Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 6.237
Filtrar
1.
Autoimmun Rev ; 19(1): 102431, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31734403

RESUMO

INTRODUCTION: Little is known about systemic sclerosis (SSc)-associated myopathy (SScAM) treatment. Herein we evaluated the use of intravenous immunoglobulin (IVIg) in SScAM. METHODS: We conducted a retrospective study of patients with SScAM in the Internal medicine department of Cochin University Hospital between 1993 and 2017. RESULTS: Fifty-two patients were included comprising 18 (34.6%) with limited SSc and 34 (65.4%) with diffuse SSc. SScAM occurred at a median [interquartile range (IQR)] time of 1 month [0-15] after SSc diagnosis. Thirty-four patients (65.4%) had muscle weakness, 28 (53.8%) had myalgia and 24 (46.2%) had dysphagia. Fifty patients (96.2%) had increased creatine kinase, 22/26 (84.6%) had myopathic electromyography, 10/12 (83.3%) had a high intensity signal of girdle muscles on MRI and 49/50 (98%) had abnormal muscle biopsy. Eighteen (34.6%) patients received IVIg. Severe adverse events occurred in 3/18 (16.7%) patients. When compared to patients who did not receive IVIg, patients who received IVIg had a significantly higher maximal corticosteroid (CS) dose ever, a greater decrease of CS at 3 months, and a lower CS dose at one year and at the end of follow up. CONCLUSIONS: This study suggests the benefit of IVIg as adjunctive therapy, with an acceptable tolerance profile, and supports its use as a CS-sparing agent, in SScAM.


Assuntos
Imunoglobulinas Intravenosas/uso terapêutico , Doenças Musculares/tratamento farmacológico , Escleroderma Sistêmico/complicações , Corticosteroides/uso terapêutico , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento
2.
Z Rheumatol ; 78(10): 967-978, 2019 Dec.
Artigo em Alemão | MEDLINE | ID: mdl-31712897

RESUMO

Raynaud's phenomenon (RP) is a frequent and painful vasospasm of small arteries localized in acral body regions (most frequently the fingers). The more frequent so-called primary RP is caused merely by a functional dysregulation of the tonus of vascular walls. In contrast, the rarer secondary RP is additionally associated with structural abnormalities of blood vessels. Knowledge of RP is important for rheumatologists because secondary RP can be associated with the presence or development of severe underlying diseases, especially with systemic sclerosis. Thus, the rheumatologist has to be aware of this condition. In this article the diagnostic procedures and the most important treatment approaches are summarized.


Assuntos
Doença de Raynaud , Escleroderma Sistêmico , Humanos , Doença de Raynaud/complicações , Doença de Raynaud/terapia , Reumatologistas , Escleroderma Sistêmico/complicações
3.
Internist (Berl) ; 60(12): 1251-1269, 2019 Dec.
Artigo em Alemão | MEDLINE | ID: mdl-31754753

RESUMO

Systemic sclerosis (SSc) is a rare fibrosing rheumatic multi-systemic disease involving many medical specialties. The mortality of SSc is determined by lung fibrosis, pulmonary arterial hypertension and cardiac involvement. With early and intensive treatment, the disease can be stabilized and symptoms relieved. This review summarizes insights into pathophysiology, disease classification, clinical manifestations and successful therapies, as well as recent studies on new immunosuppressant, biological and vasoactive therapies.


Assuntos
Hipertensão Pulmonar/fisiopatologia , Fibrose Pulmonar/fisiopatologia , Escleroderma Sistêmico/fisiopatologia , Humanos , Hipertensão Pulmonar/etiologia , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais/etiologia , Fibrose Pulmonar/etiologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/terapia
5.
Presse Med ; 48(10): e284-e291, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31653544

RESUMO

OBJECTIVE: The Prevalence of systemic sclerosis (SSc) and different clinical subsets varies across the world. Few data have been published on SSc patients in North Africa. Our objective was to describe a SSc cohort in south of Tunisia and to compare clinical findings, disease subsets and antibodies with other international SSc populations. METHODS: In this retrospective observational study, Folders of patients with SSc seen in the internal medicine section of the Hedi Chaker Hospital between 2000 and 2013 were retrospectively analyzed. The diagnosis of SSc was retained according to ACR/EULAR 2013 criteria. Patients were classified into diffuse cutaneous SSc and limited cutaneous SSc subsets. Comparison with other cohorts was made based on published information. RESULTS: A higher female: male ratio (8:1) and a higher diffuse subset prevalence (82%) was found in this Tunisian cohort comparing with others. We also found a lower prevalence of calcinosis and anticentromere antibodies. Within each subset, diffuse cutaneous and limited cutaneous scleroderma clinical findings were similar with other systemic sclerosis populations except for a very low prevalence in renal crisis and pulmonary hypertension. Our results indicate overlap syndrome defined as scleroderma associated with others connective tissue disorder's is a relatively common condition. CONCLUSION: With slight variations, perhaps due to genetic, environmental or referral factors, SSc in this cohort appears to be similar to that described in other part of the world.


Assuntos
Esclerodermia Limitada , Escleroderma Sistêmico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Calcinose/epidemiologia , Centrômero/imunologia , Estudos Transversais , DNA Topoisomerases Tipo I/imunologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Retrospectivos , Esclerodermia Limitada/complicações , Esclerodermia Limitada/diagnóstico , Esclerodermia Limitada/imunologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/imunologia , Tunísia , Adulto Jovem
6.
J Drugs Dermatol ; 18(10): 995-998, 2019 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-31584777

RESUMO

Background: Autoimmune connective tissue diseases (ACTDs) are a broad range of diseases featured by immune dysregulation, and often have multisystem involvement with prominent skin manifestations. Pruritus is one of the most common symptoms in these diseases, with significant impact on the quality of life of patients. Objective: To characterize the frequency, location, severity, and timing relative to disease onset of pruritus in different ACTDs. Methods: A chart review of all patients seen in the Rheumatology-Dermatology clinic at Massachusetts General Hospital. Results: Itch was a troubling symptom in 83% of dermatomyositis (DM), 61% of systemic lupus erythematosus (SLE), 59% of Sjogren syndrome (SJO), 22% of systemic sclerosis (SSc), and 60% of mixed connective tissue disease. In DM and SLE, itch paralleled the course of inflammatory skin manifestations in 83% and 45%, respectively. Itch in DM is more intense and more treatment resistant in 12% vs 1% in SLE. In contrast, itch in SSc and SJO tended to occur later in the disease course, 86% vs 42%, respectively. Conclusion: Itch is common in all ACTDs and often under-evaluated and under treated. Pruritus is more common and more severe in DM than in SLE. Treatment of pruritus in ACTDs can be challenging, and sometimes multi-modal therapy is warranted. J Drugs Dermatol. 2019;18(10):995-998.


Assuntos
Dermatomiosite/complicações , Lúpus Eritematoso Sistêmico/complicações , Prurido/diagnóstico , Escleroderma Sistêmico/complicações , Síndrome de Sjogren/complicações , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Terapia Combinada/métodos , Dermatomiosite/imunologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Pessoa de Meia-Idade , Prurido/imunologia , Prurido/terapia , Qualidade de Vida , Estudos Retrospectivos , Escleroderma Sistêmico/imunologia , Índice de Gravidade de Doença , Síndrome de Sjogren/imunologia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
7.
BMC Neurol ; 19(1): 234, 2019 Oct 14.
Artigo em Inglês | MEDLINE | ID: mdl-31607267

RESUMO

BACKGROUND: This article reports a case diagnosis of a 44-year-old female who presented with intractable hiccups and vomit complicated with an acute onset of paraplegia. Transverse myelitis was evident on MRI and serological studies were consistent with Neuromyelitis Optica (NMO) based on NMO-IgG sero-positivity. Further studies revealed positive ANA, anti-RNA polymerase III autoantibodies, and Scl-70, leading to a concurrent diagnosis of systemic sclerosis (SSc). The coexistence of these two disease processes and their underlying clinical manifestations and therapeutic interventions are seldom reported in literature and are worth reporting. CASE PRESENTATION: The patient was treated with high dose steroids, and subsequently developed malignant hypertension and acute renal failure, later identified on biopsy as steroids-induced scleroderma renal crisis. Although Neuromyelitis Optica spectrum disorder (NMOSD) has often been associated with various collagen and autoimmune diseases, the coexistence of NMOSD and SSc presented a challenge where the patient underwent aggressive physical therapy and necessitated an intervention with Rituximab to achieve an appropriate clinical response. We have received a written consent forms from the participant in our study, and we have them on file in case they are requested. We have also received the patient's written consent for the data and images presented in this article. CONCLUSION: This article expands on NMOSD associated autoimmune diseases. Systemic Sclerosis is an insidious disease that is often diagnosed late as not all patients often report skin manifestation. The finding suggests that patients presenting with acute neurological manifestations get tested for NMO-IgG/AQP-4 antibodies and other immunological studies based on clinical findings.


Assuntos
Mielite Transversa/etiologia , Neuromielite Óptica/complicações , Escleroderma Sistêmico/complicações , Adulto , Autoanticorpos/sangue , Feminino , Humanos , Fatores Imunológicos/uso terapêutico , Imagem por Ressonância Magnética , Mielite Transversa/tratamento farmacológico , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/tratamento farmacológico , Rituximab/uso terapêutico , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/tratamento farmacológico , Síndrome
9.
Clin Exp Rheumatol ; 37 Suppl 119(4): 108-114, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31587696

RESUMO

OBJECTIVES: The aim of our study was to assess the role of videofluorography (VFG) in the evaluation of swallowing and oesophageal peristalsis in patients with systemic sclerosis (SSc). METHODS: From June 2014 to September 2017, 55 consecutive SSc patients, defined according to the 2013 ACR/EULAR classification criteria, underwent VFG study using a remote-controlled digital device. In order to evaluate possible abnormalities, 18 dynamic parameters were chosen, dividing the act of swallowing into three phases: oral, pharyngeal and oesophageal phases. The following dynamic radiological findings were considered: veil motility in phonation, leakage, drooling, salivation and presence of residues in the oral cavity, pharyngeal residues, penetration, aspiration, altered motility of the upper oesophageal sphincter, efficacy of primary peristaltic contractions, oesophageal clearance capacity, reflux, oesophagitis and motility of the lower oesophageal sphincter. RESULTS: The VFG study was well tolerated in all patients. Dysfunctions of oesophageal motility were common and included abnormal motility of UES (12.7%) and LES (76.4%), inadequate primary peristalsis (52.7%), abnormal secondary peristalsis (29.1%) and non-peristaltic contractions (40%). A defective oesophageal clearance was observed in 69.4% of patients. Moreover, most patients presented signs of oesophageal reflux (63.6%), oesophagitis (81.8%) and hiatal hernia (80%). Pharyngeal abnormalities were less common and involved up to 50% of patients. Oesophageal dysfunction and defective clearance were associated with dcSSc and pulmonary involvement. CONCLUSIONS: The VFG study is a useful technique for the morphological and functional evaluation of swallowing in SSc patients.


Assuntos
Cinerradiografia/métodos , Transtornos de Deglutição , Fluoroscopia/métodos , Escleroderma Sistêmico , Transtornos de Deglutição/diagnóstico por imagem , Transtornos de Deglutição/etiologia , Feminino , Refluxo Gastroesofágico , Humanos , Masculino , Manometria , Pessoa de Meia-Idade , Peristaltismo , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico por imagem
10.
Clin Exp Rheumatol ; 37 Suppl 119(4): 115-124, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31573469

RESUMO

OBJECTIVES: Nintedanib is approved for the treatment of idiopathic pulmonary fibrosis (IPF) and was demonstrated to slow disease progression in patients with IPF by reducing decline in forced vital capacity by 50%. Recently, nintedanib has been reported to exert anti-fibrotic activity on systemic sclerosis (scleroderma, SSc) skin fibroblasts and to diminish skin and lung fibrosis in mouse models. The goal of the present study was to determine the effects of nintedanib on a cellular model of SSc-associated interstitial lung disease (ILD). METHODS: Study was performed using lung fibroblasts (LF) isolated from five patients with SSc-ILD and from three control subjects. RESULTS: Nintedanib inhibited LF proliferation and migration in a concentration- and time-dependent manner. The proliferation rate of LF stimulated with PDGF in the presence of nintedanib was reduced 1.9-fold within 24 h as compared to cells stimulated with PDGF alone. Migration of SSc-ILD LF incubated with 100 nM nintedanib was reduced from 62.8±12.5% to 39.1±9.0% in the presence of PDGF and from 38.2±7.9% to 26.6±7.2% in serum-free medium. Nintedanib attenuated PDGF-induced Ca2+ efflux, reduced α-SMA promoter activity and α-SMA protein expression. Furthermore, nintedanib blocked PDGF-induced differentiation of normal LF to myofibroblasts, reduced production of collagen and fibronectin, and decreased contractility of SSc-ILD LF in both floating and fixed collagen gels. CONCLUSIONS: Our data demonstrate significant antifibrotic efficacy of nintedanib in SSc-ILD LF suggesting that nintedanib has the potential not only to prevent but also to reverse the increased activity of LF consequently attenuating excessive lung fibrosis observed in SSc-ILD.


Assuntos
Fibrose Pulmonar Idiopática , Indóis/uso terapêutico , Doenças Pulmonares Intersticiais , Inibidores de Proteínas Quinases/uso terapêutico , Escleroderma Sistêmico , Células Cultivadas , Fibroblastos/efeitos dos fármacos , Humanos , Fibrose Pulmonar Idiopática/tratamento farmacológico , Fibrose Pulmonar Idiopática/etiologia , Pulmão/citologia , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/etiologia , Escleroderma Sistêmico/complicações
11.
Wiad Lek ; 72(9 cz 1): 1683-1686, 2019.
Artigo em Polonês | MEDLINE | ID: mdl-31586983

RESUMO

Systemic sclerosis is a multi-organ connective tissue disease characterised by dysfunction and impaired morphology of the blood vessels with non-specific inflammation and progressive fibrosis. In the majority of cases, the onset is observed between 30-50 years of age; in many cases, however, the diagnosis is established in patients < 20 years of age or > 75 years of age. The course of late-onset systemic sclerosis is markedly different from that in early- onset disease. In late-onset patients, limited systemic sclerosis, pulmonary hypertension, primary heart involvement, and anti-centromere antibodies are more commonly observed. Moreover, the diagnosis of systemic sclerosis in patients > 60 years of age is associated with poor prognosis, higher mortality rates, and an increased risk of neoplasms, as compared to younger patients.


Assuntos
Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Idoso , Anticorpos Antinucleares/sangue , Humanos , Hipertensão Pulmonar/complicações , Pessoa de Meia-Idade
12.
Lakartidningen ; 1162019 Sep 26.
Artigo em Sueco | MEDLINE | ID: mdl-31573670

RESUMO

Systemic sclerosis is an autoimmune systemic disease with an annual incidence in Sweden of only 20 cases per million and a standardised mortality rate of 3-4. Disease onset is usually preceded by a period with Raynaud's phenomenon, combined with structurally abnormal nailbed capillaries and accompanied by presence of scleroderma related autoantibodies. The presenting symptoms are skin thickness, puffy fingers, digital ulcers, dysphagia, joint stiffness and pain, and pruritus. Optimal management involves a number of specialists including allied health professionals. Early recognition, diagnosis and treatment are important. The dominating causes of death are cardiopulmonary.


Assuntos
Escleroderma Sistêmico , Autoanticorpos/imunologia , Humanos , Atenção Primária à Saúde , Doenças Raras/complicações , Doenças Raras/diagnóstico , Doenças Raras/patologia , Doenças Raras/terapia , Doença de Raynaud/etiologia , Encaminhamento e Consulta , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/terapia
13.
Presse Med ; 48(9): 897-903, 2019 Sep.
Artigo em Francês | MEDLINE | ID: mdl-31471090

RESUMO

Ineffective esophageal motility is the most frequent esophageal motility disorder. It is currently defined by 50% or more ineffective esophageal contractions and a normal lower esophageal sphincter relaxation on high-resolution esophageal manometry. Although reported in patients without symptoms, it is typically associated with gastro-esophageal reflux disease, and might be a consequence of the reflux. Ineffective esophageal motility can account for a certain degree of dysphagia, and is associated with a more severe gastroesophageal reflux. Todate, no specific endoscopic or pharmacologic treatment is available, and proton pump inhibitor are advisable when gastro-esophageal reflux is associated.


Assuntos
Transtornos da Motilidade Esofágica/etiologia , Refluxo Gastroesofágico/complicações , Doenças Assintomáticas , Transtornos de Deglutição/etiologia , Esofagite Eosinofílica/complicações , Transtornos da Motilidade Esofágica/tratamento farmacológico , Refluxo Gastroesofágico/tratamento farmacológico , Humanos , Inibidores da Bomba de Prótons/uso terapêutico , Escleroderma Sistêmico/complicações , Síndrome
14.
Rinsho Shinkeigaku ; 59(9): 604-606, 2019 Sep 25.
Artigo em Japonês | MEDLINE | ID: mdl-31474645

RESUMO

A 68-year-old woman with a medical history of interstitial pneumonia associated with systemic sclerosis (SSc) presented with numbness of the lower limbs and left drop foot. She was diagnosed with multiple mononeuropathy based on the laterality of her symptoms, muscle weakness, thermal hypoalgesia, and nerve conduction study findings. Left sural nerve biopsy showed vasculitis, and steroid therapy was effective. This case highlights the importance of histopathological assessment to select an appropriate treatment strategy.


Assuntos
Biópsia , Glucocorticoides/administração & dosagem , Mononeuropatias/etiologia , Mononeuropatias/patologia , Prednisolona/administração & dosagem , Escleroderma Sistêmico/complicações , Nervo Sural/patologia , Vasculite/complicações , Idoso , Feminino , Humanos , Mononeuropatias/diagnóstico , Mononeuropatias/tratamento farmacológico , Condução Nervosa , Resultado do Tratamento
15.
BMJ Case Rep ; 12(8)2019 Aug 12.
Artigo em Inglês | MEDLINE | ID: mdl-31409620

RESUMO

Digital ulcers are one of the earliest and most disabling manifestations of systemic sclerosis (SSc). We report the clinical case of a female patient with SSc and severe digital ulcers, recurrent and refractory to the classic treatments to whom it was prescribed off-label macitentan with complete resolution of the condition.


Assuntos
Pirimidinas/uso terapêutico , Escleroderma Sistêmico/complicações , Úlcera Cutânea/tratamento farmacológico , Sulfonamidas/uso terapêutico , Idoso , Feminino , Dedos/patologia , Humanos , Úlcera Cutânea/patologia
16.
Oral Dis ; 25(8): 1995-2002, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31407451

RESUMO

OBJECTIVE: The aim of this study was to evaluate the orofacial parameters of systemic sclerosis (SSc) and its related systemic features. SUBJECTS AND METHODS: A descriptive case-control study was performed from November 2015 to October 2016. Ninety-three individuals were included and divided into SSc group (n = 50) and healthy controls (C, n = 43). RESULTS: Systemic sclerosis individuals were mostly women (43/50, 86%), with a mean age of 46 years (±11.6 years). Telangiectasia (42/50, 84%) and reduced mouth opening (35/50, 70%) were the most frequent orofacial findings. The periodontitis frequency was much higher in SSc individuals than in healthy controls (90.7% × 48.83%; p < .001). In addition, SSc individuals presented a distinctive pattern of periodontitis, with low probing pocket depth (2 ± 0.65 mm × 2 ± 0.24; p < .001), higher gingival recession (4 ± 2.13 × 0.14 ± 0,22; p < .001), higher periodontal attachment loss (6 ± 1.34 mm × 2 ± 0.43, p < .001), and lower gingival bleeding index values (7.05 ± 7.25 × 21.57 ± 15.66; p < .001). CONCLUSIONS: Orofacial manifestations were common in SSc and included a unique pattern of periodontal manifestation, characterized by lower gingival bleeding index, higher periodontal attachment loss, and low probing depth.


Assuntos
Hemorragia Gengival/epidemiologia , Perda da Inserção Periodontal , Doenças Periodontais/epidemiologia , Periodontite/epidemiologia , Escleroderma Sistêmico/complicações , Xerostomia/epidemiologia , Adulto , Idoso , Brasil/epidemiologia , Estudos de Casos e Controles , Índice de Placa Dentária , Feminino , Nível de Saúde , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Periodontais/complicações , Doenças Periodontais/diagnóstico , Escleroderma Sistêmico/epidemiologia
18.
Braz J Med Biol Res ; 52(8): e8513, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31365695

RESUMO

Phenotypic differences have been described between patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and SSc-associated pulmonary hypertension, including performance differences in the 6-min walk test (6MWT). Moreover, the correlations between the 6MWT and traditional pulmonary function tests (PFTs) are weak, indicating the need to search for new parameters that explain exercise performance. Thus, our objective was to evaluate the impact of ventilation distribution heterogeneity assessed by the nitrogen single-breath washout (N2SBW) test and peripheral muscle dysfunction on the exercise capacity in patients with SSc-ILD and limited involvement of the pulmonary parenchyma. In this cross-sectional study, 20 women with SSc-ILD and 20 matched controls underwent PFTs (including spirometry, diffusing capacity for carbon monoxide (DLco), and the N2SBW test) and performed the 6MWT and knee isometric dynamometry. The 6-min walking distance (6MWD, % predicted) was strongly correlated with the phase III slope of the single-breath nitrogen washout (phase III slopeN2SBW) (r=-0.753, P<0.0001) and reasonably correlated with the forced vital capacity (FVC) (r=0.466, P=0.008) and DLco (r=0.398, P=0.011). The peripheral oxygen saturation (SpO2) during exercise was not significantly correlated with any of the pulmonary or muscle function parameters. The phase III slopeN2SBW was the only predictive variable for the 6MWD, whereas quadriceps strength and FVC/DLco were predictive variables for SpO2. Ventilation distribution heterogeneity is one factor that contributes to a lower 6MWD in SSc-ILD patients. In addition, muscle dysfunction and abnormal lung diffusion at least partly explain the decreased SpO2 of these patients.


Assuntos
Tolerância ao Exercício/fisiologia , Hipertensão Pulmonar/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Pulmão/fisiologia , Testes de Função Respiratória/métodos , Escleroderma Sistêmico/complicações , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/etiologia , Medidas de Volume Pulmonar/métodos , Pessoa de Meia-Idade , Ventilação Pulmonar , Doença de Raynaud/complicações , Tomografia Computadorizada por Raios X/métodos , Capacidade Vital/fisiologia , Teste de Caminhada/métodos
19.
Stomatologiia (Mosk) ; 98(3): 104-108, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31322605

RESUMO

The aim of this study was to conduct literature review about oral manifestations of scleroderma. Systemic sclerosis is a multisystem autoimmune disorder characterized by widespread fibrosis, vascular alterations and inflammation. Systemic sclerosis mainly affects people in age from 30 to 50 years, but the onset of disease can occur in any age. The disturbance of microcirculation with the activation and proliferation of endothelium and smooth muscle cells plays an important role in pathogenesis of sclerodrma. These changes lead to sclerosis and fibrosis of various part in human body. Orofacial manifestations of scleroderma include: reduced mouth opening, widening of periodontal ligament, teleangiectasia, bone lesions. Systemic scleroderma is often accompanied by Sjogren's syndrome. Oral manifestations of Sjogren's syndrome are recurrent parotitis, angular cheilitis, xerostomia and multiple caries. Sjogren's syndrome leads to negative impact on patients health and mental status. Orofacial manifestations of systemic sclerosis are still poorly known, that is why more researches should be made to improve dental treatment of patients with systemic sclerosis.


Assuntos
Cárie Dentária , Saúde Bucal , Escleroderma Sistêmico , Síndrome de Sjogren , Xerostomia , Cárie Dentária/complicações , Humanos , Escleroderma Sistêmico/complicações , Síndrome de Sjogren/complicações , Xerostomia/complicações
20.
Medicine (Baltimore) ; 98(29): e16423, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31335694

RESUMO

To test whether texture analysis (TA) can discriminate between Systemic Sclerosis (SSc) and non-SSc patients in computed tomography (CT) with different radiation doses and reconstruction algorithms.In this IRB-approved retrospective study, 85 CT scans at different radiation doses [49 standard dose CT (SDCT) with a volume CT dose index (CTDIvol) of 4.86 ±â€Š2.1 mGy and 36 low-dose (LDCT) with a CTDIvol of 2.5 ±â€Š1.5 mGy] were selected; 61 patients had Ssc ("cases"), and 24 patients had no SSc ("controls"). CT scans were reconstructed with filtered-back projection (FBP) and with sinogram-affirmed iterative reconstruction (SAFIRE) algorithms. 304 TA features were extracted from each manually drawn region-of-interest at 6 pre-defined levels: at the midpoint between lung apices and tracheal carina, at the level of the tracheal carina, and 4 between the carina and pleural recesses. Each TA feature was averaged between these 6 pre-defined levels and was used as input in the machine learning algorithm artificial neural network (ANN) with backpropagation (MultilayerPerceptron) for differentiating between SSc and non-SSc patients.Results were compared regarding correctly/incorrectly classified instances and ROC-AUCs.ANN correctly classified individuals in 93.8% (AUC = 0.981) of FBP-LDCT, in 78.5% (AUC = 0.859) of FBP-SDCT, in 91.1% (AUC = 0.922) of SAFIRE3-LDCT and 75.7% (AUC = 0.815) of SAFIRE3-SDCT, in 88.1% (AUC = 0.929) of SAFIRE5-LDCT and 74% (AUC = 0.815) of SAFIRE5-SDCT.Quantitative TA-based discrimination of CT of SSc patients is possible showing highest discriminatory power in FBP-LDCT images.


Assuntos
Doenças Pulmonares Intersticiais , Pulmão/diagnóstico por imagem , Escleroderma Sistêmico , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Algoritmos , Diagnóstico Diferencial , Diagnóstico Precoce , Feminino , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Masculino , Pessoa de Meia-Idade , Doses de Radiação , Intensificação de Imagem Radiográfica/métodos , Interpretação de Imagem Radiográfica Assistida por Computador/métodos , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA