RESUMO
Objective: Real-world studies on the effectiveness of omalizumab in Egyptian population with asthma are limited. This study aimed to evaluate the real-world effectiveness and safety of omalizumab as an add-on treatment in pediatric and adult patients with severe, persistent allergic asthma in Egypt. Methods: The primary endpoint of this 16-week, prospective, open-label, multicenter, non-interventional study was the reduction in oral corticosteroid (OCS) dose. Secondary endpoints included reduction in exacerbation, improvements in quality of life and global assessment of omalizumab therapy. Results: Of the 59 patients, 53 completed the study. Add-on omalizumab significantly reduced the proportion of patients receiving OCS at Week 16 versus baseline (81.1% at baseline versus 52.8% at Week 16; p < 0.001). A 55% decrease in the total daily prednisolone-equivalent dose of OCS was observed at the end of the study compared to baseline (p < 0.001). No patients reported exacerbations or missed days from work or school after receiving omalizumab for 16 weeks compared to baseline (both p < 0.001). A statistically significant decrease was observed in asthma control questionnaire-5 scores (p < 0.001). Almost all physicians and patients rated omalizumab therapy as 'good,' 'very good' or 'excellent' in tolerability and effectiveness. No new safety signals were observed in the safety analysis of omalizumab as add-on treatment. Conclusions: Outcomes of this real-world study were consistent with previous effectiveness and safety studies of omalizumab. Omalizumab was effective and well tolerated for the management of severe, persistent IgE-mediated asthma in pediatric and adult patients in Egypt.
Assuntos
Antiasmáticos/uso terapêutico , Asma/tratamento farmacológico , Asma/epidemiologia , Hipergamaglobulinemia/epidemiologia , Omalizumab/uso terapêutico , Absenteísmo , Adolescente , Corticosteroides/uso terapêutico , Agonistas de Receptores Adrenérgicos beta 2/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Antiasmáticos/administração & dosagem , Antiasmáticos/efeitos adversos , Criança , Quimioterapia Combinada , Egito , Feminino , Volume Expiratório Forçado/efeitos dos fármacos , Humanos , Imunoglobulina E/sangue , Masculino , Pessoa de Meia-Idade , Omalizumab/administração & dosagem , Omalizumab/efeitos adversos , Estudos Prospectivos , Qualidade de Vida , Testes de Função Respiratória , Adulto JovemAssuntos
Hematologia , Doença Relacionada a Imunoglobulina G4 , Humanos , Hipergamaglobulinemia/complicações , Hipergamaglobulinemia/diagnóstico , Hipergamaglobulinemia/epidemiologia , Doença Relacionada a Imunoglobulina G4/complicações , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/epidemiologia , Plasmócitos , Estudos Retrospectivos , Centros de Atenção TerciáriaRESUMO
OBJECTIVES: Onset of primary SS is usually between 40 and 60 years of age, with severe systemic complications in 15% of cases. We sought to determine whether early-onset disease is related to a specific phenotype and if it is predictive of a poor outcome. METHODS: Biological and clinical data from 393 patients recruited in the ASSESS cohort, a French multicentre prospective cohort, were compared according to age at diagnosis. RESULTS: Fifty-five patients had early-onset disease, defined as age ⩽35 years at diagnosis, and presented a significantly higher frequency of salivary gland enlargement (47.2% vs 33.3%, P = 0.045), adenopathy (25.5% vs 11.8%, P = 0.006), purpura (23.6% vs 9.2%, P = 0.002) and renal involvement (16.4% vs 4.4%, P = 0.003). They had a higher frequency of hypergammaglobulinaemia (60.8% vs 26.6%, P < 0.001), RF positivity (41.5% vs 20.2%, P < 0.001), low C3 level (18.9% vs 9.1%, P = 0.032), low C4 level (54.7% vs 40.2%, P = 0.048) and autoantibodies [84.6% with anti-SSA vs 54.4% (P < 0.001) and 57.7% with anti-SSB vs 29.7% (P < 0.001)]. The change in ESSDAI scores between baseline and the 5-year follow-up was significantly different (P = 0.005) with a trend for worsening in the early-onset group (0.72, P = 0.27) and a significant improvement in the later onset group (-1.27, P < 0.0001). CONCLUSION: Early-onset primary SS is associated with a specific phenotype defined by clinical and biological features known to be predictive factors of severe systemic disease. Interestingly, we showed a different evolution of the ESSDAI score depending on the age at disease onset, patients with early-onset disease tending to worsen over time.
Assuntos
Síndrome de Sjogren/diagnóstico , Adulto , Distribuição por Idade , Fatores Etários , Idade de Início , Idoso , Idoso de 80 Anos ou mais , Autoanticorpos/sangue , Complemento C3/análise , Complemento C4/análise , Seguimentos , França/epidemiologia , Humanos , Hipergamaglobulinemia/epidemiologia , Hipergamaglobulinemia/etiologia , Linfadenopatia/epidemiologia , Linfadenopatia/etiologia , Pessoa de Meia-Idade , Fenótipo , Prognóstico , Estudos Prospectivos , Púrpura/epidemiologia , Púrpura/etiologia , Fator Reumatoide/sangue , Índice de Gravidade de Doença , Síndrome de Sjogren/complicações , Síndrome de Sjogren/epidemiologia , Síndrome de Sjogren/imunologiaRESUMO
INTRODUCTION: Autoimmune hepatitis (AIH) is one of the most common disorder resulting in end stage liver disease (ESLD) among children. Scarce data is available in this regard from Pakistan. In this study we have analyzed clinical and biochemical parameters of children suffering from this disorder. METHODS: It was a cross sectional study conducted in the Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation (SIUT) Karachi from January 2005 to June 2016. Patients aged up-to 18 years were included. AIH was diagnosed by using International Autoimmune hepatitis group (IAIHG) pre-treatment and simplified AIH score. Patients with both probable and definite score were included. Biochemical, serological, sonographic and demographics were recorded at the time of diagnosis, liver biopsy was also performed in most of the cases. Data was analyzed by using SPSS ver.20 and p-value of < 0.05 was considered significant. RESULTS: Total 51 patients were enrolled most of them were females (68.6%). Mean age of presentation was around 10 years. Males had statistically significant earlier age of presentation, p-value = 0.007. The most common presenting complain was jaundice. Hypergammaglobulinemia is seen in almost all patients. Type I AIH was the most common entity while Type II AIH was statistically more significant in males p-value = 0.019. Raised GGT was also seen in male patient specifically in Type II AIH, p-value = 0.001. CONCLUSION: Autoimmune hepatitis predominantly affects female children who have late age of presentation as compare to the males. Type I AIH was the most common while Type II AIH was more common in males and they also had raised GGT.
Assuntos
Hepatite Autoimune/diagnóstico , Hipergamaglobulinemia/etiologia , Icterícia/etiologia , Adolescente , Fatores Etários , Idade de Início , Biópsia , Criança , Pré-Escolar , Estudos Transversais , Feminino , Hepatite Autoimune/fisiopatologia , Humanos , Hipergamaglobulinemia/epidemiologia , Icterícia/epidemiologia , Masculino , Paquistão , Fatores SexuaisRESUMO
OBJECTIVE: To explore changes in the phenotypic features of Sjögren's syndrome (SS), and in SS status among participants in the Sjögren's International Collaborative Clinical Alliance (SICCA) registry over a 2-3-year interval. METHODS: All participants in the SICCA registry who were found to have any objective measures of salivary hypofunction, dry eye, focal lymphocytic sialadenitis in minor salivary gland biopsy, or anti-SSA/SSB antibodies were recalled over a window of 2 to 3 years after their baseline examinations to repeat all clinical examinations and specimen collections to determine whether there was any change in phenotypic features and in SS status. RESULTS: As of September 15, 2013, a total of 3,514 participants had enrolled in SICCA, and among 3,310 eligible, 771 presented for a followup visit. Among participants found to have SS using the 2012 American College of Rheumatology (ACR) classification criteria, 93% again met the criteria after 2 to 3 years, and this proportion was 89% when using the 2016 ACR/European League Against Rheumatism (EULAR) criteria. Among those who did not meet ACR or ACR/EULAR criteria at baseline, 9% and 8%, respectively, had progressed and met them at followup. Those with hypergammaglobulinemia and hypocomplementemia at study entry were, respectively, 4 and 6 times more likely to progress to SS by ACR criteria than those without these characteristics (95% confidence interval 1.5-10.1 and 1.8-20.4, respectively). CONCLUSION: While there was stability over a 2-3-year period of both individual phenotypic features of SS and of SS status, hypergammaglobulinemia and hypocomplementemia at study entry were predictive of progression to SS.
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Síndrome de Sjogren/diagnóstico , Adulto , Argentina/epidemiologia , Ásia/epidemiologia , Autoimunidade , Biomarcadores/sangue , Proteínas do Sistema Complemento/deficiência , Proteínas do Sistema Complemento/imunologia , Dinamarca/epidemiologia , Progressão da Doença , Feminino , Humanos , Hipergamaglobulinemia/diagnóstico , Hipergamaglobulinemia/epidemiologia , Hipergamaglobulinemia/imunologia , Masculino , Pessoa de Meia-Idade , Fenótipo , Valor Preditivo dos Testes , Prognóstico , Sistema de Registros , Fatores de Risco , Síndrome de Sjogren/epidemiologia , Síndrome de Sjogren/imunologia , Síndrome de Sjogren/fisiopatologia , Fatores de Tempo , Estados Unidos/epidemiologiaRESUMO
We investigated the correlation between the increased proportion of peripheral B cell subsets and clinical and immunological features in primary Sjögren's syndrome (pSS). We found that the proportion of CD19+ B cells was significantly increased in pSS as compared with HC and was correlated with serum IgG levels. Moreover, in vitro IgG production by CD19+ B cells was significantly increased in pSS and was positively and significantly correlated with serum IgG levels. FACS analysis revealed that the proportions of peripherally CD38highIgD+ B cells and CD38highIgD- B cells were significantly increased in pSS. In addition, the proportion of CD38highIgD+ B cells positively correlated with ESSDAI scores and serum levels of IgG, anti-Ro/SSA and anti-La/SSB antibodies while that of CD38highIgD- B cells showed no correlation with these parameters. Our data suggest that increased proportion of CD38highIgD+ B cells in pSS is involved in IgG overproduction including autoantibodies, and correlates with disease progression.
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Subpopulações de Linfócitos B/imunologia , Síndrome de Sjogren/imunologia , ADP-Ribosil Ciclase 1/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticorpos Antinucleares/imunologia , Antígenos CD19/imunologia , Linfócitos B/imunologia , Estudos de Casos e Controles , Feminino , Humanos , Hipergamaglobulinemia/epidemiologia , Imunoglobulina D/imunologia , Imunoglobulina G/imunologia , Masculino , Pessoa de Meia-Idade , Prevalência , Síndrome de Sjogren/epidemiologiaRESUMO
AIM: The aim of the study was to characterize quantitative and qualitative immunoglobulinopathies in patients with AITL at the onset of the disease. MATERIALS AND METHODS: 55 patients with newly diagnosed AITL were enrolled in the study, the male/female ratio was 30/25; median age was 61 (29-81) years. Diagnosis was based on standard WHO criteria. Immunochemical studies of blood serum included serum protein electrophoresis/immunofixation, nephelometric quantification of total immunoglobulins, serum free light chain assay. RESULTS: Quantitative and qualitative immunoglobulinopathies were determined in 49 (89,1%) of 55 pts. Quantitative immunoglobulinopathies were revealed in 47 (85.5%) of 55 cases, qualitative - in 14 (25,5%). Combination quantitative and qualitative immunoglobulinopathies was observed in 12 (21,8%) of 55 pts. The detected immunoglobulinopathies were divided into 4 groups: polyclonal hypergammaglobulinaemia, hypogammaglobulinaemia, oligoclonal gammapathy, and monoclonal gammapathy. Polyclonal hypergammaglobulinaemia was marked in 41 (74.5%) of 55 pts, elevated level of IgG was determined in 27 (49,15%) of 55 cases, IgM - in 18 (32,7%) and IgA - in 21 (38.2%). Interestingly, polyclonal IgE hypergammaglobulinaemia was detected in 12 (48,0%) of 25 cases of performed studies. Hypogammaglobulinaemia was detected in 8 (14,5%) of 55 cases. Oligoclonal gammapathy was determined in 4 (7.3%) of 55 pts. Monoclonal gammapathy was revealed in 11 (20,0%) of 55 cases. The amount of monoclonal immunoglobulin varied from 2.6 to 14.1 g/l. Monoclonal immunoglobulin Gk was detected in 5 of 11 pts, Gλ - in 2, Mλ - in 2, Mk - in 2. Monoclonal gammapathy was accompanied by polyclonal hypergammaglobulinaemia in 9 of 11 cases, hypogammaglobulinaemia - in 2. CONCLUSION: Quantitative and qualitative immunoglobulinopathies are observed in most patients at the onset of AITL. Quantitative abnormalities were determined more often than qualitative. Monoclonal gammapathy can be a manifestation of lymphoproliferation and other concomitant disorders. The prognostic value of immunochemical parameters is still unclear and requires dynamic observation and study.
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Agamaglobulinemia/complicações , Hipergamaglobulinemia/complicações , Linfadenopatia Imunoblástica/complicações , Linfoma de Células T/complicações , Paraproteinemias/complicações , Adulto , Agamaglobulinemia/sangue , Agamaglobulinemia/epidemiologia , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Hipergamaglobulinemia/sangue , Hipergamaglobulinemia/epidemiologia , Linfadenopatia Imunoblástica/sangue , Linfadenopatia Imunoblástica/epidemiologia , Cadeias Leves de Imunoglobulina/sangue , Linfoma de Células T/sangue , Linfoma de Células T/epidemiologia , Masculino , Pessoa de Meia-Idade , Paraproteinemias/sangue , Paraproteinemias/epidemiologiaRESUMO
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded. Demographics and pSS characteristics were recorded. The EGM were defined by EULAR-SS disease activity index (ESSDAI). Patients were divided into two groups according to their use or not of HCQ therapy. We evaluated the use of HCQ and its relationship to EGM. HCQ therapy was defined as the continuous use of the drug for at least 3 months. A descriptive analysis of demographics and pSS characteristics was performed. We compared the incidence of EGM between groups defined by HCQ therapy using chi2 test or Fisher's exact test. A total of 221 patients were included (97.3% women), mean age, 55.7 years (SD 14). Mean age at diagnosis, 48.8 years (SD 15); median disease duration, 60 months (IQR 35-84). One hundred and seventy patients (77%) received HCQ. About half of the patients had at least one EGM during the course of the disease, 20% of them developed an EGM before the onset of the sicca syndrome and 26% simultaneously with dryness symptom. Overall, EGM were less frequent in those on HCQ therapy (36.5% vs 63.5%, p < 0.001). Considering each EGM individually, the following manifestations were more frequent in the non-treated group: arthritis (p < 0.001), fatigue (p < 0.001), purpura (p = 0.01), Raynaud phenomenon (p = 0.003), and hypergammaglobulinemia (p = 0.006). Immunosuppressive treatment was indicated on 28 patients (12.7%), 13 of which were receiving also HCQ. The first reason for those treatments was the presence of arthritis in 12/28 patients (42.8%), and the drug used in all the cases was methotrexate. Only three patients required immunosuppressive therapy with cyclophosphamide, due to the presence of glomerulonephritis, vasculitis, and interstitial lung disease. None of the patients received biologic therapy. The lower incidence of EGM was observed in patients on HCQ therapy supports its efficacy in pSS. However, further large scale prospective studies are needed to confirm these findings.
Assuntos
Antirreumáticos/uso terapêutico , Hidroxicloroquina/uso terapêutico , Síndrome de Sjogren/complicações , Síndrome de Sjogren/tratamento farmacológico , Adulto , Fadiga/epidemiologia , Fadiga/etiologia , Feminino , Humanos , Hipergamaglobulinemia/epidemiologia , Hipergamaglobulinemia/etiologia , Incidência , Masculino , Pessoa de Meia-Idade , Púrpura/epidemiologia , Púrpura/etiologia , Doença de Raynaud/epidemiologia , Doença de Raynaud/etiologia , Estudos RetrospectivosRESUMO
AIM: To test the hypothesis that systemic auto-antibodies or hypergammaglobulinemia are related to the prevalence of extra-glandular tissue organ damage (EGOD) in primary Sjögren's syndrome (SS). METHODS: A real practice-based investigation of a relatively large (n = 110) Dutch cohort of primary SS patients systematically followed up in a large non-academic hospital. RESULTS: After a follow up of mean 8.2 years a significant correlation was found between disease duration and the prevalence of EGOD. We did not observe a relationship between the total number or type of systemic auto-antibodies or hypergammaglobulinemia and the total number of EGOD. However, there was a correlation between the prevalence of polyneuropathy (PNP) and antinuclear antibodies (ANA) as well as anti-Ro/SS-A positivity and there was an inverse relationship between the presence of anti-Ro/SS-A antibodies and primary biliary cirrhosis (PBC). All PBC cases were anti-Ro/SS-A and anti-La/SS-B negative but ANA positive. There was a trend for a higher occurrence of pleuro-pulmonary disease in the ANA negative cases. CONCLUSIONS: Although we did not find a relationship between the total number or type of systemic auto-antibodies and the total number of EGOD, there were correlations between specific systemic auto-antibodies and specific types of EGOD. The presence of ANA and anti-Ro/SS-A was associated with the occurrence of PNP, as well as was the absence of anti-Ro/SS-A with PBC.
Assuntos
Autoanticorpos/sangue , Hipergamaglobulinemia/epidemiologia , Hipergamaglobulinemia/imunologia , Síndrome de Sjogren/epidemiologia , Síndrome de Sjogren/imunologia , Adulto , Idoso , Anticorpos Antinucleares/sangue , Biomarcadores/sangue , Feminino , Humanos , Hipergamaglobulinemia/sangue , Hipergamaglobulinemia/diagnóstico , Cirrose Hepática Biliar/epidemiologia , Cirrose Hepática Biliar/imunologia , Masculino , Pessoa de Meia-Idade , Países Baixos/epidemiologia , Polineuropatias/epidemiologia , Polineuropatias/imunologia , Prevalência , Estudos Retrospectivos , Fatores de Risco , Síndrome de Sjogren/sangue , Síndrome de Sjogren/diagnóstico , Fatores de TempoAssuntos
Colite Ulcerativa/epidemiologia , Doença de Crohn/epidemiologia , Hipergamaglobulinemia/sangue , Hipergamaglobulinemia/epidemiologia , gama-Globulinas/análise , Biomarcadores/sangue , Colite Ulcerativa/diagnóstico , Doença de Crohn/diagnóstico , Estudos Transversais , Humanos , Hipergamaglobulinemia/diagnóstico , Prevalência , Estudos Retrospectivos , Fatores de Risco , Cidade de Roma/epidemiologia , Estudos Soroepidemiológicos , Testes SorológicosRESUMO
This retrospective single-center study assessed the incidence and clinical features of immune manifestations of refractory cytopenia of childhood (RCC) and childhood aplastic anemia (AA). We evaluated 72 children with RCC and 123 with AA between February 2008 and March 2013. RCC was associated with autoimmune disease in 4 children, including 1 case each with autoimmune hemolytic anemia, rheumatoid arthritis, systemic lupus erythematosus, and anaphylactoid purpura. No children with AA were diagnosed with autoimmune diseases. Immune abnormalities were common in both RCC and AA; the most significant reductions were in the relative numbers of CD3-CD56+ subsets found in RCC. Despite the many similar immunologic abnormalities in AA and RCC, the rate of autoimmune disease was significantly lower in childhood AA than RCC (p=0.008, χ2=6.976). The relative numbers of natural killer cells were significantly lower in RCC patients than AA patients. By month 6, there was no significant difference in autoimmune manifestations between RCC and AA in relation to the response to immunosuppressive therapy (p=0.907, χ2=0.014). The large overlap of analogous immunologic abnormalities indicates that RCC and childhood AA may share the same pathogenesis.
Assuntos
Anemia Aplástica/epidemiologia , Doenças Autoimunes/epidemiologia , Síndromes Mielodisplásicas/epidemiologia , Adolescente , Agamaglobulinemia/epidemiologia , Anemia Aplástica/tratamento farmacológico , Anemia Aplástica/etiologia , Anemia Aplástica/imunologia , Anemia Aplástica/patologia , Soro Antilinfocitário/uso terapêutico , Autoanticorpos/sangue , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/imunologia , Medula Óssea/patologia , Criança , Pré-Escolar , Comorbidade , Complemento C3/deficiência , Complemento C4/deficiência , Ciclosporina/uso terapêutico , Feminino , Rearranjo Gênico da Cadeia delta dos Receptores de Antígenos dos Linfócitos T , Humanos , Hipergamaglobulinemia/epidemiologia , Imunoglobulinas/sangue , Imunossupressores/uso terapêutico , Incidência , Lactente , Contagem de Linfócitos , Subpopulações de Linfócitos/patologia , Masculino , Síndromes Mielodisplásicas/tratamento farmacológico , Síndromes Mielodisplásicas/etiologia , Síndromes Mielodisplásicas/imunologia , Síndromes Mielodisplásicas/patologia , Estudos Retrospectivos , Fatores de TempoRESUMO
El síndrome de Hiper EgE (SHIE) es una rara inmunodeficiencia caracterizada por abscesos cutáneos, neumonías recurrentes, conformación de neumatoceles y elevados niveles de IgE en suero. Se ha reconocido la asociación de rasgos faciales, esqueléticos y dentales, pero su frecuencia es poco conocida. Objetivo: describir la epidemiología, presentación clínica, hallazgos de laboratorio y tratamiento de este síndrome desde el punto de vista médico y odontológico, con énfasis en las manifestaciones estomatológicas y dentales. Métodos. Métodos: realizamos una revisión científica sistemática de todos los reporte y series de casos de SHIE en las bases de datos Lilacs, Medline, SciELO y Biblioteca Cochrane, utilizando como descriptores DeCS/MeSH las palabras claves: hyper IgE AND immunodeficiency, hypereosinophilia, Job´s syndrome, hiperinmunoglobulinemia E. Resultados: revisamos 31 publicaciones con 328 pacientes. Los abscesos cutáneos se encontraron en 89 pior ciento de los casos, la neumonía en el 87 por ciento y la IgE sérica dio una mediana de 3.417 Ul/mL. Los rasgos faciales característicos estuvieron en el 69 por ciento, las alteraciones dentarias en 58 por ciento y la candidiasis oral fue reportada en 53 por ciento de los pacientes. Conclusión: el síndrome de IgE es un desorden multisistémico que afecta a la dentición, el esqueleto, el tejido conectivo y el sistema inmune. Por esto, el odontopediatra debe ser capaz de reconocer el fenotipo orofacial para mejorar la calidad del diagnóstico y brindar el abordaje terapéutico apropiado...
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Humanos , Masculino , Feminino , Hipergamaglobulinemia/complicações , Imunoglobulina E , Manifestações Bucais , Anormalidades Dentárias/etiologia , Hipergamaglobulinemia/epidemiologia , Hipergamaglobulinemia/terapia , Interpretação Estatística de Dados , Síndrome de Job/patologia , Hipergamaglobulinemia/sangueAssuntos
Doenças Autoimunes/imunologia , Imunoglobulina G/sangue , Pancreatite Crônica/imunologia , Ásia/epidemiologia , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/epidemiologia , Doenças Autoimunes/etiologia , Doenças Autoimunes/patologia , Biópsia , Colangite Esclerosante/etiologia , Diagnóstico Diferencial , Hepatite Autoimune , Humanos , Hipergamaglobulinemia/complicações , Hipergamaglobulinemia/epidemiologia , Infiltração de Neutrófilos , Pâncreas/patologia , Neoplasias Pancreáticas/diagnóstico , Pancreatite Crônica/classificação , Pancreatite Crônica/diagnóstico , Pancreatite Crônica/epidemiologia , Pancreatite Crônica/etiologia , Pancreatite Crônica/patologia , Plasmócitos/patologia , EscleroseAssuntos
Doenças Autoimunes/imunologia , Hipergamaglobulinemia/imunologia , Imunoglobulina G/sangue , Doença de Mikulicz/imunologia , Plasmócitos/patologia , Sialadenite/imunologia , Idoso , Doenças Autoimunes/epidemiologia , Doenças Autoimunes/patologia , Diagnóstico Diferencial , Fibrose , Humanos , Hipergamaglobulinemia/diagnóstico , Hipergamaglobulinemia/epidemiologia , Hipergamaglobulinemia/patologia , Hiperplasia , Hipertrofia , Linfócitos/patologia , Doença de Mikulicz/patologia , Neoplasias das Glândulas Salivares/diagnóstico , Glândulas Salivares/patologia , Esclerose , Sialadenite/diagnóstico , Sialadenite/epidemiologia , Sialadenite/patologia , Síndrome de Sjogren/diagnósticoRESUMO
OBJECTIVE: To study the prevalence of extraglandular manifestations in primary Sjögren's syndrome (SS) among participants enrolled in the Sjögren's International Collaborative Clinical Alliance (SICCA) Registry. METHODS: A total of 1,927 participants in the SICCA registry were studied, including 886 participants who met the 2002 American-European Consensus Group (AECG) criteria for primary SS, 830 "intermediate" cases who had some objective findings of primary SS but did not meet AECG criteria, and 211 control individuals. We studied the prevalence of immunologic and hematologic laboratory abnormalities, specific rheumatologic examination findings, and physician-confirmed thyroid, liver, and kidney disease, as well as lymphoma among SICCA participants. RESULTS: Laboratory abnormalities, including hematologic abnormalities, hypergammaglobulinemia, and hypocomplementemia, frequently occurred among primary SS cases and were more common among the intermediate cases than among control participants. Cutaneous vasculitis and lymphadenopathy were also more common among primary SS cases. In contrast, the frequency of physician-confirmed diagnoses of thyroid, liver, and kidney disease and lymphoma was low and only primary biliary cirrhosis was associated with primary SS case status. Rheumatologic and neurologic symptoms were common among all SICCA participants, regardless of case status. CONCLUSION: Data from the international SICCA registry support the systemic nature of primary SS, manifested primarily in terms of specific immunologic and hematologic abnormalities. The occurrence of other systemic disorders among this cohort is relatively uncommon. Previously reported associations may be more specific to select patient subgroups, such as those referred for evaluation of certain neurologic, rheumatologic, or other systemic manifestations.
Assuntos
Hipergamaglobulinemia/epidemiologia , Doenças Linfáticas/epidemiologia , Síndrome de Sjogren/epidemiologia , Vasculite/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , América/epidemiologia , Ásia/epidemiologia , Comorbidade , Europa (Continente)/epidemiologia , Feminino , Humanos , Agências Internacionais , Masculino , Pessoa de Meia-Idade , Prevalência , Sistema de RegistrosRESUMO
BACKGROUND: Undifferentiated arthritis (UA) comprises arthritis not yet identifiable as a specific rheumatic disease. Few reports exist on the natural course of UA in Thai patients. OBJECTIVE: To study the clinical features and natural course of UA in Thai patients. METHOD: A retrospective, analytical study was performed among Thai patients diagnosed with UA seen at Srinagarind Hospital, Khon Kaen, Thailand, between January 2002 and December 2007. RESULTS: The medical records of 95 UA patients were reviewed. The mean age at onset was 40.7 ± 14.7 years (range, 15-78). The female:male ratio was 1.25 : 1.00. Common presentations included asymmetrical oligoarthritis followed by polyarthritis. The knee was the most commonly affected joint, followed by the wrist and ankle. Complete remission occurred within 6 months of onset in 4.2% of cases. A diagnosis was specified in 29 patients (30.5%) during the follow-up period (which averaged 17.1 ± 24.0 months [range, 6-84]), including reactive arthritis (in 9 patients), undifferentiated spondyloarthropathy (7), rheumatoid arthritis (6), psoriatic arthritis (4), ankylosing spondylitis (1), gout (1) and unclassified connective tissue disease (1). UA was the default diagnosis for 66 patients (69.5%) after 24 months of follow-up. Hyperglobulinemia was correlated with persistent arthritis (i.e., > 6 months, P = 0.045). The only predictive factor for RA development was old-age at onset (P = 0.038). CONCLUSION: The most common presentation of Thai UA was asymmetrical oligoarthritis and most patients had persistent arthritis correlated with hyperglobulinemia. Elderly-onset, without any radiographic changes or rheumatoid factor, was predictive of RA development during follow-up.
Assuntos
Artrite/patologia , Articulações/patologia , Adolescente , Adulto , Idade de Início , Idoso , Artrite/epidemiologia , Artrite/fisiopatologia , Feminino , Humanos , Hipergamaglobulinemia/epidemiologia , Hipergamaglobulinemia/patologia , Hipergamaglobulinemia/fisiopatologia , Articulações/fisiopatologia , Masculino , Pessoa de Meia-Idade , Remissão Espontânea , Estudos Retrospectivos , Tailândia/epidemiologia , Adulto JovemRESUMO
Chronic antigenic stimulation is associated with hypergamma-globulinemia. Higher rates of hypergamma-globulinemia in tropical populations are maintained even with migration to temperate regions. We conducted a population-based screening study to assess the prevalence and risk factors for hypergamma-globulinemia in Ghana, Africa. 917 Ghanaian males (50-74 years) underwent in-person interviews and health examinations. Serum from all persons was analyzed by electrophoresis performed on agarose gel; serum with a discrete/localized band was subjected to immunofixation. 54 persons with monoclonal proteins were excluded and 17 samples were insufficient for analysis. Using logistic regression and Chi-square statistics we analyzed patterns of hypergamma-globulinemia. Among 846 study subjects, the median γ-globulin level was 1.86 g/dL. On the basis of a U.S. reference, 616 (73%) had hypergamma-globulinemia (>1.6 g/dL) and 178 (21%) had γ-globulin levels >2.17 gm/dl. On multivariate analyses, lower education status (P = 0.0013) and never smoking (P = 0.038) were associated with increased γ-globulin levels. Self-reported history of syphilis was associated with hypergamma-globulinemia. We conclude that three quarters of this population-based adult Ghanaian male sample had hypergamma-globulinemia with γ-globulin levels >1.6 g/dL. Future studies are needed to uncover genetic and environmental underpinnings of our finding, and to define the relationship between hypergamma-globulinemia, monoclonal gammopathy of undetermined significance (MGUS), and multiple myeloma.
Assuntos
Hipergamaglobulinemia/sangue , Hipergamaglobulinemia/epidemiologia , Imunoglobulina G/sangue , Adulto , Idoso , Gana/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Análise MultivariadaRESUMO
Autoinflammatory syndromes are characterised by recurrent or persistent inflammation with no increase in the antibody titers or antigen-specific T lymphocytes, and absence of infection. Initially, they included the hereditary periodic fever syndromes, a group of innate immune system monogenic diseases characterised by recurrent febrile episodes, with different characteristics, duration and interval, accompanied by other symptoms. Secondary amyloidosis is a complication in this group. The advances in the last few years has led to the identification of susceptible genes, new proteins, and characterising mechanisms and pathogenic routes that have led to an improvement in the diagnosis and establishing more effective treatments. Among these routes, are the changes in the inflammasome components, a group of cytoplasmic proteins that regulate the production of several inflammatory response mediators. The initial group of monogenic autoinflammatory diseases have increased in the last few years, due to including several polygenic hereditary diseases.
Assuntos
Doenças Autoimunes/classificação , Doenças Hereditárias Autoinflamatórias , Inflamassomos/fisiologia , Inflamação/classificação , Amiloidose/etiologia , Doenças Autoimunes/imunologia , Doenças Autoimunes/fisiopatologia , Proteínas do Sistema Complemento/fisiologia , Citocinas/fisiologia , Proteínas do Citoesqueleto/genética , Febre Familiar do Mediterrâneo/diagnóstico , Febre Familiar do Mediterrâneo/epidemiologia , Febre Familiar do Mediterrâneo/genética , Febre Familiar do Mediterrâneo/fisiopatologia , Doenças Hereditárias Autoinflamatórias/classificação , Doenças Hereditárias Autoinflamatórias/complicações , Humanos , Hipergamaglobulinemia/epidemiologia , Hipergamaglobulinemia/genética , Imunidade Inata , Imunoglobulina D , Inflamassomos/antagonistas & inibidores , Inflamassomos/genética , Inflamação/imunologia , Inflamação/fisiopatologia , Interleucina-1/fisiologia , Peptídeos e Proteínas de Sinalização Intracelular/genética , Ativação de Macrófagos , NF-kappa B/fisiologia , Fosfotransferases (Aceptor do Grupo Álcool)/genética , Pirina , Receptores Tipo I de Fatores de Necrose Tumoral/genéticaRESUMO
OBJECTIVE: A proliferation-inducing ligand (APRIL) is a new member of the tumour necrosis factor family which is intimately connected to the regulation of cellular pathways. The aim of this study was to assess serum concentrations of APRIL in systemic sclerosis patients, and to correlate them with the main clinical and serological features of the disease. METHODS: Sera from 35 patients with systemic sclerosis, 25 had limited cutaneous and 10 had diffuse cutaneous subtypes, and 35 normal healthy subjects were assayed for APRIL by Enzyme Linked Immunosorbant Assay. Demographic, clinical, autoantibodies and serological data were prospectively assessed. RESULTS: Serum APRIL concentrations were higher in patients with systemic sclerosis and in both its subtypes compared to the healthy controls (p<0.0001 in all). Patients with elevated APRIL levels had significantly higher incidences of myositis than those with normal levels (p=0.04). We did not find significant differences in other organ involvement prevalence between systemic sclerosis patients with elevated vs. normal APRIL levels. In addition, the frequencies of autoantibodies (i.e., anti-topoisomerase I, anti-centromere) were comparable between both groups. Serum APRIL levels were correlated with serum γ-globulins concentrations (r=0.404, p=0.016) but not with C-reactive protein, skin score, nor pulmonary functions. Serum APRIL was also correlated with creatine kinase levels only in systemic sclerosis patients with myositis (r=0.786, p=0.02). CONCLUSION: Our preliminary results suggest increased serum APRIL levels in systemic sclerosis patients, particularly in those associated with myositis and hypergammaglobinemia. To confirm our results, we propose that larger scale, multicentre studies with longer evaluation periods are needed.
Assuntos
Miosite/metabolismo , Escleroderma Sistêmico/metabolismo , Membro 13 da Superfamília de Ligantes de Fatores de Necrose Tumoral/sangue , Adulto , Idoso , Proteína C-Reativa/metabolismo , Humanos , Hipergamaglobulinemia/epidemiologia , Hipergamaglobulinemia/imunologia , Hipergamaglobulinemia/metabolismo , Incidência , Pneumopatias/epidemiologia , Pneumopatias/imunologia , Pneumopatias/metabolismo , Pessoa de Meia-Idade , Miosite/epidemiologia , Miosite/imunologia , Prevalência , Estudos Prospectivos , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/imunologia , Membro 13 da Superfamília de Ligantes de Fatores de Necrose Tumoral/imunologia , Adulto Jovem , gama-Globulinas/metabolismoRESUMO
The diverse hematologic manifestations of primary Sjögren syndrome (pSS) have not been systematically investigated, and their prognostic relevance remains unclear. We conducted a retrospective study of 536 consecutive patients followed in our institution to assess the prevalence of hematologic abnormalities and their associations with various disease manifestations in pSS. We also aimed to identify risk factors for the development of non-Hodgkin lymphoma (NHL) overall and by subtype. Anemia of chronic disease and hypergammaglobulinemia were the most prevalent hematologic manifestations encountered at diagnosis and during the course of pSS. Univariate analysis between cytopenias and glandular manifestations revealed a statistically significant correlation between lymphocytopenia and parotid gland enlargement (p = 0.002), as well as between neutropenia and xerostomia (p = 0.019). Anemia, lymphocytopenia, thrombocytopenia, hypergammaglobulinemia, the presence of monoclonal serum proteins, and cryoglobulinemia correlated significantly with the presence of extraglandular symptoms such as palpable purpura, lymphadenopathy, and splenomegaly. Lymphoma was diagnosed in 7.5% (95% confidence interval [CI], 5.4%-10%) of patients. Marginal zone B-cell lymphomas (MZBCLs) were the predominant histologic type (65%; 95% CI, 48.3%-79.4%), while diffuse large B-cell lymphomas (DLBCLs) accounted for 17.5% (95% CI, 7.3%-32.8%) of all cases. The development of NHL in patients with pSS could be predicted by the presence of simple clinical and laboratory factors at diagnosis: neutropenia (p = 0.041), cryoglobulinemia (p = 0.008), splenomegaly (p = 0.006), lymphadenopathy (p = 0.021), and low C4 levels (p = 0.009). Patients carrying any of these factors had a more than 5-fold increased risk of NHL compared to patients with no risk factors at all. The above set of disease characteristics could predict subsequent development of MZBCL; the presence of lymphocytopenia (p = 0.044) at diagnosis served as a risk factor for the development of a non-MZBCL, most commonly DLBCL. Anemia of chronic disease and hypergammaglobulinemia are common hematologic manifestations at diagnosis and during the course of pSS. Neutropenia and cryoglobulinemia at diagnosis are significantly associated with an increased risk of lymphoma development.