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1.
Artigo em Inglês | MEDLINE | ID: mdl-38970381

RESUMO

BACKGROUND: The definition of Takayasu arteritis (TAK) remission and disease activity is still unclear. Vascular imaging is an essential tool for following-up patients. Herein, we aimed to compare the evolution of vascular lesions (ie vessel wall thickening and stenosis) under conventional disease-modifying anti-rheumatic drugs (cDMARDs) relatively to biological DMARDs (bDMARDs) in TAK patients followed with the same CT angiography modalities. METHOD: We compared 75 lines of therapy in TAK patients who received cDMARDs (n = 40 lines) and bDMARDs (n = 35 lines) using CT angiography. We established 1-3 main target vessels with vessel wall thickening and/or stenosis. Every targeted vessel had its thickness and its lumen diameter measured at the initiation of immunosuppressive treatment and at 12 months. RESULTS: We observed an overall reduction of arterial wall thickness in 73% of cases and 31% had >25% of wall thickness relative decrease. Using a linear mixed effects model, first line immunosuppressive therapy (p= 0.012) and bDMARDs relatively to cDMARDs (p= 0.026) were independently associated with vessel wall thickness reduction in TAK. Thirty-eight percent of the stenotic vessels had a > 25% relative increase in lumen diameter under immunosuppressive therapy. The relative increase >25% in lumen diameter was noted in 56% vs 17% with bDMARDs compared with cDMARDs. CONCLUSION: Immunosuppressive treatments can reduce arterial wall thickness and widen lumen diameter in TAK. bDMARDs seems to be more effective than cDMARDs to improve arterial lesions in TAK.

2.
Rheumatology (Oxford) ; 62(12): 3932-3939, 2023 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-37010495

RESUMO

OBJECTIVE: Among specific autoantibodies in DM, the anti-small ubiquitin-like modifier activating enzyme (SAE) antibody is rare. We aim to describe the clinical characteristics, cancer prevalence, and muscle pathology of anti-SAE-positive DM. METHODS: Patients with a diagnosis of DM and sera positive for the anti-SAE antibody were recruited from 19 centres in this retrospective observational study. The available muscular biopsies were reviewed. We conducted a comparison with anti-SAE-negative DM and a review of the literature. RESULTS: Of the patients in the study (n = 49), 84% were women. Skin involvement was typical in 96% of patients, with 10% having calcinosis, 18% ulceration and 12% necrosis; 35% presented with a widespread skin rash. Muscular disease affected 84% of patients, with mild weakness [Medical Research Council (MRC) scale 4 (3, 5)], although 39% of patients had dysphagia. Muscular biopsies showed typical DM lesions. Interstitial lung disease was found in 21% of patients, mainly with organizing pneumonia pattern, and 26% of patients showed dyspnoea. Cancer-associated myositis was diagnosed in 16% of patients and was responsible for the majority of deaths, its prevalence being five times that of the general population. IVIG therapy was administered to 51% of the patients during the course of the disease. Comparison with anti-SAE-negative DM (n = 85) showed less and milder muscle weakness (P = 0.02 and P = 0.006, respectively), lower creatinine kinase levels (P < 0.0001) and less dyspnoea (P = 0.003). CONCLUSION: Anti-SAE positive DM is a rare subgroup associated with typical skin features but a potentially diffuse rash, a mild myopathy. Interstitial lung disease defines an organizing pneumonia pattern. Cancer associated DM prevalence is five times that of the general population. TRIAL REGISTRATION: ClinicalTrials.gov, http://clinicaltrials.gov, NCT04637672.


Assuntos
Dermatomiosite , Exantema , Doenças Pulmonares Intersticiais , Miosite , Neoplasias , Humanos , Feminino , Masculino , Autoanticorpos , Dermatomiosite/complicações , Miosite/diagnóstico , Exantema/epidemiologia , Neoplasias/epidemiologia , Neoplasias/complicações , Enzimas Ativadoras de Ubiquitina , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/complicações , Dispneia , Estudos Observacionais como Assunto
3.
Circulation ; 143(3): 267-282, 2021 01 19.
Artigo em Inglês | MEDLINE | ID: mdl-33464968

RESUMO

Systemic vasculitides are multisystem blood vessel disorders, which are defined by the size of the vessel predominantly affected, namely small, medium, or large vessels. The term "large vessel" relates to the aorta and its major branches; "medium vessel" refers to the main visceral arteries and veins and their initial branches. The most common causes of large-vessel vasculitis are giant cell arteritis and Takayasu arteritis, and those of medium-vessel arteritis are polyarteritis nodosa and Kawasaki disease. However, there is some overlap, and arteries of any size can potentially be involved in any of the 3 main categories of dominant vessel involvement. In addition to multisystem vasculitides, other forms of vasculitis have been defined, including single-organ vasculitis (eg, isolated aortitis). Prompt identification of vasculitides is important because they are associated with an increased risk of mortality. Left undiagnosed or mismanaged, these conditions may result in serious adverse outcomes that might otherwise have been avoided or minimized. The ethnic and regional differences in the incidence, prevalence, and clinical characteristics of patients with vasculitis should be recognized. Because the clinical presentation of vasculitis is highly variable, the cardiovascular clinician must have a high index of suspicion to establish a reliable and prompt diagnosis. This article reviews the pathophysiology, epidemiology, diagnostic strategies, and management of vasculitis.


Assuntos
Arterite de Células Gigantes/diagnóstico por imagem , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Poliarterite Nodosa/diagnóstico por imagem , Arterite de Takayasu/diagnóstico por imagem , Animais , Arterite de Células Gigantes/terapia , Glucocorticoides/uso terapêutico , Humanos , Síndrome de Linfonodos Mucocutâneos/terapia , Poliarterite Nodosa/terapia , Arterite de Takayasu/terapia , Vasculite/diagnóstico por imagem , Vasculite/terapia
4.
Rheumatology (Oxford) ; 61(4): 1376-1384, 2022 04 11.
Artigo em Inglês | MEDLINE | ID: mdl-34363461

RESUMO

OBJECTIVE: To assess the safety and the efficacy of TNF-α antagonists and tocilizumab in patients with Takayasu arteritis (TAK). METHODS: A total of 209 patients with TAK [median age 29 years (interquartile range 7-62)], 186 (89%) females] were included. They received either TNF-α antagonists [n = 132 (63%) with 172 lines; infliximab (n = 109), adalimumab (n = 45), golimumab (n = 8), certolizumab (n = 6) and etanercept (n = 5)] or tocilizumab [n = 77 (37%) with 121 lines; i.v. and s.c. in 95 and 26 cases, respectively]. RESULTS: A complete response at 6 months was evidenced in 101/152 (66%) patients on TNF-α antagonists and 75/107 (70%) patients on tocilizumab. Age ≥30 years [odds ratio 2.09 (95% CI 1.09, 3.99)] was associated with complete response, whereas vascular signs [OR 0.26 (95% CI 0.1, 0.65)], baseline prednisone ≥20 mg/day [OR 0.51 (95% CI 0.28, 0.93)] were negatively associated with the complete response to TNF-α antagonists or tocilizumab. During a median follow-up of 36 months, 103 relapses were noted. Supra-aortic branches and thoracic aorta involvement [HR 2.44 (95% CI 1.06, 5.65) and 3.66 (1.18, 11.4), respectively] and systemic signs at baseline [HR 2.01 (95% CI 1.30, 3.11)] were significantly associated with relapse. The cumulative incidence of treatment discontinuation and relapse were similar in TNF-α antagonists and tocilizumab. Fifty-eight (20%) adverse effects occurred on biologic targeted therapies [37 (21%) on TNF-α antagonists and 21 (17%) on tocilizumab (P = 0.4), respectively]. CONCLUSION: This large multicentre study shows high efficacy of biologic targeted treatments in refractory TAK. Efficacy, relapse and drug retention rate were equivalent with TNF-α antagonists and tocilizumab.


Assuntos
Arterite de Takayasu , Fator de Necrose Tumoral alfa , Adulto , Anticorpos Monoclonais Humanizados , Feminino , Humanos , Recidiva , Estudos Retrospectivos , Arterite de Takayasu/complicações , Arterite de Takayasu/tratamento farmacológico , Resultado do Tratamento , Inibidores do Fator de Necrose Tumoral
5.
Clin Exp Rheumatol ; 40(8): 1491-1496, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35200121

RESUMO

OBJECTIVES: Vascular Behçet's disease (VBD) is a systemic vasculitis involving both arterial and venous vessels of all sizes and occurring in up to 40% of patients with BD. VBD is the main cause of mortality in BD. Although commonly seen around the Mediterranean region, comparative studies in VBD are lacking. We aimed to compare the course and therapeutic approaches of VBD in two large cohorts from Turkey and France. METHODS: We included 291 VBD patients (female/male:63/228, mean age: 41.2±11.3 years) who were followed up in the Department of Internal Medicine and Clinical Immunology at Pitié-Salpêtrière Hospital, Sorbonne University, Paris, France (n=131) and Rheumatology Division of Marmara University School of Medicine, Istanbul, Turkey (n=160). All clinical and demographical data were acquired from patient charts retrospectively. RESULTS: Smoking, family history for BD, HLA-B*51 presence and pathergy positivity were significantly higher in Turkish patients (TR), while neurologic involvement was more prominent in the French (FR) group. After a median follow-up of 77 months, 562 vascular events occurred including 440 venous events, 115 arterial events and 7 cardiac thrombi. In 79 (29%) patients, first vascular event developed before BD diagnosis and for 77 (28%) of them, vascular involvement was the presenting sign of the disease. First relapse developed in 130 (44.7%) patients after median 24.5 (1-276) months of follow-up (TR: 46.3% (n=74), FR: 42.7% (n=56), p=0.56). Survival graph revealed that FR cohort has 1.64 times increased recurrent event risk compared to TR cohort (HR=1.64 (1.1-2.44), p=.014) and although did not reach to statistical significance, IS treatment after the first vascular event decreased further vascular events (HR= 0.66 (0.43-1.01, p=.057). CONCLUSIONS: Almost half of patients relapsed of VBD within 2 years after the first vascular event. Immunosuppressants decrease VBD relapses.


Assuntos
Síndrome de Behçet , Trombose , Adulto , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/tratamento farmacológico , Síndrome de Behçet/epidemiologia , Feminino , França/epidemiologia , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Turquia/epidemiologia
6.
J Autoimmun ; 116: 102577, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33248866

RESUMO

OBJECTIVE: To report the efficacy of rituximab plus belimumab in patients with refractory cryoglobulinemia vasculitis (CV). METHODS: Belimumab was administered intravenously at a dose of 10 mg/kg on days 0, 14, 28 and then every month in association with rituximab in 4 patients with refractory CV. Demographic, clinical and laboratory characteristics, treatment modalities and outcomes were recorded. RESULTS: All patients had type II IgM Kappa cryoglobulinemia, which was associated with primary Sjögren syndrome (n = 1), hepatitis C virus infection (n = 1), and essential (n = 2). Main manifestations of CV included purpura (n = 4), arthralgia and peripheral neuropathy (n = 3), and glomerulonephritis and skin ulcers (n = 1). In all cases, CV was refractory and/or relapse following rituximab. Intravenous belimumab infusion along with rituximab resulted in rapid clinical response in the four patients. Osteitis and recurrent urinary tract infections occurred in two patients. CONCLUSION: Belimumab along with rituximab showed promising results in refractory patients with CV.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Crioglobulinemia/tratamento farmacológico , Rituximab/uso terapêutico , Vasculite/tratamento farmacológico , Idoso , Anticorpos Monoclonais Humanizados/administração & dosagem , Crioglobulinemia/imunologia , Crioglobulinemia/patologia , Quimioterapia Combinada , Feminino , Humanos , Fatores Imunológicos/administração & dosagem , Fatores Imunológicos/uso terapêutico , Imunossupressores/administração & dosagem , Imunossupressores/uso terapêutico , Projetos Piloto , Indução de Remissão , Rituximab/administração & dosagem , Fatores de Tempo , Resultado do Tratamento , Vasculite/imunologia , Vasculite/patologia
7.
J Autoimmun ; 108: 102419, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32035747

RESUMO

OBJECTIVES: To assess prognosis factors and outcome of large vessel involvement (LVI) in large vessels vasculitis (LVV) patients. METHODS: Retrospective multicenter study of characteristics and outcomes of 417 patients with LVI including 299 Takayasu arteritis (TAK) and 118 Giant cell arteritis (GCA-LVI) were analyzed. Logistic regression analysis assessed prognosis factors in LVV patients. Outcome of LVI among TAK and GCA-LVI patients (ischemic complications, aneurysms complications, relapses and revascularization) were assessed. RESULTS: In multivariable analysis, stroke/transient ischemic attack [HR: 3.63 (1.46-9.04), p = 0.006] was independently associated with vascular complications in LVV. The 10-years aneurysm free survival was significantly lower [67% (48-93) vs 89% (84-95), p = 0.02] in GCA-LVI compare to TAK patients. The 5-years relapse free survival was significantly lower [47% (37-60) vs 69% (63-75), p < 0.001,] in GCA-LVI compare to TAK patients. The 10-years revascularization free survival was significantly lower [55% (48-64) vs 76% (59-99), p < 0.001] in TAK compare to GCA-LVI patients. After a median follow-up of 5 years, 16 (5.4%) TAK and 7 (5.9%) GCA-LVI patients died, mainly of aneurysm (26%) and ischemic complications (26%). CONCLUSION: This large nationwide cohort of LVI provided prognosis factors of vascular complications in LVV patients. TAK and GCA-LVI have different long-term outcome in term of aneurysm development, relapse and revascularization.


Assuntos
Arterite de Células Gigantes/epidemiologia , Arterite de Takayasu/epidemiologia , Comorbidade , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/etiologia , Humanos , Mortalidade , Avaliação de Resultados da Assistência ao Paciente , Prognóstico , Vigilância em Saúde Pública , Arterite de Takayasu/diagnóstico , Arterite de Takayasu/etiologia
8.
Crit Care ; 24(1): 231, 2020 05 18.
Artigo em Inglês | MEDLINE | ID: mdl-32423434

RESUMO

BACKGROUND: Diffuse alveolar hemorrhage (DAH) occurs during the course of autoimmune disease and may be life threatening. The objective was to assess characteristics and prognosis factors of DAH who required intensive care unit (ICU) admission in patients with autoimmune diseases. METHODS: French multicenter retrospective study including patients presenting DAH related to autoimmune diseases requiring ICU admission from 2000 to 2016. RESULTS: One hundred four patients (54% of men) with median age of 56 [32-68] years were included with 79 (76%) systemic vasculitis and 25 (24%) connective tissue disorders. All patients received steroids, and 72 (69%), 12 (11.5%), and 57 (55%) patients had cyclophosphamide, rituximab, and plasma exchanges, respectively. During ICU stay, 52 (50%), 36 (35%), and 55 (53%) patients required mechanical ventilation, vasopressor use, and renal replacement therapy, respectively. Factors associated with mechanical ventilation weaning were age (HR [95%CI] 0.97 [0.96-0.99] per 10 years, p < 0.0001), vasculitis-related DAH (0.52 [0.27-0.98], p = 0.04), and time from dyspnea onset to ICU admission (0.99 [0.99-1] per day, p = 0.03). ICU mortality was 15%. Factors associated with alive status at ICU discharge were chronic cardiac failure (HR [95%CI] 0.37 [0.15-0.94], p = 0.04), antiphospholipid syndrome-related DAH (3.17 [1.89-5.32], p < 0.0001), SAPS II (0.98 [0.97-0.99], p = 0.007), and oxygen flow at ICU admission (0.95 [0.91-0.99] per liter/min, p = 0.04). CONCLUSION: DAH in autoimmune diseases is a life-threatening complication which requires mechanical ventilation in half of the cases admitted to ICU.


Assuntos
Doenças Autoimunes/complicações , Hemorragia/etiologia , Alvéolos Pulmonares/anormalidades , Adulto , Idoso , Doenças Autoimunes/fisiopatologia , Feminino , França , Hemorragia/fisiopatologia , Humanos , Unidades de Terapia Intensiva/organização & administração , Unidades de Terapia Intensiva/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Alvéolos Pulmonares/fisiopatologia , Estudos Retrospectivos
9.
J Clin Rheumatol ; 26(6): 248-254, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31124918

RESUMO

OBJECTIVE: The aims of this study were to describe and assess the vascular outcomes of patients with giant cell arteritis (GCA) presenting with only symptomatic isolated limb involvement (LI-GCA). METHODS: We recruited patients from 5 tertiary centers who were diagnosed with GCA based on histology or vasculitis demonstration on imaging and who presented with isolated symptomatic limb involvement at diagnosis. For each included patient, we randomly selected 3 control patients who satisfied the 5 criteria from the American College of Rheumatology at diagnosis. RESULTS: We included 27 LI-GCA patients and 81 control patients. Compared with the controls, the patients with LI-GCA were younger (p = 0.005), exhibited a more delayed diagnosis (p = 0.006), and had lower C-reactive protein levels (p = 0.001), but they did not show more cardiovascular risk factors. Glucocorticoid use (starting and tapering doses) and relapse rates did not differ in the 2 groups, but the patients with LI-GCA received longer treatment (p = 0.02). Cardiovascular complications occurred in 67% of the patients with LI-GCA versus 21% of the control patients (p < 0.0001), especially ischemic events (p < 0.0001) including stroke (p = 0.03) and myocardial infarction (p = 0.01). Vascular surgery was required in 44% of the patients with LI-GCA versus 2% of the controls (p < 0.0001). Excluding vascular surgery, the cumulative incidence of cardiovascular complications was higher in the patients with LI-GCA (log-rank test: p < 0.0001) than in the controls (hazard ratio, 5.73; 95% confidence interval, 2.94-11.28; p < 0.0001). CONCLUSIONS: Compared with the typical cranial form of GCA, LI-GCA has a worse cardiovascular-related prognosis. Further studies are required to determine the best management of these patients.


Assuntos
Arterite de Células Gigantes , Acidente Vascular Cerebral , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/tratamento farmacológico , Glucocorticoides , Humanos , Prognóstico , Estudos Retrospectivos
10.
J Autoimmun ; 103: 102283, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31130367

RESUMO

Large-vessel involvement (LVI) in giant cell arteritis (GCA) includes different clinical and imaging patterns that are rarely described separately at diagnosis and whose specific cardiovascular outcomes are unknown. We conducted a nationwide retrospective study and included GCA patients with LVI demonstrated on imaging at diagnosis between 2007 and 2017. We analyzed the prognosis of three different imaging patterns of LVI present at diagnosis, with some of them overlapping but with the first one present in all patients: 1) inflammation of the aorta and/or its branches; 2) dilation of the aorta; and 3) stenosis of the aortic branches. A control group of GCA patients without LVI was constituted. We included 183 patients with LVI and 105 controls without LVI. Altogether, among the 183 patients who all showed inflammation of the aorta and/or its main branches, concomitant aortic dilation and large-vessel stenosis were observed in 27 (15%) and 55 (30%) patients, respectively. During the follow-up period, new cardiovascular events occurred in 49% and 11% of LVI patients and controls, respectively (p < 0.0001). Inflammation of the aorta and/or its branches (HR: 3.42 [2.09-5.83], p < 0.0001) and large-artery stenosis (HR: 2.75 [1.80-4.15], p < 0.0001) were independent predictive factors of new cardiovascular events. Conversely, the use of an immunosuppressant besides corticosteroids was a protective factor against new cardiovascular events (HR: 0.44 [0.29-0.66], p < 0.0001) and the development of aortic dilation (HR: 0.43 [0.23-0.77], p = 0.005). This study suggests different forms of cardiovascular events according to the initial imaging pattern of LVI.


Assuntos
Aorta/patologia , Doenças Cardiovasculares/diagnóstico , Diagnóstico por Imagem/métodos , Arterite de Células Gigantes/diagnóstico , Inflamação/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Doenças Cardiovasculares/epidemiologia , Constrição Patológica , Feminino , Seguimentos , França/epidemiologia , Arterite de Células Gigantes/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos
12.
Liver Int ; 37(12): 1805-1813, 2017 12.
Artigo em Inglês | MEDLINE | ID: mdl-28467688

RESUMO

BACKGROUND: The net benefits of new hepatitis C virus (HCV) direct-acting antiviral drugs (DAA) in patients with cryoglobulinaemia vasculitis (CryoVas) are unknown. OBJECTIVE: To analyse the effectiveness and cost of all treatments used for HCV-CryoVas in the DAA vs pre-DAA era. METHODS: A chart review of all HCV-CryoVas patients who received antivirals from 1993 to 2016 in a tertiary centre was performed. Treatment effectiveness was analysed for clinical, immunological and virological responses. Cost analyses included anti-HCV treatments, non-antiviral drugs, plasmapheresis, dialysis and hospitalizations. We compared main data in the pre-DAA vs DAA period. RESULTS: About 201 HCV-CryoVas patients were included (women, 53.2%; mean age, 59.2 years; Metavir score F3-F4, 36.7%; genotype 1, 64.2%). Patients in the DAA era (n=27) compared to those in the pre-DAA era (n=174) showed higher rates of clinical (96.3% vs. 78.6%), immunological (89.5% vs. 77.1%), and sustained virological response (75.0% vs. 42.8%). Death rate was 14.8% vs. 24.4% respectively. In the DAA compared to pre-DAA era, mean cost of anti-HCV drugs increased from 11 855 to 57 632 € while mean CryoVas-related cost decreased for both hospitalizations (from 33 510 to 21 347€) and non-antiviral treatments (from 17 347 to 11 397€). CONCLUSION: Improved antiviral efficacy of HCV drugs in the DAA era led to increased clinical and immunological efficacy and a lower death rate. Use of DAAs was associated to higher costs for HCV drugs while costs related to both hospitalizations and non-antiviral treatments decreased.


Assuntos
Antivirais/uso terapêutico , Crioglobulinemia/tratamento farmacológico , Hepatite C/complicações , Vasculite Sistêmica/tratamento farmacológico , Idoso , Antivirais/economia , Crioglobulinemia/complicações , Crioglobulinemia/economia , Crioglobulinemia/virologia , Feminino , Hepatite C/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Vasculite Sistêmica/economia , Vasculite Sistêmica/virologia , Resultado do Tratamento
16.
Neurology ; 103(2): e209548, 2024 Jul 23.
Artigo em Inglês | MEDLINE | ID: mdl-38900992

RESUMO

BACKGROUND AND OBJECTIVES: Cerebral amyloid angiopathy-related inflammation (CAA-RI) and biopsy-positive primary angiitis of the CNS (BP-PACNS) have overlapping clinicoradiologic presentations. It is unknown whether clinical and radiologic features can differentiate CAA-RI from BP-PACNS and whether both diseases have different relapse rates. The objectives of this study were to compare clinicoradiologic presentations and relapse rates in patients with CAA-RI vs BP-PACNS. METHODS: Patients with CAA-RI and BP-PACNS were enrolled from 2 retrospective multicenter cohorts. Patients with CAA-RI were biopsy-positive or met probable clinicoradiologic criteria. Patients with BP-PACNS had histopathologic confirmation of CNS angiitis, with no secondary etiology. A neuroradiologist read brain MRIs, blinded to the diagnosis of CAA-RI or BP-PACNS. Clinicoradiologic features were compared using univariable logistic regression models. Relapse rates were compared using a univariable Fine-Gray subdistribution hazard model, with death as a competing risk. RESULTS: This study enrolled 104 patients with CAA-RI (mean age 73 years, 48% female sex) and 52 patients with BP-PACNS (mean age 45 years, 48% female sex). Patients with CAA-RI more often had white matter hyperintense lesions meeting the probable CAA-RI criteria (93% vs 51%, p < 0.001), acute subarachnoid hemorrhage (15% vs 2%, p = 0.02), cortical superficial siderosis (27% vs 4%, p < 0.001), ≥1 lobar microbleed (94% vs 26%, p < 0.001), past intracerebral hemorrhage (17% vs 4%, p = 0.04), ≥21 visible centrum semiovale perivascular spaces (34% vs 4%, p < 0.01), and leptomeningeal enhancement (70% vs 27%, p < 0.001). Patients with BP-PACNS more often had headaches (56% vs 31%, p < 0.01), motor deficits (56% vs 36%, p = 0.02), and nonischemic parenchymal gadolinium enhancement (82% vs 16%, p < 0.001). The prevalence of acute ischemic lesions was 18% in CAA-RI and 22% in BP-PACNS (p = 0.57). The features with the highest specificity for CAA-RI were acute subarachnoid hemorrhage (98%), cortical superficial siderosis (96%), past intracerebral hemorrhage (96%), and ≥21 visible centrum semiovale perivascular spaces (96%). The probable CAA-RI criteria had a 71% sensitivity (95% CI 44%-90%) and 91% specificity (95% CI 79%-98%) in differentiating biopsy-positive CAA-RI from BP-PACNS. The rate of relapse in the first 2 years after remission was lower in CAA-RI than in BP-PACNS (hazard ratio 0.46, 95% CI 0.22-0.96, p = 0.04). CONCLUSION: Clinicoradiologic features differed between patients with CAA-RI and those with BP-PACNS. Specific markers for CAA-RI were hemorrhagic signs of subarachnoid involvement, past intracerebral hemorrhage, ≥21 visible centrum semiovale perivascular spaces, and the probable CAA-RI criteria. A biopsy remains necessary for diagnosis in some cases of CAA-RI. The rate of relapse in the first 2 years after disease remission was lower in CAA-RI than in BP-PACNS.


Assuntos
Angiopatia Amiloide Cerebral , Vasculite do Sistema Nervoso Central , Humanos , Feminino , Masculino , Angiopatia Amiloide Cerebral/diagnóstico por imagem , Angiopatia Amiloide Cerebral/patologia , Angiopatia Amiloide Cerebral/complicações , Idoso , Pessoa de Meia-Idade , Vasculite do Sistema Nervoso Central/diagnóstico por imagem , Vasculite do Sistema Nervoso Central/patologia , Estudos Retrospectivos , Biópsia , Imageamento por Ressonância Magnética , Idoso de 80 Anos ou mais , Encéfalo/patologia , Encéfalo/diagnóstico por imagem , Adulto , Recidiva
17.
J Rheumatol ; 50(7): 916-923, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-36858435

RESUMO

OBJECTIVE: To evaluate tocilizumab (TCZ) efficacy in patients with refractory Behçet disease (BD). METHODS: This is a multicenter study of 30 patients fulfilling the international criteria for BD and treated with TCZ at different European referral centers. The clinical response was evaluated at 6 months from TCZ initiation. RESULTS: Ninety percent of patients with BD were refractory or intolerant to anti-tumor necrosis factor (anti-TNF) agents. Overall, TCZ was effective in 25 (83%) patients with BD of whom 18 (60%) and 7 (23%) were complete and partial responders, respectively. The complete response was 67%, 60%, and 42% in patients with uveitis (18/30), neurological manifestations (5/30), and mucocutaneous and/or articular (7/30) manifestations, respectively. TCZ had a significant steroid-sparing effect allowing patients to decrease their median daily prednisone dose from 20 (IQR 10-40) mg/day to 9 (IQR 5-13) mg at 6 months (P < 0.001). The number of patients with BD needing concomitant disease-modifying antirheumatic drug therapy fell from 7 (23%) to 4 (13%) at 6 months. Mild to moderate side effects were observed in 6 (20%) patients, and 3 (10%) presented with serious adverse events (pneumonia, intestinal perforation, and septicemia) requiring therapy discontinuation in 2 cases. CONCLUSION: TCZ seems to be an effective alternative to anti-TNF agents in treating BD-related uveitis and neurological manifestations.


Assuntos
Antirreumáticos , Síndrome de Behçet , Uveíte , Humanos , Síndrome de Behçet/complicações , Síndrome de Behçet/tratamento farmacológico , Inibidores do Fator de Necrose Tumoral/uso terapêutico , Resultado do Tratamento , Uveíte/etiologia , Uveíte/complicações , Antirreumáticos/uso terapêutico , Fator de Necrose Tumoral alfa , Estudos Retrospectivos
18.
J Clin Med ; 10(21)2021 Oct 24.
Artigo em Inglês | MEDLINE | ID: mdl-34768435

RESUMO

(1) Background: Takayasu arteritis (TA) is a chronic inflammatory large-vessel vasculitis. Ultra-wide-field imaging allows describing the retinal lesions in these patients and correlating them with vascular supra-aortic stenosis. (2) Methods: In total, 54 eyes of 27 patients diagnosed with TA were included, and a complete ophthalmological examination was performed, including UWF color fundus photography (UWF-CFP), fluorescein angiography (UWF-FA), and computed tomography angiography measuring supra-aortic stenosis. Eleven patients underwent Doppler ultrasound imaging assessing the blood flow velocity (BFV) in the central retinal artery (CRA). (3) Results: Microaneurysms were detected in 18.5% of eyes on fundus examination, in 24.4% of eyes on UWF-CFP, and in 94.4% of eyes on UWF-FA. The number of microaneurysms significantly correlated with the presence of an ipsilateral supra-aortic stenosis (p = 0.026), the presence of hypertension (p = 0.0011), and the duration of the disease (p = 0.007). The number of microaneurysms per eye negatively correlated with the BFV in the CRA (r = -0.61; p = 0.003). (4) Conclusions: UWF-FA improved the assessment of TA-associated retinal findings. The significant correlation between the number of microaneurysms and the BFV in the CRA gives new insight to our understanding of Takayasu retinopathy. The total number of microaneurysms could be used as an interesting prognostic factor for TA.

19.
J Rheumatol ; 48(10): 1583-1588, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34210830

RESUMO

OBJECTIVE: To assess the spectrum and long-term outcome of patients with noninfectious aortitis. METHODS: We performed a retrospective multicenter study of 353 patients (median age at diagnosis was 62 [IQR 46-71] yrs and 242 [68.6%] patients were women) with noninfectious aortitis. Factors associated with vascular complications were assessed in multivariate analysis. RESULTS: We included 136 patients with giant cell arteritis (GCA), 96 with Takayasu arteritis (TA), 73 with clinically isolated aortitis (CIA), and 48 with aortitis secondary to inflammatory diseases (including Behçet disease, relapsing polychondritis, IgG4-related disease, Cogan syndrome, ankylosing spondylitis). After a median follow-up of 52 months, vascular complications were observed in 32.3%, revascularizations in 30% of patients, and death in 7.6%. The 5-year cumulative incidence of vascular complications was 58% (95% CI 41-71), 20% (95% CI 13-29), and 19% (95% CI 11-28) in CIA, GCA, and TA, respectively. In multivariate analysis, male sex (HR 2.10, 95% CI 1.45-3.05, P < 0.0001) and CIA (HR 1.76, 95% CI 1.11-2.81, P = 0.02) were independently associated with vascular complications. CONCLUSION: Noninfectious aortitis accounts for significant morbidity and mortality. CIA seems to carry the highest rate of vascular complications.


Assuntos
Aortite , Arterite de Células Gigantes , Policondrite Recidivante , Arterite de Takayasu , Aortite/epidemiologia , Feminino , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/epidemiologia , Humanos , Masculino , Estudos Retrospectivos , Arterite de Takayasu/complicações , Arterite de Takayasu/epidemiologia
20.
Lancet Rheumatol ; 3(7): e481-e488, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33942031

RESUMO

BACKGROUND: The COVID-19 pandemic has raised numerous questions among patients with immune-mediated inflammatory diseases regarding potential reciprocal effects of COVID-19 and their underlying disease, and potential effects of immunomodulatory therapy on outcomes related to COVID-19. The seroprevalence of SARS-CoV-2 and factors associated with symptomatic COVID-19 in patients with immune-mediated inflammatory diseases are still unclear. The Euro-COVIMID study aimed to determine the serological and clinical prevalence of COVID-19 among patients with immune-mediated inflammatory diseases, as well as factors associated with COVID-19 occurrence and the impact of the pandemic in its management. METHODS: In this multicentre cross-sectional study, patients aged 18 years or older with a clinical diagnosis of rheumatoid arthritis, axial spondyloarthritis, systemic lupus erythematosus, Sjögren's syndrome, or giant cell arteritis were recruited from six tertiary referral centres in France, Germany, Italy, Portugal, Spain, and the UK. Demographics, comorbidities, treatments, and recent disease flares, as well as information on COVID-19 symptoms, were collected through a questionnaire completed by participants. SARS-CoV-2 serology was systematically tested. The main outcome was the serological and clinical prevalence of COVID-19. Factors associated with symptomatic COVID-19 were assessed by multivariable logistic regression, and incidence of recent disease flares, changes in treatments for underlying disease, and the reasons for treatment changes were also assessed. This study is registered with ClinicalTrials.gov, NCT04397237. FINDINGS: Between June 7 and Dec 8, 2020, 3136 patients with an immune-mediated inflammatory disease answered the questionnaire. 3028 patients (median age 58 years [IQR 46-67]; 2239 [73·9%] women and 789 [26·1%] men) with symptomatic COVID-19, serological data, or both were included in analyses. SARS-CoV-2 antibodies were detected in 166 (5·5% [95% CI 4·7-6·4]) of 3018 patients who had serology tests. Symptomatic COVID-19 occurred in 122 (4·0% [95% CI 3·4-4·8]) of 3028 patients, of whom 24 (19·7%) were admitted to hospital and four (3·3%) died. Factors associated with symptomatic COVID-19 were higher concentrations of C-reactive protein (odds ratio 1·18, 95% CI 1·05-1·33; p=0·0063), and higher numbers of recent disease flares (1·27, 1·02-1·58; p=0·030), whereas use of biological therapy was associated with reduced risk (0·51, 0·32-0·82; p=0·0057). At least one disease flare occurred in 654 (21·6%) of 3028 patients. Over the study period, 519 (20·6%) of 2514 patients had treatment changes, of which 125 (24·1%) were due to the pandemic. INTERPRETATION: This study provides key insights into the epidemiology and risk factors of COVID-19 among patients with immune-mediated inflammatory diseases. Overall, immunosuppressants do not seem to be deleterious in this scenario, and the control of inflammatory activity seems to be key when facing the pandemic. FUNDING: Pfizer, Sanofi, Amgen, Galapagos, and Lilly.

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