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1.
BJOG ; 127(8): 941-949, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32128978

RESUMO

OBJECTIVE: This study assessed prevalence of connective tissue disease (CTDs), systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS) and antiphospholipid antibodies (aPL) in women with previous adverse pregnancy outcome compared with uncomplicated livebirths. DESIGN: Retrospective case-control study. SETTING: UK Primary Care. POPULATION OR SAMPLE: Records of women, 18 years and older, within the Clinical Practice Research Datalink (CPRD) (1 January 2000-31 December 2013). METHODS: Clinical Practice Research Datalink was searched for pregnancy terms to identify adverse pregnancy outcome. Each identified case was matched to five livebirths. MAIN OUTCOME MEASURES: Diagnosis of SLE, CTD, APS or autoimmune antibodies. Poisson regression was performed to calculate relative risk ratios (RR), comparing adverse pregnancy outcome with livebirth cohorts. RESULTS: Clinical Practice Research Datalink identified 20 123 adverse pregnancy outcomes matched to 97 323 livebirths, with a total of 875 590 person-years follow up. Median follow up from study entry was 7.29 years (SD 4.39). Compared with women with an uncomplicated livebirth, women with adverse pregnancy outcome had an increased risk of developing CTD or autoimmune antibodies (RR 3.20, 95% CI 2.90-3.51). Risk was greatest following a stillbirth (RR 5.82, 95% CI 4.97-6.81). For CTD and SLE, the risk was greatest within the first 5 years of adverse pregnancy outcome. Risk for aPL and APS diagnosis was highest ≥5 years from adverse pregnancy outcome. CONCLUSIONS: Adverse pregnancy outcome is associated with increased risk of developing maternal CTD, including SLE. Either immunological factors predispose women to adverse pregnancy outcome and subsequent CTD diagnosis or, alternatively, adverse pregnancy outcome initiates autoimmune events which culminate in CTD in later life. TWEETABLE ABSTRACT: Stillbirth is associated with increased maternal risk of developing systemic lupus erythematosus (SLE).


Assuntos
Doenças do Tecido Conjuntivo/epidemiologia , Suscetibilidade a Doenças/epidemiologia , Complicações na Gravidez/epidemiologia , Adulto , Estudos de Casos e Controles , Doenças do Tecido Conjuntivo/etiologia , Doenças do Tecido Conjuntivo/fisiopatologia , Suscetibilidade a Doenças/imunologia , Feminino , Humanos , Razão de Chances , Gravidez , Complicações na Gravidez/imunologia , Complicações na Gravidez/fisiopatologia , Resultado da Gravidez , Prevalência , Estudos Retrospectivos , Natimorto , Reino Unido/epidemiologia
2.
Ann Rheum Dis ; 79(5): 626-634, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32161055

RESUMO

OBJECTIVES: To evaluate initial combination therapy with ambrisentan plus tadalafil (COMB) compared with monotherapy of either agent (MONO), and the utility of baseline characteristics and risk stratification in predicting outcomes, in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) and the systemic sclerosis (SSc)-pulmonary arterial hypertension (PAH) subpopulation. METHODS: This post hoc analysis of the Ambrisentan and Tadalafil in Patients with Pulmonary Arterial Hypertension (AMBITION) study included patients with CTD-PAH from the modified intention-to-treat population. Time to clinical failure (TtCF) was assessed by baseline characteristics, treatment assignment and risk group (low, intermediate and high) at baseline and week 16. TtCF was compared between groups using Kaplan-Meier curves and Cox proportional hazards regression modelling. RESULTS: The analysis included 216 patients (COMB, n=117; MONO, n=99). The risk of clinical failure was lower with COMB versus MONO (risk reduction: CTD-PAH 51.7%, SSc-PAH 53.7%), particularly in patients with haemodynamic parameters characteristic of typical PAH without features of left heart disease and/or restrictive lung disease at baseline. The risk of clinical failure was lower with COMB versus MONO in the baseline low-risk group (HR not calculated due to no events in COMB), baseline intermediate-risk group (HR 0.519, 95% CI 0.297 to 0.905) and in the week 16 low-risk group (HR 0.069, 95% CI 0.009 to 0.548). CONCLUSIONS: The benefit of COMB over MONO was demonstrated in patients with CTD-PAH, particularly in those with typical PAH haemodynamic characteristics at baseline. COMB is appropriate for patients categorised as low risk and intermediate risk at baseline and low risk at follow-up. TRIAL REGISTRATION NUMBER: NCT01178073.


Assuntos
Fenilpropionatos/administração & dosagem , Hipertensão Arterial Pulmonar/tratamento farmacológico , Hipertensão Arterial Pulmonar/epidemiologia , Piridazinas/administração & dosagem , Escleroderma Sistêmico/tratamento farmacológico , Escleroderma Sistêmico/epidemiologia , Tadalafila/administração & dosagem , Adulto , Comorbidade , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/tratamento farmacológico , Doenças do Tecido Conjuntivo/epidemiologia , Relação Dose-Resposta a Droga , Método Duplo-Cego , Esquema de Medicação , Quimioterapia Combinada , Feminino , Seguimentos , Humanos , Análise de Intenção de Tratamento , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Hipertensão Arterial Pulmonar/diagnóstico , Medição de Risco , Escleroderma Sistêmico/diagnóstico , Resultado do Tratamento , Vasodilatadores/administração & dosagem
3.
BMC Med Genet ; 21(1): 27, 2020 02 10.
Artigo em Inglês | MEDLINE | ID: mdl-32039712

RESUMO

BACKGROUND: Stickler syndrome is the most common genetic cause of rhegmatogenous retinal detachment (RRD) in children, and has a high risk of blindness. Type I (STL1) is the most common subtype, caused by COL2A1 mutations. This study aims to analyze the mutation spectrum of COL2A1 and further elucidate the genotype-phenotype relationships in the East Asian populations with STL1, which is poorly studied at present. METHODS: By searching MEDLINE, Web of Science, CNKI, Wanfang Data, HGMD and Clinvar, all publications associated with STL1 were collected. Then, they were carefully screened to obtain all reported STL1-related variants in COL2A1 and clinical features in East Asian patients with STL1. RESULTS: There were 274 COL2A1 variants identified in 999 patients with STL1 from 466 unrelated families, and more than half of them were truncation mutations. Of the 107 STL1 patients reported in the East Asian population, it was found that patients with truncation mutations had milder systemic phenotypes, whereas patients with splicing mutations had severer phenotypes. In addition, several recurrent variants (c.3106C > T, c.1833 + 1G > A, c.2710C > T and c.1693C > T) were found. CONCLUSIONS: Genotype-phenotype correlations should certainly be studied carefully, contributed to making personalized follow-up plans and predicting prognosis of this disorder. Genome editing holds great potential for treating inherited diseases caused by pathogenic mutations. In this study, several recurrent variants were found, providing potential candidate targets for genetic manipulation in the future.


Assuntos
Artrite/genética , Doenças do Tecido Conjuntivo/genética , Análise Mutacional de DNA , Perda Auditiva Neurossensorial/genética , Mutação/genética , Descolamento Retiniano/genética , Artrite/epidemiologia , Artrite/patologia , Doenças do Tecido Conjuntivo/epidemiologia , Doenças do Tecido Conjuntivo/patologia , Oftalmopatias Hereditárias , Estudos de Associação Genética , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/patologia , Humanos , Fenótipo , Descolamento Retiniano/epidemiologia , Descolamento Retiniano/patologia
4.
Am J Ophthalmol ; 209: 55-61, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31526795

RESUMO

PURPOSE: Sagging eye syndrome (SES), horizontal and/or vertical strabismus caused by orbital connective tissue degeneration, was first defined 10 years ago. This study investigated SES and other causes of acquired binocular diplopia in adults presenting to a single institution since the description of SES. DESIGN: Retrospective observational case series. METHODS: Medical records were reviewed of all new patients over the age of 40 who presented to the Stein Eye Institute with binocular diplopia between January 2015 and December 2018. Clinical causes of diplopia were tabulated in patients grouped by age and sex. In patients with SES, we tabulated binocular alignment, types of treatment, and surgical outcomes. RESULTS: There were 945 patients of mean age 66.5 years, of whom 514 (54.4%) were female. The most common cause of diplopia was SES (31.4%). The 297 patients with SES were older at 71.2 years (P < 0.0001) and more predominantly female at 59.9% than other patients (52.0%; P = 0.023). The relative proportion of SES patients among all diplopic patients increased with age from 4.7% under age 50 years to 60.9% over the age of 90. Age-related distance esotropia was present in 35% and cyclovertical strabismus in 65% of cases of SES. Strabismus surgery was performed in 50% of cases of SES. Mean esotropia at distance decreased from 6.9 ± 0.7Δ preoperatively to 0.3 ± 0.3Δ postoperatively. Preoperative hypertropia decreased from 3.0 ± 0.3Δ to 0.7 ± 0.2Δ postoperatively. Surgery resolved diplopia in all cases. CONCLUSIONS: It is important to recognize that SES is a very common cause of adult binocular diplopia.


Assuntos
Doenças do Tecido Conjuntivo/epidemiologia , Diplopia/epidemiologia , Doenças Orbitárias/epidemiologia , Estrabismo/epidemiologia , Visão Binocular/fisiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Envelhecimento/fisiologia , Doenças do Tecido Conjuntivo/fisiopatologia , Doenças do Tecido Conjuntivo/cirurgia , Diplopia/fisiopatologia , Diplopia/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculos Oculomotores/fisiopatologia , Músculos Oculomotores/cirurgia , Procedimentos Cirúrgicos Oftalmológicos , Doenças Orbitárias/fisiopatologia , Doenças Orbitárias/cirurgia , Prevalência , Estudos Retrospectivos , Estrabismo/fisiopatologia , Estrabismo/cirurgia , Síndrome
5.
BMC Pulm Med ; 19(1): 215, 2019 Nov 14.
Artigo em Inglês | MEDLINE | ID: mdl-31727051

RESUMO

BACKGROUND: Acute exacerbation (AE) is the major cause of morbidity and mortality in patients with idiopathic pulmonary fibrosis (IPF). AEs also occur in other forms of fibrosing interstitial lung disease (fILD). The clinical features and prognosis of AE patients with connective tissue diseases (CTDs) associated-ILD has not been fully described. METHODS: We retrospectively reviewed 177 patients with either IPF or a characterized CTD-ILD admitted to Nanjing Drum Tower Hospital with an AE from January 2010 to December 2016. RESULTS: The study cohort included 107 subjects with AE-IPF and 70 cases with AE-CTD-ILD. Female gender, prior use of corticosteroid and immunosupressants, lower serum albumin, higher D-dimer level, TLC% pred, survival, and treatment with immunosupressants and caspofungin were more common in the CTD-ILD group (all p<0.05). The incidences of AE-CTD-ILD and AE-IPF were similar in our single center (p = 0.526). TLC% pred was the risk factor for AE after ILD diagnosis for 1 year in CTD patients (p = 0.018). Log-rank tests showed patients with CTD-ILD had a significantly lower mortality rate compared with IPF patients after AEs (p = 0.029). No significant difference in survival was noted among CTD subgroups (p = 0.353). The survival was negatively correlated with WBC count, LDH and CT score, (p = 0.006, p = 0.013 and p = 0.035, respectively), and positively correlated with PaO2/FiO2 ratio (p<0.001) in the CTD-ILD group. WBC count and PO2/FiO2 ratio were the independent predictors for survival in AE-CTD-ILD after adjusting for other clinical variates in Cox regression Models (p = 0.038 and p < 0.001, respectively). CONCLUSIONS: The clinical characteristics of patients with AE-CTD-ILD differed from those with AE-IPF, while AE incidences were similar between the two groups. Subjects with AE-CTD-fILD tended to have a better prognosis, and WBC count and PO2/FiO2 ratio were the independent survival predictors for these patients.


Assuntos
Doenças do Tecido Conjuntivo/epidemiologia , Fibrose Pulmonar Idiopática/epidemiologia , Doenças Pulmonares Intersticiais/epidemiologia , Oxigênio/sangue , Doença Aguda , Idoso , China , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/terapia , Progressão da Doença , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Incidência , Contagem de Leucócitos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/terapia , Masculino , Prognóstico , Testes de Função Respiratória , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida , Tomografia Computadorizada por Raios X
6.
BMC Res Notes ; 12(1): 747, 2019 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-31730479

RESUMO

OBJECTIVES: The association between post-traumatic stress disorder (PTSD) and medical comorbidities is controversial since most studies focused on specific comorbidity and victim types. In Italy, data on this issue are scarce. A comprehensive evaluation of all the ICD medical categories co-occurring in PTSD may orient assessment and treatment during clinical and forensic practice. This is the first study evaluating all the ICD physical comorbidities and gender-related differences in Italian PTSD patients. Eighty-four PTSD patients (36 females, 48 males) were included. The Clinician-Administered PTSD Scale, Mini International Neuropsychiatric Interview and Davidson Trauma Scale were administered. RESULTS: Most patients had a PTSD consequent to an accident and half of them presented extreme symptom severity. No gender differences emerged on symptom severity/duration and age at the event. Metabolic (39.29%), circulatory (20.24%) and musculoskeletal systems/connective tissue diseases (17.86%) were the most frequent comorbidities. Metabolic/circulatory diseases were more frequent among males (p = 0.019 and p = 0.027, respectively) while females more frequently showed neoplasms (p = 0.039). Physical comorbidities represent a serious complication in PTSD patients and are more prevalent than in the Italian population. While gender is not associated with symptom presentation, it seems to play a key role in specific comorbidities including metabolic, circulatory and neoplastic diseases.


Assuntos
Doenças Cardiovasculares/epidemiologia , Doenças do Tecido Conjuntivo/epidemiologia , Doenças Metabólicas/epidemiologia , Doenças Musculoesqueléticas/epidemiologia , Transtornos de Estresse Pós-Traumáticos/epidemiologia , Adulto , Idoso , Doenças Cardiovasculares/diagnóstico , Doenças Cardiovasculares/fisiopatologia , Comorbidade , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/fisiopatologia , Feminino , Humanos , Classificação Internacional de Doenças , Itália/epidemiologia , Masculino , Doenças Metabólicas/diagnóstico , Doenças Metabólicas/fisiopatologia , Pessoa de Meia-Idade , Doenças Musculoesqueléticas/diagnóstico , Doenças Musculoesqueléticas/fisiopatologia , Prevalência , Escalas de Graduação Psiquiátrica , Índice de Gravidade de Doença , Fatores Sexuais , Transtornos de Estresse Pós-Traumáticos/diagnóstico , Transtornos de Estresse Pós-Traumáticos/fisiopatologia
8.
Rheumatol Int ; 39(10): 1777-1781, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31385080

RESUMO

We sought to determine the prevalence of additional connective tissue diseases (CTDs) in patients with idiopathic inflammatory myopathies (IIM), and to study the muscle biopsy patterns in various clinico-serologic subsets of myositis. We undertook a retrospective cohort study of 648 patients with a histological diagnosis of IIM. The following was determined from the South Australian Myositis Database: presence of associated CTDs, histological details and presence of myositis-specific (MSA) or myositis-associated (MAA) antibodies. Among patients with IIM, a significantly greater proportion had systemic sclerosis 32/648 (4.9%) than mixed connective tissue disease (12/648, p = 0.003), primary Sjogren's syndrome (12/648, p = 0.003), systemic lupus erythematosus (10/648, p < 0.001) or rheumatoid arthritis (6/648, p = 0.0001). Polymyositis was the most common IIM diagnosis regardless of the presence or absence of CTD. MSA/MAA was more commonly detected in those with systemic sclerosis than those with IIM alone (OR 5.35, p < 0.005). The higher prevalence of SSc (compared with other CTDs) in IIM, together with the more frequent detection of autoantibodies in this group, suggests that these conditions may be linked.


Assuntos
Doenças do Tecido Conjuntivo/epidemiologia , Miosite/epidemiologia , Escleroderma Sistêmico/epidemiologia , Autoanticorpos/sangue , Biomarcadores/sangue , Biópsia , Doenças do Tecido Conjuntivo/sangue , Doenças do Tecido Conjuntivo/diagnóstico , Bases de Dados Factuais , Humanos , Miosite/sangue , Miosite/diagnóstico , Prevalência , Estudos Retrospectivos , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/diagnóstico , Austrália do Sul/epidemiologia
9.
Clin Respir J ; 13(11): 683-692, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31364275

RESUMO

INTRODUCTION: Fever of unknown origin (FUO) with pulmonary lesions is a common syndrome in respiratory diseases, which can be caused by infection, cancer, connective tissue disease and other rare diseases of South China. In patients with FUO associated with pulmonary lesions, pathogeny should be identified and followed by an appropriate treatment strategy. OBJECTIVE: This study aimed to investigate the etiological distribution and clinical features of FUO with pulmonary lesions and to analyze the efficiency of different diagnostic methods for certain disease categories. METHODS: Patients hospitalized at the Guangzhou Institute of Respiratory Health from July 2012 to December 2016 who had fever ≥38.3°C that lasted ≥21 days, in whom the chest X-ray or computed tomography (CT) revealed definite pulmonary infiltration, and for whom, despite being examined for a week, no definitive diagnosis could be made, were considered for this study. RESULTS: A total of 104 patients were identified as having FUO with lung lesions, and 89.4% (93/104) patients were definitively diagnosed. Infectious disease was the most common cause (46.2%, 48/104). Histopathology was instrumental in the diagnosis of the causes of FUO with pulmonary manifestations, 47.3% (44/93) patients were diagnosed through histopathology, 35.4% (17/48) with infectious disease and 55.2% (16/29) with connective tissue diseases as the etiology were definitely diagnosed using histopathology. CONCLUSION: Most FUO with pulmonary lesions are identified during infections and autoimmune diseases. The most important diagnostic measure for FUO with pulmonary lesions is histopathology. Additionally, lung biopsy must be encouraged in multi-level hospitals in the future.


Assuntos
Febre de Causa Desconhecida/diagnóstico , Febre de Causa Desconhecida/etiologia , Pulmão/patologia , Doenças Respiratórias/complicações , Adulto , Doenças Autoimunes/complicações , Doenças Autoimunes/epidemiologia , Biópsia , China/epidemiologia , Doenças Transmissíveis/complicações , Doenças Transmissíveis/epidemiologia , Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/epidemiologia , Estudos Transversais , Feminino , Febre de Causa Desconhecida/epidemiologia , Hospitalização , Humanos , Pulmão/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Neoplasias/complicações , Neoplasias/epidemiologia , Radiografia Torácica/métodos , Doenças Respiratórias/diagnóstico por imagem , Doenças Respiratórias/epidemiologia , Doenças Respiratórias/patologia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos
10.
Int J Rheum Dis ; 22(9): 1741-1745, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31328423

RESUMO

BACKGROUND: Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is associated with high mortality, but there is limited clinical data on AE of interstitial lung disease (ILD) in connective tissue disease-associated ILD (CTD ILD). The present study was conducted to provide prevalence and clinical features of AE, as well as various risk factors associated with mortality among AE CTD ILD patients. METHODS: Between May 2013 and April 2018, 15 patients who developed AE among 105 consecutive patients with CTD with chronic ILD were included. AE was defined using the criteria recently proposed by the IPF net, with slight modification for adaptation to CVD-IP6 (collagen vascular disease-associated interstitial pneumonia), and patients having CTD with AE met all the criteria. RESULTS: Fifteen patients with mean age of 45.8 ± 13.9 years developed AE; the most common subgroup (n = 5, 33%) was systemic sclerosis. The mean duration (months) between diagnosis of ILD and AE was 56.5 ± 38.0 with mean follow-up duration of 24 ± 18.1 months. The baseline arterial oxygen pressure (PaO2 ) was 81.7 ± 8.1 mm Hg and mean forced vital capacity (%) was 57.9 ± 8.9. Five patients requiring mechanical ventilation died. Patients with shorter duration (months) of disease between onset of ILD to AE had higher mortality, 40.4 ± 45.1 vs 64.6 ± 33.6. Those who had significantly lower baseline PaO2 (mean ± SD), 72.6 ± 3.4 vs 86.2 ± 5.3 mm Hg (P = .002) had higher mortality. CONCLUSIONS: In our study, the majority of patients with AE CTD ILD had systemic sclerosis. Patients with lower baseline PaO2 and those requiring mechanical ventilation had higher mortality.


Assuntos
Doenças do Tecido Conjuntivo/epidemiologia , Fibrose Pulmonar Idiopática/epidemiologia , Doenças Pulmonares Intersticiais/epidemiologia , Adulto , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/mortalidade , Doenças do Tecido Conjuntivo/terapia , Progressão da Doença , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/terapia , Imunossupressores/uso terapêutico , Índia/epidemiologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/terapia , Masculino , Pessoa de Meia-Idade , Prevalência , Prognóstico , Estudos Prospectivos , Respiração Artificial , Medição de Risco , Fatores de Risco , Fatores de Tempo
11.
Pan Afr Med J ; 32: 181, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31312295

RESUMO

Introduction: The term anti-nuclear antibody (ANA) is used to define a large group of autoantibodies which specifically bind to nuclear elements. Although healthy individuals may also have ANA positivity, the measurement of ANA is generally used in the diagnosis of autoimmune disorders. However, various studies have shown that ANA testing may be overused, especially in pediatrics clinics. Our aim was to investigate the reasons for antinuclear antibody (ANA) testing in the general pediatrics and pediatric rheumatology clinics of our hospital and to determine whether ANA testing was ordered appropriately by evaluating chief complaints and the ultimate diagnoses of these cases. Methods: The medical records of pediatric patients in whom ANA testing was performed between January 2014 and June 2016 were retrospectively evaluated. Subjects were grouped according to the indication for ANA testing and ANA titers. Results: ANA tests were ordered in a total of 409 patients during the study period, with 113 positive ANA results. The ANA test was ordered mostly due to joint pain (50% of the study population). There was an increased likelihood of autoimmune rheumatic diseases (ARDs) with higher ANA titer. The positive predictive value of an ANA test was 16% for any connective tissue disease and 13% for lupus in the pediatric setting. Conclusion: in the current study, more than one-fourth of the subjects were found to have ANA positivity, while only 15% were ultimately diagnosed with ARDs. Our findings underline the importance of an increased awareness of correct indications for ANA testing.


Assuntos
Anticorpos Antinucleares/sangue , Doenças Autoimunes/diagnóstico , Doenças do Tecido Conjuntivo/diagnóstico , Doenças Reumáticas/diagnóstico , Adolescente , Instituições de Assistência Ambulatorial , Doenças Autoimunes/epidemiologia , Doenças Autoimunes/imunologia , Criança , Pré-Escolar , Doenças do Tecido Conjuntivo/epidemiologia , Doenças do Tecido Conjuntivo/imunologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Valor Preditivo dos Testes , Estudos Retrospectivos , Doenças Reumáticas/epidemiologia , Doenças Reumáticas/imunologia , Turquia/epidemiologia
12.
Pathology ; 51(5): 518-523, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31230817

RESUMO

The role of autoantibody testing for patients with interstitial lung disease is an evolving area. Recent guidelines recommend routine anti-nuclear antibodies, rheumatoid factor, and anti-citrullinated cyclic peptide antibody testing for patients undergoing diagnostic evaluation for interstitial lung disease, with further autoantibody testing reserved for selected cases guided by rheumatological features. Even this approach may miss patients with clinically significant autoantibodies when interstitial lung disease is the dominant or first manifestation of autoimmune disease. We retrospectively performed autoimmune serology in a clinically well characterised cohort of interstitial lung disease patients. Using stored serum, additional testing was performed to ensure all patients had complete autoantibody profiles including anti-nuclear antibodies, extractable nuclear antigen antibodies, double-stranded DNA antibodies, rheumatoid factor, anti-citrullinated cyclic peptide antibodies, anti-neutrophil cytoplasmic antibodies, and myositis antibodies. Eighty patients with interstitial lung disease, and available stored serum, were assessed. Mean age at interstitial lung disease diagnosis was 65.2 years and 42 patients were male. Positive autoimmune serology was found in 56 of 80 (70.0%) patients; the most common positive result was anti-nuclear antibodies (n=34; 42.5%). Myositis antibodies were detected in 13 of 80 (16.2%) patients. Four (5%) patients had elevated anti-citrullinated cyclic peptide antibodies, and two (2.5%) patients had detectable myeloperoxidase antibodies. Eleven (13.7%) patients with negative anti-nuclear antibodies had other significant disease associated autoantibodies. An extended panel of autoantibody testing may detect cases of connective tissue disease associated interstitial lung disease, regardless of clinical or radiological subtype, and prior to extra-pulmonary manifestations of systemic autoimmunity.


Assuntos
Autoanticorpos/sangue , Doenças do Tecido Conjuntivo/complicações , Doenças Pulmonares Intersticiais/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Doenças Autoimunes/complicações , Doenças Autoimunes/epidemiologia , Doenças do Tecido Conjuntivo/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
13.
Arthritis Res Ther ; 21(1): 93, 2019 04 11.
Artigo em Inglês | MEDLINE | ID: mdl-30975190

RESUMO

BACKGROUND: Interstitial lung disease (ILD) is a common complication of connective tissue disease (CTD) and a leading cause of morbidity and mortality. There are various lung ultrasound (LUS) scoring systems with different lung intercostal spaces (LIS). The purpose of this meta-analysis was to find a simplified LUS method for the assessment of CTD-ILD. METHODS: We systematically retrieved lung ultrasound diagnostic studies on CTD-ILD in PubMed, Embase, and Web of Science databases. Summary diagnostic accuracy, including sensitivity, specificity, and area under the curve (AUC), was analyzed. Subgroup analysis was conducted according to different LIS and diseases. RESULTS: The 11 studies included in this meta-analysis comprised a total of 487 patients with CTD. The pooled sensitivity and specificity of the LUS were 0.859 (95% confidence interval (CI) 0.812-0.898) and 0.839 (95% CI 0.782-0.886), respectively, illustrating its great value for CTD-ILD diagnosis. In addition, there were six methods to evaluate LIS, including 72, 65, 50, 14, 10, and all LIS. The pooled sensitivity and specificity of 14 LIS were 0.982 (95% CI 0.904-1.000) and 0.875 (95% CI 0.710-0.965), respectively. The pooled positive likelihood ratio (PLR), negative likelihood ratio (NLR), and diagnostic odd ratio (DOR) of 14 LIS were 7.297 (95% CI 6.050-17.45), 0.029 (95% CI 0.006-0.147), and 292.30 (95% CI 35.53-2403.8), respectively. Moreover, the AUC for systemic sclerosis (SSc) and rheumatoid arthritis (RA) was 0.929 and 0.981, respectively; the DOR for SSc and RA was 42.93 (95% CI 17.75-103.79) and 80.24 (95% CI 8.107-796.09), respectively. CONCLUSIONS: We found a modified and simplified method of LUS, by scanning 14 LIS in a short time, which had a very high sensitivity and specificity.


Assuntos
Doenças do Tecido Conjuntivo/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Ultrassonografia/métodos , Área Sob a Curva , Doenças do Tecido Conjuntivo/epidemiologia , Humanos , Doenças Pulmonares Intersticiais/epidemiologia
14.
Respir Med ; 150: 154-160, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30961944

RESUMO

BACKGROUND: The term Interstitial Pneumonia with Autoimmune Features (IPAF) describes patients with Interstitial Lung Diseases (ILDs) and clinical or serological features of autoimmune diseases insufficient to reach a specific classification of a Connective Tissue Disease (CTD). Currently, retrospective studies on IPAF patients have proven to be heterogeneous in general characteristics, outcomes and High-Resolution Computed Tomography (HRCT) pattern. This study aims to describe for the first time the clinical, serological and radiological features of a prospective cohort of IPAF patients. This cohort is then compared to a group of patients with Idiopathic Pulmonary Fibrosis (IPF). MATERIAL AND METHODS: From 626 consecutive ILD patients evaluated, 45 IPAF and a comparison cohort of 143 IPF patients were enrolled. All patients underwent clinical assessment with rheumatologic and respiratory evaluation, HRCT, Pulmonary Function Tests and Nailfold Videocapillaroscopy. RESULTS: The IPAF patients had a predominance of female gender (62.12%) with a median age of 66 years. The most common findings were: Nonspecific Interstitial Pneumonia (NSIP, 68.89%), Antinuclear Antibody positivity (17.77%) and Raynaud Phenomenon (31.11%). In comparison with IPF, IPAF patients showed younger age, better performances in Pulmonary Function Tests, less necessity of O2 support and predominance of female sex and NSIP pattern. DISCUSSION: This is the first report of a prospective cohort of IPAF patients. IPAF patients seem to have a less severe lung disease than IPF. IPAF criteria probably need to be revisited and validated, but their capacity to recruit patients with incomplete forms or early onset of CTD could be useful for further research.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/imunologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/imunologia , Idoso , Anticorpos Antinucleares/imunologia , Doenças Autoimunes/imunologia , Doenças do Tecido Conjuntivo/classificação , Doenças do Tecido Conjuntivo/epidemiologia , Feminino , Humanos , Fibrose Pulmonar Idiopática/sangue , Fibrose Pulmonar Idiopática/fisiopatologia , Doenças Pulmonares Intersticiais/sangue , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Angioscopia Microscópica/métodos , Pessoa de Meia-Idade , Estudos Prospectivos , Radiografia/métodos , Doença de Raynaud/epidemiologia , Testes de Função Respiratória/métodos , Tomografia Computadorizada por Raios X/métodos
15.
Clin Immunol ; 203: 1-8, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30922961

RESUMO

BACKGROUND: We investigated the pattern of reported immune diseases in the international ASIA syndrome registry. METHODS: Data from 500 subjects exposed to adjuvants from the ASIA syndrome international registry were analysed. RESULTS: The patient mean age was 43 ±â€¯17 years and 89% were female. Within the reported immune diseases, 69% were well-defined immune diseases (autoimmune, autoinflammation, and mixed pattern diseases). Among the well-defined immune diseases following the exposure to adjuvants, polygenic autoimmune diseases were significantly higher than autoinflammatory disorders (92.7% vs 5.8%, respectively, p < 0.001). Polygenic autoimmune diseases such as connective tissue diseases were significantly linked to the exposure to HBV vaccine (OR 3.15 [95%CI 1.08-9.23], p = 0.036). Polygenic autoinflammatory diseases were significantly associated with the exposure to influenza vaccination (OR 10.98 [95%CI 3.81-31.67], p < 0.0001). CONCLUSIONS: Immune conditions following vaccination are rare, and among these, polygenic autoimmune diseases represent the vast majority of the well-defined immune diseases reported under the umbrella ASIA syndrome. However, vaccines benefit outweighs their autoimmune side effects.


Assuntos
Doenças Autoimunes/epidemiologia , Doenças do Tecido Conjuntivo/epidemiologia , Arterite de Células Gigantes/epidemiologia , Inflamação/epidemiologia , Vacinação/estatística & dados numéricos , Adjuvantes Imunológicos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Vacinas contra Hepatite B/imunologia , Humanos , Lactente , Recém-Nascido , Vacinas contra Influenza/imunologia , Israel/epidemiologia , Masculino , Pessoa de Meia-Idade , Síndrome , Adulto Jovem
16.
Arthritis Res Ther ; 21(1): 71, 2019 03 11.
Artigo em Inglês | MEDLINE | ID: mdl-30867045

RESUMO

BACKGROUND: A retrospective study was performed to investigate the clinical features and associated factors of invasive mycoses (IM) in patients with connective tissue disease (CTD) from Southern China. METHODS: Demographic and clinical data were recorded. Associated factors were analyzed by logistic regression analysis. RESULTS: A total of 6911 patients with CTD were included. IM was diagnosed in 32 patients (incidence, 0.5%). IM was predominant in patients with ANCA-associated vasculitis (AAV) (incidence, 1.5%, 7/480). Lung was commonly involved (30/32, 93.8%). Aspergillus spp. (81.3%) were the leading strain. The positive rate of fungi detection in sputum culture was 69.0%. Serum galactomannan (GM) test was positive in bronchoalveolar lavage fluid (BALF) from seven (7/10, 70.0%) patients. Ten patients died (31.3%), including three with AAV (42.9%) and seven with SLE (36.8%). Penicillium marneffei was the most fatal (mortality, 100%). Non-survivors had higher prevalence of leukopenia (30.0% vs 4.5%, P = 0.04), lymphopenia (100.0% vs 59.1%, P = 0.02), elevated serum creatinine (70.0% vs 27.3%, P = 0.02), and co-infection (70.0% vs 18.2%, P = 0.004) than survivors. Multivariate logistic regression analysis showed that lymphopenia [odds ratio (OR) = 3.28, 95% confidence interval (CI) 1.29-8.38, P = 0.01] and median-to-high dose of glucocorticoid (GC) [OR = 3.40, 95% CI 1.04-11.13, P = 0.04] were associated with IM in patients with CTD. CONCLUSIONS: IM tended to develop in patients with AAV, resulting in high mortality. Sputum culture and GM test in BALF were effective methods to distinguish IM. Vigilance against lymphopenia, impaired kidney function, and co-infection improved the prognosis of IM.


Assuntos
Doenças do Tecido Conjuntivo/epidemiologia , Infecções Fúngicas Invasivas/epidemiologia , Adulto , Grupo com Ancestrais do Continente Asiático/estatística & dados numéricos , Aspergillus/isolamento & purificação , Aspergillus/fisiologia , Líquido da Lavagem Broncoalveolar/química , Líquido da Lavagem Broncoalveolar/microbiologia , China/epidemiologia , Comorbidade , Doenças do Tecido Conjuntivo/etnologia , Feminino , Humanos , Incidência , Infecções Fúngicas Invasivas/etnologia , Infecções Fúngicas Invasivas/microbiologia , Masculino , Mananas/análise , Mananas/sangue , Pessoa de Meia-Idade , Penicillium/isolamento & purificação , Penicillium/fisiologia , Estudos Retrospectivos , Escarro/microbiologia , Adulto Jovem
17.
Arthritis Rheumatol ; 71(2): 182-195, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30604506
18.
Respir Med ; 146: 23-27, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30665514

RESUMO

INTRODUCTION: Idiopathic interstitial pneumonias (IIP) are diffuse lung diseases whose cause is unknown and often present with features of autoimmunity despite not meeting criteria for a connective tissue disease (CTD). Recent studies suggest that anti-RNA binding protein (anti-RBP) antibodies, which include anti-SSA, anti-SSB, anti-Sm, and anti-RNP, play a role in the loss of immune tolerance and severity of pulmonary hypertension (PH) in CTDs. We hypothesized that anti-RBP positive (RBP+) subjects would have worse measures of lung function, radiographic findings, PH, and survival than anti-RBP negative (RBP-) subjects. METHODS: Subjects with both IIP and serologies for review were identified retrospectively and stratified based on anti-RBP antibody seropositivity. Baseline cohort characteristics, pulmonary function tests (PFT), ambulatory oxygen requirement, radiographic characteristics, markers of PH, and transplant-free survival were compared between anti-RBP positive and negative groups. RESULTS: Five hundred twenty patients with IIP were identified, of which ten percent (n = 53) were anti-RBP positive. RBP+ as compared to RBP- subjects had significantly worse PFTs as indicated by FEV1 (59.6 vs. 64.9, p = 0.046) and FVC (71.6 vs. 78.8, p = 0.018). There was a higher prevalence of radiographic honeycombing (49.1% vs. 38.3%, p = 0.006) and emphysema (22.6% vs. 5.1%, p < 0.001) in the RBP+ group despite no difference in smoking history. The Pulmonary Artery-Aorta ratio was also larger in the RBP+ group (0.93 vs. 0.88, p = 0.040). There was no difference in transplant-free survival between groups (log rank = 0.912). CONCLUSION: Anti-RBP+ IIP patients may have worse lung function, increased chest radiographic abnormalities, and PH compared with those without these antibodies.


Assuntos
Proteínas de Transporte/metabolismo , Pneumonias Intersticiais Idiopáticas/sangue , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Idoso , Anticorpos Antinucleares/imunologia , Autoimunidade/imunologia , Proteínas de Transporte/sangue , Doenças do Tecido Conjuntivo/epidemiologia , Feminino , Humanos , Hipertensão Pulmonar/fisiopatologia , Pneumonias Intersticiais Idiopáticas/diagnóstico por imagem , Pulmão/irrigação sanguínea , Pulmão/diagnóstico por imagem , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prevalência , Radiografia Torácica/métodos , Testes de Função Respiratória/métodos , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
19.
J Neurol ; 266(2): 515-521, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30612143

RESUMO

OBJECTIVE: To investigate if progressive multifocal leucoencephalopathy (PML) incidence has increased in Finland like in the neighbouring Sweden. METHODS: National administrative registries were searched for all PML admissions aged 16 years or more in 2004-2014 on all neurological and internal medicine wards in Finland. The mortality data of the patients was extracted from the national causes of death registry. National level data on annual predisposing drug use was obtained from the national pharmaceutical authority. RESULTS: We identified 35 PML cases (57% male) with a peak in 2010-2011 that amounted to 49% of all cases. The annual incidence for the entire study period was 0.072/100,000 person-years (95% CI 0.050-0.10) with no temporal trend (p = 0.18). Mean age was 57 years (22-88 years) with no sex difference (p = 0.42). Neoplasms (60%), HIV infection (17%) and systemic connective tissue disorders (CTD, 14%) were the most common predisposing conditions. MS was recorded in three cases (9%). The national level use of drugs that predispose to PML increased during the study period, with the exceptions of alemtuzumab and fludarabine. Overall survival was 85% at 90 days, 79% at 1 year, and 66% at 5 years. Survival was worst in patients with malignancy and best in patients with CTD. CONCLUSIONS: PML most often occurs in patients with malignancies and patients with HIV or CTD cover a third. PML incidence in Finland is lower than in Sweden and shows no temporal trend despite increasing use of predisposing drugs. Mortality after PML varies according to the predisposing condition.


Assuntos
Doenças do Tecido Conjuntivo/epidemiologia , Infecções por HIV/epidemiologia , Leucoencefalopatia Multifocal Progressiva/epidemiologia , Neoplasias/epidemiologia , Sistema de Registros , Adulto , Idoso , Idoso de 80 Anos ou mais , Doenças do Tecido Conjuntivo/complicações , Estudos Transversais , Feminino , Finlândia/epidemiologia , Infecções por HIV/complicações , Humanos , Incidência , Leucoencefalopatia Multifocal Progressiva/etiologia , Masculino , Pessoa de Meia-Idade , Neoplasias/complicações , Adulto Jovem
20.
ANZ J Surg ; 89(3): 191-195, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30685890

RESUMO

BACKGROUND: Patella infera is a known complication of total knee arthroplasty, and the method of soft tissue closure is a possible contributing factor. The aim of our study was to evaluate the effect of knee position during surgical closure of capsule, subcutaneous tissue and skin on patella tendon length after total knee arthroplasty. METHODS: A three arm retrospective cohort study was conducted in a single institution over a 3-year period; 75 patients were divided, by surgeon preference, into three groups (Flexed, Extended and Hybrid) of 25 patients. All groups had standardized prosthesis, intraoperative and postoperative protocols, and differed in knee position at closure. Patellar tendon length was assessed radiologically using Insall Salvati ratio (ISR) and modified Insall Salvati ratio, with a 12-month follow-up. Intraclass correlation coefficients were used to assess intraobserver variability. RESULTS: There was a small but significant difference in preoperative to initial postoperative ISR change between Flexed and Extended groups (Extended group mean ISR change = -0.05; t = -2.31, P = 0.025, independent samples t-test), which was not sustained at 12 months. The incidence of patella infera was similar in Flexed and Extended groups at 12 months with only one case seen in the Hybrid group. CONCLUSION: Our study suggests that knee position during soft tissue closure does not have a sustained impact on patella tendon length after knee replacement. A small but statistically significant reduction in patella height was found in the Extended group initially after surgery but this effect was not sustained at 12 months.


Assuntos
Artroplastia do Joelho , Patela , Ligamento Patelar , Posicionamento do Paciente , Técnicas de Fechamento de Ferimentos , Idoso , Artroplastia do Joelho/métodos , Estudos de Coortes , Doenças do Tecido Conjuntivo/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Patela/anatomia & histologia , Ligamento Patelar/anatomia & histologia , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos
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