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1.
Case Rep Dermatol ; 16(1): 97-101, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38590388

RESUMEN

Introduction: Multicentric Castleman's disease (MCD) with cutaneous involvement has rarely been discussed in dermatologic literature, with few reports. Cutaneous lesions in MCD may induce deep scars, causing a significant impact in the daily life of the patients. The treatment of Castleman's disease (CD) is usually a challenge, especially in case of cutaneous involvement. Case Presentation: We report the case of a 35-year-old Caucasian man with a 3-year-old history of MCD with cutaneous involvement that we treated with a combined therapy characterized by siltuximab and 1,927 nm fractional laser. The patient showed a therapeutic response, characterized by a reduction of systemic symptoms and cutaneous manifestations. Conclusion: We believe that the combination of siltuximab and 1,927 nm fractional laser might have a synergistic beneficial role in patients with cutaneous iMCD and maximize esthetic outcomes. Anyway, additional evidence is needed to validate our findings.

2.
Vaccine ; 41(32): 4693-4699, 2023 07 19.
Artículo en Inglés | MEDLINE | ID: mdl-37349223

RESUMEN

Basophil activation test (BAT) can tackle multiple mechanisms underlying acute and delayed hypersensitivity to drugs and vaccines and might complement conventional allergy diagnostics but its role in anti-severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine-related hypersensitivity is ill-defined. Therefore, 89 patients with possible hypersensitivity (56 % with delayed mucocutaneous manifestations) to anti-SARS-CoV-2 vaccines were tested with BAT for Macrogol 3350, DMG-PEG 2000, PEG 20000, polysorbate-80 and trometamol and compared to 156 subjects undergoing pre-vaccine BAT. A positive BAT was associated with delayed reaction onset (p = 0.010) and resolution (p = 0.011). BAT was more frequently positive to DMG-PEG 2000 than to other excipients in both groups (p < 0.001). DMG-PEG 2000 reactivity was less frequent in vaccine-naïve (6 %) than vaccinated subjects (35 %, p < 0.001) and associated with mRNA-1273 vaccination. DMG-PEG 2000 BAT might therefore have a diagnostic role in subjects with delayed hypersensitivity reactions. Natural immunity might be a key player in basophil activation.


Asunto(s)
COVID-19 , Hipersensibilidad Tardía , Hipersensibilidad , Humanos , Basófilos , Excipientes/efectos adversos , Vacunas contra la COVID-19/efectos adversos , COVID-19/prevención & control , SARS-CoV-2
3.
Vaccines (Basel) ; 11(3)2023 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-36992151

RESUMEN

Anti-Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) vaccination is the world's most important strategy for stopping the pandemic. Vaccination challenges the body's immune response and can be complicated by hypersensitivity reactions. The autonomic nervous system can modulate the inflammatory immune response, therefore constituting a potential marker to characterize individuals at high risk of hypersensitivity reactions. Autonomic nervous system functionality was assessed through measurement of the heart rate variability (HRV) in subjects with a history of severe allergic reactions and 12 control subjects. HRV parameters included the mean electrocardiograph RR interval and the standard deviation of all normal R-R intervals (SDNN). All measurements were performed immediately before the anti-SARS-CoV-2 vaccination. The median RR variability was lower in the study than in the control group: 687 ms (645-759) vs. 821 ms (759-902); p = 0.02. The SDNN was lower in the study group than in the control group: 32 ms (23-36) vs. 50 ms (43-55); p < 0.01. No correlation was found between age and the SDNN. Autonomic nervous system activity is unbalanced in people with a severe allergy background.

4.
Clin Exp Rheumatol ; 41(3): 543-553, 2023 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-36916322

RESUMEN

Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient's representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final OP and PtC agreed by the Task Force.


Asunto(s)
Enfermedades Autoinmunes , COVID-19 , Síndrome de Dificultad Respiratoria , Enfermedades Reumáticas , Humanos , Enfermedades Autoinmunes/tratamiento farmacológico , Enfermedades Reumáticas/tratamiento farmacológico , Enfermedades Reumáticas/epidemiología , Antivirales/uso terapéutico
5.
Lupus ; 32(2): 263-269, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36519201

RESUMEN

OBJECTIVES: Neuropsychiatric symptoms develop in up to 20% of the patients with Systemic Lupus Erythematosus (SLE). Growing evidence is accruing on the association of SLE with Post-traumatic Stress Disorder (PTSD), but little is known about its contribution on patient-reported outcomes. This study focuses on PTSD prevalence in our SLE cohort and on its impact on quality of life. METHODS: Trauma and Loss Spectrum - Self Reported (TALS-SR) and Lupus Quality of Life (Lupus QoL) questionnaires were administered via web to the patients with SLE in our cohort, along with questions on demographical and disease-related aspects. RESULTS: Among 99 patients who completed the questionnaire, fatigue prevalence was 75% and 31% scored TALS-SR test consistently with PTSD. Patients with PTSD achieved lower scores compared to those without PTSD in three Lupus QoL domains: planning (83.3 vs. 100, p = .035), body image (85.0 vs. 95.0, p = .031) and fatigue (66.7 vs. 91.7, p = .001). An inverse correlation was found between TALS-SR domains and Lupus QoL scores, particularly regarding fatigue with reaction to losses or upsetting events (ρ -0.458, p < .001). CONCLUSIONS: PTSD is possibly far more frequent in patients with SLE than in general population and exerts a detrimental influence on quality of life.


Asunto(s)
Lupus Eritematoso Sistémico , Calidad de Vida , Trastornos por Estrés Postraumático , Humanos , Estudios Transversales , Fatiga/psicología , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/epidemiología , Lupus Eritematoso Sistémico/diagnóstico , Calidad de Vida/psicología , Trastornos por Estrés Postraumático/epidemiología , Trastornos por Estrés Postraumático/complicaciones , Trastornos por Estrés Postraumático/psicología , Encuestas y Cuestionarios
6.
Emerg Med J ; 40(3): 202-209, 2023 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-36522147

RESUMEN

BACKGROUND: Respiratory infections can be complicated by acute brain failure. We assessed delirium prevalence, predictors and outcomes in COVID-19 ED patients. METHODS: This was a retrospective observational study conducted at the San Raffaele ED (Italy). Patients age >18 years attending the ED between 26 February 2020 and 30 May 2020 and who had a positive molecular nasopharyngeal swab for SARS-CoV-2 were included. The Chart-Based Delirium Identification Instrument (CHART-DEL) was used to retrospectively assess delirium. Univariable and multivariable logistic regression analyses were used to evaluate delirium predictors. Univariable binary logistic regression analyses, linear regression analyses and Cox regression analyses were used to assess the association between delirium and clinical outcomes. Age-adjusted and sex-adjusted models were then run for the significant predictors of the univariable models. RESULTS: Among the 826 included patients, 123 cases (14.9%) of delirium were retrospectively detected through the CHART-DEL method. Patients with delirium were older (76.9±13.15 vs 61.3±14.27 years, p<0.001) and more frequently living in a long-term health facility (32 (26%) vs 22 (3.1%), p<0.001). Age (OR 1.06, 95% CI 1.04 to 1.09, p<0.001), dementia (OR 17.5, 95% CI 7.27 to 42.16, p<0.001), epilepsy (OR 6.96, 95% CI 2.48 to 19.51, p<0.001) and the number of chronic medications (OR 1.09, 95% CI 1.01 to 1.17, p=0.03) were significant predictors of delirium in multivariable analyses. Delirium was associated with increased in-hospital mortality (adjusted HR 2.16, 95% CI 1.55 to 3.03, p<0.001) and with a reduced probability of being discharged home compared with being institutionalised (adjusted OR 0.39, 95% CI 0.25 to 0.61, p<0.001). CONCLUSIONS: Chart review frequently identified ED delirium in patients with COVID-19. Age, dementia, epilepsy and polypharmacy were significant predictors of ED delirium. Delirium was associated with an increased in-hospital mortality and with a reduced probability of being discharged home after hospitalisation. The findings of this single-centre retrospective study require validation in future studies.


Asunto(s)
COVID-19 , Delirio , Demencia , Humanos , Adolescente , COVID-19/epidemiología , Estudios Retrospectivos , SARS-CoV-2 , Pandemias , Delirio/complicaciones , Delirio/epidemiología , Demencia/complicaciones , Servicio de Urgencia en Hospital
7.
J Autoimmun ; 132: 102900, 2022 10.
Artículo en Inglés | MEDLINE | ID: mdl-36087539

RESUMEN

Mechanisms for the generation of anti-dsDNA autoantibodies are still not completely elucidated. One theory states that dsDNA interacts for mimicry with antibodies raised versus other antigens but molecular features for mimicry are unknown. Here we show that, at physiological acid-base balance, anti-Annexin A1 binds IgG2 dsDNA in a competitive and dose-dependent way with Annexin A1 and that the competition between the two molecules is null at pH 9. On the other hand, these findings also show that dsDNA and Annexin A1 interact with their respective antibodies on a strictly pH-dependent basis: in both cases, the binding was minimal at pH 4 and maximal at pH9-10. The anionic charge of dsDNA is mainly conferred by the numerous phosphatidic residues. The epitope binding site of Annexin A1 for anti-Annexin A1 IgG2 was here characterized as a string of 34 amino acids at the NH2 terminus, 10 of which are anionic. Circulating levels of anti-dsDNA and anti-Annexin A1 IgG2 antibodies were strongly correlated in patients with systemic lupus erythematosus (n 496) and lupus nephritis (n 425) stratified for age, sex, etc. These results show that dsDNA competes with Annexin A1 for the binding with anti-Annexin A1 IgG2 on a dose and charged mediated base, being able to display an inhibition up to 75%. This study provides the first demonstration that dsDNA may interact with antibodies raised versus other anionic molecules (anti-Annexin A1 IgG2) because of charge mimicry and this interaction may contribute to anti-dsDNA antibodies generation.


Asunto(s)
Anexina A1 , Lupus Eritematoso Sistémico , Nefritis Lúpica , Humanos , Anticuerpos Antinucleares , Autoanticuerpos , Inmunoglobulina G , Anexina A1/metabolismo , ADN
8.
J Clin Med ; 11(13)2022 Jun 22.
Artículo en Inglés | MEDLINE | ID: mdl-35806873

RESUMEN

Tackling active disease to prevent damage accrual constitutes a major goal in the management of patients with systemic lupus erythematosus (SLE). Patients with early onset disease or in the early phase of the disease course are at increased risk of developing severe manifestations and subsequent damage accrual, while less is known about the course of the disease in the long term. To address this issue, we performed a multicentre retrospective observational study focused on patients living with SLE for at least 20 years and determined their disease status at 15 and 20 years after onset and at their last clinical evaluation. Disease activity was measured through the British Isles Lupus Assessment Group (BILAG) tool and late flares were defined as worsening in one or more BILAG domains after 20 years of disease. Remission was classified according to attainment of lupus low-disease-activity state (LLDAS) criteria or the Definitions Of Remission In SLE (DORIS) parameters. Damage was quantitated through the Systemic Lupus Erythematosus International Collaborating Clinics/American College of Rheumatology damage index (SLICC/ACR-DI). LLAS/DORIS remission prevalence steadily increased over time. In total, 84 patients had a late flare and 88 had late damage accrual. Lack of LLDAS/DORIS remission status at the 20 year timepoint (p = 0.0026 and p = 0.0337, respectively), prednisone dose ≥ 7.5 mg (p = 9.17 × 10-5) or active serology (either dsDNA binding, low complement or both; p = 0.001) were all associated with increased late flare risk. Late flares, in turn, heralded the development of late damage (p = 2.7 × 10-5). These data suggest that patients with longstanding SLE are frequently in remission but still at risk of disease flares and eventual damage accrual, suggesting the need for tailored monitoring and therapeutic approaches aiming at effective immunomodulation besides immunosuppression, at least by means of steroids.

9.
Vaccines (Basel) ; 10(5)2022 Apr 22.
Artículo en Inglés | MEDLINE | ID: mdl-35632419

RESUMEN

Vulnerable subjects, including systemic lupus erythematosus (SLE) patients, have been prioritised to receive anti-SARS-CoV-2 vaccines. Few data about the safety of these vaccines in SLE are available. The aim of our study is to investigate the safety of anti-SARS-CoV-2 vaccines in SLE. We included 452 SLE patients, referring to seven tertiary centres, who were immunised. A total of 119 (26%) reported side effects (SE) after the first and/or the second shot (the most frequent SE were fever, local reaction, fatigue, and arthralgia). Patients with constitutional symptoms and those on an immunosuppressive regimen (especially belimumab) showed more SE. In addition, 19 (4%) had a flare after the immunisation (flares classified by organ involvement: six musculoskeletal with constitutional symptoms, four renal, three cardio-respiratory, three haematological, two mucocutaneous). None of the patients needed hospitalisation and none died. Moreover, 15 required a transient increase in corticosteroids and four were treated with steroid pulses. One patient required an additional rituximab course. Anti-dsDNA, moderate/high DAS before vaccine, and belimumab were found more frequently in patients with disease flare. Anti-SARS-CoV-2 vaccines are safe in SLE patients, and they should be recommended in these patients, as the potential benefits widely outweigh the risk of SE. Treatment adjustment might be considered with the aim of minimising SE risk and flare.

10.
Maturitas ; 158: 37-39, 2022 04.
Artículo en Inglés | MEDLINE | ID: mdl-35241236

RESUMEN

Depression and selective serotonin reuptake inhibitors (SSRI) reduce bone mass and increase fracture risk. We analyzed the association between SSRI use and fractures development in nursing homes residents during a one-year prospective observational study. Sixty-four of the 800 participants developed a fracture during the one-year follow-up. Individuals who developed fractures used SSRIs more often than residents who did not (40.6% vs 28.7%, p =0.045). SSRIs were associated with fractures (adjusted OR 1.76, 95% CI 1.04 - 2.98, p = 0.036). A regular medication review should be performed to reduce inappropriate prescriptions and related adverse consequences.


Asunto(s)
Fracturas Óseas , Inhibidores Selectivos de la Recaptación de Serotonina , Anciano , Densidad Ósea , Fracturas Óseas/inducido químicamente , Fracturas Óseas/epidemiología , Humanos , Casas de Salud , Estudios Prospectivos , Inhibidores Selectivos de la Recaptación de Serotonina/efectos adversos
11.
Aging Clin Exp Res ; 34(4): 945-949, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-35298829

RESUMEN

OBJECTIVE: Exploring the association between frailty and mortality in a cohort of patients with COVID-19 respiratory insufficiency treated with continuous positive airway pressure. METHODS: Frailty was measured using a Frailty Index (FI) created by using the baseline assessment data on comorbidities and body mass index and baseline blood test results (including pH, lactate dehydrogenase, renal and liver function, inflammatory indexes and anemia). FI > 0.25 identified frail individuals. RESULTS: Among the 159 included individuals (81% men, median age of 68) frailty was detected in 69% of the patients (median FI score 0.3 ± 0.08). Frailty was associated to an increased mortality (adjusted HR 1.99, 95% CI 1.02-3.88, p = 0.04). CONCLUSIONS: Frailty is highly prevalent among patients with COVID-19, predicts poorer outcomes independently of age. A personalization of care balancing the risk and benefit of treatments (especially the invasive ones) in such complex patients is pivotal.


Asunto(s)
COVID-19 , Fragilidad , Insuficiencia Respiratoria , Anciano , Comorbilidad , Presión de las Vías Aéreas Positiva Contínua , Femenino , Anciano Frágil , Fragilidad/epidemiología , Evaluación Geriátrica/métodos , Humanos , Masculino , Insuficiencia Respiratoria/terapia
13.
Clin Nutr ; 41(12): 2965-2972, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-34465493

RESUMEN

BACKGROUND & AIMS: Sarcopenia, a loss of muscle mass, quality and function, which is particularly evident in respiratory muscles, has been associated with many clinical adverse outcomes. In this study, we aimed at evaluating the role of reduced muscle mass and quality in predicting ventilation weaning, complications, length of intensive care unit (ICU) and of hospital stay and mortality in patients admitted to ICU for SARS-CoV-2-related pneumonia. METHODS: This was an observational study based on a review of medical records of all adult patients admitted to the ICU of a tertiary hospital in Milan and intubated for SARS-CoV-2-related pneumonia during the first wave of the COVID-19 pandemic. Muscle mass and quality measurement were retrieved from routine thoracic CT scans, when sections passing through the first, second or third lumbar vertebra were available. RESULTS: A total of 81 patients were enrolled. Muscle mass was associated with successful extubation (OR 1.02, 95% C.I. 1.00-1.03, p = 0.017), shorter ICU stay (OR 0.97, 95% C.I. 0.95-0.99, p = 0.03) and decreased hospital mortality (HR 0.98, 95% C.I. 0.96-0.99, p = 0.02). Muscle density was associated with successful extubation (OR 1.07, 95% C.I. 1.01-1.14; p = 0.02) and had an inverse association with the number of complications in ICU (Β -0.07, 95% C.I. -0.13 - -0.002, p = 0.03), length of hospitalization (Β -1.36, 95% C.I. -2.21 - -0.51, p = 0.002) and in-hospital mortality (HR 0.88, 95% C.I. 0.78-0.99, p = 0.046). CONCLUSIONS: Leveraging routine CT imaging to measure muscle mass and quality might constitute a simple, inexpensive and powerful tool to predict survival and disease course in patients with COVID-19. Preserving muscle mass during hospitalisation might have an adjuvant role in facilitating remission from COVID-19.


Asunto(s)
COVID-19 , Desconexión del Ventilador , Adulto , Humanos , Desconexión del Ventilador/métodos , Pandemias , SARS-CoV-2 , Unidades de Cuidados Intensivos , Tiempo de Internación , Músculos , Respiración Artificial/métodos , Estudios Observacionales como Asunto
14.
Arthritis Rheumatol ; 74(2): 295-306, 2022 02.
Artículo en Inglés | MEDLINE | ID: mdl-34347947

RESUMEN

OBJECTIVE: Mepolizumab proved to be an efficacious treatment for eosinophilic granulomatosis with polyangiitis (EGPA) at a dose of 300 mg every 4 weeks in the randomized, controlled MIRRA trial. In a few recently reported studies, successful real-life experiences with the approved dose for treating severe eosinophilic asthma (100 mg every 4 weeks) were observed. We undertook this study to assess the effectiveness and safety of mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks in a large European EGPA cohort. METHODS: We included all patients with EGPA treated with mepolizumab at the recruiting centers in 2015-2020. Treatment response was evaluated from 3 months to 24 months after initiation of mepolizumab. Complete response to treatment was defined as no disease activity (Birmingham Vasculitis Activity Score [BVAS] = 0) and a prednisolone or prednisone dose (or equivalent) of ≤4 mg/day. Respiratory outcomes included asthma and ear, nose, and throat (ENT) exacerbations. RESULTS: Two hundred three patients, of whom 191 received a stable dose of mepolizumab (158 received 100 mg every 4 weeks and 33 received 300 mg every 4 weeks) were included. Twenty-five patients (12.3%) had a complete response to treatment at 3 months. Complete response rates increased to 30.4% and 35.7% at 12 months and 24 months, respectively, and rates were comparable between mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks. Mepolizumab led to a significant reduction in BVAS score, prednisone dose, and eosinophil counts from 3 months to 24 months, with no significant differences observed between 100 mg every 4 weeks and 300 mg every 4 weeks. Eighty-two patients (40.4%) experienced asthma exacerbations (57 of 158 [36%] who received 100 mg every 4 weeks; 17 of 33 [52%] who received 300 mg every 4 weeks), and 31 patients (15.3%) experienced ENT exacerbations. Forty-four patients (21.7%) experienced adverse events (AEs), most of which were nonserious AEs (38 of 44). CONCLUSION: Mepolizumab at both 100 mg every 4 weeks and 300 mg every 4 weeks is effective for the treatment of EGPA. The 2 doses should be compared in the setting of a controlled trial.


Asunto(s)
Anticuerpos Monoclonales Humanizados/administración & dosificación , Eosinofilia/tratamiento farmacológico , Granulomatosis con Poliangitis/tratamiento farmacológico , Adulto , Esquema de Medicación , Eosinofilia/complicaciones , Femenino , Granulomatosis con Poliangitis/complicaciones , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del Tratamiento
16.
Curr Rheumatol Rep ; 23(7): 52, 2021 07 01.
Artículo en Inglés | MEDLINE | ID: mdl-34196907

RESUMEN

PURPOSE OF REVIEW: To deepen the comprehension of the role of specific psychological conditions in the pathogenesis and in the treatment of systemic lupus erythematosus (SLE). Specifically, the present comprehensive review aims at examining the association between SLE, alexithymia (AT)-a personality construct referring to the inability to identify, describe, and express sensations, emotions, and physical state-and post-traumatic stress disorder (PTSD), to infer potential biological relationships between these psychopathological issues and disease course, and to draw up a research agenda on gray areas of these topics. RECENT FINDINGS: Whereas several studies document the presence of neuropsychiatric symptoms in patients with SLE, psychological distress, alexithymia, and post-traumatic manifestations are usually neglected by healthcare professionals and poorly investigated in research contexts. However, the interplay of these aspects, which affect physiologic stress coping mechanisms, potentially plays an important role in SLE pathogenesis. In particular, research documents that cytokine repertoire pattern alteration and hypothalamic-pituitary-adrenal axis impairment leading to inflammation and pain represent the main links between emotional health and immunity. AT and PTSD seem to be common in patients with SLE and account for multiple aspects of SLE-related morbidity. Furthermore, abnormal processing of stressful stimuli as hallmarks of PTSD and AT might promote neuroendocrine dysfunction and dysregulated immunity, thus contributing to the pathogenesis of SLE. A comprehensive, multidisciplinary clinical approach, based on a cooperation between immunologists, rheumatologists, neurologists, and mental health professionals, is crucial to promote patients' global health.


Asunto(s)
Lupus Eritematoso Sistémico , Trastornos por Estrés Postraumático , Síntomas Afectivos/etiología , Humanos , Sistema Hipotálamo-Hipofisario , Lupus Eritematoso Sistémico/complicaciones , Sistema Hipófiso-Suprarrenal , Trastornos por Estrés Postraumático/epidemiología , Trastornos por Estrés Postraumático/etiología
17.
Semin Arthritis Rheum ; 51(4): 761-765, 2021 08.
Artículo en Inglés | MEDLINE | ID: mdl-34144386

RESUMEN

BACKGROUND: The 2019 ACR/EULAR Classification Criteria for IgG4-related disease (IgG4-RD) represent a fundamental tool for patient enrollment in research studies and in clinical trials but their usefulness in daily clinical practice remains unknown. OBJECTIVE: To validate the 2019 ACR/EULAR Classification Criteria for IgG4-RD in a real-life setting and to anticipate their utility for orienting disease diagnosis and patient management. METHODS: Four experts were asked to classify 200 patients diagnosed with IgG4-RD according to the 2019 ACR/EULAR Classification Criteria for IgG4-RD. Agreement between experts was calculated and the Classification score of each patient was correlated with the following variables and outcomes: serum IgG4 and IgE; inflammatory markers; eosinophils; plasmablasts; IgG4-RD responder index; diabetes, osteoporosis, relapses; and use of rituximab. RESULTS: Among the 157/200 cases equally rated by at least three experts, 94 (59.9%) achieved IgG4-RD classification and 63 (40.1%) did not. Strong agreement among IgG4-RD experts was observed in classifying patients (k = 0.711, p<0.0001). Clinical presentations not included in the classification algorithm and lack of informative histology were the most common reasons for not achieving classification. In patients achieving classification, the Classification score did not correlate with variables of disease activity and was not associated with specific outcomes. CONCLUSIONS: The ACR/EULAR Classification Criteria represent a replicable instrument for classifying patients and a useful framework for orienting diagnosis but are of limited utility for assessing IgG4-RD activity, for predicting disease outcomes, and for defining personalized therapeutic approaches.


Asunto(s)
Enfermedad Relacionada con Inmunoglobulina G4 , Humanos , Inmunoglobulina G , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Enfermedad Relacionada con Inmunoglobulina G4/tratamiento farmacológico , Recurrencia , Rituximab
18.
Intern Med J ; 51(11): 1810-1815, 2021 11.
Artículo en Inglés | MEDLINE | ID: mdl-33961728

RESUMEN

BACKGROUND: COVID-19 long-term sequelae are ill-defined since only a few studies have explored the long-term consequences of this disease so far. AIMS: To evaluate the 6-month respiratory outcome and exercise capacity of COVID-19 acute respiratory failure (ARF) patients treated with continuous positive airway pressure (CPAP) during the first wave of the ongoing COVID-19 pandemic. METHODS: A retrospective observational study included COVID-19 patients with ARF. Interventions included CPAP during hospitalisation and 6-month follow up. Frailty assessment was carried out through frailty index (FI), pO2 /FiO2 during hospitalisation and at follow up, respiratory parameters, 6-min walking test (6MWT) and the modified British Medical Research Council (mMRC) and Borg scale at follow up. RESULTS: More than half of the patients had no dyspnoea according to the mMRC scale. Lower in-hospital pO2 /FiO2 correlated with higher Borg scale levels after 6MWT (ρ 0.27; P 0.04) at the follow-up visit. FI was positively correlated with length of hospitalisation (ρ 0.3; P 0.03) and negatively with the 6MWT distance walked (ρ -0.36; P 0.004). CONCLUSIONS: Robust and frail patients with COVID-19 ARF treated with CPAP outside the intensive care unit setting had good respiratory parameters and exercise capacity at 6-month follow up, although more severe patients had slightly poorer respiratory performance compared with patients with higher PaO2 /FiO2 and lower FI.


Asunto(s)
COVID-19 , Insuficiencia Respiratoria , Presión de las Vías Aéreas Positiva Contínua , Tolerancia al Ejercicio , Humanos , Pandemias , Insuficiencia Respiratoria/epidemiología , Insuficiencia Respiratoria/terapia , Estudios Retrospectivos , SARS-CoV-2
19.
Lupus ; 30(8): 1233-1243, 2021 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-33884900

RESUMEN

OBJECTIVE: To report baseline data of SLE patients enrolled in the Lupus Italian Registry (LIRE). METHODS: Patients affected by SLE aged ≥ 16 years were consecutively recruited in a multicenter prospective study comparing two cohorts: patients starting biologic immunosuppressants (BC) and patients starting non-biologic immunosuppresants (NBC). RESULTS: 308 patients were enrolled, 179 in NBC and 129 in BC. Mean age at disease onset and at diagnosis was significantly higher in NBC (p = 0.023, p = 0.045, respectively). Disease duration was longer in BC (p = 0.022). Patients in BC presented arthritis more frequently (p = 0.024), those in NBC nephropathy (p = 0.03). Quality of life was worse in BC (p = 0.031). Anti-dsDNA, low C3, were significantly more frequent in BC (p < 0.001, p = 0.009, respectively). Mycophenolate, methotrexate and azathioprine were the drugs more frequently prescribed in NBC, Belimumab and Rituximab in BC. CONCLUSION: The predominant organ involvement was different in the two cohorts: kidney involvement predominated in NBC, joint involvement in BC. Despite the younger age at disease onset, patients of the BC had a longer disease duration and more frequently had taken a cumulative prednisone dosage greater than 10 g. Even the pattern of clinical manifestations inducing to prescribe biological rather than conventional immunosuppressants was quite different.Keywords: Autoantibody(ies), autoimmune disease, belimumab, cohort studies, glucocorticoids, immunosuppressants, rituximab, systemic lupus erythematosus.


Asunto(s)
Lupus Eritematoso Sistémico , Reumatología , Humanos , Inmunosupresores/efectos adversos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/epidemiología , Estudios Prospectivos , Calidad de Vida , Sistema de Registros , Rituximab/uso terapéutico
20.
Clin Immunol ; 217: 108490, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32492478
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