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2.
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
3.
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
4.
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
5.
Ann Agric Environ Med ; 26(3): 468-471, 2019 Sep 19.
Artigo em Inglês | MEDLINE | ID: mdl-31559805

RESUMO

INTRODUCTION AND OBJECTIVE: Fascia Manipulation® is one of the methods focusing on the deep fascia. The assumption is that fascial manipulation is carried out on precisely determined points - coordination centres (cc), and on a limited area so as the friction occurring during manipulation would cause a local rise in temperature due to the inflammatory reaction. Rise in temperature influences modification in consistency of elementary matter in the manipulated area, and by the same token causing a decrease in the negative effects of fascia densification which stems from accumulation of hyaluronic acid. The purpose of the research is to prove the thesis that fascial manipulation causes local rise in temperature due to inflammatory reaction. MATERIAL AND METHODS: For the research, 25 individuals with densification in lower limb area were qualified. They were exposed to a single, 3-minute facial manipulation®. By means of a thermal-imaging camera, changes in the temperature of the body in the examined area were evaluated. The body's temperature evaluation was carried out 8 times: before the treatment, 5 minutes after the treatment, and, next, 6, 12, 18, 24, 36, 48 hours after the treatment. RESULTS: The average surface temperature of the treated area before mobilization was 33.4°C. A statistically relevant increase in temperature was already observed 5 minutes after the treatment (increase of 0.5°C; p<0.001). However, the highest temperature was observed 24 hours after mobilization (increase of 2.4°C). The difference between the first and 7 other measurements was statistically relevant (p<0.001). CONCLUSIONS: The statistically relevant increase in temperature under the influence of fascial manipulation® in the treatment area can confirm the occurrence of inflammatory reaction.


Assuntos
Doenças do Tecido Conjuntivo/terapia , Fáscia/fisiopatologia , Manipulações Musculoesqueléticas , Adulto , Temperatura Corporal , Doenças do Tecido Conjuntivo/imunologia , Doenças do Tecido Conjuntivo/fisiopatologia , Fáscia/imunologia , Feminino , Humanos , Masculino , Termografia , Adulto Jovem
6.
Indian J Pediatr ; 86(9): 857-859, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31077005

RESUMO

Connective tissue diseases are rarely suspected and diagnosed in childhood and adolescence. Rarity of occurrence and poor disease acceptance among parents make them extremely difficult to treat in the early stages. An adolescent girl presented with features of pneumonia, was worked up and diagnosed as an evolving connective tissue disease. Her clinical characteristics did not fit into any specific disease. She was started on steroids and immunoglobulin as she had fulminant myocarditis with rapid downhill clinical course. High index of suspicion and aggressive immunosuppression can be life saving in exceptional situations even though a specific diagnosis cannot be ascertained.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/diagnóstico , Miocardite/complicações , Miocardite/diagnóstico , Adolescente , Doenças do Tecido Conjuntivo/tratamento farmacológico , Doenças do Tecido Conjuntivo/fisiopatologia , Cianose/diagnóstico por imagem , Feminino , Humanos , Imunoglobulinas/uso terapêutico , Miocardite/tratamento farmacológico , Miocardite/fisiopatologia , Esteroides/uso terapêutico
7.
Cardiol Young ; 29(5): 564-569, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31111802

RESUMO

INTRODUCTION: This research investigated patients who underwent surgery for a dilated aorta associated with a connective tissue disease or inflammatory vasculitis in children and adolescents. MATERIALS AND METHODS: The medical records of 11 patients who underwent aortic surgery for dilatation resulting from a connective tissue disease or inflammatory vasculitis between 2000 and 2017 were retrospectively reviewed. RESULTS: The median age and body weight of the patients were 9.6 years (range 5.4 months-15.5 years) and 25.8 kg (range 6.8-81.5), respectively. The associated diseases were Marfan syndrome (n = 3), Loeys-Dietz syndrome (n = 3), Kawasaki disease (n = 1), Takayasu arteritis (n = 1), PHACE syndrome (n = 1), tuberous sclerosis (n = 1), and unknown (n = 1). The most common initially affected area was the ascending aorta. During the 66.4 ± 35.9 months of follow-up, two Marfan syndrome patients died, and four patients (one Marfan syndrome and three Loeys-Dietz syndrome) had repeated aortic operation. Except for one patient, the functional class was well maintained in all patients who were followed up. CONCLUSION: Cases of surgical treatment for a dilated aorta associated with a connective tissue disease and inflammatory vasculitis are rare in children and adolescents at our institution. Most of the patients in this study showed a tolerable postoperative course. However, the aorta showed progressive dilation over time even after surgical treatment, especially in patients with Loeys-Dietz syndrome. In these patients, close and more frequent regular follow-up is required.


Assuntos
Aorta Torácica/patologia , Aorta Torácica/cirurgia , Doenças do Tecido Conjuntivo/complicações , Dilatação Patológica/cirurgia , Vasculite/complicações , Adolescente , Criança , Pré-Escolar , Doenças do Tecido Conjuntivo/fisiopatologia , Dilatação Patológica/etiologia , Feminino , Humanos , Masculino , Reoperação , República da Coreia , Estudos Retrospectivos , Vasculite/fisiopatologia
9.
Ter Arkh ; 91(3): 93-100, 2019 Mar 30.
Artigo em Inglês | MEDLINE | ID: mdl-31094466

RESUMO

This review presents an analysis of the literature on the topic of respiratory muscle (RM) dysfunction in various forms of respiratory pathology: chronic obstructive pulmonary disease (COPD), asthma, community-acquired pneumonia, idiopathic pulmonary fibrosis (IPF), sarcoidosis and interstitial lung diseases (ILD), associated with systemic connective tissue diseases (polymyositis, dermatomyositis and systemic lupus erythematosus - SLE). Various clinical and pathophysiological aspects of RM dysfunction and general patterns of its pathogenesis were examined. It was proved that the role of RM in the development of respiratory failure depends on the form and stage of the pulmonary pathology and the severity of systemic manifestations of these diseases: excessive proteolysis, oxidative stress, hypoxia, chronic systemic inflammation. These factors modify the morphofunctional status of RM, worsens their contractile function, which is contributed to the development of respiratory failure. In some cases, the primary weakness of RM precedes the clinical manifestation of pulmonary pathology, which is distinctive for some variants of myositis-associated ILD and SLE. Endogenous intoxication syndrome plays a significant role in the development of RM dysfunction during community-acquired pneumonia. It is noted that sarcoid pulmonary ventilation disorders associate with the RM weakness, but not with the degree of lung damage. In most cases, secondary RM dysfunction predominates that contributes to respiratory failure progression, which is especially noticeable in case of COPD, asthma and IPF.


Assuntos
Pulmão/fisiopatologia , Insuficiência Respiratória/fisiopatologia , Músculos Respiratórios/fisiopatologia , Asma/fisiopatologia , Doenças do Tecido Conjuntivo/fisiopatologia , Humanos , Fibrose Pulmonar Idiopática/fisiopatologia , Pneumopatias Obstrutivas/fisiopatologia , Doença Pulmonar Obstrutiva Crônica/fisiopatologia
10.
Dermatol Ther ; 32(4): e12935, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30983128

RESUMO

Connective tissue disorders (CTDs) are chronic inflammatory conditions that can lead to scarring and disfiguration. Although conventional methods are often of little benefit in cutaneous manifestations, the use of cosmetic procedures is still controversial. Concerns have also been raised concerning cosmetic treatments in CTDs, and particularly regarding lasers and fillers, due to photosensitivity and potential reactivation. This article reviews the cosmetic treatment of various CTDs under three headings - lasers, fillers, and botulinum toxin.


Assuntos
Doenças do Tecido Conjuntivo/terapia , Técnicas Cosméticas , Preenchedores Dérmicos/administração & dosagem , Toxinas Botulínicas/administração & dosagem , Doenças do Tecido Conjuntivo/fisiopatologia , Humanos , Terapia a Laser/métodos
11.
Respir Med ; 150: 15-20, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30961943

RESUMO

BACKGROUND: Patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) often experience impaired health status. In daily clinical practice, a short and easy instrument for assessing health status would be useful to help better understand the patient's condition. The COPD Assessment Test (CAT) is a simple questionnaire about respiratory symptoms and their impact. We aimed to examine the CAT's performance characteristics and to generate data to support its reliability and validity in patients with CTD-ILD. METHODS: We used data from 132 CTD-ILD patients evaluated at Tosei General Hospital from July 2011 to July 2016 to assess the cross-sectional and longitudinal validity of the CAT. RESULTS: The mean age of the patients was 64.5 years and 87 (66%) were women. There were no significant differences in CAT score between any of the CTD subgroups. Internal consistency (Cronbach's α = 0.881) and repeatability (intraclass correlation coefficient [ICC] = 0.803) for the CAT score were acceptable. At baseline, CAT score was significantly associated with clinically meaningful measures of physiologic function, exercise capacity, and dyspnea. Change in CAT score over 6-12 months was also associated with change in other measures. In the distribution- and anchor-based analyses, the estimated minimal clinically important difference of CAT score was 1-4 points. CONCLUSION: These data support the validity and reliability of CAT as a sensitive measure for assessing health status in patients with CTD-ILD.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Dispneia/diagnóstico , Doenças Pulmonares Intersticiais/complicações , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Inquéritos e Questionários/normas , Adulto , Idoso , Gasometria/métodos , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/fisiopatologia , Estudos Transversais , Dispneia/fisiopatologia , Dispneia/psicologia , Exercício Físico , Feminino , Nível de Saúde , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Resistência Física/fisiologia , Doença Pulmonar Obstrutiva Crônica/sangue , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Doença Pulmonar Obstrutiva Crônica/psicologia , Qualidade de Vida , Reprodutibilidade dos Testes , Testes de Função Respiratória/métodos , Estudos Retrospectivos , Inquéritos e Questionários/estatística & dados numéricos
12.
Genet Med ; 21(8): 1832-1841, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30675029

RESUMO

PURPOSE: Heritable factors play an important etiologic role in connective tissue disorders (CTD) with vascular involvement, and a genetic diagnosis is getting increasingly important for gene-tailored, personalized patient management. METHODS: We analyzed 32 disease-associated genes by using targeted next-generation sequencing and exome sequencing in a clinically relevant cohort of 199 individuals. We classified and refined sequence variants according to their likelihood for pathogenicity. RESULTS: We identified 1 pathogenic variant (PV; in FBN1 or SMAD3) in 15 patients (7.5%) and ≥1 likely pathogenic variant (LPV; in COL3A1, FBN1, FBN2, LOX, MYH11, SMAD3, TGFBR1, or TGFBR2) in 19 individuals (9.6%), together resulting in 17.1% diagnostic yield. Thirteen PV/LPV were novel. Of PV/LPV-negative patients 47 (23.6%) showed ≥1 variant of uncertain significance (VUS). Twenty-five patients had concomitant variants. In-depth evaluation of reported/calculated variant classes resulted in reclassification of 19.8% of variants. CONCLUSION: Variant classification and refinement are essential for shaping mutational spectra of disease genes, thereby improving clinical sensitivity. Obligate stringent multigene analysis is a powerful tool for identifying genetic causes of clinically related CTDs. Nonetheless, the relatively high rate of PV/LPV/VUS-negative patients underscores the existence of yet unknown disease loci and/or oligogenic/polygenic inheritance.


Assuntos
Aorta/fisiopatologia , Doenças do Tecido Conjuntivo/genética , Sequenciamento de Nucleotídeos em Larga Escala , Síndrome de Marfan/genética , Adulto , Aorta/metabolismo , Biomarcadores/metabolismo , Estudos de Coortes , Tecido Conjuntivo/metabolismo , Tecido Conjuntivo/patologia , Doenças do Tecido Conjuntivo/fisiopatologia , Feminino , Testes Genéticos , Humanos , Masculino , Síndrome de Marfan/diagnóstico , Síndrome de Marfan/fisiopatologia
13.
Gen Thorac Cardiovasc Surg ; 67(3): 271-276, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30689200

RESUMO

We discuss the etiology of aortic dissection (AD) from various points of view. The development of AD requires two pathological conditions: medial degeneration and mechanical wall stress. First, histopathological findings of medial degeneration are hypothesized to be due to a loss of elastic fibers and interconnecting elastic fibers. Damage to the vasa vasorum plays a key role in creating an entry site. The clinical causes of medial degeneration include hypertension, aortic aneurysms, obstructive sleep apnea, and connective tissue disorders. Second, mechanical wall stress is supposedly induced by shear stress caused by blood flow, together with hypertension and aortic root movement. Further investigation is necessary in the search for mechanisms responsible for medial degeneration prior to AD development.


Assuntos
Aneurisma Dissecante/fisiopatologia , Aneurisma Dissecante/etiologia , Aneurisma Aórtico/complicações , Aneurisma Aórtico/fisiopatologia , Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/fisiopatologia , Humanos , Hipertensão/complicações , Hipertensão/fisiopatologia , Apneia Obstrutiva do Sono/complicações , Apneia Obstrutiva do Sono/fisiopatologia , Estresse Mecânico
14.
Am J Med Genet A ; 179(1): 50-56, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30548383

RESUMO

Biallelic variants in the AEBP1 gene cause a novel autosomal-recessive connective tissue disorder (CTD) reminiscent of Ehlers-Danlos Syndrome (EDS). The four previously reported individuals show considerable clinical variability. Unbiased high-throughput sequencing enables the rapid identification of additional cases for such rare entities. We identified the homozygous nonsense variant c.917dup, p.Tyr306* in AEBP1 using clinical exome sequencing in a female individual with previously unsolved CTD. Segregation testing confirmed homozygosity in the clinically affected brother and heterozygous carrier status in the healthy mother. Chromosomal microarray showed that the variant lies in a run of homozygosity, suggesting a common origin of this genomic segment. RT-PCR analysis in the mother revealed a monoallelic expression of the normal transcript supporting a nonsense-mediated mRNA decay and functional nullizygosity as disease mechanism. We describe two individuals from a fourth family with AEBP1-associated CTD. Our results further verify that autosomal-recessive inherited LOF variants in the AEBP1 gene cause clinical features of different EDS subtypes, but also of the marfanoid spectrum. As identification of further individuals is necessary to inform the clinical characterization, we stress the added value of exome sequencing for such rare diseases.


Assuntos
Carboxipeptidases/genética , Doenças do Tecido Conjuntivo/genética , Síndrome de Ehlers-Danlos/genética , Predisposição Genética para Doença , Proteínas Repressoras/genética , Adulto , Códon sem Sentido/genética , Tecido Conjuntivo/metabolismo , Tecido Conjuntivo/patologia , Doenças do Tecido Conjuntivo/fisiopatologia , Síndrome de Ehlers-Danlos/fisiopatologia , Exoma/genética , Feminino , Genes Recessivos , Heterozigoto , Sequenciamento de Nucleotídeos em Larga Escala , Homozigoto , Humanos , Mutação com Perda de Função/genética , Masculino , Linhagem , Fenótipo , Irmãos
15.
Pan Afr Med J ; 30: 176, 2018.
Artigo em Francês | MEDLINE | ID: mdl-30455805

RESUMO

This study aimed to highlight the epidemiological , clinical, therapeutic and evolutionary profile of connective tissue diseases in the hospitals setting in Lomé. We conducted a retrospective and descriptive study from January 1, 1993 to December 30, 2012. We focused on the medical records of patients with connective tissue diseases hospitalized in five Departments of Dermatology and Rheumatology at the Hospitals in Lomé. During the study period, we identified 231 cases of connective tissue diseases in the five study centers, corresponding to a frequency of 0.19% of consultations. The average age of patients was 36,96±15 years, the sex-ratio was 0,2. Major connective tissue diseases included lupus disease (50.22%), sclerodermas (21,64%) and rheumatoid polyarthritis (20.35%). Clinically, the main clinical manifestations of connective tissue diseases included discoid lupus lesions (87.50%) and photosensitivity (82.50%) in patients with systemic lupus erythematosus, skin sclerosis (90.48%) in patients with systemic scleroderma and distal joint involvement (100%) in patients with rheumatoid arthritis. Treatment in patients with systemic lupus erythematosus (92.5%) and rheumatoid arthritis (73.47%) was based on systemic corticosteroids. Connective tissue diseases are rare in people living in Lomé, with a predominance of lupus disease. They are more frequent in young women. Treatment is based on systemic corticosteroids.


Assuntos
Artrite Reumatoide/epidemiologia , Doenças do Tecido Conjuntivo/epidemiologia , Lúpus Eritematoso Sistêmico/epidemiologia , Escleroderma Sistêmico/epidemiologia , Adolescente , Corticosteroides/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Artrite Reumatoide/terapia , Criança , Pré-Escolar , Doenças do Tecido Conjuntivo/fisiopatologia , Doenças do Tecido Conjuntivo/terapia , Feminino , Hospitais , Humanos , Lúpus Eritematoso Sistêmico/terapia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Escleroderma Sistêmico/terapia , Togo/epidemiologia , Adulto Jovem
16.
Clin Exp Rheumatol ; 36 Suppl 114(5): 165-170, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30296990

RESUMO

Interstitial lung disease (ILD) is one of the most serious pulmonary complications associated with connective tissue diseases (CTDs), resulting in significant morbidity and mortality. Recently, lung ultrasound examination has appeared as a useful, feasible, non-invasive, radiation-free imaging technique with high sensitivity and specificity for the diagnosis of CTD-associated ILD. In this review, we discuss the literature concerning lung ultrasound findings (B-lines and pleural irregularities) in systemic sclerosis. We also examine the possible utility and potential limitations of lung ultrasound in other CTDs including: inflammatory myopathies, mixed connective tissue disease, and Sjögren's syndrome. Specifically, we compare the diagnostic accuracy of lung ultrasound with traditional imaging techniques and pulmonary functional tests.


Assuntos
Doenças do Tecido Conjuntivo/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Ultrassonografia/métodos , Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/fisiopatologia , Doenças do Tecido Conjuntivo/terapia , Humanos , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/terapia , Valor Preditivo dos Testes , Prognóstico , Reprodutibilidade dos Testes , Índice de Gravidade de Doença
17.
Monaldi Arch Chest Dis ; 88(3): 970, 2018 09 04.
Artigo em Inglês | MEDLINE | ID: mdl-30183156

RESUMO

Patients with Interstitial Lung Disease (ILD) without a definitive diagnosis of connective tissue diseases (CTD) were historically described as Undifferentiated Connective Tissue Disease (UCTD-ILD). Recently a new classification, Interstitial Pneumonia with Autoimmune Features (IPAF), has been proposed. Aim of this study was to describe the prevalence, clinical characteristics and prognostic factors of UCTD and IPAF subjects in a cohort of Non-Specific Interstitial Pneumonia (NSIP) patients. This retrospective, observational study enrolled 102 adult patients characterized by NSIP pattern on High Resolution Computed Tomography, without a specific diagnosis of CTD. Three groups were identified according to patients' characteristics: IPAF, UCTD or idiopathic NSIP (iNSIP). Forty percent, 27% and 55% of patients showed diagnostic criteria for IPAF, UCTD and iNSIP, respectively. No significant differences in age, gender, smoking habit, pulmonary function tests and three-year survival rate were observed among study groups. IPAF patients with antisynthetase antibodies positivity, in comparison to IPAF without antisynthetase antibodies positivity, showed more frequently an acute onset (44% vs 9%, p<0.012). The presence of autoimmune features seems not to be associated with better outcomes in NSIP patients. IPAF criteria seem to be more representative than UCTD criteria in identifying patients with autoimmune features. Further studies are needed to verify if IPAF should include patients with positive antisynthetase serology.


Assuntos
Doenças Autoimunes/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças do Tecido Conjuntivo Indiferenciado/diagnóstico por imagem , Idoso , Anticorpos Anti-Proteína Citrulinada/imunologia , Anticorpos Antinucleares/imunologia , Antígenos Nucleares/imunologia , Doenças Autoimunes/imunologia , Doenças Autoimunes/fisiopatologia , Estudos de Coortes , Doenças do Tecido Conjuntivo/diagnóstico por imagem , Doenças do Tecido Conjuntivo/imunologia , Doenças do Tecido Conjuntivo/fisiopatologia , Feminino , Humanos , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Capacidade de Difusão Pulmonar , Estudos Retrospectivos , Fator Reumatoide/imunologia , Doenças do Tecido Conjuntivo Indiferenciado/imunologia , Doenças do Tecido Conjuntivo Indiferenciado/fisiopatologia , Capacidade Vital , Teste de Caminhada
18.
PLoS One ; 13(9): e0203947, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30235235

RESUMO

BACKGROUND: Hemodynamic assessment during exercise may unmask an impaired functional reserve of the right ventricle and the pulmonary vasculature in patients with connective tissue disease. We assessed the effect of intravenous sildenafil on the hemodynamic response to exercise in patients with connective tissue disease. METHODS: In this proof-of-concept study, patients with connective tissue disease and mean pulmonary arterial pressure (mPAP) >20 mm Hg were subjected to a supine exercise hemodynamic evaluation before and after administration of intravenous sildenafil 10 mg. RESULTS: Ten patients (four with moderately elevated mPAP 21-24 mm Hg; six with mPAP >25 mm Hg) underwent hemodynamic assessment. All of them showed markedly abnormal exercise hemodynamics. Intravenous sildenafil was well tolerated and had significant hemodynamic effects at rest and during exercise, although without pulmonary selectivity. Sildenafil reduced median total pulmonary resistance during exercise from 6.22 (IQR 4.61-8.54) to 5.24 (3.95-6.96) mm Hg·min·L-1 (p = 0.005) and increased median pulmonary arterial capacitance during exercise from 1.59 (0.93-2.28) to 1.74 (1.12-2.69) mL/mm Hg (p = 0.005). CONCLUSIONS: In patients with connective tissue disease who have an abnormal hemodynamic response to exercise, intravenous sildenafil improved adaption of the right ventricular-pulmonary vascular unit to exercise independent of resting mPAP. The impact of acute pharmacological interventions on exercise hemodynamics in patients with pulmonary vascular disease warrants further investigation. TRIAL REGISTRATION: Clinicaltrials.gov NCT01889966.


Assuntos
Pressão Arterial/efeitos dos fármacos , Doenças do Tecido Conjuntivo/fisiopatologia , Exercício Físico/fisiologia , Hemodinâmica/efeitos dos fármacos , Citrato de Sildenafila/administração & dosagem , Vasodilatadores/administração & dosagem , Idoso , Pressão Arterial/fisiologia , Teste de Esforço , Feminino , Hemodinâmica/fisiologia , Humanos , Injeções Intravenosas , Masculino , Pessoa de Meia-Idade
20.
Turk J Med Sci ; 48(4): 826-832, 2018 Aug 16.
Artigo em Inglês | MEDLINE | ID: mdl-30119159

RESUMO

Background/aim: Diffuse parenchymal lung diseases (DPLDs) comprise a broad, heterogeneous group of diseases with common functional characteristics and a common final pathway, usually leading to irreversible fibrosis. We investigated the effects of the physiological and functional parameters and of pulmonary hypertension (PH) on survival in DPLDs. Materials and methods: The study included 158 patients with DPLDs. Patient data were examined retrospectively, and survival status was obtained through phone calls. Results: Patients were divided into five groups according to their diagnosis: idiopathic pulmonary fibrosis (IPF), non-IPF idiopathic interstitial pneumonias, connective tissue diseases, sarcoidosis, and other DPLDs. Median survival was 42.9 months. The significant negative effects of older age, presence of delta saturation (DeltaSat; difference between oxygen saturation at rest and after the 6-min walking test), 6-min walking distance (<350 m), systolic pulmonary artery pressure (sPAP; ≥50 mmHg), and baseline percentage of diffusing capacity of the lungs for carbon monoxide (<80%) with percentage of forced vital capacity (FVC%; <80%) were detected on survival (P < 0.05). A one-unit decrease in FVC% was related to a 6% increase in mortality. Another unique finding indicated that higher DeltaSat (>10%) correlated strongly with sPAP (>50 mmHg) and thus with a worse survival rate. Conclusion: The current study determined that FVC% is important in the prediction of mortality. Moreover, it demonstrated a strong relationship between exercise desaturation and PH.


Assuntos
Monóxido de Carbono/metabolismo , Exercício Físico/fisiologia , Pneumopatias/mortalidade , Pulmão/fisiopatologia , Oxigênio/metabolismo , Capacidade Vital , Fatores Etários , Idoso , Pressão Sanguínea , Doenças do Tecido Conjuntivo/metabolismo , Doenças do Tecido Conjuntivo/mortalidade , Doenças do Tecido Conjuntivo/fisiopatologia , Teste de Esforço , Feminino , Fibrose , Humanos , Concentração de Íons de Hidrogênio , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/mortalidade , Fibrose Pulmonar Idiopática/metabolismo , Fibrose Pulmonar Idiopática/mortalidade , Fibrose Pulmonar Idiopática/fisiopatologia , Pneumopatias/metabolismo , Pneumopatias/fisiopatologia , Doenças Pulmonares Intersticiais/metabolismo , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Consumo de Oxigênio , Descanso , Estudos Retrospectivos , Sarcoidose/metabolismo , Sarcoidose/mortalidade , Sarcoidose/fisiopatologia , Análise de Sobrevida , Caminhada
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