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We conducted a national in-depth analysis including pharmacovigilance reports and clinical study to assess the reporting rate (RR) and to determine the clinical profile of polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) in COVID-19-vaccinated individuals. First, based on the French pharmacovigilance database, we estimated the RR of PMR and GCA cases in individuals aged over 50 who developed their initial symptoms within one month of receiving the BNT162b2 mRNA, mRNA-1273, ChAdOx1 nCoV-19, and Ad26.COV2.S vaccines. We then conducted a nationwide survey to gather clinical profiles, therapeutic management, and follow-up data from individuals registered in the pharmacovigilance study. A total of 70 854 684 COVID-19 vaccine doses were administered to 25 260 485 adults, among which, 179 cases of PMR (RR 7. 1 cases/1 000 000 persons) and 54 cases of GCA (RR 2. 1 cases/1 000 000 persons) have been reported. The nationwide survey allowed the characterization of 60 PMR and 35 GCA cases. Median time to the onset of first symptoms was 10 (range 2-30) and 7 (range 2-25) days for PMR and GCA, respectively. Phenotype, GCA-related ischemic complications and -large vessel vasculitis as well as therapeutic management and follow-up seemed similar according to the number of vaccine shots received and when compared to the literature data of unvaccinated population. Although rare, the short time between immunization and the onset of first symptoms of PMR and GCA suggests a temporal association. Physician should be aware of this potential vaccine-related phenomenon.
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COVID-19 , Arteritis de Células Gigantes , Polimialgia Reumática , Adulto , Humanos , Persona de Mediana Edad , Arteritis de Células Gigantes/epidemiología , Polimialgia Reumática/epidemiología , Vacunas contra la COVID-19/efectos adversos , Ad26COVS1 , Vacuna BNT162 , ChAdOx1 nCoV-19 , COVID-19/epidemiología , COVID-19/prevención & control , Vacunación/efectos adversosRESUMEN
INTRODUCTION: Immunocompromised patients can show prolonged shedding of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and persistent symptoms, which is called persistent COVID-19. CASE PRESENTATION: We report a case of an immunocompromised patient who was treated for mantle cell lymphoma and was suffering from B-cell depletion. The patient developed persistent COVID-19, which was confirmed by real-time polymerase chain reaction (RT-PCR) tests in only sputum and bronchoalveolar fluid which remained positive for at least 112 days. The patient was successfully treated with SARS-CoV-2 convalescent plasma. CONCLUSION: It could be of interest to investigate the RT-PCR results of SARS-CoV-2 in sputum/bronchoalveolar lavage samples from immunocompromised patients with unexplained pneumonia.
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COVID-19 , Huésped Inmunocomprometido , Linfoma de Células del Manto , SARS-CoV-2 , Humanos , COVID-19/inmunología , COVID-19/diagnóstico , COVID-19/complicaciones , SARS-CoV-2/aislamiento & purificación , SARS-CoV-2/inmunología , Masculino , Linfoma de Células del Manto/diagnóstico , Líquido del Lavado Bronquioalveolar/virología , Sueroterapia para COVID-19 , Persona de Mediana Edad , Inmunización Pasiva , EsputoRESUMEN
OBJECTIVES: Non-Hodgkin's lymphoma (NHL) risk assessment is crucial in Sjögren's syndrome (SS). We studied the prevalence of clonal immunoglobulin gene rearrangements in minor salivary glands (MSG) and their correlations with lymphoma occurrence and with previously established NHL predictors. METHODS: Molecular B-cell expansion was studied in fresh-frozen MSG of 207 patients with either suspected SS or with suspected lymphoma during SS, using a standardised multiplex PCR assay combined with heteroduplex analysis by microcapillary electrophoresis. The assignation of clonal cases was based on EuroClonality consortium guidelines. RESULTS: Among 207 studied patients, 31 (15%) had MSG monoclonal B-cell infiltration. Monoclonality was significantly more frequent in patients with SS (28/123, 22.8%) compared with patients without SS (3/84, 3.6%, P<0.001). Monoclonal B-cell infiltration in MSG of SS patients correlated significantly with ongoing salivary gland NHL, salivary gland swelling, CD4+ T-cell lymphopenia, rheumatoid factor (RF) activity, low complement levels and type 2 mixed cryoglobulinemia. The accumulation of biological risk factors was associated with a higher rate of MSG B-cell monoclonality given that patients with only positive RF had no probability of MSG B-cell monoclonality, RF-positive patients with 1 or 2 other risk factors had a 25.0% and 85.7% probability of MSG B-cell monoclonality, respectively. CONCLUSION: The detection of MSG monoclonal B-cell expansion by this easy-to-perform molecular assay is useful, both at the time of diagnosis and during the course of SS. Monoclonal B-cell expansion is associated with a subset of SS patients presenting either ongoing lymphoma or other established lymphoma predictive factors.
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Linfocitos B , Glándulas Salivales Menores , Síndrome de Sjögren , Humanos , Síndrome de Sjögren/inmunología , Síndrome de Sjögren/diagnóstico , Síndrome de Sjögren/genética , Femenino , Persona de Mediana Edad , Medición de Riesgo/métodos , Masculino , Linfocitos B/inmunología , Anciano , Adulto , Glándulas Salivales Menores/patología , Linfoma no Hodgkin/patología , Linfoma no Hodgkin/diagnóstico , Linfoma no Hodgkin/genética , Linfoma no Hodgkin/inmunología , Neoplasias de las Glándulas Salivales/genética , Neoplasias de las Glándulas Salivales/patología , Neoplasias de las Glándulas Salivales/inmunología , Anciano de 80 o más AñosRESUMEN
Neutrophil recruitment to sites of infection and inflammation is an essential process in the early innate immune response. Upon activation, a subset of neutrophils rapidly assembles the multiprotein complex known as the NLRP3 inflammasome. The NLRP3 inflammasome forms at the microtubule organizing center, which promotes the formation of interleukin (IL)-1ß and IL-18, essential cytokines in the immune response. We recently showed that mice deficient in NLRP3 (NLRP3-/-) have reduced neutrophil recruitment to the peritoneum in a model of thioglycolate-induced peritonitis. Here, we tested the hypothesis that this diminished recruitment could be, in part, the result of defects in neutrophil chemotaxis. We find that NLRP3-/- neutrophils show loss of cell polarization, as well as reduced directionality and velocity of migration toward increasing concentrations of leukotriene B4 (LTB4) in a chemotaxis assay in vitro, which was confirmed through intravital microscopy of neutrophil migration toward a laser-induced burn injury of the liver. Furthermore, pharmacologically blocking NLRP3 inflammasome assembly with MCC950 in vitro reduced directionality but preserved nondirectional movement, indicating that inflammasome assembly is specifically required for polarization and directional chemotaxis, but not cell motility per se. In support of this, pharmacological breakdown of the microtubule cytoskeleton via nocodazole treatment induced cell polarization and restored nondirectional cell migration in NLRP3-deficient neutrophils in the LTB4 gradient. Therefore, NLRP3 inflammasome assembly is required for establishment of cell polarity to guide the directional chemotactic migration of neutrophils.
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Quimiotaxis , Leucotrieno B4 , Proteína con Dominio Pirina 3 de la Familia NLR , Animales , Ratones , Inflamasomas , Leucotrieno B4/metabolismo , Neutrófilos , Proteína con Dominio Pirina 3 de la Familia NLR/metabolismoRESUMEN
BACKGROUND: Anti-Jo-1 autoantibodies represent essential markers in the diagnosis of antisynthetase syndrome (ASS). In this retrospective study, we aimed to investigate whether their concentrations and fluctuations could both respectively reflect the severity and evolution of ASS. METHODS: Between 2015 and 2020, clinical and biological features of ASS patients with at least one positive measure of anti-Jo-1 autoantibody were collected. At each serum sampling, we assessed myositis activity by using the Myositis Intention to Treat Activities Index (MITAX) and compared anti-Jo-1 concentrations with ASS severity, anti-Jo-1 concentrations between patients with and without active disease, and changes in anti-Jo-1 concentrations with disease activity. RESULTS: Forty-eight patients with ASS had at least one positive determination of anti-Jo-1 concentration. Among them, twenty-nine patients had at least two determinations of anti-Jo-1 autoantibody in their follow-up. We showed that these autoantibody concentrations were significantly correlated with MITAX (r = 0.4, p = 0.03) and creatine kinase concentration (r = 0.34, p = 0.002) and that they were significantly higher in patients with active disease than in those with inactive disease (91.7 IU/L vs 44.4 IU/L, p = 0.016). During follow-up, we found a significant correlation between fluctuations of anti-Jo-1 autoantibody concentrations and MITAX score (r = 0.7, p < 0.0001). CONCLUSION: Our results suggest that anti-Jo-1 autoantibody concentration could be a predictive marker of the severity and evolution of ASS and show that their quantification could represent a precious tool for disease monitoring and for improving the therapeutic management of ASS patients.
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Autoanticuerpos , Miositis , Humanos , Biomarcadores , Estudios RetrospectivosRESUMEN
OBJECTIVE: Giant cell arteritis (GCA) is the most common systemic vasculitis in individuals aged ≥50 years. Its course is marked by a high relapse rate requiring long-term glucocorticoid use with its inherent adverse effects. We aimed to identify factors associated with relapses or recurrences in GCA at diagnosis. METHODS: We reviewed the medical records of consecutive patients with GCA diagnosed between 2009 and 2019 and followed for at least 12 months. We recorded their characteristics at onset and during follow-up. Factors associated with relapses or recurrences were identified using multivariable analysis. RESULTS: We included 153 patients, among whom 68% were female with a median age of 73 (47-98) years and a median follow-up of 32 (12-142) months. Seventy-four patients (48.4%) had at least 1 relapse or recurrence. Headache and polymyalgia rheumatica were the most frequent manifestations of relapses. The first relapse occurred at a median time of 13 months after the diagnosis, with a median dose of 5.5 (0-25) mg/d of glucocorticoids.In multivariable analysis, patients with relapses or recurrences had a higher frequency of cough and scalp tenderness at diagnosis (20.3% vs 5.1%; odds ratio [OR], 4.73; 95% confidence interval [CI], 1.25-17.94; p = 0.022; and 41.9% vs 29.1%; OR, 2.4; 95% CI, 1.07-5.39; p = 0.034, respectively). Patients with diabetes mellitus at diagnosis had fewer relapses or recurrences during follow-up (5.4% vs 19%; OR, 0.24; 95% CI, 0.07-0.83; p = 0.024). CONCLUSIONS: Cough and scalp tenderness at diagnosis were associated with relapses or recurrences, whereas patients with diabetes experienced fewer relapses or recurrences.
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Arteritis de Células Gigantes , Polimialgia Reumática , Humanos , Femenino , Anciano , Anciano de 80 o más Años , Masculino , Arteritis de Células Gigantes/diagnóstico , Arteritis de Células Gigantes/tratamiento farmacológico , Arteritis de Células Gigantes/epidemiología , Tos/inducido químicamente , Tos/complicaciones , Glucocorticoides/efectos adversos , Polimialgia Reumática/diagnóstico , Polimialgia Reumática/tratamiento farmacológico , Polimialgia Reumática/epidemiología , Dolor , Recurrencia , Registros MédicosRESUMEN
PURPOSE: The purpose of this study is to assess the diagnostic accuracy of paracentral acute middle maculopathy (PAMM) in the setting of anterior ischemic optic neuropathy (AION) to distinguish arteritic (A-AION) from nonarteritic (NA-AION) type. DESIGN: Retrospective cross-sectional diagnostic evaluation. METHODS: PAMM was evaluated by 3 physicians blinded to diagnosis using macular spectral-domain optical coherence tomography. We studied 45 patients with AION. Of those, 28 had NA-AION and 17 had A-AION. The study was conducted in the Department of Ophthalmology at the Hospital of Marseille-Assistance Publique, France, from January 1, 2018, to March 31, 2022. RESULTS: PAMM were only found in the A-AION group (N = 4) (P = .0143). As a distinctive sign of A-AION, we found a specificity of 100% (95% IC, 88.06%-100%) and a positive predictive value of 100%. In contrast, sensitivity and negative predictive value were lower, 19.1% (95% IC, 5.5-42.0) and 63.0% (95% CI, 58.1-67.7), respectively. CONCLUSIONS: The PAMM finding is highly specific for A-AION in the setting of AION. According to our results, macular spectral-domain optical coherence tomography looking for PAMM should be performed with any patient presenting with AION.
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Arteritis , Degeneración Macular , Neuropatía Óptica Isquémica , Humanos , Estudios Retrospectivos , Neuropatía Óptica Isquémica/diagnóstico , Estudios Transversales , Arteritis/diagnóstico , Tomografía de Coherencia Óptica/métodosRESUMEN
During SARS-CoV-2 infection, eosinopenia may reflect a hyperactive immune response. In this study of hospitalized COVID-19 patients, we aimed to better understand the prognostic value of severe eosinopenia (absolute eosinophil count = 0 G/L) and decipher its underlying mechanisms. We retrospectively analyzed the records of COVID-19 patients hospitalized from March to June 2020 in three university hospitals in Marseille, France. We assessed the association between severe eosinopenia and a composite poor outcome in these patients, including the need for oxygen supplementation at >6 L/min, ICU admission, and in-hospital death. Among the 551 COVID-19 patients included in this study, severe eosinopenia was found in 228 (51%) of them on admission to hospital and was associated with a composite poor outcome using multivariate analysis (OR = 2.58; CI95 [1.77−3.75]; p < 0.0001). We found a significant association between the presence of severe eosinopenia on admission and the elevation in C-reactive protein, ferritin, IP-10, and suPAR. The histological findings in a series of 37 autopsies from patients who died from severe COVID-19 and presented with severe eosinopenia showed no pulmonary eosinophil trapping. Severe eosinopenia can be a reliable biomarker associated with a composite poor outcome in hospitalized COVID-19 adult patients. It may reflect the magnitude of immune hyperactivation during severe-to-critical COVID-19.
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OBJECTIVE: Giant cell arteritis (GCA) is the most frequent vasculitis affecting adults aged > 50 years. Cardiac involvement in GCA is considered rare, and only a few cases of pericarditis have been reported. The aim of this study was to determine the characteristics and prognosis of GCA patients suffering from pericardial involvement at diagnosis. METHODS: We conducted a single-centre, retrospective chart review of patients with GCA in internal medicine departments (from 2000 to 2020). Patients were identified through a centralized hospital database. We retrospectively collected demographic, clinicobiological, histological, imaging, treatment and outcome data. Patients with pericardial effusion, defined as an effusion visible on the CT-scan performed at GCA diagnosis were compared to those without pericardial involvement. RESULTS: Among the 250 patients with GCA, 23 patients (9.2%) had pericardial effusion on CT-scan. The comparison between the groups revealed similar distribution of age, gender, cranial symptoms and ocular ischaemic complications. Patients with pericardial effusion had a higher frequency of weight loss. They also had lower haemoglobin levels and higher platelet levels (p = 0.006 and p = 0.002, respectively), and they more frequently had positive temporal artery biopsy. There were no differences concerning the treatment, relapses, follow-up duration or deaths. CONCLUSIONS: This case series sheds light on GCA as a cause of unexplained pericardial effusion or symptomatic pericarditis among adults aged > 50 years and elevated inflammatory biological markers. Fortunately, pericardial involvement is a benign GCA manifestation. In that context, the search for constitutional symptoms, cranial symptoms and associated signs of polymyalgia rheumatica is crucial for rapidly guiding GCA diagnosis.
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Arteritis de Células Gigantes , Derrame Pericárdico , Pericarditis , Polimialgia Reumática , Biomarcadores , Arteritis de Células Gigantes/complicaciones , Arteritis de Células Gigantes/diagnóstico por imagen , Hemoglobinas , Humanos , Derrame Pericárdico/diagnóstico por imagen , Derrame Pericárdico/etiología , Pericarditis/complicaciones , Polimialgia Reumática/diagnóstico , Estudios RetrospectivosRESUMEN
Dexamethasone has demonstrated efficacy in reducing mortality in COVID-19. However, its practical use is badly defined. We aimed to investigate factors associated with dexamethasone efficacy in real life. Our retrospective study was conducted in two university hospitals between September and November 2020 and included all the consecutive hospitalized patients with a laboratory-confirmed SARS-CoV-2 infection assessed by RT-PCR, treated with intravenous dexamethasone (6 mg/day). Among 111 patients, 10.6% necessitated a transfer into the intensive care unit (ICU) and the 28-day mortality rate was 17.1%. The 28-day mortality rate was significantly lower in patients who demonstrated improvement at 48 h (hazard ratio [HR]: 0.17, 95% confidence interval [CI]: 0.04-0.78, p = 0.02) and 96 h (HR: 0.07, 95% CI: 0.02-0.31, p = 0.0005) after dexamethasone initiation. Apart from well-known risk factors (age, hypertension, active cancer, severe lesions on chest computed tomography [CT] scan), we found that a high viral load in nasopharyngeal swab (Cycle threshold <30) at dexamethasone initiation was associated with higher 28-day mortality (66.6% vs. 36.7%, p = 0.03). Patients who did not receive antibiotics at dexamethasone initiation had a higher rate of transfer into the ICU (55.6% vs. 23.5%, p = 0.045) with a trend towards higher mortality in case of severe or critical lesions on CT scan (75.0% vs. 25.0%, p = 0.053). Patients who did not improve within 2-4 days after steroid initiation have a bad prognosis and should receive additional anti-inflammatory drugs. Our data suggest better efficacy of dexamethasone in patients with a low or negative viral load, receiving broad-spectrum antibiotics.
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Tratamiento Farmacológico de COVID-19 , Antibacterianos/uso terapéutico , Estudios de Cohortes , Dexametasona/uso terapéutico , Humanos , Estudios Retrospectivos , SARS-CoV-2RESUMEN
Aortitis is a classic manifestation of large vessel vasculitis. Antiphospholipid syndrome (APS), sometimes known as Hughes syndrome, is an acquired autoimmune disorder that manifests clinically as recurrent venous or arterial thrombosis. Patients with APS may also suffer from various underlying diseases, most frequently systemic lupus erythematosus (SLE). Catastrophic antiphospholipid syndrome (CAPS) is a rare but serious complication of APS characterized by failure of several organs due to diffuse microcirculatory thrombi. Its main manifestations involve the kidneys, lungs, heart and central nervous system, and require early diagnosis and rapid therapeutic management. While APS can affect virtually any blood vessel, aortitis is not a known symptom of APS. We report the case of a 36-year-old patient with APS and SLE who presented with CAPS during pregnancy, with no concomitant SLE flare. The first manifestation of CAPS was aortitis, preceding renal, cardiac and haematological manifestations. The outcome was favourable with combined treatment including corticosteroids, anticoagulants, plasma exchange and rituximab. We then carried out a literature search for papers describing the presence of aortitis in APS and/or SLE. In the cases of aortic involvement identified in the literature, including another case of CAPS, the occurrence of aortitis in SLE, often associated with the presence of antiphospholipid antibodies/APS, suggests that aortitis should be considered as an under-recognized manifestation and potential non-criterion feature of APS.
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Síndrome Antifosfolípido , Aortitis , Lupus Eritematoso Sistémico , Trombosis , Adulto , Anticuerpos Antifosfolípidos , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnóstico , Aortitis/complicaciones , Aortitis/etiología , Femenino , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Microcirculación , EmbarazoRESUMEN
BACKGROUND: Hypocomplementemic urticarial vasculitis (HUV) is a rare systemic vasculitis. We aimed to describe the kidney involvement of HUV in a multicenter national cohort with an extended follow-up. METHODS: All patients with HUV (international Schwartz criteria) with a biopsy-proven kidney involvement, identified through a survey of the French Vasculitis Study Group (FVSG), were included. A systematic literature review on kidney involvement of HUV was performed. RESULTS: Twelve patients were included, among whom 8 had positive anti-C1q antibodies. All presented with proteinuria, from mild to nephrotic, and 8 displayed acute kidney injury (AKI), requiring temporary haemodialysis in 2. Kidney biopsy showed membrano-proliferative glomerulonephritis (MPGN) in 8 patients, pauci-immune crescentic GN or necrotizing vasculitis in 3 patients (with a mild to severe interstitial inflammation), and an isolated interstitial nephritis in 1 patient. C1q deposits were observed in the glomeruli (n = 6), tubules (n = 4) or renal arterioles (n = 3) of 8 patients. All patients received corticosteroids, and 9 were also treated with immunosuppressants or apheresis. After a mean follow-up of 8.9 years, 6 patients had a preserved renal function, but 2 patients had developed stage 3-4 chronic kidney disease (CKD) and 4 patients had reached end-stage kidney disease (ESKD), among whom 1 had received a kidney transplant. CONCLUSION: Renal involvement of HUV can be responsible for severe AKI, CKD and ESRD. It is not always associated with circulating anti-C1q antibodies. Kidney biopsy shows mostly MPGN or crescentic GN, with frequent C1q deposits in the glomeruli, tubules or arterioles.
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Glomerulonefritis Membranoproliferativa/complicaciones , Urticaria/complicaciones , Vasculitis/complicaciones , Corticoesteroides/uso terapéutico , Adulto , Anciano , Biopsia , Eliminación de Componentes Sanguíneos , Niño , Preescolar , Complemento C1q/metabolismo , Ciclofosfamida/uso terapéutico , Femenino , Estudios de Seguimiento , Glomerulonefritis Membranoproliferativa/tratamiento farmacológico , Glomerulonefritis Membranoproliferativa/inmunología , Glomerulonefritis Membranoproliferativa/patología , Humanos , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Rituximab/uso terapéutico , Síndrome , Urticaria/inmunología , Vasculitis/inmunologíaRESUMEN
Specificities of COVID-19 disease course in patients with haematologic malignancies are still poorly studied. So, we aimed to compare patients with haematologic malignancies to patients without malignancies, matched by sex and age and hospitalised for COVID-19 at the same time and in the same centre. Among 25 patients with haematologic malignancies, we found that mortality (40% versus 4%, p < 0.01), number of days with RT-PCR positivity (21.2 ± 15.9 days [range, 3-57] versus 7.4 ± 5.6 days [range, 1-24], p < 0.01), maximal viral load (mean minimal Ct, 17.2 ± 5.2 [range, 10-30] versus 26.5 ± 5.1 [range, 15-33], p < 0.0001) and the delay between symptom onset and clinical worsening (mean time duration between symptom onset and first day of maximum requirement in inspired oxygen fraction, 14.3 ± 10.7 days versus 9.6 ± 3.7 days, p = 0.0485) were higher than in other patients. COVID-19 course in patients with haematologic malignancies has a delayed onset and is more severe with a higher mortality, and patients may be considered as super-spreaders. Clinicians and intensivists need to be trained to understand the specificity of COVID-19 courses in patients with haematological malignancies.
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COVID-19/epidemiología , Neoplasias Hematológicas/epidemiología , Leucemia/epidemiología , Linfoma/epidemiología , Mieloma Múltiple/epidemiología , SARS-CoV-2/patogenicidad , Adulto , Anciano , Anciano de 80 o más Años , COVID-19/terapia , COVID-19/virología , Enfermedades Cardiovasculares/epidemiología , Comorbilidad , Diabetes Mellitus/epidemiología , Femenino , Mortalidad Hospitalaria , Humanos , Masculino , Desnutrición/epidemiología , Persona de Mediana Edad , SARS-CoV-2/aislamiento & purificación , Fumar/epidemiología , Resultado del Tratamiento , Carga ViralRESUMEN
OBJECTIVE: To assess the efficacy and tolerance of tocilizumab (TCZ) in giant cell arteritis (GCA) patients over 80. METHOD: GCA patients over 80 years old from the French Study Group for Large Vessel Vasculitis register who received TCZ were analyzed. RESULTS: Twenty-one GCA patients (median age 84 [81-90] years old, including nine over 85) received TCZ for the following nonexclusive reasons: glucocorticoid (GC)-sparing effect in 14, relapsing disease in 8, disease severity in 4, and/or failure of another immunosuppressant in 4. TCZ was introduced with GCs at diagnosis in 6 patients and at 8 [3-37] months after GC initiation in 15 others. After a median delay of 8 [2-21] months post-TCZ introduction, 14 (67%) patients were able to definitively stop GCs, including 6 who were GC-dependent before TCZ. At the last follow-up (median 20 [3-48] months), 11 (52%) patients had definitively stopped TCZ, and 2 additional patients had stopped but relapsed and resumed TCZ. Seven (33%) patients experienced 11 adverse events: hypercholesterolemia in 4 patients; infections, i.e., pyelonephritis, bronchitis, and fatal septic shock associated with mesenteric infarction following planned surgery (GCs were stopped for 1 year and TCZ infusions for 2 months), respectively, in 3 patients; moderate thrombocytopenia and moderate neutropenia in 2 patients; and a 5-fold increase in transaminase levels in another that improved after TCZ dose reduction. CONCLUSION: TCZ remains a valuable GC-sparing option in the oldest GCA patients with an interesting risk-benefit ratio. Mild-to-moderate adverse events were observed in one-third of patients.
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Arteritis de Células Gigantes , Anciano de 80 o más Años , Anticuerpos Monoclonales Humanizados/efectos adversos , Arteritis de Células Gigantes/tratamiento farmacológico , Humanos , Inmunosupresores , Resultado del TratamientoAsunto(s)
Médula Ósea/virología , COVID-19/complicaciones , Pancitopenia/etiología , SARS-CoV-2/patogenicidad , Macroglobulinemia de Waldenström/complicaciones , Médula Ósea/patología , COVID-19/patología , COVID-19/virología , Resultado Fatal , Femenino , Humanos , Persona de Mediana Edad , Pancitopenia/patología , Pancitopenia/virología , SARS-CoV-2/aislamiento & purificación , Macroglobulinemia de Waldenström/patología , Macroglobulinemia de Waldenström/virologíaRESUMEN
OBJECTIVE: To determine the clinical significance of anti-nuclear mitotic apparatus (NuMA) antibodies (AC-26 or AC-25) in patients with primary Sjögren's syndrome (pSS) and SLE. METHODS: Between 2013 and 2018, clinical and immunological features of pSS and SLE patients with anti-NuMA antibodies were compared with anti-NuMA antibodies-negative pSS and SLE cohorts. RESULTS: Among 31 284 sera positive for antinuclear antibodies, 90 patients (0.29%) had anti-AC-26 (anti-NuMA1) and AC-25 (anti-HsEg5) antibodies (73.3% and 26.7%, respectively). Autoimmune diseases, mainly consisting in pSS (28.9%) and SLE (21.1%), were found in 67.8%. Anti-NuMA antibodies represented the unique ANA in 60% and 50% of patients with pSS and SLE patients, respectively. Compared with 137 anti-NuMA-negative pSS patients, 20 anti-NuMA-positive pSS presented with less frequent ocular sicca syndrome (70.0% vs 89.1%, P=0.031), dryness complications (15.0% vs 39.4%, P=0.045), or detectable anti-SSa and/or anti-SSb antibodies (40.0% vs 66.4%, P=0.027). Compared with 80 anti-NuMA-negative SLE patients, 14 anti-NuMA-positive SLE patients had no lupus nephritis (0.0% vs 28.8%, P=0.049), less frequent dsDNA antibodies (42.9% vs 75.0%, P=0.025) and complement consumption (21.4% vs 53.8%, P=0.040). Anti-NuMA-positive pSS and SLE patients less frequently required treatments compared with anti-NuMA-negative patients. CONCLUSION: Although rare, anti-NuMA antibodies are mainly associated with pSS and SLE and may be useful for diagnosis when other auto-antibodies are negative. PSS and SLE patients with anti-NuMA antibodies have less severe clinical and biological profiles, suggesting that anti-NuMA antibodies may constitute a good prognosis marker in both autoimmune diseases.
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Autoanticuerpos/sangre , Proteínas de Ciclo Celular/inmunología , Lupus Eritematoso Sistémico/inmunología , Síndrome de Sjögren/inmunología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores/sangre , Niño , Preescolar , Femenino , Humanos , Lactante , Lupus Eritematoso Sistémico/sangre , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Síndrome de Sjögren/sangre , Adulto JovenRESUMEN
OBJECTIVE: To assess the prevalence of axial articular manifestations (AAMs) in patients with primary Sjögren syndrome (pSS), to investigate whether these symptoms reveal an associated spondyloarthritis (SpA), and to assess their therapeutic management. METHODS: Among 148 consecutive patients with pSS fulfilling European League Against Rheumatism (EULAR)/American College of Rheumatology 2019 classification criteria followed between 2010 and 2018, we selected those who presented with AAMs. The association with SpA was retained when patients fulfilled Assessment of SpA international Society criteria. RESULTS: A total of 29 patients (20%, 28 women) with a median age of 43 years (range 15-65 yrs), were identified. The main extraglandular features were peripheral arthralgia and arthritis in 93% and 90% of patients, respectively. Positive anti-Ro/SSA (anti-SSA) antibody was reported in 62%. AAMs were inaugural in 7%, delayed from the diagnostic of pSS in 7%, and occurred concomitantly in 86% of patients. AAMs were not associated to multisystemic involvement of pSS. Radiographic sacroiliitis was mentioned in 65%, and HLA-B27 was positive in 13%. The diagnosis of SpA was retained in 23/29 patients (79%), among which 74% and 26% fulfilled psoriatic arthritis and ankylosing spondylitis criteria, respectively. There was no phenotypic difference according to the anti-SSA antibody status. With a median follow-up of 60 months (range: 5-96), 61% of patients with associated SpA required biotherapies, mainly of anti-tumor necrosis factor-α or anti-interleukin 17A molecules with a good clinical outcome in 64% and no effect on pSS. CONCLUSION: AAMs are not uncommon in patients with pSS and may reveal an associated SpA. Treatment of AAMs, especially when clearly associated with SpA, may necessitate biologics, following SpA-management therapeutic guidelines.
Asunto(s)
Sacroileítis , Síndrome de Sjögren , Espondiloartritis , Espondilitis Anquilosante , Adolescente , Adulto , Anciano , Femenino , Antígeno HLA-B27 , Humanos , Masculino , Persona de Mediana Edad , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/epidemiología , Espondiloartritis/complicaciones , Espondiloartritis/epidemiología , Espondilitis Anquilosante/complicaciones , Espondilitis Anquilosante/epidemiología , Adulto JovenRESUMEN
BACKGROUND: Daily management to shield chronic dialysis patients from SARS-CoV-2 contamination makes patient care cumbersome. There are no screening methods to date and a molecular biology platform is essential to perform RT-PCR for SARS-CoV-2; however, accessibility remains poor. Our goal was to assess whether the tools routinely used to monitor our hemodialysis patients could represent reliable and quickly accessible diagnostic indicators to improve the management of our hemodialysis patients in this pandemic environment. METHODS: In this prospective observational diagnostic study, we recruited patients from La Conception hospital. Patients were eligible for inclusion if suspected of SARS-CoV-2 infection when arriving at our center for a dialysis session between March 12th and April 24th 2020. They were included if both RT-PCR result for SARS-CoV-2 and cell blood count on the day that infection was suspected were available. We calculated the area under the curve (AUC) of the receiver operating characteristic curve. RESULTS: 37 patients were included in the final analysis, of which 16 (43.2%) were COVID-19 positive. For the day of suspected COVID-19, total leukocytes were significantly lower in the COVID-19 positive group (4.1 vs. 7.4 G/L, p = 0.0072) and were characterized by lower neutrophils (2.7 vs. 5.1 G/L, p = 0.021) and eosinophils (0.01 vs. 0.15 G/L, p = 0.0003). Eosinophil count below 0.045 G/L identified SARS-CoV-2 infection with AUC of 0.9 [95% CI 0.81-1] (p < 0.0001), sensitivity of 82%, specificity of 86%, a positive predictive value of 82%, a negative predictive value of 86% and a likelihood ratio of 6.04. CONCLUSIONS: Eosinophil count enables rapid routine screening of symptomatic chronic hemodialysis patients suspected of being COVID-19 within a range of low or high probability.