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1.
J Clin Rheumatol ; 27(1): 40-41, 2021 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-33347033

RESUMO

BACKGROUND/AIMS: A role for Helicobacter pylori in triggering systemic sclerosis (SSc) has been proposed, but data are conflicting. In previous studies, infection has been generally searched for by using serology. We designed this study to assess H. pylori prevalence in SSc patients with histology of gastric mucosa, considered the criterion standard for infection diagnosis. METHODS: This cross-sectional study enrolled 30 SSc patients who complained of upper gastrointestinal symptoms. All underwent upper endoscopy with gastric biopsies. Endoscopic alterations were recorded, and gastric mucosa biopsies were used for both histological examination and searching for H. pylori. The role for proton-pump inhibitor (PPI) therapy was considered. Fisher exact test was used for statistical analysis. RESULTS: Data of 28 SSc patients were available, 14 with ongoing PPI therapy. Helicobacter pylori infection at histology was detected in 14.3% patients, and it equally occurred in patients with or without PPI therapy. Erosive esophagitis/Barrett esophagus was detected in 26.6% of cases. Among patients with PPI therapy, 30% received half dose only. The prevalence of intestinal metaplasia was low (14.3%). Endoscopic esophageal alterations were significantly more frequent in those patients showing anti-Scl70 antibody positivity. CONCLUSIONS: This study showed that prevalence of H. pylori is very low in SSc patients, so that it seems not having a role in triggering SSc. Management of gastroesophageal diseases in SSc patients needs to be improved, and looking to the autoimmune profile may be of help. Thus, collaboration between rheumatologist and gastroenterologist is highly recommended.


Assuntos
Infecções por Helicobacter/patologia , Helicobacter pylori/isolamento & purificação , Proteínas Nucleares/imunologia , Inibidores da Bomba de Prótons/uso terapêutico , Escleroderma Sistêmico , Trato Gastrointestinal Superior , Autoanticorpos/sangue , Esôfago de Barrett/patologia , Biópsia/métodos , Endoscopia do Sistema Digestório/métodos , Feminino , Mucosa Gástrica/microbiologia , Mucosa Gástrica/patologia , Refluxo Gastroesofágico/tratamento farmacológico , Infecções por Helicobacter/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Medição de Risco , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/microbiologia , Escleroderma Sistêmico/fisiopatologia , Trato Gastrointestinal Superior/diagnóstico por imagem , Trato Gastrointestinal Superior/microbiologia , Trato Gastrointestinal Superior/patologia
2.
High Blood Press Cardiovasc Prev ; 27(6): 597-599, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33006010

RESUMO

Systemic sclerosis (SSc) is a rare autoimmune disease that causes fibrosis in the skin and subcutaneous tissue, involving other organs such as the heart, lungs, kidneys, and gastrointestinal tract. Additionally, it can cause pulmonary arterial hypertension. Scleroderma renal crisis (SRC) is one of the most dreadful complications of SSc. SRC is a medical emergency that can present as a clinical picture of hypertensive encephalopathy. The pathophysiology involves an abrupt onset of moderate to severe hypertension that ranges from days to weeks; it is associated with an increase in plasma renin activity and acute kidney injury. It is known that by introducing angiotensin-converting enzyme inhibitors, the mortality decreases significantly in SRC. The renal biopsy plays an important role on the diagnosis and opportune treatment. We present a clinical case of SRC with a typical presentation of hypertensive emergency and acute kidney injury.


Assuntos
Lesão Renal Aguda/etiologia , Pressão Sanguínea , Hipertensão/etiologia , Escleroderma Sistêmico/complicações , Lesão Renal Aguda/diagnóstico , Lesão Renal Aguda/fisiopatologia , Lesão Renal Aguda/terapia , Administração Intravenosa , Idoso , Inibidores da Enzima Conversora de Angiotensina/administração & dosagem , Anti-Hipertensivos/administração & dosagem , Bloqueadores dos Canais de Cálcio/administração & dosagem , Emergências , Feminino , Humanos , Hipertensão/diagnóstico , Hipertensão/tratamento farmacológico , Hipertensão/fisiopatologia , Diálise Renal , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/fisiopatologia , Resultado do Tratamento , Vasodilatadores/administração & dosagem
3.
Lancet Respir Med ; 8(10): 963-974, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32866440

RESUMO

BACKGROUND: A phase 2 trial of tocilizumab showed preliminary evidence of efficacy in systemic sclerosis. We assessed skin fibrosis and systemic sclerosis-associated interstitial lung disease (SSc-ILD) in a phase 3 trial to investigate the safety and efficacy of tocilizumab, an anti-interleukin-6 receptor antibody, in the treatment of systemic sclerosis. METHODS: In this multicentre, randomised, double-blind, placebo-controlled, phase 3 trial, participants were recruited from 75 sites in 20 countries across Europe, North America, Latin America, and Japan. Adults with diffuse cutaneous systemic sclerosis for 60 months or less and a modified Rodnan skin score (mRSS) of 10-35 at screening were randomly assigned (1:1) with a voice-web-response system to receive subcutaneous tocilizumab 162 mg or placebo weekly for 48 weeks, stratified by IL-6 levels; participants and investigators were masked to treatment group. The primary endpoint was the difference in change from baseline to week 48 in mRSS. Percentage of predicted forced vital capacity (FVC% predicted) at week 48, time to treatment failure, and patient-reported and physician-reported outcomes were secondary endpoints. This trial is registered with ClinicalTrials.gov (number NCT02453256) and is closed to accrual. FINDINGS: Between Nov 20, 2015, and Feb 14, 2017, 210 individuals were randomly assigned to receive tocilizumab (n=104) or placebo (n=106). In the intention-to-treat population, least squares mean [LSM] change from baseline to week 48 in mRSS was -6·14 for tocilizumab and -4·41 for placebo (adjusted difference -1·73 [95% CI -3·78 to 0·32]; p=0·10). The shift in distribution of change from baseline in FVC% predicted at week 48 favoured tocilizumab (van Elteren nominal p=0·002 vs placebo), with a difference in LSM of 4·2 (95% CI 2·0-6·4; nominal p=0·0002), as did time to treatment failure (hazard ratio 0·63 [95% CI 0·37-1·06]; nominal p=0·08). Change in LSM from baseline to week 48 in Health Assessment Questionnaire-Disability Index and in patient-global and physician-global visual analogue scale assessments did not differ between tocilizumab and placebo. In the safety set, infections were the most common adverse events (54 [52%] of 104 participants in the tocilizumab group, 53 [50%] of 106 in the placebo group). Serious adverse events were reported in 13 participants treated with tocilizumab and 18 with placebo, primarily infections (three events, eight events) and cardiac events (two events, seven events). INTERPRETATION: The primary skin fibrosis endpoint was not met. Findings for the secondary endpoint of FVC% predicted indicate that tocilizumab might preserve lung function in people with early SSc-ILD and elevated acute-phase reactants. Safety was consistent with the known profile of tocilizumab. FUNDING: F Hoffmann-La Roche Ltd.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Escleroderma Sistêmico/tratamento farmacológico , Adulto , Estudos de Coortes , Método Duplo-Cego , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Capacidade Vital
4.
Clin Rheumatol ; 39(11): 3195-3204, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32852623

RESUMO

INTRODUCTION: Covid-19 infection poses a serious challenge for immune-compromised patients with inflammatory autoimmune systemic diseases. We investigated the clinical-epidemiological findings of 1641 autoimmune systemic disease Italian patients during the Covid-19 pandemic. METHOD: This observational multicenter study included 1641 unselected patients with autoimmune systemic diseases from three Italian geographical areas with different prevalence of Covid-19 [high in north (Emilia Romagna), medium in central (Tuscany), and low in south (Calabria)] by means of telephone 6-week survey. Covid-19 was classified as (1) definite diagnosis of Covid-19 disease: presence of symptomatic Covid-19 infection, confirmed by positive oral/nasopharyngeal swabs; (2) highly suspected Covid-19 disease: presence of highly suggestive symptoms, in absence of a swab test. RESULTS: A significantly higher prevalence of patients with definite diagnosis of Covid-19 disease, or with highly suspected Covid-19 disease, or both the conditions together, was observed in the whole autoimmune systemic disease series, compared to "Italian general population" (p = .030, p = .001, p = .000, respectively); and for definite + highly suspected diagnosis of Covid-19 disease, in patients with autoimmune systemic diseases of the three regions (p = .000, for all comparisons with the respective regional general population). Moreover, significantly higher prevalence of definite + highly suspected diagnosis of Covid-19 disease was found either in patients with various "connective tissue diseases" compared to "inflammatory arthritis group" (p < .000), or in patients without ongoing conventional synthetic disease-modifying anti-rheumatic drugs treatments (p = .011). CONCLUSIONS: The finding of a higher prevalence of Covid-19 in patients with autoimmune systemic diseases is particularly important, suggesting the need to develop valuable prevention/management strategies, and stimulates in-depth investigations to verify the possible interactions between Covid-19 infection and impaired immune-system of autoimmune systemic diseases. Key Points • Significantly higher prevalence of Covid-19 is observed in a large series of patients with autoimmune systemic diseases compared to the Italian general population, mainly due to patients' increased susceptibility to infections and favored by the high exposure to the virus at medical facilities before the restriction measures on individual movement. • The actual prevalence of Covid-19 in autoimmune systemic diseases may be underestimated, possibly due to the wide clinical overlapping between the two conditions, the generally mild Covid-19 disease manifestations, and the limited availability of virological testing. • Patients with "connective tissue diseases" show a significantly higher prevalence of Covid-19, possibly due to deeper immune-system impairment, with respect to "inflammatory arthritis group". • Covid-19 is more frequent in the subgroup of autoimmune systemic diseases patients without ongoing conventional synthetic disease-modifying anti-rheumatic drugs, mainly hydroxyl-chloroquine and methotrexate, which might play some protective role against the most harmful manifestations of Covid-19.


Assuntos
Doenças Autoimunes/epidemiologia , Infecções por Coronavirus/epidemiologia , Pneumonia Viral/epidemiologia , Doenças Reumáticas/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Antirreumáticos/uso terapêutico , Artrite Psoriásica/tratamento farmacológico , Artrite Psoriásica/epidemiologia , Artrite Psoriásica/fisiopatologia , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/epidemiologia , Artrite Reumatoide/fisiopatologia , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/fisiopatologia , Betacoronavirus , Infecções por Coronavirus/fisiopatologia , Dermatomiosite/tratamento farmacológico , Dermatomiosite/epidemiologia , Dermatomiosite/fisiopatologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Itália/epidemiologia , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/fisiopatologia , Doenças Reumáticas/tratamento farmacológico , Doenças Reumáticas/fisiopatologia , Escleroderma Sistêmico/tratamento farmacológico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/fisiopatologia , Síndrome de Sjogren/tratamento farmacológico , Síndrome de Sjogren/epidemiologia , Síndrome de Sjogren/fisiopatologia , Espondilite Anquilosante/tratamento farmacológico , Espondilite Anquilosante/epidemiologia , Espondilite Anquilosante/fisiopatologia , Doenças do Tecido Conjuntivo Indiferenciado/tratamento farmacológico , Doenças do Tecido Conjuntivo Indiferenciado/epidemiologia , Doenças do Tecido Conjuntivo Indiferenciado/fisiopatologia
5.
Ann Rheum Dis ; 79(9): 1210-1217, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32606043

RESUMO

OBJECTIVE: To prospectively investigate whether differences in pulmonary vasculature exist in systemic sclerosis (SSc) and how they are distributed in patients with different pulmonary function. METHODS: Seventy-four patients with SSc undergoing chest CT scan for interstitial lung disease (ILD) screening or follow-up were prospectively enrolled. A thorough clinical, laboratory and functional evaluation was performed the same day. Chest CT was spirometry gated at total lung capacity and images were analysed by two automated software programs to quantify emphysema, ILD patterns (ground-glass, reticular, honeycombing), and pulmonary vascular volume (PVV). Patients were divided in restricted (FVC% <80, DLco%<80), isolated DLco% reduction (iDLco- FVC%≥80, DLco%<80) and normals (FVC%≥80, DLco%≥80). Spearman ρ, Mann-Whitney tests and logistic regressions were used to assess for correlations, differences among groups and relationships between continuous variables. RESULTS: Absolute and lung volume normalised PVV (PVV/LV) correlated inversely with functional parameters and positively with all ILD patterns (ρ=0.75 with ground glass, ρ=0.68 with reticular). PVV/LV was the only predictor of DLco at multivariate analysis (p=0.007). Meanwhile, the reticular pattern prevailed in peripheral regions and lower lung thirds, PVV/LV prevailed in central regions and middle lung thirds. iDLco group had a significantly higher PVV/LV (2.2%) than normal (1.6%), but lower than restricted ones (3.8%). CONCLUSIONS: Chest CT in SSc detects a progressive increase in PVV/LV as DLco decreases. Redistribution of perfusion to less affected lung regions rather than angiogenesis nearby fibrotic lung may explain the results. Further studies to ascertain whether the increase in PVV/LV reflects a real increase in blood volume are needed.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico por imagem , Pulmão/irrigação sanguínea , Escleroderma Sistêmico/diagnóstico por imagem , Espirometria/estatística & dados numéricos , Tomografia Computadorizada por Raios X/estatística & dados numéricos , Adulto , Feminino , Humanos , Modelos Logísticos , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/etiologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Testes de Função Respiratória , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/fisiopatologia , Espirometria/métodos , Estatísticas não Paramétricas , Tomografia Computadorizada por Raios X/métodos , Capacidade Vital
6.
Microvasc Res ; 131: 104029, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32531354

RESUMO

OBJECTIVE: Finger systolic blood pressure measurement (FSBP) has been shown helpful in the detection of distal arterial insufficiency in upper limbs. This work assesses the possibility to measure FSBP on the 2nd phalanx instead of the first one in order to improve its sensitivity and to verify this would not alter the repeatability of the measurement. METHODS: In this multicenter study, FSBP was measured twice in all fingers but the thumbs in consecutive systemic sclerosis patients on the first phalanx and the second phalanx in alternate order using laser-Doppler flowmetry. RESULTS: Thirty-seven patients were enrolled. The repeatability of FSBP was excellent and similar on the first and 2nd phalanxes with coefficients of variation respectively of 7.1% and 7.6%. While the correlation between the FSBP at the two sites was fair (Pearson coefficient 0.69; p < 0.001). The agreement was poor, with a mean difference of 14 mm Hg between the two sites. Significantly higher differences were found in fingers with digital ulcers. The ROC curves showed a better prediction of the 2nd phalanx measurements. CONCLUSION: FSBP has an excellent repeatability whatever the site of phalanx. However, measurements performed on the 2nd phalanx have a better sensitivity for the prediction of digital ulcers.


Assuntos
Determinação da Pressão Arterial/métodos , Pressão Sanguínea , Dedos/irrigação sanguínea , Fluxometria por Laser-Doppler , Escleroderma Sistêmico/diagnóstico , Úlcera Cutânea/diagnóstico , Velocidade do Fluxo Sanguíneo , Humanos , Estudos Longitudinais , Paris , Valor Preditivo dos Testes , Estudos Prospectivos , Reprodutibilidade dos Testes , Escleroderma Sistêmico/fisiopatologia , Úlcera Cutânea/fisiopatologia , Fatores de Tempo
7.
Adv Exp Med Biol ; 1253: 375-403, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32445102

RESUMO

Scleroderma (systemic sclerosis; SSc) is a complex and highly heterogeneous multisystem rheumatic disease characterized by vascular abnormality, immunologic derangement, and excessive deposition of extracellular matrix (ECM) proteins. To date, the etiology of this life-threatening disorder remains not fully clear. More and more studies show epigenetic modifications play a vital role. The aberrant epigenetic status of certain molecules such as Fli-1, BMPRII, NRP1, CD70, CD40L, CD11A, FOXP3, KLF5, DKK1, SFRP1, and so on contributes to the pathogenesis of progressive vasculopathy, autoimmune dysfunction, and tissue fibrosis in SSc. Meanwhile, numerous miRNAs including miR-21, miR-29a, miR-196a, miR-202-3p, miR-150, miR-let-7a, and others are involved in the process. In addition, the abnormal epigenetic biomarker levels of CD11a, Foxp3, HDAC2, miR-30b, miR-142-3p, miR-150, miR-5196 in SSc are closely correlated with disease severity. In this chapter, we not only review new advancements on the epigenetic mechanisms involved in the pathogenesis of SSc and potential epigenetic biomarkers, but also discuss the therapeutic potential of epigenetic targeting therapeutics such as DNA methylation inhibitors, histone acetylase inhibitors, and miRNA replacement.


Assuntos
Epigênese Genética , Escleroderma Sistêmico/genética , Escleroderma Sistêmico/terapia , Biomarcadores , Humanos , MicroRNAs , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/fisiopatologia
8.
Arthritis Rheumatol ; 72(7): 1049-1058, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32134199

RESUMO

Systemic sclerosis (SSc) is an autoimmune rheumatic disease with heterogeneous clinical manifestations and a variable course in which the severity of the pathology dictates the disease prognosis and course. Among autoimmune rheumatic diseases, SSc has the highest mortality rate among all rheumatic diseases, though there are exciting new therapeutic targets that appear to halt the progression of SSc manifestations such as skin or lung fibrosis. In selected patients, high-intensity regimens with autologous stem cell transplantation can favorably modify the course. In what was once thought to be an untreatable disease, targeted therapies have now changed the outlook of SSc to a treatable disorder. Herein, we discuss the targeted therapies modifying the outlook on selected organ involvement and creating opportunities for future treatment. We also present a framework for defining low disease activity in SSc.


Assuntos
Cardiopatias/terapia , Nefropatias/terapia , Doenças Pulmonares Intersticiais/terapia , Hipertensão Arterial Pulmonar/terapia , Doença de Raynaud/terapia , Escleroderma Sistêmico/terapia , Úlcera Cutânea/terapia , Doença Aguda , Anti-Hipertensivos/uso terapêutico , Progressão da Doença , Antagonistas dos Receptores de Endotelina/uso terapêutico , Fibrose , Dedos , Cardiopatias/etiologia , Cardiopatias/fisiopatologia , Transplante de Células-Tronco Hematopoéticas , Humanos , Imunossupressores/uso terapêutico , Nefropatias/etiologia , Nefropatias/fisiopatologia , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Miocárdio/patologia , Avaliação de Resultados em Cuidados de Saúde , Prostaglandinas I/uso terapêutico , Hipertensão Arterial Pulmonar/etiologia , Hipertensão Arterial Pulmonar/fisiopatologia , Doença de Raynaud/etiologia , Doença de Raynaud/fisiopatologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/fisiopatologia , Índice de Gravidade de Doença , Úlcera Cutânea/etiologia , Úlcera Cutânea/fisiopatologia
9.
Autoimmun Rev ; 19(5): 102515, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32173517

RESUMO

Systemic sclerosis (SSc) is an autoimmune disease which is characterized by vasculopathy, tissue fibrosis and activation of the innate and adaptive immune system. Clinical features of the disease consists of skin thickening and internal organ involvement. Due to the heterogeneous nature of the disease it is difficult to predict disease progression and complications. Despite the discovery of novel autoantibodies associated with SSc, there is an unmet need for biomarkers for diagnosis, disease progression and response to treatment. To date, the use of single (surrogate) biomarkers for these purposes has been unsuccessful. Combining multiple biomarkers in to predictive panels or ultimately algorithms could be more precise. Given the limited therapeutic options and poor prognosis of many SSc patients, a better understanding of the immune-pathofysiological profiles might aid to an adjusted therapeutic approach. Therefore, we set out to explore immunological fingerprints in various clinically defined forms of SSc. We used multilayer profiling to identify unique immune profiles underlying distinct autoantibody signatures. These immune profiles could fill the unmet need for prognosis and response to therapy in SSc. Here, we present 3 pathophysiological fingerprints in SSc based on the expression of circulating antibodies, vascular markers and immunomodulatory mediators.


Assuntos
Medicina de Precisão , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/fisiopatologia , Autoanticorpos/imunologia , Biomarcadores/análise , Feminino , Fibrose/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Escleroderma Sistêmico/imunologia , Escleroderma Sistêmico/patologia
10.
Rev Assoc Med Bras (1992) ; 66(1): 48-54, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32130381

RESUMO

INTRODUCTION: Systemic sclerosis (SSC) is an autoimmune disorder that affects several organs of unknown etiology, characterized by vascular damage and fibrosis of the skin and organs. Among the organs involved are the esophagus and the lung. OBJECTIVES: To relate the profile of changes in esophageal electromanometry (EM), the profile of skin involvement, interstitial pneumopathy (ILD), and esophageal symptoms in SSC patients. METHODS: This is an observational, cross-sectional study carried out at the SSC outpatient clinic of the Hospital de Clínicas of the Federal University of Uberlândia. After approval by the Ethics Committee and signed the terms of consent, 50 patients were initially enrolled, from 04/12/2014 to 06/25/2015. They were submitted to the usual investigations according to the clinical picture. The statistical analysis was descriptive in percentage, means, and standard deviation. The Chi-square test was used to evaluate the relationship between EM, high-resolution tomography, and esophageal symptoms. RESULTS: 91.9% of the patients had some manometric alterations. 37.8% had involvement of the esophageal body and lower esophageal sphincter. 37.8% had ILD. 24.3% presented the diffuse form of SSC. No association was found between manometric changes and clinical manifestations (cutaneous, pulmonary, and gastrointestinal symptoms). CONCLUSION: The present study confirms that esophageal motility alterations detected by EM are frequent in SSC patients, but may not be related to cutaneous extension involvement, the presence of ILD, or the gastrointestinal complaints of patients.


Assuntos
Transtornos da Motilidade Esofágica/fisiopatologia , Esôfago/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Manometria/métodos , Escleroderma Sistêmico/fisiopatologia , Adulto , Idoso , Estudos Transversais , Ensaio de Imunoadsorção Enzimática , Transtornos da Motilidade Esofágica/complicações , Transtornos da Motilidade Esofágica/diagnóstico por imagem , Esfíncter Esofágico Inferior/patologia , Esfíncter Esofágico Inferior/fisiopatologia , Esôfago/diagnóstico por imagem , Esôfago/patologia , Feminino , Hemaglutinação , Humanos , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos
11.
Autoimmun Rev ; 19(4): 102494, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32062031

RESUMO

Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease which is characterised by autoimmunity, widespread tissue fibrosis of the skin and internal organs, and vasculopathic alterations. SSc is more common in women but has a more severe expression of disease including internal organ-based complications and higher mortality in men. The extant literature shows that although important pathophysiological sex differences are present in SSc, behavioural differences (e.g. higher smoking rates in men) and occupational exposures may contribute to poorer outcomes in men with SSc. The higher death male death rate in the general population and greater prevalence of lung fibrosis are likely the key factors responsible for excess mortality found in men. Other important factors include (but are not limited to) a greater prevalence of the disease subset, delayed time to diagnosis, and higher disease activity in early disease in men. SSc carries a significant burden of disease-related morbidity; however, no qualitative studies to date have focussed on gender differences in SSc. The purpose of this review is to provide a comprehensive overview of gender differences in SSc including (but not limited to) epidemiology, pathophysiology, clinical expression of disease, mortality, SSc in transgender individuals, and psychosocial aspects of disease.


Assuntos
Escleroderma Sistêmico , Caracteres Sexuais , Feminino , Fibrose , Humanos , Masculino , Morbidade , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/fisiopatologia
12.
Sci Rep ; 10(1): 2561, 2020 02 13.
Artigo em Inglês | MEDLINE | ID: mdl-32054932

RESUMO

The clinical assessment of fibrosis is critical to the diagnosis and management of patients with systemic sclerosis. Current clinical standards for patient assessment is to use skin fibrosis as an indicator of organ involvement, though this approach is highly subjective and relies on manual palpation. The development of a new method for accurately quantifying collagen content may therefore significantly improve the accuracy of the traditional skin score in patients with systemic sclerosis and may additionally aid in the monitoring of anti-fibrotic therapies in clinical practice. Polarization-sensitive optical coherence tomography (PS-OCT) is a high-speed volumetric imaging modality that can be used to assess birefringent tissues including collagen. In this work we demonstrate a novel computational approach using PS-OCT for the assessment of fibrosis. This approach, based on the measured distribution of optic axis values associated with a given volume of collagen orientation, characterizes fibrotic changes independently from the depth of the region of interest in the tissue. This approach has the potential to accurately quantify collagen content and orientation faster and more robustly compared to traditional PS-OCT metrics. We investigate the viability of this approach for assessing the development of fibrosis in a bleomycin induced skin fibrosis mouse model.


Assuntos
Olho/fisiopatologia , Processamento de Imagem Assistida por Computador/métodos , Escleroderma Sistêmico/fisiopatologia , Tomografia de Coerência Óptica/métodos , Animais , Bleomicina/toxicidade , Colágeno/metabolismo , Colágeno/ultraestrutura , Progressão da Doença , Olho/diagnóstico por imagem , Fibrose/induzido quimicamente , Fibrose/diagnóstico por imagem , Fibrose/fisiopatologia , Humanos , Camundongos , Refração Ocular/fisiologia , Escleroderma Sistêmico/diagnóstico por imagem
13.
Int J Mol Sci ; 21(4)2020 Feb 16.
Artigo em Inglês | MEDLINE | ID: mdl-32079137

RESUMO

Autonomic neuropathy has been reported in autoimmune rheumatic diseases (ARD) including Sjögren's syndrome, systemic sclerosis, rheumatoid arthritis, and systemic lupus erythematosus. However, the pathophysiological mechanism underlying autonomic dysfunction remains unknown to researchers. On the other hand, autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder, which causes dysautonomia that is mediated by autoantibodies against ganglionic acetylcholine receptors (gAChRs). The purpose of this review was to describe the characteristics of autonomic disturbance through previous case reports and the functional tests used in these studies and address the importance of anti-gAChR antibodies. We have established luciferase immunoprecipitation systems to detect antibodies against gAChR in the past and determined the prevalence of gAChR antibodies in various autoimmune diseases including AAG and rheumatic diseases. Autonomic dysfunction, which affects lower parasympathetic and higher sympathetic activity, is usually observed in ARD. The anti-gAChR antibodies may play a crucial role in autonomic dysfunction observed in ARD. Further studies are necessary to determine whether anti-gAChR antibody levels are correlated with the severity of autonomic dysfunction in ARD.


Assuntos
Autoanticorpos/imunologia , Doenças Autoimunes do Sistema Nervoso/fisiopatologia , Gânglios Autônomos/fisiopatologia , Receptores Colinérgicos/imunologia , Doenças Reumáticas/fisiopatologia , Animais , Artrite Reumatoide/imunologia , Artrite Reumatoide/fisiopatologia , Doenças Autoimunes do Sistema Nervoso/imunologia , Sistema Nervoso Autônomo/imunologia , Sistema Nervoso Autônomo/fisiopatologia , Gânglios Autônomos/imunologia , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Doenças Reumáticas/imunologia , Escleroderma Sistêmico/imunologia , Escleroderma Sistêmico/fisiopatologia , Síndrome de Sjogren/imunologia , Síndrome de Sjogren/fisiopatologia
14.
Int J Cardiovasc Imaging ; 36(5): 883-888, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32060775

RESUMO

This study examined the relationship between global longitudinal strain (GLS) and pulmonary function tests (PFT) in patients with systemic sclerosis (SS) and normal ejection fraction (EF) and pulmonary artery pressure (PAP) and healthy controls. Sixty patients in two groups underwent extensive screening, including echocardiography, physical examination, the modified Rodnan Skin Score, and pulmonary function tests. Pulmonary interstitial disease was diagnosed by the pulmonary function test and by CT scan in case of indication. GLS score was computed as the mean peak systolic strain for 17 segments. The mean GLS score was - 18.36 ± 2.1 in the case group and - 20.66 ± 1.6 in the control group (P value < 0.001). GLS scores had a significant inverse relationship with the forced expiratory volume in one second (FEV1) to forced vital capacity (FVC) ratio (P value = 0.049) and both FEV and FVC in patients younger than 35 years old (P = 0.046 and 0.049, respectively). GLS scores had no significant relationship with time elapsed since the onset of skin manifestations, and Raynaud phenomenon, Rodnan score, EF, systolic PAP, or the six-minute walk test results. The patients' six-minute walk test had a significant positive relationship with FVC and right ventricular end diastolic diameter (P value = 0.018 and 0.047, respectively). According to our findings, GLS is significantly lower in patients with SS (with normal EF & PAP) than in healthy individuals. It is also related with certain pulmonary function indices including FEV1/FVC. The reduction in GLS is associated with reduced pulmonary function strength.


Assuntos
Pressão Arterial , Doenças Pulmonares Intersticiais/etiologia , Pulmão/irrigação sanguínea , Contração Miocárdica , Hipertensão Arterial Pulmonar/etiologia , Artéria Pulmonar/fisiopatologia , Escleroderma Sistêmico/complicações , Disfunção Ventricular Esquerda/etiologia , Função Ventricular Esquerda , Adulto , Estudos de Casos e Controles , Tolerância ao Exercício , Feminino , Volume Expiratório Forçado , Humanos , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Hipertensão Arterial Pulmonar/diagnóstico , Hipertensão Arterial Pulmonar/fisiopatologia , Fatores de Risco , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/fisiopatologia , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Direita , Capacidade Vital
15.
Scand J Rheumatol ; 49(3): 239-243, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-31928291

RESUMO

Objective: Despite being a cardinal clinical sign of systemic sclerosis (SSc), digital pitting has been little studied. Our objective was to test, in a pilot study, the hypothesis that pitting is painful and associated with digital vascular disease severity.Method: Fifty patients with SSc were recruited: 25 with and 25 without digital pitting. Fingertip pain was assessed on a 0-10 scale. Thermography of both hands assessed surface temperature, allowing calculation of the distal-dorsal difference (temperature gradient) for each finger. Nailfold capillaroscopy was performed in each finger using a dermatoscope, and graded on a 0-3 scale (0 = normal; 3 = grossly abnormal).Results: In the 25 patients with digital pitting, 65 fingers in total were affected (mainly the index and middle fingers). Pain scores were higher in 'pitting' patients [median 4 (interquartile range 3-8) vs 0 (0-2), p < 0.001], and pitting patients reported that pitting impacted on activities of everyday living. Temperature gradients along the fingers did not differ significantly between patients with and without pitting (p = 0.248). Pitting patients were more likely to have 'grossly abnormal' capillaries than those without pitting, and less likely to have 'no/mild' nailfold capillary changes.Conclusions: Digital pitting is painful and impacts on hand function. Capillaroscopy findings provide further support for an association between pitting and severity of digital vascular change. Larger, more comprehensive studies are required to examine the pathophysiology of pitting and to pave the way to therapeutic intervention, ideally including preventive strategies.


Assuntos
Dedos/fisiopatologia , Dor/fisiopatologia , Esclerodermia Difusa/fisiopatologia , Esclerodermia Limitada/fisiopatologia , Idoso , Estudos de Casos e Controles , Feminino , Dedos/irrigação sanguínea , Dedos/patologia , Humanos , Masculino , Angioscopia Microscópica , Pessoa de Meia-Idade , Dor/etiologia , Projetos Piloto , Esclerodermia Difusa/complicações , Esclerodermia Difusa/patologia , Esclerodermia Limitada/complicações , Esclerodermia Limitada/patologia , Escleroderma Sistêmico/fisiopatologia , Venenos de Escorpião , Índice de Gravidade de Doença , Úlcera Cutânea/etiologia , Termografia
16.
Lupus ; 29(3): 225-235, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31933408

RESUMO

Chronic inflammation has profound tumor-promoting effects. Inflammatory cells are the key players in immunosurveillance against tumors, and immunosuppression is known to increase the risk of tumors. Autoimmune diseases, which manifest as loss of self-tolerance and chronic immune dysregulation, provide a perfect environment for tumor development. Aside from managing the direct inflammatory consequences of autoimmune pathogenesis, cancer risk profiles should be considered as a part of a patient's treatment. In this review, we describe the various associations of malignancies with autoimmune diseases, specifically systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, and Sjögren's syndrome, as well as discuss the mechanisms contributing to the pathogenesis of both disorders.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Neoplasias Hematológicas/etiologia , Doenças Autoimunes/complicações , Doenças do Tecido Conjuntivo/fisiopatologia , Neoplasias Hematológicas/fisiopatologia , Humanos , Inflamação/complicações , Inflamação/fisiopatologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/fisiopatologia , Doenças Reumáticas/complicações , Doenças Reumáticas/fisiopatologia , Fatores de Risco , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/fisiopatologia , Síndrome de Sjogren/complicações , Síndrome de Sjogren/fisiopatologia
17.
Rheumatology (Oxford) ; 59(3): 478-486, 2020 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-31943100

RESUMO

SSc is an autoimmune disease characterized by microvascular damage, endothelial dysfunction and fibrosis of the skin and the internal organs. Cardiac manifestation in patients with SSc is one of the major organ involvements. Approximately 20% of SSc patients suffer from primary cardiovascular disease and another 20% may have secondary cardiac involvement. Although cardiac arrhythmias are mostly linked to myocardial fibrosis, atrioventricular conduction abnormalities are secondary to the fibrosis of the pulse conduction system. Despite the severe consequences of ventricular rhythm disturbances in patients with SSc, the exact role of electrocardiographic markers in the prediction of these arrhythmias has not yet been clearly elucidated. Therefore, the question is whether certain ECG parameters reflecting ventricular repolarization may help to recognize scleroderma patients with increased risk for ventricular arrhythmias and sudden cardiac death.


Assuntos
Arritmias Cardíacas/diagnóstico , Escleroderma Sistêmico/complicações , Arritmias Cardíacas/complicações , Arritmias Cardíacas/fisiopatologia , Eletrocardiografia , Humanos , Escleroderma Sistêmico/fisiopatologia
19.
Acta Clin Belg ; 75(1): 19-25, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30376766

RESUMO

Objectives: Autoimmune diseases include a spectrum of disorders in which immune response to the autoantigens leads to tissue damage or dysfunction. Xerostomia, salivary gland dysfunction and lack of saliva are some common symptoms associated with many autoimmune diseases.Methods: In this review study, the meta-analysis technique is used to objectively review the relationship between autoimmune diseases and salivary gland dysfunction. We have searched Medline and Embase and Google Scholar. By Revman 5.3, meta-analysis was performed to investigate the salivary flow rate in both stimulatory and non-stimulatory saliva. The sample size obtained from these studies was 130 people with autoimmune diseases and 100 healthy individuals.Results: The results showed a significant decrease in the level of non-stimulatory saliva in people with autoimmune diseases.Conclusions: A complete and comprehensive understanding of the clinical manifestation of systemic diseases is crucial in early diagnosis of diseases and identifying the mechanisms that develop the disease. Other than xerostomia, there is a significant reduction in salivary flow rate in patients with autoimmune diseases. As saliva plays a very important role in oral health and has significant functions, more attention is needed for monitoring and managing of hyposalivation in autoimmune patients.


Assuntos
Doenças Autoimunes/fisiopatologia , Glândulas Salivares/fisiopatologia , Xerostomia/fisiopatologia , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Humanos , Doenças das Glândulas Salivares/complicações , Doenças das Glândulas Salivares/diagnóstico , Doenças das Glândulas Salivares/fisiopatologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/fisiopatologia , Xerostomia/diagnóstico , Xerostomia/etiologia
20.
Clin Rheumatol ; 39(1): 57-67, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31129793

RESUMO

INTRODUCTION: The aim of this study was to evaluate the associations between malnutrition and the clinical features of the disease and depression in patients with systemic sclerosis (SSc). METHOD: Patients with SSc who were followed up in our clinic were enrolled in the study. Malnutrition risk was assessed using the Malnutrition Universal Screening Tool (MUST). Skin involvement was assessed using the modified Rodnan skin score (mRSS) and interincisal distance (ID) measurements were used to assess the maximal mouth opening capacity. The Beck Depression Inventory (BDI) was used for measuring the severity of depression. RESULTS: Ninety-eight patients with SSc (84.7% women; mean age 52.67 ± 11.26 years) were included in the study. According to the MUST scores, 61.2%, 15.3%, and 23.5% of patients had low, medium, and high risk for malnutrition, respectively. The mRSS was significantly higher in the group with high malnutrition risk compared with low-risk group (p = 0.014). Malnutrition risk was associated with interstitial lung disease and bowel involvement (p = 0.044 and p = 0.021, respectively). Interincisal distance was lower in the group with high malnutrition risk compared with the low-risk group (p = 0.003). Malnutrition risk was higher in patients who had mild-to-severe depressive symptoms than in those without (p = 0.012). Interincisal distance and bowel involvement were the most relevant factors for malnutrition. CONCLUSIONS: The risk of malnutrition is increased in patients with SSc. In our study, microstomia and bowel involvement were the most relevantly associated factors with malnutrition. KEY POINTS: • The risk of malnutrition is increased in patients with systemic sclerosis (SSc). • Microstomia and bowel involvement are found to be the most important factors associated with malnutrition. • Depressive symptoms are seen frequently among patients with SSc, and depression seems to be one of the etiologic factors or the result of malnourishment in SSc. • Assessment of nutritional status and the presence of depression should be a part of routine clinical visits of patients with SSc.


Assuntos
Depressão/diagnóstico , Desnutrição/diagnóstico , Escleroderma Sistêmico/fisiopatologia , Escleroderma Sistêmico/psicologia , Adulto , Estudos Transversais , Depressão/epidemiologia , Feminino , Humanos , Modelos Logísticos , Masculino , Desnutrição/epidemiologia , Pessoa de Meia-Idade , Estado Nutricional , Escalas de Graduação Psiquiátrica , Fatores de Risco , Índice de Gravidade de Doença
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