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OBJECTIVES: To investigate the effects of passive immunization with the anti-SARS-CoV-2 monoclonal antibodies tixagevimab/cilgavimab on humoral responses and on COVID-19 outcomes in vaccine-refractory patients with immune-mediated inflammatory diseases (IMID) at high risk of severe COVID-19. METHODS: A prospective cohort study was performed on a cohort of high-risk vaccine-refractory IMID patients treated with a single dose of tixagevimab/cilgavimab (150 mg/150 mg). COVID-19 outcomes as well as serum and salivary anti-SARS-CoV-2 IgG were assessed at baseline and for at least 6 months. Results were compared with an untreated high-risk vaccine-refractory IMID population. Standardised incidence ratios (SIR) of COVID-19 compared with the general population were calculated for both groups. RESULTS: 38 high-risk IMID patients received tixagevimab/cilgavimab and were compared with 114 untreated high-risk IMID controls. Serum anti-Spike IgG increased to 6.6 OD (SD: ±0.8) at day one and remained positive up to month 6 (6.3 ± 1.4 OD). Salivary anti-Spike IgG peaked at month 2 (1.6 ± 1.1 OD)) and decreased from month 3 (0.8 ± 0.3 OD)). No severe or extended infection was observed in the tixagevimab/cilgavimab group. Compared with the general population, the SIR of COVID-19 in treated patients was 0.76 (95% CI: 0.24-1.58) despite the increased risk profile. The SIR of the control group was 1.51 (1.07-2.02), corresponding to a significantly increased incidence. CONCLUSIONS: Passive immunization with tixagevimab/cilgavimab is safe and effective in inducing anti-SARS-CoV-2 immunity and potentially in preventing COVID-19 in high-risk vaccine-refractory IMID patients. These data provide a proof of concept for the use of monoclonal antibodies as a preventative strategy against SARS-CoV-2 in vulnerable populations.
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Coronavirus disease-2019 (COVID-19) associated pneumonia may progress into acute respiratory distress syndrome (ARDS). Some patients develop features of macrophage activation syndrome (MAS). Elevated levels of IL-6 were reported to be associated with severe disease, and anti-IL-6R tocilizumab has been shown to be effective in some patients. This retrospective multicenter case-control study aimed to evaluate the efficacy of tocilizumab in hospitalized COVID-19 patients, who received standard of care with or without tocilizumab. Primary outcome was the progression to intubation or death. PSMATCH (SAS) procedure was used to achieve exact propensity score (PS) matching. Data from 1289 patients were collected, and study population was reduced to 1073 based on inclusion-exclusion criteria. The composite outcome was observed more frequently in tocilizumab-users, but there was a significant imbalance between arms in all critical parameters. Primary analyses were carried out in 348 patients (174 in each arm) after exact PS matching according to gender, ferritin, and procalcitonin. Logistic regression models revealed that tocilizumab significantly reduced the intubation or death (OR 0.40, p = 0.0017). When intubation is considered alone, tocilizumab-users had > 60% reduction in odds of intubation. Multiple imputation approach, which increased the size of the matched patients up to 506, provided no significant difference between arms despite a similar trend for intubation alone group. Analysis of this retrospective cohort showed more frequent intubation or death in tocilizumab-users, but PS-matched analyses revealed significant results for supporting tocilizumab use overall in a subset of patients matched according to gender, ferritin and procalcitonin levels.
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Anticorpos Monoclonais Humanizados/uso terapêutico , Antivirais/uso terapêutico , Tratamento Farmacológico da COVID-19 , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Purpose: To evaluate the affected extraocular muscles (EOMs) of patients with Graves' ophthalmopathy (GO) using real-time ultrasound elastography (UE) and to compare these results with those of healthy subjects.Methods: This prospective, comparative case series included 70 eyes of 35 patients with moderate-to-severe GO with type 2 orbitopathy (myogenic variant) according to their computed tomography (CT) scans. Forty-six eyes of 23 healthy subjects were evaluated as the control group. The strain ratio of orbital fat to medial rectus was calculated as the ratio of the medial rectus to orbital fat tissue, and the strain ratio of orbital fat to lateral rectus was calculated as the ratio of lateral rectus to orbital fat tissue.Results: The strain ratio of orbital fat to medial rectus was 3.03 ± 1.25 (range: 1.05-5.07) in the GO group, and 0.54 ± 0.20 (range: 0.14-1.08) in the control group (p = .0001). The strain ratio of orbital fat to lateral rectus was 0.97 ± 0.29 (range: 0.56-1.55) in the GO group, and 0.63 ± 0.23 (range: 0.18-1.09) in the control group (p = .0001).Conclusion: By using real-time UE, we found the medial and the lateral recti of GO patients to be stiffer compared to those of healthy subjects.
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Técnicas de Imagem por Elasticidade , Oftalmopatia de Graves/diagnóstico por imagem , Músculos Oculomotores/diagnóstico por imagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos ProspectivosRESUMO
PURPOSE: To analyze the relationship between the seasonal allergic conjunctivitis (SAC), eotaxin-2, matrix metalloproteinase-9 (MMP-9), and the topographical findings in the keratoconus patients. METHODS: Thirty-four eyes of patients without SAC (Group 1), 34 eyes of patients with SAC (Group 2), and 20 eyes of control subjects (control group) were enrolled. Tear samples of the subjects were collected by Schirmer method. Corneal topography parameters, tear MMP-9, and tear eotaxin-2 levels were analyzed. RESULTS: The mean tear MMP-9 levels in Groups 1 and 2 were significantly higher than in the control group (p = 0.004). MMP-9 level exhibited a positive correlation with the keratoconus stage and a negative correlation with the thinnest corneal thickness (r = 0.294, p = 0.018, and r = - 0.302, p = 0.006, respectively). The tear eotaxin-2 level was higher in Group 2 than in Group 1 and control group which is not statistically significant (p = 0.17). CONCLUSION: The tear eotaxin-2 did not exhibit any difference in the presence of keratoconus. The tear MMP-9 level was higher in the keratoconic eyes, and it showed a correlation with the stage of the disease and the corneal thickness.
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Quimiocina CCL24/metabolismo , Conjuntivite Alérgica/metabolismo , Córnea/patologia , Topografia da Córnea/métodos , Ceratocone/metabolismo , Metaloproteinase 9 da Matriz/metabolismo , Lágrimas/metabolismo , Adolescente , Adulto , Biomarcadores/metabolismo , Conjuntivite Alérgica/complicações , Conjuntivite Alérgica/diagnóstico , Ensaio de Imunoadsorção Enzimática , Feminino , Seguimentos , Humanos , Ceratocone/complicações , Ceratocone/diagnóstico , Masculino , Estudos Prospectivos , Adulto JovemRESUMO
PURPOSE: To investigate the long-term effects that CXL has on the tear function and ocular surface in keratoconus. METHODS: Twenty-one consecutive patients (24 eyes) with progressive keratoconus scheduled for CXL were included. All patients underwent the following procedures: conjunctival impression cytology analysis, ocular surface disease index (OSDI) score, tear osmolarity test, Schirmer test, tear film breakup time (TBUT), ophthalmic surface fluorescein (Fl) staining, and topographical corneal evaluation before as well as 3 and 18 months after accelerated CXL. RESULTS: There was no change in the median OSDI score, tear osmolarity test, Schirmer test, and the Fl staining score after CXL. The median TBUT increased from 9.00 s at baseline to 12.00 s at 18 months postoperative (P < 0.001). The cytological features of the temporal and superior bulbar conjunctiva deteriorated at 3 months post-CXL (P < 0.001). An improvement in impression cytology analysis of the temporal conjunctiva was noted at 18-month follow-up (P < 0.001). Significant improvements in the median maximum keratometry and mean keratometry (K-mean) readings were also noted 18 months after CXL (P < 0.001). The changes in the K-mean correlated significantly with the changes in TBUT levels at 18-month follow-up as compared to baseline (r = - 0.688, P < 0.001). CONCLUSIONS: The improvement in TBUT, conjunctival squamous metaplasia, and the goblet cell density indicates a favorable effect of CXL on the ocular surface and tear film in keratoconus, presumably due to the reduced corneal irregularity after CXL.
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Colágeno/farmacologia , Córnea/patologia , Topografia da Córnea/métodos , Reagentes de Ligações Cruzadas/farmacologia , Ceratocone/tratamento farmacológico , Fotoquimioterapia/métodos , Lágrimas/metabolismo , Adolescente , Adulto , Córnea/metabolismo , Feminino , Seguimentos , Humanos , Ceratocone/diagnóstico , Ceratocone/metabolismo , Masculino , Fármacos Fotossensibilizantes/uso terapêutico , Estudos Prospectivos , Riboflavina/uso terapêutico , Fatores de Tempo , Raios Ultravioleta , Acuidade Visual , Adulto JovemRESUMO
PURPOSE: To evaluate the cytologic features of conjunctival epithelium in patients with unilateral primary acquired nasolacrimal duct obstruction (PANDO) using impression cytology. METHODS: Twenty-four patients with unilateral PANDO for at least 1 year were enrolled in this study. The healthy eyes of the patients are assessed as control group. All patients were subjected to ophthalmic surface examination with conjunctival impression cytology analysis. Squamous metaplasia and goblet cell density were graded according to Nelson grading system. RESULTS: The mean age was 52.7 ± 16.3 (range 21-70) years. The mean period for complaints of epiphora was 3.2 ± 2.4 (range 1-8) years. The eye with PANDO had a mean squamous metaplasia grade of 2.38 ± 0.59 versus 1.91 ± 0.82 for control eyes (p = 0.011). The mean grade of goblet cell density was 2.0 ± 0.51 for eyes with PANDO and 2.38 ± 0.65 for control eyes (p = 0.013). There was no statistically significant correlation between conjunctival cytological features in terms of squamous metaplasia and goblet cell density grades and duration of epiphora complaint (r = -0.04, p = 0.82; r = 0.09, p = 0.66, respectively). CONCLUSIONS: PANDO may cause alterations in conjunctival impression cytology. Successful dacryocystorhinostomy surgeries will help to protect ocular surface health.
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Túnica Conjuntiva/patologia , Epitélio/patologia , Células Caliciformes/patologia , Obstrução dos Ductos Lacrimais/diagnóstico , Ducto Nasolacrimal/patologia , Adulto , Idoso , Contagem de Células , Dacriocistorinostomia , Progressão da Doença , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Ducto Nasolacrimal/cirurgia , Estudos Prospectivos , Fatores de Tempo , Adulto JovemRESUMO
The purpose of the study is to evaluate the efficacy and safety of as-needed dexamethasone (DEX) retreatment compared with standard DEX retreatment combined with PRN ranibizumab injections among patients with persistent diabetic macular edema (DME). Twenty-eight patients with persistent macular edema having recurrence earlier than 6 months after initial DEX implantation were included in this retrospective study. Group I consisted of 13 patients retreated using monthly PRN ranibizumab injections combined with standard 6-monthly DEX implantation; Group II consisted of 15 patients retreated with DEX implantation earlier than 6 months on an "as-needed" basis. There was no significant difference between the groups with regarding to age, gender, HbA1C levels, duration of diabetes, duration of macular edema, baseline central macular thickness (CMT), best-corrected visual acuity (BCVA), and intraocular pressure (IOP) values (p > 0.05). The mean follow-up time of the whole study population was 10.13 ± 1.75 months (range 9-15). The mean CMT values were significantly decreased in both groups compared to baseline values except for the 6th-month CMT in Group I (p < 0.05). The mean logMAR BCVA values were not statistically different between groups during the follow-up compared to baseline BCVA values (p > 0.05). However, a significant change in mean BCVA from baseline was seen at 4th, 6th, and 9th months in Group II (p ≤ 0.05). The mean number of total intravitreal treatments was 3.50 ± 0.77 in Group I and 2.53 ± 0.51 in Group II (p = 0.001). During the follow-up period, one patient in Group I and five patients in Group II had increased IOP (≥25 mmHg). Early DEX retreatment improved vision with superior anatomical improvement at 6th month and with fewer intravitreal treatments in eyes with DME. However, improvement in visual acuity is similar with standard DEX retreatment combined with PRN ranibizumab group.
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Inibidores da Angiogênese/administração & dosagem , Dexametasona/administração & dosagem , Retinopatia Diabética/tratamento farmacológico , Glucocorticoides/administração & dosagem , Edema Macular/tratamento farmacológico , Ranibizumab/administração & dosagem , Idoso , Retinopatia Diabética/fisiopatologia , Esquema de Medicação , Implantes de Medicamento , Quimioterapia Combinada , Feminino , Humanos , Pressão Intraocular/fisiologia , Injeções Intravítreas , Edema Macular/fisiopatologia , Masculino , Pessoa de Meia-Idade , Retratamento , Estudos Retrospectivos , Acuidade Visual/fisiologiaAssuntos
Ciclosporina , Imunossupressores , Lúpus Eritematoso Sistêmico , Mesentério/irrigação sanguínea , Doenças Peritoneais , Vasculite , Adulto , Ciclosporina/farmacologia , Ciclosporina/uso terapêutico , Feminino , Humanos , Terapia de Imunossupressão/efeitos adversos , Imunossupressores/farmacologia , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Mesentério/diagnóstico por imagem , Doenças Peritoneais/diagnóstico por imagem , Doenças Peritoneais/tratamento farmacológico , Circulação Esplâncnica/efeitos dos fármacos , Vasculite/diagnóstico , Vasculite/diagnóstico por imagem , Vasculite/tratamento farmacológicoRESUMO
To investigate the efficacy of single-dose intravitreal dexamethasone implantation in the treatment of persistent diabetic macular edema (DME) unresponsive to 3 consecutive ranibizumab injections over a period of 6 months. Forty-one patients with a previous history of treatment for DME including at least three consecutive intravitreal ranibizumab injections were enrolled in this retrospective study. Main outcome measures were change in best-corrected visual acuity (BCVA), central macular thickness (CMT), and intraocular pressure from baseline to 6th month. At the baseline, the mean CMT was 572.4 ± 123.1 µm which improved to 264.2 ± 114.4, 317.7 ± 141.7, 410.6 ± 169.1, and 382.8 ± 181.5 µm at the 1st, 3rd, 5th, and 6th month, respectively (p < 0.05). The preoperative mean BCVA was 0.85 ± 0.54 logMAR units which improved to 0.76 ± 0.5 (p = 0.08), 0.69 ± 0.4 (p = 0.02), 0.74 ± 0.4 (p = 0.284), and 0.72 ± 0.3 (p = 0.489) logMAR units at the 1st, 3rd, 5th, and 6th months, respectively. Additional injections were required for 13 (31 %) eyes at 3rd month and 14 (34 %) eyes at 5th month due to recurrence of macular edema. Intravitreal dexamethasone implantation caused a significant improvement of BCVA and reduction of CMT in the patients with persistent DME that were unresponsive to 3 consecutive ranibizumab injections. However, retreatment before 6 months in the majority of the patients was needed despite the beneficial effects after the index procedure.
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Dexametasona/administração & dosagem , Retinopatia Diabética/tratamento farmacológico , Glucocorticoides/administração & dosagem , Edema Macular/tratamento farmacológico , Idoso , Diabetes Mellitus Tipo 2/complicações , Retinopatia Diabética/diagnóstico , Implantes de Medicamento , Feminino , Angiofluoresceinografia , Humanos , Pressão Intraocular/efeitos dos fármacos , Injeções Intravítreas , Edema Macular/diagnóstico , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Tomografia de Coerência Óptica , Resultado do Tratamento , Acuidade Visual/efeitos dos fármacosRESUMO
B cell generation of autoantibodies is a crucial step in the pathogenesis of systemic lupus erythematosus (SLE). After their differentiation in the bone marrow, B cells populate the secondary lymphatic organs, where they undergo further maturation leading to the development of memory B cells as well as antibody-producing plasmablasts and plasma cells. Targeting B cells is an important strategy to treat autoimmune diseases such as SLE, in which B cell tolerance is disturbed and autoimmune B cells and autoantibodies emerge. This review discusses the functional aspects of antibody- and cell-based B cell-depleting therapy in SLE. It thereby particularly focuses on lessons learned from chimeric antigen receptor (CAR) T cell treatment on the role of B cells in SLE for understanding B cell pathology in SLE. CAR T cells model a deep B cell depletion and thereby allow understanding the role of aberrant B cell activation in the pathogenesis of SLE. Furthermore, the effects of B cell depletion on autoantibody production can be better described, ie, explaining the concept of different cellular sources of (auto-) antibodies in the form of short-lived plasmablasts and long-lived plasma cells, which differ in their susceptibility to B cell depletion and require different targeted therapeutic approaches. Finally, the safety of deep B cell depletion in autoimmune disease is discussed.
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Lúpus Eritematoso Sistêmico , Receptores de Antígenos Quiméricos , Humanos , Linfócitos B , Antígenos CD19 , Autoanticorpos , Linfócitos TRESUMO
OBJECTIVES: To assess the presence and anatomical distribution of activated fibroblasts in the joints and entheses of patients with psoriasis with arthralgia and to test how fibroblast activation visualised by 68gallium-labelled fibroblast activation protein inhibitor-04 (68Ga-FAPI-04)-positron emission tomography (PET)/CT correlates with clinical tenderness, musculoskeletal ultrasound findings and progression to psoriatic arthritis (PsA). METHODS: We conducted a prospective cohort study in patients with psoriasis and arthralgia who underwent clinical and ultrasound evaluation and whole-body PET/CT imaging with 68Ga-FAPI-04. 68Ga-FAPI-04 uptake at synovial and entheseal sites was assessed by maximal standardised uptake values (SUVmax) and PET/CT Joint Index (JI); logistic regression models were used to investigate its correlation with clinical and ultrasound findings. Survival analyses were performed on patients with at least 6 months of follow-up. RESULTS: 36 patients with psoriasis were enrolled. 68Ga-FAPI-04 uptake was found in 318 (7.9%) joints and 369 (7.3%) entheses in 29 (80.6%) participants, with a mean SUVmax (SD) of 3.2 (1.8) for joints and 2.9 (1.6) for entheses. Large joints and the lower limbs were predominantly affected. A significant positive relationship was found between 68Ga-FAPI-04-PET/CT signal intensity and the 68 tender joint count (SUVmax: p<0.001; PET/CT-JI: p<0.001) and tender entheses count (SUVmax: p<0.001; PET/CT-JI: p=0.002). No correlations were found with ultrasound findings (SUVmax: p=0.969; PET/CT-JI: p=0.720). Patients with relevant synovio-entheseal 68Ga-FAPI-04 uptake showed a statistically significant higher risk of developing PsA (p=0.02), independent of ultrasound findings. CONCLUSIONS: Patients with psoriasis presenting with arthralgia show localised signs of resident tissue activation in joints and entheses, which are associated with higher risk of developing PsA.
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Artrite Psoriásica , Fibroblastos , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Psoríase , Humanos , Artrite Psoriásica/patologia , Artrite Psoriásica/diagnóstico por imagem , Masculino , Feminino , Pessoa de Meia-Idade , Psoríase/patologia , Adulto , Estudos Prospectivos , Fibroblastos/metabolismo , Membrana Sinovial/patologia , Membrana Sinovial/diagnóstico por imagem , Idoso , Ultrassonografia , Progressão da DoençaRESUMO
OBJECTIVES: To train, test and validate the performance of a convolutional neural network (CNN)-based approach for the automated assessment of bone erosions, osteitis and synovitis in hand MRI of patients with inflammatory arthritis. METHODS: Hand MRIs (coronal T1-weighted, T2-weighted fat-suppressed, T1-weighted fat-suppressed contrast-enhanced) of rheumatoid arthritis (RA) and psoriatic arthritis (PsA) patients from the rheumatology department of the Erlangen University Hospital were assessed by two expert rheumatologists using the Outcome Measures in Rheumatology-validated RA MRI Scoring System and PsA MRI Scoring System scores and were used to train, validate and test CNNs to automatically score erosions, osteitis and synovitis. Scoring performance was compared with human annotations in terms of macro-area under the receiver operating characteristic curve (AUC) and balanced accuracy using fivefold cross-validation. Validation was performed on an independent dataset of MRIs from a second patient cohort. RESULTS: In total, 211 MRIs from 112 patients (14 906 region of interests (ROIs)) were included for training/internal validation using cross-validation and 220 MRIs from 75 patients (11 040 ROIs) for external validation of the networks. The networks achieved high mean (SD) macro-AUC of 92%±1% for erosions, 91%±2% for osteitis and 85%±2% for synovitis. Compared with human annotation, CNNs achieved a high mean Spearman correlation for erosions (90±2%), osteitis (78±8%) and synovitis (69±7%), which remained consistent in the validation dataset. CONCLUSIONS: We developed a CNN-based automated scoring system that allowed a rapid grading of erosions, osteitis and synovitis with good diagnostic accuracy and using less MRI sequences compared with conventional scoring. This CNN-based approach may help develop standardised cost-efficient and time-efficient assessments of hand MRIs for patients with arthritis.
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Aprendizado Profundo , Imageamento por Ressonância Magnética , Osteíte , Sinovite , Humanos , Osteíte/diagnóstico por imagem , Osteíte/etiologia , Osteíte/diagnóstico , Osteíte/patologia , Sinovite/diagnóstico por imagem , Sinovite/etiologia , Sinovite/diagnóstico , Imageamento por Ressonância Magnética/métodos , Masculino , Feminino , Pessoa de Meia-Idade , Artrite Reumatoide/diagnóstico por imagem , Artrite Reumatoide/complicações , Mãos/diagnóstico por imagem , Mãos/patologia , Artrite Psoriásica/diagnóstico por imagem , Artrite Psoriásica/diagnóstico , Adulto , Idoso , Curva ROC , Índice de Gravidade de Doença , Redes Neurais de ComputaçãoRESUMO
Background: The accruing evidence about the efficacy of anti-IL-1 agents in Familial Mediterranean Fever (FMF) patients led to their widespread off-label use. Therefore, identifying precise indications and clinical characteristics of IL-1i-warranting patients are important. This study investigated the clinical characteristics and treatment indications of patients with FMF requiring interleukin 1 inhibition therapy (IL-1i). Methods: Hospital records of FMF patients attending a tertiary care center at the Department of Rheumatology, University of Health Sciences, Basaksehir Cam and Sakura City Hospital were retrospectively analyzed. Data on symptoms and disease manifestations, age of symptom onset, time to diagnosis, MEFV variants, type of treatment, and their indications were collected. Results: Between June 2020 and March 2023, 312 FMF patients were identified. The mean age at the onset of symptoms was 14.0, and the mean time to diagnosis was 11.9 years. In total, 87.1% of patients were receiving colchicine monotherapy, while the remaining 11.8% warranted IL-1i. Clinical symptoms and flare manifestations did not show a significant difference between the two groups. However, patients receiving IL-1i started having symptoms at younger age (11.5 vs. 14.5, p = 0.042) and time to diagnosis was longer (18.2 vs. 11.0, p < 0.01). M694V homozygosity was more common in patients receiving IL-1i. Indications for patients receiving IL-1i were colchicine resistance (8.0%), secondary amyloidosis (5.1%), and colchicine intolerance (2.2%). Conclusions: This study shows that a subset of FMF patients, particularly those with a more severe phenotype with an earlier disease onset and M694V homozygosity, require IL-1i treatment despite the overall good efficacy and tolerability of colchicine, primarily due to colchicine resistance, intolerance, or complications such as amyloidosis.
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The advent of biologic disease modifying antirheumatic drugs (bDMARDs) has considerably improved patient outcomes in inflammatory arthritis. However, not all patients reach the state of remission, as disease can be resistant even to single cytokine inhibition by bDMARDs. Simultaneous or sequential inhibition of multiple cytokines may be considered in situations where disease control is not adequate under singular inhibition of cytokines. Although there have been some disappointing experiences in the past with combination of bDMARDs, the ongoing improvement of our understanding about inflammatory pathways and the overall better safety understanding of bDMARDs seem to make new biologic treatment combinations possible. This review covers the rationale and current evidence for bDMARDs combination in inflammatory arthritis.
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Antirreumáticos , Artrite Psoriásica , Artrite Reumatoide , Produtos Biológicos , Humanos , Artrite Psoriásica/tratamento farmacológico , Artrite Reumatoide/tratamento farmacológico , Antirreumáticos/uso terapêutico , Citocinas , Produtos Biológicos/uso terapêuticoRESUMO
OBJECTIVE: Pulmonary involvement is an important cause of mortality and morbidity in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). In this study, we aimed to evaluate the type and frequency of pulmonary involvement and investigate the potential relationship between thorax computed tomography (CT) signs and other systemic clinical findings in AAV. METHODS: A total of 63 patients over the age of 18 and diagnosed with AAV were included in this study. Thoracic CT imaging findings and clinical features of the patients at the time of diagnosis were retrospectively evaluated. The frequency and distribution of detected pathological findings on imaging according to disease types, in addition to the relationship of these findings with other systemic findings and disease severity, were analyzed. RESULTS: Of 63 patients, 50 (79.4%) had pulmonary symptoms at the time of presentation. Most frequently observed pulmonary finding of thorax CT was nodular opacity. Consolidation, cavitary nodules, bronchiectasis, emphysema and fibrotic sequelae change were more common in the patients with diagnosis of granulomatosis with polyangiitis. Honeycomb, atelectasis, interstitial pneumonia, pulmonary venous congestion and pleural effusion were more common in the patients with diagnosis of microscopic polyangiitis. Ground-glass appearance, central airway disease, peribronchovascular nodules, pericardial effusion and lymphatic adenomegaly (>10 mm) were more common in the patients with diagnosis of eosinophilic granulomatosis with polyangiitis. Interstitial lung disease, pulmonary hemorrhage and severe lung involvement were found significantly increased in patients with myeloperoxidase antibody (MPO)-ANCA positivity (P < 0.05). CONCLUSION: Lung involvement was detected in almost all of the patients with AAV. Both interstitial lung disease and severe lung involvement were more frequently found in patients with MPO-ANCA positivity compared to other patients. Pulmonary examination by an imaging technique in all patients with AAV may be useful to determine the vasculitis subtype and to determine the extent of the disease. PRACTITIONER POINTS: Pulmonary involvement is quite common in AAV. Every patient with suspected AAV should be examined with imaging for lung involvement, even in the absence of respiratory symptoms. Severe pulmonary involvement is associated with the presence of severe disease and MPO-ANCA positivity.
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Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Doenças Pulmonares Intersticiais , Humanos , Adulto , Pessoa de Meia-Idade , Anticorpos Anticitoplasma de Neutrófilos , Estudos Retrospectivos , Turquia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnósticoRESUMO
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly defined disorder in which treatment is still unclear. Herein, a patient with VEXAS syndrome who had atypical findings and an interesting treatment course is presented as a case report. He had fatigue, recurrent fever, pulmonary infiltrates, proteinuria, anemia, leucopenia, transient skin rush and increased acute phase reactants. The patient, who could not tolerate corticosteroid tapering, recovered rapidly after diagnostic splenectomy and the pathological examination of the spleen revealed significant findings.
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Anemia , Trombocitopenia , Masculino , Humanos , Esplenectomia/efeitos adversos , Síndrome , Anemia/etiologia , MutaçãoRESUMO
Behcet's syndrome is a variable vessel vasculitis characterized by recurrent oral and genital ulcers with concomitant skin, ocular, neurologic, gastrointestinal, and joint involvement. Herein, we present a patient who was diagnosed with Behcet's syndrome, which with magnetic resonance angiography showed occlusion of the right subclavian artery at the level of the thoracic outlet and reverse flow in the right vertebral artery consistent with subclavian steal syndrome. In addition, partial narrowing was noted in the left renal artery. The distribution of arterial involvement resembled Takayasu's arteritis, although the presence of mucocutaneous lesions, male gender, history of deep vein thrombosis, and HLA-B51 positivity favored a diagnosis of vasculo-Behçet's syndrome. We treated the patient with methylprednisolone and cyclophosphamide. After the regression of vascular inflammation with immunosuppressive therapy, stenting was performed in the left renal artery.
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OBJECTIVES: To assess the impact of low to moderate biomechanical stress on entheses in patients with psoriasis and psoriatic arthritis (PsA). METHODS: We conducted a prospective interventional study on a cohort of psoriasis and PsA patients who underwent a 60 min badminton training session. Pain assessment by Visual Analogue Scale (VAS), physical examination of 29 entheses (SPARCC, LEI, MASES) and bilateral ultrasound at the lateral humeral epicondyle, inferior patellar pole and Achilles tendon were performed before and after training. Ultrasound changes were assessed using the OMERACT scoring system. A follow-up assessment of pain and adverse events was performed at 1 week. RESULTS: Sixteen patients were included (n=7 PsA; n=9 psoriasis) and 196 entheseal ultrasound scans were acquired. At baseline, median VAS pain (IQR) was 0.5 cm (0-2.3) and the total number of tender entheses was 12/464. Mean (min; max) Disease Activity Index for Psoriatic Arthritis was 6.1 (0.8; 19) and 5/7 PsA patients had an Minimal Disease Activity status. After training, no significant change in VAS pain (0.0 cm (0.0-2.0)) nor in tender entheses (13/464) emerged. Four patients (n=2 PsA, n=2 psoriasis) developed a grade-1 power Doppler-signal at six entheses, which, however, remained non-tender. At 1 week, median VAS pain remained stable (0.0 cm (0.0-3.0); p>0.05) and only one participant with active PsA at baseline reported increased arthralgias in three joints. CONCLUSIONS: Low to moderate physical strain, as in the context of leisure sport activity, seems well tolerated in psoriatic patients without increases in tenderness, pain and ultrasound-proven inflammation. Evidence-based recommendations for physical activity in PsA are direly needed and larger controlled studies should be conducted to define safe exercise thresholds.