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1.
Clin Exp Rheumatol ; 37 Suppl 117(2): 130-136, 2019 Mar-Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31162033

RESUMO

OBJECTIVES: Cardiovascular (CV) events are highly prevalent in systemic necrotising vasculitides (SNV). Visceral/subcutaneous adipose tissue (VAT/SAT) ratio has been shown to be associated with CV events in various diseases. We aimed to assess the relevance of abdominal adipose tissue measurement to predict major CV events (MCVEs) in SNV. METHODS: Patients with SNV were successively included in a longitudinal study assessing MCVEs and other sequelae. Dual x-ray absorptiometry was performed to evaluate abdominal adipose tissue. Patients were prospectively followed for MCVEs, defined as myocardial infarction, unstable angina, stroke, arterial revascularisation and/or hospitalisation for or death from CV causes. RESULTS: One hundred and twenty consecutive SNV patients were included and analysed (54 males, mean age 53±18 years). High CV risk was found in 28 (23.3%) patients. In univariate analysis, age, male gender, VDI, VAT/SAT ratio and serum troponin level were significantly associated with high CV risk, whereas age and VAT/SAT ratio remained independently associated with high CV risk. Variables associated with high tertile of VAT/SAT ratio included age and metabolic risk factors. After median follow-up of 42 months, 19 (16%) patients experienced MCVEs. Hazard ratios for incident MCVEs compared with 1st tertile of VAT/SAT ratio were 7.22 (1.02-51.3; p=0.048) and 9.90 (3.15-31.2; p=0.0002) in the 2nd and 3rd tertile, respectively. CONCLUSIONS: Abdominal visceral adipose tissue is a reliable surrogate marker of CV risk and predicts incident MCVEs in SNV patients. Abdominal adipose tissue should be probably evaluated routinely in these patients to assess CV risk.


Assuntos
Gordura Abdominal/metabolismo , Doenças Cardiovasculares , Vasculite Sistêmica/complicações , Tecido Adiposo/metabolismo , Adulto , Idoso , Doenças Cardiovasculares/diagnóstico , Feminino , Humanos , Gordura Intra-Abdominal , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Vasculite Sistêmica/metabolismo
2.
Clin Infect Dis ; 64(6): 767-775, 2017 03 15.
Artigo em Inglês | MEDLINE | ID: mdl-28362954

RESUMO

Background: Although prognosis of Chronic Granulomatous Disease (CGD) has greatly improved, few studies have focused on its long-term outcome. We studied the clinical course and sequelae of CGD patients diagnosed before age 16, at various adult time points. Method: Cross-sectional French nationwide retrospective study of patients screened through the National Reference Center for Primary Immunodeficiencies (CEREDIH) registry. Results: Eighty CGD patients (71 males [88.7%], 59 X-linked [73.7%], median age 23.9 years [minimum, 16.6; maximum, 59.9]) were included, Median ages at diagnosis and last follow-up were 2.52 and 23.9 years, respectively. Seven patients underwent hematopoietic stem cell transplantation. A total of 553 infections requiring hospitalization occurred in 2017 patient-years. The most common site of infection was pulmonary (31%). Aspergillus spp. (17%) and Staphylococcus aureus (10.7%) were the commonest pathogens. A total of 224 inflammatory episodes occurred in 71 patients, mainly digestive (50%). Their characteristics as well as their annual frequency did not vary before and after age 16. Main sequelae were a small adult height and weight and mild chronic restrictive respiratory failure. At age 16, only 53% of patients were in high school. After age 30 years, 9/13 patients were working. Ten patients died during adulthood. Conclusions: Adult CGD patients displayed similar characteristics and rates of severe infections and inflammatory episodes that those of childhood. The high rate of handicap has become a matter of medical and social consideration. Careful follow-up in centers of expertise is strongly recommended and an extended indication of curative treatment by HSCT should be considered.


Assuntos
Doença Granulomatosa Crônica/epidemiologia , Adolescente , Fatores Etários , Antibioticoprofilaxia , Autoimunidade , Infecções Bacterianas/tratamento farmacológico , Infecções Bacterianas/epidemiologia , Infecções Bacterianas/etiologia , Infecções Bacterianas/prevenção & controle , Criança , Pré-Escolar , Efeitos Psicossociais da Doença , Estudos Transversais , Feminino , França/epidemiologia , Doença Granulomatosa Crônica/complicações , Doença Granulomatosa Crônica/diagnóstico , Doença Granulomatosa Crônica/mortalidade , Humanos , Lactente , Recém-Nascido , Masculino , Micoses/tratamento farmacológico , Micoses/epidemiologia , Micoses/etiologia , Micoses/prevenção & controle , Fenótipo , Vigilância da População , Sistema de Registros , Estudos Retrospectivos , Análise de Sobrevida , Avaliação de Sintomas
3.
Autoimmun Rev ; 16(5): 504-511, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28279837

RESUMO

OBJECTIVE: The diagnostic workup of uveitis is a challenge due to the wide range of diagnoses and the lack of a well-codified diagnostic procedure. We aimed to evaluate the relevance of diagnostic investigations for the etiological diagnosis of uveitis. METHODS: Retrospective cohort study of patients referred for etiological diagnosis of uveitis. Uveitis related to ophthalmological diseases or occurring during the course of previously diagnosed diseases were not included. RESULTS: Three hundred patients were included. Chest CT-scan was suggestive of sarcoidosis in 83 (29%). Features associated with abnormal CT-scan were: snowballs and/or peripheral multifocal choroiditis (PMC) upon ocular examination (P=0.004), blood lymphopenia (P<0.0001), angiotensin converting enzyme (ACE) level>1.5 ULN (P=0.0003). Bronchoscopy showed granuloma in 18 (11%) while alveolar lymphocytosis suggestive of sarcoidosis was reported in 45 (27%). Presence of granuloma on bronchial biopsies was always associated with chest CT-scan abnormalities, whereas 31% of patients with alveolar lymphocytosis had normal CT-scans. Features associated with contributive bronchoscopy were: snowballs and/or PMC (P=0.003), ACE>1.5 ULN (P=0.007), abnormal chest-CT scan (P<0.0001). Salivary gland biopsy revealed granuloma in 12 patients (5%). Cerebral MRI was abnormal in 15 patients (9%) who mostly presented with snowballs and/or retinal vasculitis. Finally, the main causes of uveitis were latent tuberculosis (25%) and sarcoidosis (22%), but 34% remained of undetermined origin. Uveitis relapses were observed in 31% and did not differ between patients with an identified diagnosis and those with idiopathic uveitis. CONCLUSION: Identification of factors associated with abnormal investigations might improve the optimal diagnostic workup adapted to each patient.


Assuntos
Uveíte/diagnóstico , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
4.
Rheumatology (Oxford) ; 56(3): 445-450, 2017 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-27940591

RESUMO

Objectives: Granulomatosis with polyangiitis (GPA) mainly affects white Europeans, but rarely GPA may also affect non-Europeans. This study aimed to describe GPA clinical-biological presentation and outcome in black sub-Saharan Africans and Afro-Caribbeans and in North Africans. Methods: Among 914 GPA patients included in the French Vasculitis Study Group database, geographic origin and ethnicity were known for 760. Clinical-biological presentations and outcomes of white Europeans vs black sub-Saharans and Afro-Caribbeans and vs North Africans were analysed. Results: Among the 760 patients, 689 (91%) were white Europeans, 33 (4.3%) were North Africans and 22 (2.9%) were sub-Saharans (n = 8) or Afro-Caribbeans (French West Indies, n = 14). Black sub-Saharans and Afro-Caribbeans, compared with white Europeans, were significantly younger at GPA diagnosis (P = 0.003), had more frequent central nervous system involvement (P = 0.02), subglottic stenosis (P = 0.002) and pachymeningitis (P = 0.009), and tended to have more frequent chondritis and retroorbital tumour. Median serum creatinine levels and Birmingham Vasculitis Activity Score were significantly lower in sub-Saharans and Afro-Caribbeans (P = 0.002 and P = 0.003, respectively). In contrast, in comparison with white Europeans, North Africans had only less frequent arthralgias (P = 0.004). Time to relapse was shorter for black sub-Saharans and Afro-Caribbeans compared with white Europeans [adjusted HR = 1.96 (95% CI: 1.09, 3.51) (P = 0.02)], and did not differ for North Africans. In contrast, overall survival was not significantly different according to ethnicity. Conclusion: Our findings indicated different GPA clinical presentations in white Europeans and sub-Saharans and Afro-Caribbeans, with black patients having more frequent severe granulomatous manifestations. In addition, time to relapse was significantly shorter for black sub-Saharans and Afro-Caribbeans compared with white Europeans.


Assuntos
Doenças das Cartilagens/etnologia , Granulomatose com Poliangiite/etnologia , Laringoestenose/etnologia , Meningite/etnologia , Vasculite do Sistema Nervoso Central/etnologia , Adulto , África ao Sul do Saara/etnologia , África do Norte/etnologia , Grupo com Ancestrais do Continente Africano/etnologia , Distribuição por Idade , Idoso , Doenças das Cartilagens/etiologia , Creatinina/sangue , Grupo com Ancestrais do Continente Europeu/etnologia , Feminino , França/epidemiologia , Granulomatose com Poliangiite/sangue , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/fisiopatologia , Humanos , Laringoestenose/etiologia , Masculino , Meningite/etiologia , Pessoa de Meia-Idade , Recidiva , Fatores de Tempo , Vasculite do Sistema Nervoso Central/etiologia , Índias Ocidentais/etnologia
5.
Clin Exp Rheumatol ; 35 Suppl 103(1): 40-46, 2017 Mar-Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28032843

RESUMO

OBJECTIVES: Improved therapeutic strategies for ANCA-associated vasculitis (AAV) have transformed acute and life-threatening diseases into chronic ones responsible for marked morbidity that could impact employment, work disability and quality of life (QoL). We aimed to analyse work, handicaps and QoL of AAV patients and identify their determinants. METHODS: Patients with AAV were included in a cross-sectional study assessing employment, work disability and QoL. Specific and non-specific questionnaires, including SF-36, were sent to patients, and clinical-biological data that could affect QoL and their determinants were analysed. RESULTS: Questionnaires were completed by 189 patients. Among 94 working-age (<60 years) patients, 57% had jobs, consistent with their qualifications for 81%, 77% were stably employed; 23% of workers felt that their disease qualitatively limited the nature of their work, while 43% felt it limited the quantity of work they could do; 50% thought their disease had hindered their careers and 43% that it had led to a salary reduction. These results were comparable for the different vasculitides. QoL was significantly impaired for AAV patients compared to the general population (p<0.0001). Physical health determinants for our population were diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA), long disease duration and its neurological involvement, whereas mental health determinants tended to be ear, nose and throat and cardiovascular involvement, and unemployment. CONCLUSIONS: Our findings showed that AAV patients' QoL was impaired compared to the general population, mainly for patients with EGPA and long-standing disease. In contrast, normal employment seemed to be preserved for the majority of the patients.


Assuntos
Absenteísmo , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Efeitos Psicossociais da Doença , Emprego , Saúde do Trabalhador , Qualidade de Vida , Licença Médica , Avaliação da Capacidade de Trabalho , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/fisiopatologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/psicologia , Estudos Transversais , Feminino , Nível de Saúde , Inquéritos Epidemiológicos , Humanos , Masculino , Pessoa de Meia-Idade , Paris , Valor Preditivo dos Testes , Fatores de Tempo
6.
Autoimmun Rev ; 16(1): 1-9, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27671089

RESUMO

OBJECTIVE: To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA. METHODS: Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease. RESULTS: The study population included 157 patients (mean age 49.4±14.1), with a follow-up of 7.4±6.4years. Patients with ANCA (31%) more frequently had weight loss, myalgias, arthralgias, biopsy-proven vasculitis, glomerulonephritis on biopsy, hematuria, leukocytoclastic capillaritis and/or eosinophilic infiltration of arterial wall on biopsy, and other renal disease. A total of 41% of patients had definite vasculitis manifestations (37%) or strong surrogates of vasculitis (4%), of whom only 53% had ANCA. Mononeuritis multiplex was associated with systemic vasculitis (p=0.005) and with the presence of ANCA (p<0.001). Overall, 59% of patients had polyangiitis as defined by definite vasculitis, strong surrogate of vasculitis, mononeuritis multiplex, and/or ANCA with at least one systemic manifestation other than ENT or respiratory. Patients with polyangiitis had more systemic manifestations including arthralgias (p=0.02) and renal disease (p=0.024), had higher peripheral eosinophilia (p=0.027), and a trend towards less myocarditis (p=0.057). Using predefined criteria of vasculitis and surrogates of vasculitis, ANCA alone were found to be insufficient to categorise patients with vasculitis features. CONCLUSION: We suggest a revised nomenclature and definition for EGPA and a new proposed entity referred to as hypereosinophilic asthma with systemic (non vasculitic) manifestations.


Assuntos
Síndrome de Churg-Strauss/patologia , Adulto , Asma/imunologia , Síndrome de Churg-Strauss/tratamento farmacológico , Síndrome de Churg-Strauss/imunologia , Feminino , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Resultado do Tratamento
7.
Eur Respir J ; 48(5): 1429-1441, 2016 11.
Artigo em Inglês | MEDLINE | ID: mdl-27587545

RESUMO

The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail.In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease.The study population included 157 patients (mean±sd age 49.4±14.1 years), with a mean±sd blood eosinophil count of 7.4±6.4×109 L-1 at diagnosis. There was a mean±sd of 11.8±18.2 years from the onset of asthma to the diagnosis of EGPA, of 1.4±8.4 years from the first onset of peripheral eosinophilia to the diagnosis of EGPA, and of 7.4±6.4 years from EGPA diagnosis to the final visit. Despite inhaled and oral corticosteroid treatment, the severity of asthma increased 3-6 months before the onset of the systemic manifestations. Asthma was severe in 57%, 48%, and 56% of patients at diagnosis, at 3 years, and at the final visit, respectively. Persistent airflow obstruction was present in 38%, 30%, and 46% at diagnosis, at 3 years, and at the final visit, respectively.In EGPA, asthma is severe, antedates systemic manifestations by a mean of 12 years, and progresses to long-term persistent airflow obstruction despite corticosteroids in a large proportion of patients, which affects long-term management and morbidity.


Assuntos
Síndrome de Churg-Strauss/fisiopatologia , Eosinófilos/citologia , Granulomatose com Poliangiite/fisiopatologia , Administração Oral , Adolescente , Corticosteroides/administração & dosagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Asma/fisiopatologia , Lavagem Broncoalveolar , Síndrome de Churg-Strauss/complicações , Eosinofilia/fisiopatologia , Feminino , França , Granulomatose com Poliangiite/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Testes de Função Respiratória , Estudos Retrospectivos , Índice de Gravidade de Doença , Vasculite Sistêmica/fisiopatologia , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Adulto Jovem
8.
Eur J Intern Med ; 26(7): 545-53, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25971154

RESUMO

OBJECTIVE: To develop disease-specific recommendations for the diagnosis and management of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (EGPA). METHODS: The EGPA Consensus Task Force experts comprised 8 pulmonologists, 6 internists, 4 rheumatologists, 3 nephrologists, 1 pathologist and 1 allergist from 5 European countries and the USA. Using a modified Delphi process, a list of 40 questions was elaborated by 2 members and sent to all participants prior to the meeting. Concurrently, an extensive literature search was undertaken with publications assigned with a level of evidence according to accepted criteria. Drafts of the recommendations were circulated for review to all members until final consensus was reached. RESULTS: Twenty-two recommendations concerning the diagnosis, initial evaluation, treatment and monitoring of EGPA patients were established. The relevant published information on EGPA, antineutrophil-cytoplasm antibody-associated vasculitides, hypereosinophilic syndromes and eosinophilic asthma supporting these recommendations was also reviewed. DISCUSSION: These recommendations aim to give physicians tools for effective and individual management of EGPA patients, and to provide guidance for further targeted research.


Assuntos
Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/tratamento farmacológico , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Comitês Consultivos , Consenso , Gerenciamento Clínico , Europa (Continente) , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Rituximab/uso terapêutico , Estados Unidos
9.
Autoimmun Rev ; 14(9): 774-80, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25960167

RESUMO

OBJECTIVE: To determine the diagnostic and prognostic significance of cardiac magnetic resonance imaging (CMRI) in a cohort of patients with eosinophilic granulomatosis with polyangiitis (EGPA). METHODS: We conducted a monocentric retrospective study including 42 EGPA patients who had consecutively undergone CMRI at diagnosis or during follow-up, independently of signs of cardiac involvement. RESULTS: Forty-two patients (male 59.5%, mean age at diagnosis 46.5 years) were included. ANCA was positive in 26.2%, and median EGPA duration before the 1st CMRI screening was 5 months. Seventeen (40.5%) were diagnosed with cardiomyopathy, independently of CMRI findings. CMRI showed myocardial late gadolinium enhancement (LGE) in 82.4% patients with cardiomyopathy vs. 44% without cardiomyopathy (P=0.024). Using LGE as the sole criterion, CMRI sensitivity and specificity for diagnosing cardiomyopathy were 82.4% and 56%, respectively. Among the 15 patients with cardiomyopathy who underwent additional CMRI during follow-up, CMRI-detected cardiac lesions had improved in 7 patients, while those of 8 patients worsened or stabilized despite treatment. These latter patients presented with significantly more cardiac events during follow-up (P=0.026). No differences were found between non-cardiomyopathic patients with or without CMRI anomalies concerning EGPA cardiac manifestations and outcomes. CONCLUSION: The diagnostic significance of myocardial LGE in EGPA patients remains uncertain and should not be the only criterion for cardiomyopathy diagnosis. For patients with no other signs of cardiomyopathy, CMRI-detected anomalies do not seem to adversely affect prognosis or outcome. For patients with cardiomyopathy, CMRI reassessment seems promising in detecting patients with a less favorable cardiac outcome.


Assuntos
Granulomatose com Poliangiite/patologia , Miocárdio/patologia , Eosinófilos , Humanos , Imagem por Ressonância Magnética/métodos , Prognóstico , Estudos Retrospectivos
10.
Autoimmun Rev ; 14(8): 742-50, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25916811

RESUMO

OBJECTIVES: To describe the clinical presentation, management and prognosis of patients diagnosed with both primary Sjögren's syndrome (pSS) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: French nation-wide survey completed by a systematic literature review. RESULTS: This work identified 7 new cases of coexisting pSS and AAV: 2 microscopic polyangiitis (MPA), 2 granulomatosis with polyangiitis (GPA), 2 anti-myeloperoxidase (MPO)-ANCA renal-limited AAV, and 1 eosinophilic granulomatosis with polyangiitis (EGPA). The systematic literature search identified 15 previously published cases. Among the 22 patients, 19 were females. Mean age at diagnosis of AAV was 63.9±9.8years. All individuals with available information experienced at least one extra-glandular manifestation attributable to pSS. p-ANCA with anti-MPO specificity were found in 76.2% (16/21), c-ANCA with anti-PR3 specificity in 14.3% (3/21) and isolated c-ANCA in 13.6% (3/22). Vasculitis involved kidneys (n=13), lungs (n=8), skin (n=6), peripheral nerves (n=5), central nervous system (n=2), small bowel (n=1), muscle (n=1), ear chondritis (n=1) and sinuses (n=1). The mean AAV follow-up was 73.5 (±120.0) months. While on treatment, disease remission occurred in 77.3% of cases, and one death was reported in the first 6months after diagnosis. CONCLUSION: This work shows that AAV may occur in patients with pSS. These are most commonly p-ANCA associated vasculitis with anti-MPO specificity. AAV may reveal an underlying pSS or arise during its evolution, but did not precede pSS in any of these cases. AAV occurrence appears to be correlated with extra-glandular manifestations of pSS.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Síndrome de Sjogren/complicações , Humanos , Nefropatias/complicações , Prognóstico
11.
Clin Rev Allergy Immunol ; 49(3): 317-26, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25672591

RESUMO

Hydroxychloroquine (HCQ) and chloroquine have been used for more than 50 years to treat systemic lupus erythematosus (SLE) and other rheumatic diseases. In general, these drugs are well tolerated and rarely need to be discontinued because of an adverse systemic reaction. However, both medications can be irreversibly toxic to the retina. A new study indicates that toxicity is not as rare as once believed, but depends critically on daily dosage and duration of use, as well as other risk factors. With attention to dosage and other factors, and with proper screening for early signs of toxicity, HCQ can be prescribed with relative safety even over long periods of time.


Assuntos
Cloroquina/uso terapêutico , Oftalmopatias/prevenção & controle , Hidroxicloroquina/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Retina/efeitos dos fármacos , Animais , Cloroquina/efeitos adversos , Cálculos da Dosagem de Medicamento , Oftalmopatias/etiologia , Humanos , Hidroxicloroquina/efeitos adversos , Lúpus Eritematoso Sistêmico/complicações , Retina/patologia
12.
Eur Respir J ; 45(6): 1613-23, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25614174

RESUMO

Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by failure of superoxide production in phagocytic cells. The disease is characterised by recurrent infections and inflammatory events, frequently affecting the lungs. Improvement of life expectancy now allows most patients to reach adulthood. We aimed to describe the pattern of pulmonary manifestations occurring during adulthood in CGD patients. This was a retrospective study of the French national cohort of adult patients (≥16 years old) with CGD. Medical data were obtained for 67 adult patients. Pulmonary manifestations affected two-thirds of adult patients. Their incidence was significantly higher than in childhood (mean annual rate 0.22 versus 0.07, p=0.01). Infectious risk persisted despite anti-infectious prophylaxis. Invasive fungal infections were frequent (0.11 per year per patient) and asymptomatic in 37% of the cases. They often required lung biopsy for diagnosis (10 out of 30). Noninfectious respiratory events concerned 28% of adult patients, frequently associated with a concomitant fungal infection (40%). They were more frequent in patients with the X-linked form of CGD. Immune-modulator therapies were required in most cases (70%). Respiratory manifestations are major complications of CGD in adulthood. Noninfectious pulmonary manifestations are as deleterious as infectious pneumonia. A specific respiratory monitoring is necessary.


Assuntos
Doença Granulomatosa Crônica/complicações , Pneumopatias Fúngicas/etiologia , Pulmão/patologia , Pneumonia Bacteriana/etiologia , Adolescente , Adulto , Anti-Infecciosos/uso terapêutico , Doenças Assintomáticas , Biópsia , Estudos de Coortes , Feminino , Doença Granulomatosa Crônica/tratamento farmacológico , Doença Granulomatosa Crônica/genética , Humanos , Fatores Imunológicos/uso terapêutico , Pneumopatias/etiologia , Pneumopatias Fúngicas/diagnóstico , Pneumopatias Fúngicas/tratamento farmacológico , Masculino , Glicoproteínas de Membrana/genética , Pessoa de Meia-Idade , NADPH Oxidase 2 , NADPH Oxidases/genética , Pneumonia Bacteriana/diagnóstico , Pneumonia Bacteriana/tratamento farmacológico , Estudos Retrospectivos , Adulto Jovem
13.
Presse Med ; 43(6 Pt 2): e167-80, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24855048

RESUMO

The efficacy of antimalarials, especially hydroxychloroquine (HCQ), in preventing systemic lupus erythematosus (SLE) flares is well demonstrated. However, many studies show that the percentage of SLE patients treated with HCQ remains low. By blocking the toll-like receptor 7 and 9 in plasmacytoid dendritic cells, HCQ inhibits interferon-alpha production which plays a crucial role in SLE pathogenesis. In addition to reducing damage accrual in SLE patients, HCQ appears to protect against the occurrence of diabetes, thrombotic events, and dyslipidemia. As a consequence, some studies have suggested that HCQ, which is inexpensive, has a protective effect on survival in SLE patients. Thanks to the pharmacokinetic properties of HCQ (long half-life) and to the availability of its blood assay, very low or undetectable blood HCQ concentrations are a valuable marker of non-adherence to treatment, thus adding a new benefit to HCQ prescriptions. The main side effect of HCQ is retinal toxicity. This complication is very rare, but may be potentially severe, thus requiring regular screening. Retinal toxicity remains the only absolute contra-indication of HCQ in adult SLE patients. Other contra-indications are few and rare. During pregnancy and breast-feeding, HCQ continuation is not only allowed but recommended. In conclusion, the risk/benefit ratio of HCQ is excellent. Many now believe that all SLE patients should be offered this treatment.


Assuntos
Antirreumáticos/uso terapêutico , Hidroxicloroquina/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Antimaláricos/uso terapêutico , Antirreumáticos/efeitos adversos , Antirreumáticos/sangue , Biomarcadores/sangue , Meia-Vida , Humanos , Hidroxicloroquina/efeitos adversos , Hidroxicloroquina/sangue , Interferon-alfa/antagonistas & inibidores , Lúpus Eritematoso Sistêmico/prevenção & controle , Adesão à Medicação , Doenças Retinianas/induzido quimicamente , Receptor 7 Toll-Like/antagonistas & inibidores , Receptor Toll-Like 9/antagonistas & inibidores
14.
J Autoimmun ; 48-49: 99-103, 2014 Feb-Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24530234

RESUMO

Recently, a group of experts in the field suggested to rename Churg-Strauss syndrome as eosinophilic granulomatosis with polyangiitis (EGPA). This condition, first described in 1951, is a rare small- and medium-sized-vessel vasculitis characterized by an almost constant association with asthma and eosinophilia, and, by the presence of anti-myeloperoxidase (MPO) antineutrophil cytoplasm antibodies (ANCA) in 30-38% of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient. Asthma is severe, associated with eosinophilia and extrapulmonary symptoms. Most frequently EGPA involves the peripheral nerves and skin. Other organs, however, may be affected and must be screened for vasculitis, especially those associated with a poorer prognosis, such as the heart, kidney and gastrointestinal tract, as assessed by the recently revised Five-Factor Score (FFS). Recent insights, particularly concerning clinical differences associated with ANCA status, showed that EGPA patients might constitute a heterogeneous group. Thus, EGPA patients with anti-MPO ANCA suffered more, albeit not exclusively, from vasculitis symptoms, such as glomerulonephritis, mononeuritis multiplex and alveolar hemorrhage, whereas ANCA-negative patients more frequently develop heart involvement. This observation led to the hypothesis that EGPA might be divided into different clinical and pathophysiological subtypes, which could be managed better with more specifically adapted therapies. For now, EGPA treatment still relies mainly on corticosteroids and, when necessary for patients with poorer prognoses, combined immunosuppressant drugs, especially cyclophosphamide. Overall survival of EGPA patients is good, despite not uncommon relapses.


Assuntos
Síndrome de Churg-Strauss/classificação , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/epidemiologia , Diagnóstico Diferencial , Humanos , Incidência , Poliangiite Microscópica/classificação , Poliangiite Microscópica/diagnóstico , Prevalência , Terminologia como Assunto
16.
Arthritis Rheum ; 65(1): 270-81, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23044708

RESUMO

OBJECTIVE: Earlier studies of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), with limited patient numbers and followup durations, demonstrated that clinical presentation at diagnosis, but not outcome, differed according to antineutrophil cytoplasmic antibody (ANCA) status. This study was undertaken to describe the main characteristics of a larger patient cohort and their long-term outcomes. METHODS: A retrospective study of EGPA patients in the French Vasculitis Study Group cohort who satisfied the American College of Rheumatology criteria and/or Chapel Hill definitions was conducted. Patient characteristics and outcomes were compared according to ANCA status and year of diagnosis. RESULTS: We identified 383 patients diagnosed between 1957 and June 2009 (128 [33.4%] before 1997 or earlier) and followed up for a mean±SD of 66.8±62.5 months. At diagnosis, their mean±SD age was 50.3±15.7 years, and 91.1% had asthma (duration 9.3±10.8 years). Main manifestations included peripheral neuropathy (51.4%); ear, nose, and throat (ENT) signs (48.0%); skin lesions (39.7%); lung infiltrates (38.6%); and cardiomyopathy (16.4%). Among the 348 patients tested at diagnosis for ANCA, the 108 ANCA-positive patients (31.0%) had significantly more frequent ENT manifestations, peripheral neuropathy, and/or renal involvement, but less frequent cardiac manifestations, than the ANCA-negative patients. Vasculitis relapses occurred in 35.2% of the ANCA-positive versus 22.5% of the ANCA-negative patients (P=0.01), and 5.6% versus 12.5%, respectively, died (P<0.05). The 5-year relapse-free survival rate was 58.1% (95% confidence interval [95% CI] 45.6-68.6) for ANCA-positive and 67.8% (95% CI 59.8-74.5) for ANCA-negative patients (P=0.35). Multivariable analysis identified cardiomyopathy, older age, and diagnosis during or prior to 1996 as independent risk factors for death and lower eosinophil count at diagnosis as predictive of relapse. CONCLUSION: The characteristics and long-term outcomes of EGPA patients differ according to their ANCA status. Although EGPA relapses remain frequent, mortality has declined, at least since 1996.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Síndrome de Churg-Strauss/diagnóstico , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Síndrome de Churg-Strauss/mortalidade , Feminino , Seguimentos , França/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Recidiva , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Resultado do Tratamento , Adulto Jovem
17.
J Clin Immunol ; 32(5): 936-41, 2012 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22526593

RESUMO

BACKGROUND: Acquired C1-inhibitor deficiency can occur secondary to excessive C1-inhibitor consumption (type I) and be associated with a lymphoid hemopathy, or linked to the presence of anti-C1-inhibitor autoantibodies (type II) in a context of an isolated monoclonal gammopathy, sometimes associated with lymphoproliferation. Efficacy of danazol, tranexamic acid and/or corticosteroids is inconstant. Rituximab efficacy against type II angioedema has been reported. METHODS: Description of 7 rituximab-treated patients, 6 with type II acquired angioedema and 1 with type I. RESULTS: Clinical efficacy (only for type II) was complete for 3, partial for 2 and 2 were therapeutic failures. Only 2 patients had improved biological parameters, with normalization of their C1-inhibitor levels and diminished anti-C1-inhibitor autoantibodies, observed 1-9 months after the last infusion of the second rituximab cycle. An associated lymphoproliferation did not affect the response to treatment. CONCLUSION: Rituximab efficacy in the treatment of acquired angioedema is inconstant and might require repeated cycles.


Assuntos
Angioedema/tratamento farmacológico , Anticorpos Monoclonais Murinos/uso terapêutico , Proteínas Inativadoras do Complemento 1/deficiência , Fatores Imunológicos/uso terapêutico , Idoso , Idoso de 80 Anos ou mais , Antígenos CD20/imunologia , Proteína Inibidora do Complemento C1 , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Rituximab
18.
Semin Respir Crit Care Med ; 32(3): 298-309, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21674415

RESUMO

Churg-Strauss syndrome (CSS), first described in 1951, is a rare vasculitis of small- and medium-sized vessels. It is characterized by a constant association with asthma and eosinophilia, and by the presence of anti-myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibodies (ANCA) in ~40% of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient and most frequently involves the peripheral nerves and skin. Other organs, however, may be affected and must be screened for vasculitis, especially those associated with a poorer prognosis, such as the heart, kidney, and gastrointestinal tract, as assessed by the recently revised Five-Factor Score (FFS). Overall survival of CSS patients is excellent, but relapses are not uncommon and require maintenance or steroid-sparing therapies, depending on the original FFS-based prognosis at diagnosis. All patients require corticosteroids, often for prolonged periods, combined with immunosuppressants [e.g., induction (cyclophosphamide) and maintenance therapy (azathioprine)], for those with poorer prognoses. Recent insights, especially concerning clinical differences associated with ANCA status, showed that CSS patients might constitute a heterogeneous group, both clinically and pathogenically. Future therapies might reflect these differences more strongly.


Assuntos
Corticosteroides/administração & dosagem , Síndrome de Churg-Strauss , Imunossupressores/administração & dosagem , Corticosteroides/uso terapêutico , Asma/complicações , Azatioprina/administração & dosagem , Azatioprina/uso terapêutico , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/epidemiologia , Síndrome de Churg-Strauss/fisiopatologia , Síndrome de Churg-Strauss/terapia , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Diagnóstico Diferencial , Eosinofilia/etiologia , Humanos , Imunossupressores/uso terapêutico , Incidência , Modalidades de Fisioterapia , Troca Plasmática , Prevalência , Prognóstico , Resultado do Tratamento
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