneration lesions, according to the European Eye Epidemiology SD-OCT classification of macular diseases, and subfoveal choroidal thickness were recorded. Moreover, the association of SDD and both clinical and demographic factors as well as plasma levels of vitamin E and lutein/zeaxanthin (L/Z) were analyzed.

Results: The mean age of patients was 82.3 ± 3.8 years and 62.7% were female. The prevalence of SDD was 18.1% (n = 205) in the subjects with at least one eye interpretable (n = 1135). In multivariate analysis, SDD was positively associated with increasing age (OR, 4.6; 95% CI, 2.8-7.7; P < 0.001 for subjects aged >85 years), female sex (OR, 1.7; 95% CI, 1.2-2.4; P = 0.005), and plasma L/Z level (OR, 1.2; 95% CI, 1.0-1.5; P = 0.039), and negatively associated with lipid-lowering drugs use (OR, 0.5; 95% CI, 0.3-0.9; P = 0.014 for statin medications) and subfoveal choroidal thickness (OR, 0.8; 95% CI, 0.7-0.9; P = 0.002).

Conclusions: The prevalence of SDD was high in subjects older than 75 years, more frequent in women, and was associated with a thinner choroid. The association with lipid-lowering drugs deserves further investigation.
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http://dx.doi.org/10.1167/iovs.19-27283DOI Listing
November 2019

Disease-specific quality of life following a flare in systemic lupus erythematosus: an item response theory analysis of the French EQUAL cohort.

Rheumatology (Oxford) 2020 06;59(6):1398-1406

Clinical Investigation Center, INSERM CIC-EC 1432, University Hospital Dijon-Burgundy, Dijon.

Objective: To explore, at an item-level, the effect of disease activity (DA) on specific health-related quality of life (HRQoL) in SLE patients using an item response theory longitudinal model.

Methods: This prospective longitudinal multicentre French cohort EQUAL followed SLE patients over 2 years. Specific HRQoL according to LupusQoL and SLEQOL was collected every 3 months. DA according to SELENA-SLEDAI flare index (SFI) and revised SELENA-SLEDAI flare index (SFI-R) was evaluated every 6 months. Regarding DA according to SFI and each SFI-R type of flare, specific HRQoL of remitting patients was compared with non-flaring patients fitting a linear logistic model with relaxed assumptions for each domain of the questionnaires.

Results: Between December 2011 and July 2015, 336 patients were included (89.9% female). LupusQoL and SLEQOL items related to physical HRQoL (physical health, physical functioning, pain) were most affected by musculoskeletal and cutaneous flares. Cutaneous flares had significant influence on self-image. Neurological or psychiatric flares had a more severe impact on specific HRQoL. Patient HRQoL was impacted up to 18 months after a flare.

Conclusion: Item response theory analysis is able to pinpoint items that are influenced by a given patient group in terms of a latent trait change. Item-level analysis provides a new way of interpreting HRQoL variation in SLE patients, permitting a better understanding of DA impact on HRQoL. This kind of analysis could be easily implemented for the comparison of groups in a clinical trial.

Trial Registration: ClinicalTrials.gov, http://clinicaltrials.gov, NCT01904812.
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http://dx.doi.org/10.1093/rheumatology/kez451DOI Listing
June 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

Usual interstitial pneumonia in ANCA-associated vasculitis: A poor prognostic factor.

J Autoimmun 2020 01 27;106:102338. Epub 2019 Sep 27.

Department of Internal Medicine and Clinical Immunology, CHU Dijon Bourgogne, Dijon, France.

Background: Progressive fibrosing interstitial lung disease (ILD) is rarely associated with antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). This study focused on the outcomes of ILD patients with associated AAV (AAV-ILD).

Methods: AAV-ILD (cases: microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) with ILD) were compared to AAV patients without ILD (controls). ILD was defined as a usual interstitial pneumonia (UIP) or non-specific interstitial pneumonia (NSIP) pattern. Two controls were matched to each case for age (>or ≤65 years), ANCA status (PR3-or MPO-positive) and creatininemia (≥or <150 μmol/L).

Results: Sixty-two cases (89% MPO-ANCA+) were included. Median age at AAV diagnosis was 66 years. ILD (63% UIP), was diagnosed before (52%) or simultaneously (39%) with AAV. Cases versus 124 controls less frequently had systemic vasculitis symptoms. One-, 3- and 5-year overall survival rates, respectively, were: 96.7%, 80% and 66% for cases versus 93.5%, 89.6% and 83.8% for controls (p = 0.008). Multivariate analyses retained age >65 years (hazard ratio (HR) 4.54; p < 0.001), alveolar haemorrhage (HR 2.25; p = 0.019) and UIP (HR 2.73; p = 0.002), but not immunosuppressant use, as factors independently associated with shorter survival.

Conclusion: For AAV-ILD patients, only UIP was associated with poorer prognosis. Immunosuppressants did not improve the AAV-ILD prognosis. But in analogy to idiopathic pulmonary fibrosis, anti-fibrosing agents might be useful and should be assessed in AAV-ILD patients with a UIP pattern.
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http://dx.doi.org/10.1016/j.jaut.2019.102338DOI Listing
January 2020

Validation of the "Good2Go": the first French-language transition readiness questionnaire.

Eur J Pediatr 2020 Jan 13;179(1):61-71. Epub 2019 Sep 13.

RECaP Network-Perceived health measurement Working Group, Paris, France.

The use of transition readiness questionnaires is strongly recommended in adolescents with chronic conditions. The aim of our study was to validate "Good2Go," the first French-language transition readiness questionnaire. We analyzed the data from 2 multicentric studies (Canada and France) involving adolescents with chronic conditions (type 1 diabetes, inflammatory bowel disease, cystic fibrosis, epilepsy, juvenile idiopathic arthritis). Content and construct validity were examined using factorial and Rasch analysis (structural validity), Spearman's correlation, and Mann-Whitney test (external validity). Cronbach's α and intra-class correlation coefficients explored reliability. Cognitive interviews assessed wording comprehension and item appropriateness. Good2Go was completed by 321 participants (boys = 51%; mean age = 16.4 years (standard deviation = 1.5; min = 14.0; max = 18.0); Canada = 51.1%). Factor analysis identified 3 domains: "health self-advocacy," "knowledge about chronic conditions," and "self-management skills." The 3-domain structure showed a satisfying Rasch fit, internal consistency, and test-retest reliability. Good2Go domain scores were significantly higher in participants over 17 years of age, indicating satisfactory external validity.Conclusion: Good2Go is a valid 20-item questionnaire to assess transition readiness in adolescents with chronic conditions and may be useful in routine care to propose individually tailored preparation for their transfer to adult healthcare. Further research is now needed to analyze correlation between domain scores and success of transition.What is Known:• In adolescents with chronic conditions, the use of transition readiness questionnaires is recommended to propose individually tailored preparation for their transfer to adult healthcare.• However, no French-language questionnaire has been so far validated.What is New:• Based on a complete validation methodology, this study highlights that the French-language 20-items Good2Go questionnaire has good psychometric properties.• It explores all transition key points though 3 scored domains: "health self-advocacy", "knowledge about chronic disease" and "self-management skills".
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http://dx.doi.org/10.1007/s00431-019-03450-4DOI Listing
January 2020

What are the characteristics that lead physicians to perceive an ICU stay as non-beneficial for the patient?

PLoS One 2019 6;14(9):e0222039. Epub 2019 Sep 6.

Department of Intensive Care, Dieppe General Hospital, Dieppe, France.

Purpose: We sought to describe the characteristics that lead physicians to perceive a stay in the intensive care unit (ICU) as being non-beneficial for the patient.

Materials And Methods: In the first step, we used a multidisciplinary focus group to define the characteristics that lead physicians to consider a stay in the ICU as non-beneficial for the patient. In the second step, we assessed the proportion of admissions that would be perceived by the ICU physicians as non-beneficial for the patient according to our focus group's definition, in a large population of ICU admissions in 4 French ICUs over a period of 4 months.

Results: Among 1075 patients admitted to participating ICUs during the study period, 155 stays were considered non-beneficial for the patient, yielding a frequency of 14.4% [95% confidence interval (CI) 8.9, 19.9]. Average age of these patients was 72 ±12.8 years. Mortality was 43.2% in-ICU [95%CI 35.4, 51.0], 55% [95%CI 47.2, 62.8] in-hospital. The criteria retained by the focus group to define a non-beneficial ICU stay were: patient refusal of ICU care (23.2% [95%CI 16.5, 29.8]), and referring physician's desire not to have the patient admitted (11.6% [95%CI 6.6, 16.6]). The characteristics that led physicians to perceive the stay as non-beneficial were: patient's age (36.8% [95%CI 29.2, 44.4]), unlikelihood of recovering autonomy (61.9% [95%CI 54.3, 69.6]), prior poor quality of life (60% [95%CI 52.3, 67.7]), terminal status of chronic disease (56.1% [95%CI 48.3, 63.9]), and all therapeutic options have been exhausted (35.5% [95%CI 27.9, 43.0]). Factors that explained admission to the ICU of patients whose stay was subsequently judged to be non-beneficial included: lack of knowledge of patient's wishes (52% [95%CI 44.1, 59.9]); decisional incapacity (sedation) (69.7% [95%CI 62.5, 76.9]); inability to contact family (34% [95%CI 26.5, 41.5]); pressure to admit (from family or other physicians) (50.3% [95%CI 42.4, 58.2]).

Conclusions: Non-beneficial ICU stays are frequent. ICU admissions need to be anticipated, so that patients who would yield greater benefit from other care pathways can be correctly oriented in a timely manner.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0222039PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6730882PMC
March 2020

Osseous sarcoidosis: A multicenter retrospective case-control study of 48 patients.

Joint Bone Spine 2019 Nov 30;86(6):789-793. Epub 2019 Jul 30.

Sorbonne Universités, UPMC Univ Paris 06, UMR 7211, and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), 75005 Paris, France; INSERM, UMR_S 959, 75013 Paris, France; CNRS, FRE3632, 75005 Paris, France; AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, 75013 Paris, France. Electronic address:

Objective: To describe the clinical presentation, distribution of lesions, treatment, and outcomes of osseous sarcoidosis.

Methods: A French retrospective multicenter study of patients with biopsy-proven sarcoidosis analyzed patients with 1) a biopsy-proven granuloma without caseous necrosis, and either 2) osseous clinical manifestations, or 3) abnormal osseous imaging. Sarcoidosis patients with osseous involvement (cases) were compared with 264 age- and sex-matched sarcoidosis patients with no osseous manifestations (controls).

Results: In the osseous sarcoidosis group (n=88), forty-two (48%) patients had osseous-related symptoms involving the axial (69%) and/or appendicular (58%) skeleton. On imaging, the most commonly affected bones were in the spine (52%), pelvis (42%), hands (22%) and femur (19%). Compared with controls, cases had higher rates of mediastinal (93% vs. 47%) and extra-thoracic lymph node involvement (66% vs. 21%), pulmonary (90% vs. 65%) and cutaneous involvement (44% vs. 23%) (all P<0.0001), and hypercalcemia (8.5% vs. 2%, P=0.014). Spleen/liver and gastrointestinal involvement were less frequent in the osseous sarcoidosis group (29% vs. 45%, and 1% vs. 17%, respectively, P<0.0001). Response rates to with glucocorticoids alone, glucocorticoids plus methotrexate or glucocorticoids plus hydroxychloroquine were 23/44 (52%), 9/13 (69%) and 4/6 (67%), respectively.

Conclusion: In patients with osseous sarcoidosis the spine and pelvis were the most commonly affected bones. Compared with controls, cases with osseous sarcoidosis have higher rates of thoracic and extra-thoracic lymph node involvement, pulmonary and cutaneous involvement, and hypercalcemia. Most patients with osseous sarcoidosis had a good response to glucocorticoids in combination with methotrexate or hydroxychloroquine.
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http://dx.doi.org/10.1016/j.jbspin.2019.07.009DOI Listing
November 2019

Achieving lupus low-disease activity and remission states under belimumab in refractory systemic lupus erythematosus: time and organ involvement matter.

Ann Rheum Dis 2020 11 8;79(11):e148. Epub 2019 Jul 8.

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

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http://dx.doi.org/10.1136/annrheumdis-2019-215732DOI Listing
November 2020

Taste Perception and Cerebral Activity in the Human Gustatory Cortex Induced by Glucose, Fructose, and Sucrose Solutions.

Chem Senses 2019 09;44(7):435-447

Centre des Sciences du goût et de l'Alimentation, AgroSup Dijon, CNRS, INRA, Université Bourgogne Franche-Comté, Dijon, France.

Glucose, fructose, and sucrose are important carbohydrates in Western diets with particular sweetness intensity and metabolisms. No study has compared their cerebral detection and their taste perception. Gustatory evoked potentials (GEPs), taste detection thresholds, intensity perception, and pleasantness were compared in response to glucose, fructose, and sucrose solutions at similar sweetness intensities and at identical molar concentrations. Twenty-three healthy subjects were randomly stimulated with 3 solutions of similar sweetness intensity (0.75 M of glucose, 0.47 M of fructose and 0.29 M of sucrose - sit. A), and with an identical molar concentration (0.29 M - sit. B). GEPs were recorded at gustatory cortex areas. Intensity perception and hedonic values of each solution were evaluated as were gustatory thresholds of the solutions. No significant difference was observed concerning the GEP characteristics of the solutions according to their sweetness intensities (sit. A) or their molar concentration (sit. B). In sit. A, the 3 solutions were perceived to have similar intensities and induced similar hedonic sensations. In sit. B, the glucose solution was perceived to be less intense and pleasant than the fructose and the sucrose solutions (P < 0.001) and the fructose solution was perceived to be less intense and pleasant than the sucrose (P < 0.001). Since GEP recordings were similar for glucose, fructose, and sucrose solutions whatever the concentrations, activation of same taste receptor induces similar cortical activation, even when the solutions were perceived differently. Sweet taste perception seems to be encoded by a complex chemical cerebral neuronal network.
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http://dx.doi.org/10.1093/chemse/bjz034DOI Listing
September 2019

Large-vessel vasculitis diagnosed between 50 and 60 years: Case-control study based on 183 cases and 183 controls aged over 60 years.

Autoimmun Rev 2019 Jul 4;18(7):714-720. Epub 2019 May 4.

National Referral Center for Rare Autoimmune and Systemic Diseases, Hopital Cochin, AP-HP, Université Paris Descartes, Paris, France. Electronic address:

Background: Age at onset of large-vessel vasculitis (LVV) is commonly used to distinguish giant cell arteritis (GCA) and Takayasu arteritis (TA). However, LVV between age 50 and 60 years may be difficult to classify.

Methods: We conducted a retrospective study including LVV aged between 50 and 60 years at onset (LVV, cases) and compared them to LVV aged over 60 years (LVV, controls). LVV was defined histologically and/or morphologically. Controls fulfilled ACR 1990 criteria for GCA or presented isolated aortitis.

Results: We included 183 LVV and 183 gender-matched LVV. LVV had more frequent peripheral limb manifestations (23 vs. 5%), and less frequent cephalic (73 vs. 90%) and ocular signs (17 vs. 27%) than LVV. Compared to LVV, CT angiography and PET/CT scan were more frequently abnormal in LVV (74 vs. 38%, and 90 vs. 72%, respectively), with aorta being more frequently involved (78 vs. 47%). By multivariate analysis, absence of cephalic symptoms, presence of peripheral limb ischemia and aorta involvement, and increased CRP level were significantly associated with LVV presentation compared to LVV. At last follow-up, compared to LVV, LVV received significantly more lines of treatment (2 vs. 1), more frequent biologics (12 vs. 3%), had more surgery (10 vs. 0%), and had higher prednisone dose (8.8 vs. 6.5 mg/d) at last follow-up, CONCLUSION: LVV onset between 50 and 60 years identifies a subset of patients with more frequent aorta and peripheral vascular involvement and more refractory disease compared to patients with LVV onset after 60.
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http://dx.doi.org/10.1016/j.autrev.2019.05.008DOI Listing
July 2019

The world-wide burden of musculoskeletal diseases: a systematic analysis of the World Health Organization Burden of Diseases Database.

Ann Rheum Dis 2019 06 15;78(6):844-848. Epub 2019 Apr 15.

Department of Rheumatology - Centre National de Référence des Maladies Systémiques Rares Est Sud-Ouest (RESO), Hôpitaux Universitaires de Strasbourg, Strasbourg, France

Background: Musculoskeletal (MSK) diseases are expected to have a growing impact worldwide.

Objective: To analyse the worldwide burden of MSK diseases from 2000 to 2015.

Methods: Disability-adjusted life years (DALYs), which combines the years of life lost (YLLs) and the years lived with disability (YLDs), were extracted for 183 countries from the WHO Global Health Estimates Database. We analysed the median proportion of DALYS, YLLs and YLDs for MSK diseases (ICD-10: M00-M99) among the 23 WHO categories of diseases. Mixed models were built to assess temporal changes.

Results: Worldwide, the total number of MSK DALYs increased significantly from 80,225,634.6 in 2000 to 107,885,832.6 in 2015 (p < 0.001), with the total number of MSK YLDs increasing from 77,377,709.4 to 103,817,908.4 (p = 0.0008) and MSK diseases being the second cause of YLDs worldwide. YLLs due to MSK diseases increased from 2,847,925.2 to 4,067,924.2 (p = 0.03). In 2015, the median proportion of DALYs attributed to MSK diseases was 6.66% (IQR: 5.30 - 7.88) in Europe versus 4.66% (3.98 - 5.59) in the Americas (p < 0.0001 vs Europe), 4.17% (3.14 - 6.25) in Asia (p < 0.0001), 4.14% (2.65 - 5.57) in Oceania (p = 0.0008) and 1.33% (1.03 - 1.92) in Africa (p < 0.0001). We observed a significant correlation (r = 0.85, p < 0.0001) between the proportion of MSK DALYs and the gross domestic product per capita for the year 2015.

Conclusions: The burden of MSK diseases increased significantly between 2000 and 2015 and is high in Europe. These results are crucial to health professionals and policy makers to implement future health plan adjustments for MSK diseases.
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http://dx.doi.org/10.1136/annrheumdis-2019-215142DOI Listing
June 2019

Responsiveness to Change of 5-point MRC scale, Endurance and Functional Evaluation for Assessing Myositis in Daily Clinical Practice.

J Neuromuscul Dis 2019;6(1):99-107

Department of Internal Medicine and Clinical Immunology, National Reference Center of Neuromuscular disorders, Pitié-Salpêtrière University Hospital, Sorbonne Université, Paris, France.

Background: Manual muscle testing has been widely used for the evaluation of muscle strength in myositis, yet less attention has been devoted to the evaluation of muscle function and endurance.

Objective: Our objective was therefore to compare the responsiveness to change of muscle strength, endurance and functional testing following induction therapy for severe myositis flare (requiring high-dose corticosteroids and combined immunotherapy) in patients with a diagnosis of dermatomyositis, immune-mediated necrotizing myopathy, or overlap myositis.

Methods: Muscle status was assessed at baseline and after mean 6.4±1.9 months, using the MRC-5 scale, along with endurance (Barre and Mingazzini maneuvers) and functional evaluation (e.g. chair rise) and responsiveness to change was evaluated using the Standardized Response Mean (SRM) and Standardized Mean Difference.

Results: Among the 49 patients included, the strongest responsiveness to change was observed in the muscle testing of the psoas and deltoids (SRM: 1.23 and 1.16, respectively). Noticeably, endurance testing also demonstrated strong responsiveness (SRM: 1.05 and 0.96, respectively), compensating for the poor discriminatory ability of muscle testing and permitting to overcome its ceiling effect.

Conclusion: Functional and endurance testing provide simple and reliable measures complementing the testing of select proximal muscle groups to evaluate responsiveness to intervention in myositis patients in daily clinical practice. Interest of functional and endurance testing should be evaluated prospectively as outcome measures in myositis clinical trials.
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http://dx.doi.org/10.3233/JND-180358DOI Listing
January 2020

Systemic lupus erythematosus: state of the art on clinical practice guidelines.

RMD Open 2018 27;4(2):e000793. Epub 2018 Nov 27.

Assistance Publique-Hôpitaux de Paris (AP-HP), Internal Medicine Department, Cochin Hospital, Referral center for rare autoimmune and systemic diseases, Paris, France.

Systemic lupus erythematosus (SLE) is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsing-remitting course. The aim of the present work was to identify current available clinical practice guidelines (CPGs) for SLE, to provide their review and to identify physicians' and patients' unmet needs. Twenty-three original guidelines published between 2004 and 2017 were identified. Many aspects of disease management are covered, including global disease management, lupus nephritis and neuropsychiatric involvement, management of pregnancies, vaccinations and comorbidities monitoring. Unmet needs relate with disease management of some clinical manifestations and adherence to treatment. Many patient's unmet needs have been identified starting with faster diagnosis, need for more therapeutic options, guidelines on lifestyle issues, attention to quality of life and adequate education.
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http://dx.doi.org/10.1136/rmdopen-2018-000793DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6269635PMC
November 2018

Monitoring Disease Activity in Systemic Lupus Erythematosus With Single-Molecule Array Digital Enzyme-Linked Immunosorbent Assay Quantification of Serum Interferon-α.

Arthritis Rheumatol 2019 05;71(5):756-765

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.

Objective: No simple or standardized assay is available to quantify interferon-α (IFNα) in routine clinical practice. Single-molecule array (Simoa) digital enzyme-linked immunosorbent assay (ELISA) technology enables direct IFNα quantification at attomolar (femtogram per milliliter [fg/ml]) concentrations. This study was undertaken to assess IFNα digital ELISA diagnostic performances to monitor systemic lupus erythematosus (SLE) activity.

Methods: IFNα concentrations in serum samples from 150 consecutive SLE patients in a cross-sectional study were determined with digital ELISA and a functional biologic activity assay (bioassay). According to their Safety of Estrogens in Lupus Erythematosus National Assessment version of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) flare composite scores, patients were divided into groups with inactive SLE (SLEDAI score of <4 or clinical SLEDAI score of 0) or active SLE (SLEDAI score of ≥4 or clinical SLEDAI score of >0), and into groups with no flare or mild/moderate flare or severe flare.

Results: Based on serum samples from healthy blood donors, the abnormal serum IFNα level threshold value was 136 fg/ml. Next, using receiver operating characteristic curves for an SLE patient series that was widely heterogeneous in terms of disease activity and organ involvement, the threshold IFNα value associated with active disease was determined to be 266 fg/ml. The digital ELISA-assessed serum IFNα level was a better biomarker of disease activity than the Farr assay because its specificity, likelihood ratio for positive results, and positive predictive value better discerned active SLE or flare from inactive disease. The digital ELISA was more sensitive than the bioassay for detecting low-abnormal serum IFNα concentrations and identifying patients with low disease activity.

Conclusion: Direct serum IFNα determination with a highly sensitive assay might improve monitoring of clinical SLE activity and selection of the best candidates for anti-IFNα treatment.
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http://dx.doi.org/10.1002/art.40792DOI Listing
May 2019

Tocilizumab as an add-on therapy to glucocorticoids during the first 3 months of treatment of Giant cell arteritis: A prospective study.

Eur J Intern Med 2018 11 24;57:96-104. Epub 2018 Jul 24.

Department of Internal Medicine and Clinical Immunology, CHU Dijon Bourgogne, Dijon, France; University Bourgogne Franche-Comté, INSERM, EFS BFC, UMR1098, Interactions Hôte-Greffon-Tumeur/Ingénierie Cellulaire et Génique, F-21000 Dijon, France.

Background: The aim of this study was to evaluate tocilizumab (TCZ) as an add-on therapy to glucocorticoids (GC) during the first 3 months of treatment of giant cell arteritis (GCA).

Methods: GCA patients, as defined by ≥3/5 ACR criteria and positive temporal artery biopsy (TAB) or angio-CT-scan or PET-scan-proven aortitis, were included in this prospective open-label study. Prednisone was started at 0.7 mg/kg/day and then tapered according to a standardized protocol. All patients received four infusions of TCZ (8 mg/kg/4 weeks) after inclusion. The primary endpoint was the percentage of patients in remission with ≤0.1 mg/kg/day of prednisone at week 26 (W26). Patients were followed for 52 weeks and data prospectively recorded.

Results: Twenty patients with a median (IQR) age of 72 (69-78) years were included. TAB were positive in 17/19 (90%) patients and 7/16 (44%) had aortitis. Remission was obtained in all cases. At W26, 15 (75%) patients met the primary endpoint. Ten patients experienced relapse during follow-up, mainly patients with aortitis (P = 0.048), or CRP >70 mg/L (P = 0.036) or hemoglobin ≤10 g/dL (P = 0.015) at diagnosis. Among 64 adverse events (AE) reported in 18 patients, three were severe and 30, mostly non-severe infections (n = 15) and hypercholesterolemia (n = 8), were imputable to the study.

Conclusion: This study shows that an alternative strategy using a short-term treatment with TCZ can be proposed to spare GC for the treatment of GCA. However, 50% of patients experienced relapse during the 9 months following TCZ discontinuation, especially patients with aortitis, or CRP > 70 mg/L or Hb ≤ 10 g/dL at diagnosis.

Trial Registration: ClinicalTrials.gov (NCT01910038).
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http://dx.doi.org/10.1016/j.ejim.2018.06.008DOI Listing
November 2018