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2.
Intern Emerg Med ; 2020 Mar 29.
Artigo em Inglês | MEDLINE | ID: mdl-32227285
3.
Presse Med ; 49(1): 104018, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32234379

RESUMO

Aortitis and periaortitis are inflammatory diseases of the aorta and its main branches; they differ in the extension of inflammation, which is confined to the aortic wall in aortitis, and spreads to the periaortic space in periaortitis. Aortitis is classified as non-infectious or infectious. Non-infectious aortitis represents a common feature of large-vessel vasculitides but can also be isolated or associated with other rheumatologic conditions. Periaortitis can be idiopathic or secondary to a wide array of etiologies such as drugs, infections, malignancies, and other proliferative diseases. Notably, both aortitis and periaortitis may arise in the context of IgG4-related disease, a recently characterised fibro-inflammatory systemic disease. Prompt recognition, correct diagnosis and appropriate treatment are essential in order to avoid life-threatening complications.

5.
Laryngoscope ; 2019 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-31841234

RESUMO

OBJECTIVE: IgG4-related disease (IgG4-RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor-like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4-RD. METHODS: Review of all cases at three tertiary-referral centers since disease description in 2003. RESULTS: Eleven patients were identified at a median age at presentation of 58 years (IQR, 38-65; 55% male). Ten (91%) patients had isolated skull base masses without systemic disease. Cranial neuropathies were commonly observed in the abducens (45%), trigeminal (18%), and facial nerves (18%). Lesions frequently involved the cavernous sinus (55%; 6/11) with extension to the petroclival junction in 50% (3/6). Infiltration of the internal auditory canal was present in 27% (3/11) with one case demonstrating erosion of the bony labyrinth. Preliminary clinical diagnoses commonly included nasopharyngeal cancer, pituitary macroadenoma, cholesteatoma, and meningioma / multiple meningioma syndrome. Local biopsy demonstrated >30 IgG4-positive plasma cells per high-powered field or an IgG4:IgG ratio greater than 40% in all cases. Rapid and durable clinical improvement was seen in 91% following corticosteroid and rituximab therapy. CONCLUSIONS: IgG4-RD nonspecifically presents as a rare cause of the skull base mass. Often presenting without concomitant systemic disease, local diagnostic biopsies are required. Obtaining adequate tissue specimen is complicated by densely fibrotic cranial base lesions that are frequently in close proximity to critical neurovascular structures. Primary medical therapy with corticosteroids and rituximab is effective in most patients. LEVEL OF EVIDENCE: 4 Laryngoscope, 2019.

6.
Intern Emerg Med ; 14(8): 1193-1197, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31388893

RESUMO

The European Eosinophilic Granulomatosis with Polyangiitis (EGPA) study group first gathered in Firenze in December 2018. The discussion was centred around the clinical and therapeutic needs in EGPA which still remain unmet. Indeed, EGPA is a puzzling and rare disease which shares clinical features with other anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAVs) and hypereosinophilic syndromes (HESs). Some of the recommendations published in 2015 are based on data derived from EGPA-related diseases, rather than from EGPA itself, and therefore need to be updated. Thus, the aim of the meeting was to stimulate ongoing research, to promote collaborative European studies and to define the main issues on which future studies should be focused. Current fields of research on EGPA include potential serological biomarkers of disease activity and of specific organ involvement, possible links between different genetic variants and clinical phenotypes, and new therapeutic perspectives. Herein, we give an overview of the meeting with the goal to stimulate an international collaboration and new points of discussion.


Assuntos
Granuloma Eosinófilo/terapia , Granulomatose com Poliangiite/terapia , Consenso , Europa (Continente) , Humanos
7.
Inflamm Intest Dis ; 3(4): 187-191, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31111035

RESUMO

Background: Extraintestinal manifestations are common in ulcerative colitis (UC). Data regarding pulmonary and nasal mucosa involvement are sparse. Objectives: The aim of the study was to evaluate, by using induced sputum (IS) and nasal cytology (NC), the cytological pattern of the lung and nose in patients with UC. Materials and Methods: We enrolled 15 consecutive subjects from the outpatient department with a recent diagnosis of UC. On the same day of enrollment, we performed a global spirometry, including a lung diffusing capacity test, IS analysis, and evaluation of NC. Results: IS analysis showed an increase in lymphocytes in UC patients when compared to those of controls (2.8 ± 0.9 vs. 0.2 ± 0.4%; p < 0.01). NC showed a similar increase in lymphocytes (12.5 ± 5.30 vs. 3.5 ± 4.0%; p < 0.01). We found a positive correlation between lymphocyte counts in IS and NC (r = 0.775; p < 0.001) and between lymphocytes in IS and NC and grade of intestinal inflammation (r = 0.603, p = 0.015; r = 0.60, p = 0.013). Conclusions: Our data demonstrated that UC patients may have a subclinical nasal and lung lymphocytosis.

8.
Front Immunol ; 10: 693, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31019511

RESUMO

Objective: Chronic periaortitis (CP) is a rare fibro-inflammatory disorder that incorporates idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms, and perianeurysmal retroperitoneal fibrosis. CP is included in the spectrum of IgG4-related disease. Since CP patients rarely undergo diagnostic biopsies, serum IgG4 levels are often used to classify CP as IgG4-related. However, the clinical and prognostic significance of serum IgG4 in CP is unknown. Methods: We measured serum IgG4 in active CP patients and compared the clinical characteristics, response to therapy and outcome of patients with high and normal levels. We also tested the diagnostic significance of IgG4 by comparing its levels in CP patients, healthy and disease controls (malignancies, Erdheim-Chester disease, large-, and small-vessel vasculitis). Results: We studied 113 consecutive patients with active CP. Twenty-four (21.2%) had high serum IgG4 (>135 mg/dL). The demographic, laboratory, and clinical characteristics of patients with high and normal IgG4 were similar, and so were the rates of ureteral obstruction and the disease characteristics on CT, MRI, and 18F-FDG-PET. Patients with high IgG4 only had a higher frequency of extra-retroperitoneal fibro-inflammatory lesions (p = 0.005). There were no significant differences in response to therapy and relapses between the two groups. Serum IgG4 levels did not discriminate CP from controls. Conclusions: Serum IgG4 levels are high in a minority of CP patients and do not identify specific clinical or prognostic subgroups; only a higher frequency of extra-retroperitoneal lesions is found in high-IgG4 patients. Serum IgG4 levels do not help in the differential diagnosis between CP and its mimics.

10.
Intern Emerg Med ; 12(3): 287-299, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28070877

RESUMO

Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis. Among these, together with sclerosing pancreatitis and cholangitis, pseudotumour of the orbit, idiopathic mediastinal fibrosis and other conditions, is idiopathic retroperitoneal fibrosis (IRF). Both IRF and IgG4-RD can be associated with a wide variety of disorders, usually governed by immune-mediated (and particularly auto-immune) mechanisms. In our review, we discuss the clinical and therapeutic challenges IRF presents to the internist, as well as the meaning of its recent inclusion in the IgG4-RD spectrum from a clinical practice standpoint.


Assuntos
Doenças Autoimunes/patologia , Imunoglobulina G/efeitos adversos , Fibrose Retroperitoneal/diagnóstico , Diagnóstico Diferencial , Humanos , Doenças Raras/epidemiologia , Fibrose Retroperitoneal/complicações , Fibrose Retroperitoneal/congênito , Fibrose Retroperitoneal/diagnóstico por imagem , Fibrose Retroperitoneal/epidemiologia , Fibrose Retroperitoneal/etiologia , Fibrose Retroperitoneal/patologia , Tomografia Computadorizada por Raios X/métodos , Ultrassonografia/métodos
12.
Lung ; 193(2): 269-74, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25680417

RESUMO

OBJECTIVE: Data are sparse regarding the prevalence of pulmonary hypertension (PH) in obstructive sleep apnoea (OSA) patients without COPD and clinically manifest cardiac diseases and the role of continuous positive airway pressure (CPAP) and Uvulopalatopharyngoplasty (UPPP) in normalizing this parameter. PATIENTS/METHODS: We studied 75 consecutive OSA patients, 55 of them men, using transthoracic echocardiography. A mild PH [pulmonary artery pressure (PAPs) 38.2 ± 6.8] was found in 25 subjects (prevalence 33%). These patients were divided into two groups: group 1A (n = 17), those treated with CPAP, and group 1B (n = 8), those who have the indication for a UPPP. We scheduled a follow-up at 3, 6 and 9 months. During follow-up, we performed echocardiography, measurement of anthropometric parameters (BMI, neck and waist-hip circumference), and of biochemical parameters (uric acid, fasting glucose, cholesterol, triglycerides) and blood pressure. RESULTS: Patients with PH had a higher BMI: 32 ± 6 versus 29 ± 4 (p < 0.001) and NC: 39.8 ± 4.76 versus 37.14 ± 3.49 (p = 0.003), were predominantly men (72%) and older: 64 ± 20 versus 55 ± 16 (p = 0.025) and had a significantly higher value of uric acid: 7.91 ± 2.35 versus 6.56 ± 1.31 (p = 0.003). We found a positive correlation between PH and BMI (r = 0.456; p < 0.001) and between uric acidic and PH (r = 0.636; p < 0.001). PAPs significantly changed, from 39.8 ± 4.1 to 27.1 ± 4, to 25.2 ± 3.1 and to 22.2 ± 3 mmHg (CI 95%; 15.09-20.11; p < 0.001) in group 1A and from 39.5 ± 5.1 to 23.4 ± 3.2, to 23.0 ± 3.1 and to 21.9 ± 2.9 mmHg (CI 95%; 13.15-22.05; p < 0.001) in group 1B (difference between the groups p = 0.12). CONCLUSIONS: PH was frequent in OSA patients and normalized after 6 months with both CPAP and UPPP. A similar trend was noted in diastolic blood pressure.


Assuntos
Pressão Positiva Contínua nas Vias Aéreas , Hipertensão Pulmonar/terapia , Palato/cirurgia , Faringe/cirurgia , Apneia Obstrutiva do Sono/terapia , Adulto , Idoso , Pressão Arterial , Índice de Massa Corporal , Ecocardiografia , Feminino , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Pescoço/anatomia & histologia , Artéria Pulmonar , Apneia Obstrutiva do Sono/complicações , Úvula/cirurgia , Circunferência da Cintura
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