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1.
Dermatol Online J ; 26(3)2020 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-32609447

RESUMO

A widespread form of eruptive collagenomas in a 12-year-old man is presented for the impressive iconography, challenging differential diagnosis, and histopathological considerations associated with such rare connective tissue disorders. Syndromic forms should be carefully investigated for the different course and prognosis. Treatment is a major unsolved issue as aesthetic concerns are significant, especially in young adults.


Assuntos
Doenças do Tecido Conjuntivo/patologia , Derme/patologia , Nevo/patologia , Neoplasias Cutâneas/patologia , Dorso/patologia , Biópsia/métodos , Corantes , Fibroblastos/patologia , Humanos , Masculino , Adulto Jovem
4.
Radiol Clin North Am ; 58(4): 831-839, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32471547

RESUMO

A variety of nonatherosclerotic diseases affect the arteries of the pelvis and lower extremities. Chronic repetitive traumatic conditions, such as popliteal entrapment and external iliac artery fibroelastosis, vasculitis and connective tissue diseases, and noninflammatory vascular diseases, are a few of the more commonly encountered nonatherosclerotic peripheral vascular diseases. Ultrasound, computed tomography angiography, and magnetic resonance angiography are essential in the initial assessment and management of patients with peripheral vascular disease.


Assuntos
Doenças Vasculares Periféricas/diagnóstico por imagem , Adulto , Doenças do Tecido Conjuntivo/complicações , Feminino , Fibrose/diagnóstico por imagem , Humanos , Artéria Ilíaca/diagnóstico por imagem , Artéria Ilíaca/patologia , Masculino , Pessoa de Meia-Idade , Artéria Poplítea/diagnóstico por imagem , Vasculite/complicações , Vasculite/diagnóstico por imagem , Adulto Jovem
5.
Medicine (Baltimore) ; 99(16): e19695, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32311947

RESUMO

PURPOSE: The aim of the study was to estimate and compare the diagnostic accuracy of serum Krebs von den Lungen-6 (KL-6) and surfactant protein D (SP-D) for identifying interstitial lung disease (ILD) from non-ILD among connective tissue disease (CTD) patients. MATERIALS AND METHODS: Original articles on the diagnostic accuracy of serum KL-6 and SP-D in differentiating CTD-ILD from CTD-nonILD were identified from three public databases. The overall quality of evidence and methodologic quality of each eligible study were assessed by the Grading of Recommendations, Assessment, Development and Evaluation approach and Quality Assessment of Diagnostic Accuracy Studies, respectively. We used the bivariate model to calculate random-effect sensitivity, specificity, likelihood ratios, and area under curve. Furthermore, trial sequential analysis (TSA) was used to determine whether sample sizes incorporated in the meta-analysis were powerful for evaluating the diagnostic utility. Bayesian network analysis was performed to compare the diagnostic accuracy of 2 serum biomarkers in differentiating ILD among CTD patients and various subgroups. RESULTS: Twenty-nine studies were included in the quantitative synthesis. No threshold effects were observed (all P values >.05). For diagnosis of ILD among CTD patients, overall sensitivity and specificity of serum KL-6 were 0.76 (95% confidence interval [CI]: 0.68-0.82) and 0.89 (95% CI: 0.83-0.93), whereas those for serum SP-D were 0.65 (95% CI: 0.45-0.80) and 0.88 (95% CI: 0.80-0.93). Comprehensive comparison of 2 circulating biomarkers using back-calculated likelihood ratio (LR) demonstrated that serum KL-6 corresponded to a higher LR+ and a lower LR- in comparison to serum SP-D, as well as in SSc-ILD. TSA indicated that evidence for serum KL-6 and SP-D in identifying CTD-ILD is powerful; nonetheless, more trials were needed for validation of serum KL-6 and SP-D in differentiating CTD-ILD subtypes, including different CTD and ethnicities. CONCLUSIONS: This meta-analysis suggested that serum KL-6 had superior diagnostic accuracy to SP-D for differentiating ILD from non-ILD among CTD patients, providing a convenient and non-invasive approach for screening and management of ILD among CTD patients.


Assuntos
Doenças do Tecido Conjuntivo/sangue , Doenças Pulmonares Intersticiais/sangue , Mucina-1/sangue , Proteína D Associada a Surfactante Pulmonar/sangue , Biomarcadores/sangue , Doenças do Tecido Conjuntivo/complicações , Diagnóstico Diferencial , Humanos , Doenças Pulmonares Intersticiais/complicações
7.
Zhonghua Jie He He Hu Xi Za Zhi ; 43(4): 362-368, 2020 Apr 12.
Artigo em Chinês | MEDLINE | ID: mdl-32294819

RESUMO

Objective: To investigate the clinical features and prognosis of interstitial lung disease patients with positive anti-neutrophil cytoplasmic antibody. Methods: The patients with interstitial lung disease who visited Peking Union Medical College Hospital from March 2006 to March 2016 were divided into three groups: interstitial lung disease with ANCA-positive(ANCA-ILD), connective tissue disease associated interstitial lung disease and interstitial pneumonia with autoimmune features (CTD-ILD/IPAF) and idiopathic interstitial pneumonia (IIP). The three groups were analyzed in terms of clinical manifestations, serology, lung function, imaging, survival and recurrence. Results: Two hundred and seventy four patients were enrolled and 38 (14%) were ANCA-positive of whom 16 were male and 22 were female. The age of 38 ANCA-positive patients was (59±10) years and the follow-up time was (52±31) months. Seven among the 38 ANCA-positive patients died and the death rate is 18.42%. The ANCA-positive patients with interstitial lung disease have higher onset age (ANCA-ILD:59±10,CTD-ILD/IPAF:52±10,IIP:53±11,H=19.29, P<0.001), lower hemoglobin (ANCA-ILD: 129±21, CTD-ILD/IPAF: 138±15, IIP: 140±19, H=8.17, P=0.017), higher erythrocyte sedimentation rate (ANCA-ILD:45±35, CTD-ILD/IPAF:26±24,IIP:19±22,H=19.73, P<0.001), lower lung function improvement rate after treatment (ANCA-ILD:31%,CTD-ILD/IPAF:59%,IIP: 39%,χ(2)=11.74,P=0.003), lower absorption rate of CT lesion (ANCA-ILD:61%,CTD-ILD/IPAF:82%,IIP:67%, χ(2)=9.23,P=0.010) and higher death rate(ANCA-ILD:18%,CTD-ILD/IPAF:6%,IIP:12%, χ(2)=7.16,P=0.028). Conclusions: There are significant differences in clinical characteristics between ANCA-positive patients and other types of pulmonary interstitial disease. And both the treatment effect and the prognosis is poor for the ANCA-positive patients.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/sangue , Doenças do Tecido Conjuntivo/diagnóstico , Pneumonias Intersticiais Idiopáticas/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Biomarcadores/sangue , Doenças do Tecido Conjuntivo/sangue , Doenças do Tecido Conjuntivo/imunologia , Doenças do Tecido Conjuntivo/mortalidade , Feminino , Humanos , Pneumonias Intersticiais Idiopáticas/sangue , Pneumonias Intersticiais Idiopáticas/imunologia , Pneumonias Intersticiais Idiopáticas/mortalidade , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/sangue , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/mortalidade , Masculino , Prognóstico , Tomografia Computadorizada por Raios X
8.
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
9.
Mayo Clin Proc ; 95(3): 554-573, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32138882

RESUMO

Interstitial lung disease (ILD) is a frequent complication of patients with connective tissue disease (CTD) and significantly affects morbidity and mortality. Disease course may vary from stable or mildly progressive to more severe, with rapid loss of lung function. We conducted a search of PubMed (National Library of Medicine) and the Web of Science Core Collection using the key words lung, pulmonary, pneumonia, pneumonitis, and alveolar and subtypes of CTD. All clinical studies from January 1, 1980, through September 1, 2018, were reviewed for descriptions of specific therapies and their efficacy or safety and were categorized as controlled interventional trials, observational prospective or retrospective cohort studies, case series (>5 patients), and case reports (<5 patients). Low-quality reports (<5 patients) before 2000, reviews, editorials, popular science papers, and letters to the editor without complete descriptions of the therapies used or their outcomes were excluded. Directed therapy for CTD-ILD is dominated by empirical use of immunosuppressive agents, with the decision to treat, treatment choice, and treatment duration limited to cases and cohort observations. Only a few higher-level controlled studies were available specifically in scleroderma-related ILD. We summarize herein for the clinician the published treatment scope and experience, highlighted clinical response, and common adverse reactions for the management of CTD-ILD.


Assuntos
Doenças do Tecido Conjuntivo/terapia , Doenças Pulmonares Intersticiais/terapia , Corticosteroides/uso terapêutico , Produtos Biológicos/uso terapêutico , Doenças do Tecido Conjuntivo/complicações , Progressão da Doença , Transplante de Células-Tronco Hematopoéticas , Humanos , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais/etiologia , Prognóstico
10.
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
11.
Rev Med Suisse ; 16(685): 487-491, 2020 Mar 11.
Artigo em Francês | MEDLINE | ID: mdl-32167250

RESUMO

Targeted therapies are nowadays commonly used in connective tissue diseases and vasculitis. Experts recommend the use of belimumab and rituximab in refractory and/or severe cases of lupus. Rituximab can be also considered in difficult to treat cases of Sjögren's disease or myositis. Nintedanib seems a very promising weapon in the management of systemic sclerosis-associated pulmonary fibrosis. Regarding vasculitis, rituximab has become the preferred treatment for granulomatosis with polyangiitis and microscopic polyangiitis. Finally, tocilizumab is recommended as a steroid-sparing agent in giant cell arteritis. Further clinical trials are warranted to study the efficacy of other targeted therapies in connective tissue diseases and vasculitis.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Doenças do Tecido Conjuntivo/tratamento farmacológico , Rituximab/uso terapêutico , Vasculite/tratamento farmacológico , Arterite de Células Gigantes/tratamento farmacológico , Humanos , Miosite/tratamento farmacológico , Síndrome de Sjogren/tratamento farmacológico
12.
Internist (Berl) ; 61(3): 261-269, 2020 Mar.
Artigo em Alemão | MEDLINE | ID: mdl-32072188

RESUMO

Vasculitic neuropathies result from inflammation of the vasa nervorum followed by ischemia and destruction of the peripheral nerve. The inflammation can be systemic or localized, i.e. non-systemic. Systemic vasculitis can be divided into primary and secondary forms. The latter is associated with, e.g. connective tissue diseases, infections, cancer or induced by certain drugs. Around two thirds of patients with systemic vasculitis develop vasculitic neuropathy presenting as characteristic painful, multifocal mononeuropathy of acute onset. The group of non-systemic neuropathies has grown in recent years with the addition of diabetic and non-diabetic lumbosacral radiculoplexus neuropathies, among others. Within the group of connective tissue diseases, other non-vasculitic neuropathies can occur as nerve-entrapment syndromes and sensory ataxic neuropathy. The aim of this article is to present a condensed overview of neuropathies associated with vasculitis and connective tissue diseases and to communicate characteristic clinical symptoms supporting rapid diagnostic and therapeutic procedures.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Doenças do Sistema Nervoso Periférico/complicações , Polineuropatias/diagnóstico , Vasculite/complicações , Vasculite/diagnóstico , Humanos , Dor/diagnóstico , Dor/etiologia , Nervos Periféricos/patologia , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/terapia , Polineuropatias/terapia , Vasculite/terapia
13.
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
14.
Medicine (Baltimore) ; 99(4): e18589, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31977850

RESUMO

To date, there is no clear agreement regarding which is the best method to detect a connective tissue disease (CTD) during the initial diagnosis of interstitial lung diseases (ILD). The aim of our study was to explore the impact of a systematic diagnostic strategy to detect CTD-associated ILD (CTD-ILD) in clinical practice, and to clarify the significance of interstitial pneumonia with autoimmune features (IPAF) diagnosis in ILD patients.Consecutive patients evaluated in an ILD Diagnostic Program were divided in 3 groups: IPAF, CTD-ILD, and other ILD forms. Clinical characteristics, exhaustive serologic testing, high resolution computed tomography (HRCT) images, lung biopsy specimens, and follow-up were prospectively collected and analyzed.Among 139 patients with ILD, CTD was present in 21 (15.1%), 24 (17.3%) fulfilled IPAF criteria, and 94 (67.6%) were classified as other ILD forms. Specific systemic autoimmune symptoms such as Raynaud phenomenon (19%), inflammatory arthropathy (66.7%), and skin manifestations (38.1%) were more frequent in CTD-ILD patients than in the other groups (all P < .001). Among autoantibodies, antinuclear antibody was the most frequently found in IPAF (42%), and CTD-ILD (40%) (P = .04). Nonspecific interstitial pneumonia, detected by HRCT scan, was the most frequently seen pattern in patients with IPAF (63.5%), or CTD-ILD (57.1%) (P < .001). In multivariate analysis, a suggestive radiological pattern by HRCT scan (odds ratio [OR] 15.1, 95% confidence interval [CI] 4.7-48.3, P < .001) was the strongest independent predictor of CTD-ILD or IPAF, followed by the presence of clinical features (OR 14.6, 95% CI 4.3-49.5, P < .001), and serological features (OR 12.4, 95% CI 3.5-44.0, P < .001).This systematic diagnostic strategy was useful in discriminating an underlying CTD in patients with ILD. The defined criteria for IPAF are fulfilled by a considerable proportion of patients referred for ILD.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/diagnóstico , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Autoanticorpos/imunologia , Biópsia , Doenças do Tecido Conjuntivo/patologia , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
16.
Radiol Med ; 125(1): 66-67, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31542856

RESUMO

The Connective Tissue Diseases (CTDs)-related Interstitial Lung Disease (ILD) early diagnosis by Transthoracic Ultrasound (TUS) still arises several issues. Gutierrez et al. clearly underlined the current role of ultrasound artifacts for ILD definition according to some Authors. In this Letter to the Editor, we would like to highlight the proper role of TUS and its pitfalls.


Assuntos
Doenças do Tecido Conjuntivo , Doenças Pulmonares Intersticiais , Artefatos , Humanos , Ultrassonografia
17.
Autoimmun Rev ; 19(2): 102453, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31838163

RESUMO

INTRODUCTION: The effectiveness of CD20 targeting in connective tissue diseases (CTD) with lung involvement is controversial. This paper aims to review the current evidence about rituximab (RTX) use in CTD-related interstitial lung disease (ILD). METHODS: We performed a systematic review of papers published between January 2009 and May 2019. We included clinical trials, case/control studies and cohort studies. We excluded letters, case reports, case series, reviews, and full articles when not in English. The selected studies listed as primary or secondary outcome a variation in pulmonary function tests or in the scores used to radiologically stage lung involvement, in CTD-related ILD patients after RTX. RESULTS: Out of 1206 potentially eligible articles, 24 papers were selected: 3 retrospectively described cohorts of patients with different CTD, 14 dealt with systemic sclerosis (SSc)-related ILD, 5 with idiopathic inflammatory myopathies (IIMs)-related ILD, and 2 with Sjögren's Syndrome-related ILD. A direct comparison of the selected studies was hampered by their heterogeneity for outcomes, follow-up duration, the severity of lung involvement, and clinical features of study populations. However, an overall agreement existed concerning the effectiveness of RTX in the stabilization of lung disease, with some studies reporting an improvement of functional parameters from baseline. IIM-related ILD appeared more responsive than other CTD-related ILD to CD20 targeting. CONCLUSION: RTX is a promising therapeutic tool in CTD-related ILD. This systematic review remarks the unmet need of multicenter prospective studies aiming to evaluate the effectiveness of RTX with adequate sample size and study design.


Assuntos
Antígenos CD20/imunologia , Doenças do Tecido Conjuntivo/complicações , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/tratamento farmacológico , Rituximab/imunologia , Rituximab/uso terapêutico , Humanos , Estudos Retrospectivos
19.
Am J Obstet Gynecol ; 222(1): 77.e1-77.e11, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31310750

RESUMO

BACKGROUND: Although consensus guidelines on the management of cardiovascular disease in pregnancy reserve cesarean delivery for obstetric indications, there is a paucity of data to support this approach. OBJECTIVE: The objective of the study was to compare cardiovascular and obstetric morbidity in women with cardiovascular disease according to the plan for vaginal birth or cesarean delivery. STUDY DESIGN: We assembled a prospective cohort of women delivering at an academic tertiary care center with a protocolized multidisciplinary approach to management of cardiovascular disease between September 2011 and December 2016. Our practice is to encourage vaginal birth in women with cardiovascular disease unless there is an obstetric indication for cesarean delivery. We allow women attempting vaginal birth a trial of Valsalva in the second stage with the ability to provide operative vaginal delivery if pushing leads to changes in hemodynamics or symptoms. Women were classified according to planned mode of delivery: either vaginal birth or cesarean delivery. We then used univariate analysis to compare adverse outcomes according to planned mode of delivery. The primary composite cardiac outcome of interest included sustained arrhythmia, heart failure, cardiac arrest, cerebral vascular accident, need for cardiac surgery or intervention, or death. Secondary obstetric and neonatal outcomes were also considered. RESULTS: We included 276 consenting women with congenital heart disease (68.5%), arrhythmias (11.2%), connective tissue disease (9.1%), cardiomyopathy (8.0%), valvular disease (1.4%), or vascular heart disease (1.8%) at or beyond 24 weeks' gestation. Seventy-six percent (n = 210) planned vaginal birth and 24% (n = 66) planned cesarean delivery. Women planning vaginal birth had lower rates of left ventricular outflow tract obstruction, multiparity, and preterm delivery. All women attempting vaginal birth were allowed Valsalva. Among planned vaginal deliveries 86.2% (n = 181) were successful, with a 9.5% operative vaginal delivery rate. Five women underwent operative vaginal delivery for the indication of cardiovascular disease without another obstetric indication at the discretion of the delivering provider. Four of these patients tolerated trials of Valsalva ranging from 15 to 75 minutes prior to delivery. Adverse cardiac outcomes were similar between planned vaginal birth and cesarean delivery groups (4.3% vs 3.0%, P = 1.00). Rates of postpartum hemorrhage (1.9% vs 10.6%, P < .01) and transfusion (1.9% vs 9.1%, P = .01) were lower in the planned vaginal birth group. There were no differences in adverse cardiac, obstetric, or neonatal outcomes in the cohort overall or the subset of women with high-risk cardiovascular disease or a high burden of obstetric comorbidity. CONCLUSION: These findings suggest that cesarean delivery does not reduce adverse cardiovascular outcomes and lend support to a planned vaginal birth for the majority of women with cardiovascular disease including those with high-risk disease.


Assuntos
Cesárea/métodos , Parto Obstétrico/métodos , Cardiopatias , Complicações Cardiovasculares na Gravidez , Adulto , Arritmias Cardíacas , Cardiomiopatias , Doenças do Tecido Conjuntivo , Doença da Artéria Coronariana , Gerenciamento Clínico , Extração Obstétrica/métodos , Feminino , Cardiopatias Congênitas , Doenças das Valvas Cardíacas , Humanos , Hemorragia Pós-Parto/epidemiologia , Gravidez , Estudos Prospectivos , Manobra de Valsalva
20.
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
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