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1.
Nihon Shokakibyo Gakkai Zasshi ; 118(1): 78-85, 2021.
Artigo em Japonês | MEDLINE | ID: mdl-33431753

RESUMO

A woman in her 70s with systemic sclerosis experienced dyspnea, and consequently, she was diagnosed with an esophago-pleural fistula, which was caused by a perforated esophageal ulcer. We administered conservative treatments including continuous pleural drainage and total parenteral nutrition. The fistula was closed but recurred, at which point we attempted to close the fistula by filling and shielding using polyglycolic acid (PGA) sheets and fibrin glue (FG). We were able to safely and smoothly fill and shield the fistula using the PGA sheets with a guidewire. We show that endoscopic closure of an esophago-pleural fistula using this technique is an effective, low-invasive treatment for gastrointestinal perforation and refractory fistulas.


Assuntos
Fístula , Escleroderma Sistêmico , Feminino , Adesivo Tecidual de Fibrina/uso terapêutico , Humanos , Ácido Poliglicólico , Complicações Pós-Operatórias , Escleroderma Sistêmico/complicações , Úlcera
2.
Lancet Respir Med ; 9(1): 96-106, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33412120

RESUMO

BACKGROUND: In the Safety and Efficacy of Nintedanib in Systemic Sclerosis (SENSCIS) trial, nintedanib reduced the rate of decline in forced vital capacity (FVC) in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). Patients on stable treatment with mycophenolate for at least 6 months before randomisation could participate. The aim of this subgroup analysis was to examine the efficacy and safety of nintedanib by mycophenolate use at baseline. METHODS: The SENSCIS trial was a randomised, double-blind, placebo-controlled trial, in which patients with SSc-ILD were randomly assigned (1:1) to receive 150 mg of oral nintedanib twice daily or placebo for at least 52 weeks. In a prespecified subgroup analysis, we analysed the primary endpoint of rate of decline in FVC over 52 weeks by mycophenolate use at baseline. In a post-hoc analysis, we analysed the proportion of patients with an absolute decrease in FVC of at least 3·3% predicted at week 52 (proposed minimal clinically important difference estimate for worsening of FVC in patients with SSc-ILD) in subgroups by mycophenolate use at baseline. Adverse events were reported in subgroups by mycophenolate use at baseline. Analyses were done in all participants who received at least one dose of study drug. We analysed the annual rate of decline in FVC using a random coefficient regression model (with random slopes and intercepts) including anti-topoisomerase I antibody status, age, height, sex, and baseline FVC as covariates and terms for baseline-by-time, treatment-by-subgroup, and treatment-by-subgroup-by-time interactions. SENSCIS is registered with ClinicalTrials.gov, NCT02597933, and is now complete. FINDINGS: Between Nov 30, 2015, and Oct 31, 2017, 819 participants were screened and 576 were enrolled, randomly assigned to, and treated with nintedanib (n=288) or placebo (n=288). 139 (48%) of 288 in the nintedanib group and 140 (49%) of 288 in the placebo group were taking mycophenolate at baseline. In patients taking mycophenolate at baseline, the adjusted mean annual rate of decline in FVC was -40·2 mL per year (SE 19·8) with nintedanib and -66·5 mL per year (19·3) with placebo (difference: 26·3 mL per year [95% CI -27·9 to 80·6]). In patients not taking mycophenolate at baseline, the adjusted mean annual rate of decline in FVC was -63·9 mL per year (SE 19·3) with nintedanib and -119·3 mL per year (19·0) with placebo (difference: 55·4 mL per year [95% CI 2·3 to 108·5]). We found no heterogeneity in the effect of nintedanib versus placebo on the annual rate of decline in FVC between the subgroups by mycophenolate use (p value for interaction=0·45). In a post-hoc analysis, the proportion of patients with an absolute decrease in FVC of at least 3·3% predicted was lower with nintedanib than with placebo in both patients taking mycophenolate (40 [29%] of 138 vs 56 [40%] of 140; odds ratio 0·61 [0·37 to 1·01]) and those not taking mycophenolate (59 [40%] of 149 vs 70 [47%] of 148; 0·73 [0·46 to 1·16]) at baseline. The adverse event profile of nintedanib was similar between the subgroups. Diarrhoea, the most common adverse event, was reported in 106 (76%) of 139 patients in the nintedanib group and 48 (34%) of 140 in the placebo group among those taking mycophenolate at baseline, and in 112 (75%) of 149 in the nintedanib group and 43 (29%) of 148 in the placebo group among those not taking mycophenolate at baseline. Over the entire trial period, 19 patients died (ten in the nintedanib group and nine in the placebo group). One death in the nintedanib group was considered to be related to study drug. INTERPRETATION: Nintedanib reduced the progression of interstitial lung disease both in patients with SSc-ILD who were and were not using mycophenolate at baseline, with no heterogeneity in its treatment effect detected between the subgroups. The adverse event profile of nintedanib was similar in the subgroups by mycophenolate use. Our findings suggest that the combination of mycophenolate and nintedanib offers a safe treatment option for patients with SSc-ILD. More data are needed on the benefits of initial combination therapy versus a sequential approach to treatment of SSc-ILD. FUNDING: Boehringer Ingelheim.


Assuntos
Indóis/uso terapêutico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Ácido Micofenólico/uso terapêutico , Inibidores de Proteínas Quinases/uso terapêutico , Escleroderma Sistêmico/tratamento farmacológico , Progressão da Doença , Método Duplo-Cego , Feminino , Humanos , Indóis/efeitos adversos , Doenças Pulmonares Intersticiais/etiologia , Masculino , Pessoa de Meia-Idade , Inibidores de Proteínas Quinases/efeitos adversos , Escleroderma Sistêmico/complicações , Resultado do Tratamento
4.
J Clin Rheumatol ; 27(1): 31-33, 2021 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-33347032

RESUMO

BACKGROUND: The coronavirus disease (COVID-19) pandemic and its subsequent effects on health care systems have significantly impacted the management of chronic rheumatic diseases, including systemic sclerosis (SSc). METHODS: In this context, a 25-item anonymized e-survey was posted on the Twitter and Facebook e-groups and pages of various scleroderma organizations and patient communities to assess the problems faced by patients with SSc during the pandemic, with a focus on effects on the disease, drug procurance, continuity of medical care, and prevalent fears among patients. RESULTS: Of the 291 participants (median age of 55 [43.5-63] years, 93.8% females), limited systemic sclerosis was the most common diagnosis (42.3%). Many patients experienced problems attributable to the COVID-19 pandemic (119, 40.9%), of which 46 (38.7%) required an increase in medicines, and 12 (10.1%) of these needed hospitalizations for disease-related complications. More than one-third (36.4%) were on glucocorticoids or had underlying cardiovascular risks (39%) that would predispose them to severe COVID-19.A significant proportion (38.1%) faced hurdles in procuring medicines or experienced disruption in physiotherapy sessions (24.7%). One-quarter (24.1%) felt it was difficult to contact their specialist, whereas another 7.2% were unable to do so. Contracting COVID-19 was the most prevalent fear (71.5%), followed by infection in the family (61.9%), and a flare of the disease (45.4%). Most respondents preferred teleconsultations (55.7%) over hospital visits in the pandemic period. CONCLUSION: The results of the patient survey suggest that the COVID-19 pandemic has affected many patients with SSc and may translate to poorer outcomes in this population in the postpandemic period.


Assuntos
/epidemiologia , Acesso aos Serviços de Saúde , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/terapia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pandemias , Inquéritos e Questionários
5.
Z Rheumatol ; 80(1): 69-72, 2021 Feb.
Artigo em Alemão | MEDLINE | ID: mdl-33340058

RESUMO

In the last few years, several studies emerged for the treatment of interstitial lung disease (ILD) associated with systemic sclerosis (SSc). Some of the studied drugs showed efficacy compared to placebo or to a control group. Since early 2020, nintedanib has been approved for the treatment of SSc-ILD. Although several drugs are used to treat SSc-LD, it is now the first approved drug for SSc-ILD. Here, we aim to provide an overview about our current therapy algorithm and strategy as well as our interpretation of study results as an experienced SSc-ILD center.


Assuntos
Doenças Pulmonares Intersticiais , Escleroderma Sistêmico , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/etiologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/tratamento farmacológico
6.
Vnitr Lek ; 66(6): 53-57, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33380154

RESUMO

Mixed connective tissue diseases (MCTD) is a very rare autoimmune disease connecting clinical signs of systemic lupus, systemic sclerosis, polymyositis and rheumatoid arthritis. Clinical manifestations are very diverse. In some patients, the digestive tract is affected in varying degrees. The esophagus is affected most often, and patients are complaining of dysphagia. Morphologically, this disorder is similar to the injure in systemic scleroderma. In this case, we describe a unique case of a severe damage of digestive tract manifested by esophageal motility disorders, cachectization, ascites, and repeated ileus conditions.


Assuntos
Artrite Reumatoide , Doenças Autoimunes , Lúpus Eritematoso Sistêmico , Doença Mista do Tecido Conjuntivo , Escleroderma Sistêmico , Esôfago , Humanos , Doença Mista do Tecido Conjuntivo/complicações , Escleroderma Sistêmico/complicações
7.
Medicine (Baltimore) ; 99(43): e22443, 2020 Oct 23.
Artigo em Inglês | MEDLINE | ID: mdl-33120739

RESUMO

The objectives of this study were to describe the prevalence and characteristics of radiographic lesions of the hands, and calcifications of the spine on computer tomography scans (CT-scans), and to investigate the relationships between radiographic and CT-scan abnormalities and clinical features in a population of patients with systemic sclerosis (SSc).Subjects underwent X-ray examination of the hands, and thoracic or thoraco-abdominal and pelvic CT scan or lumbar CT scan in the year. Structural lesions on hand X ray was scored and spinal calcifications were evaluated in the anterior, intracanal and posterior segments. Intra and inter-reliability was tested for radiography and CT- scan. Prognostic factors considered were interstitial pulmonary lesions on the CT scan, pulmonary arterial hypertension (PAH) and death.This study involved 77 SSc patients, 58 (75%) with limited cutaneous SSc (lcSSc) and 19 (25%) with diffuse SSc (dSSc). The prevalences of radiographic lesions of the hand were 28.6% for periarticular calcifications and 26% for calcinosis. On CT scan, 64 (83%) patients exhibited at least 1 calcification. Spine calcifications were depicted in 80.5%, 27.3%, and 35.1% at the anterior, intracanal and posterior segments respectively. Calcifications were mainly localized on thoracic spine. Inter reader reliabilities were good for hands and moderate for spine respectively. Spine calcifications and periarticular calcifications in the hands were associated (P = .012). Calcinosis in the hands was related to PAH (P = .02). Posterior calcification segment and foraminal calcifications were associated with interstitial lung disease (ILD) (P = .029) and death (P = .001).More than 80% of systemic sclerosis patients presented spine calcifications. A significant association between hands and spinal calcifications were confirmed and some localization in the posterior segment considered as a bad prognostic factor.


Assuntos
Calcinose/diagnóstico por imagem , Ossos da Mão/diagnóstico por imagem , Escleroderma Sistêmico/complicações , Doenças da Coluna Vertebral/diagnóstico por imagem , Coluna Vertebral/diagnóstico por imagem , Fatores Etários , Feminino , Humanos , Hipertensão Pulmonar/complicações , Doenças Pulmonares Intersticiais/complicações , Masculino , Pessoa de Meia-Idade , Prognóstico , Radiografia , Reprodutibilidade dos Testes , Estudos Retrospectivos , Escleroderma Sistêmico/mortalidade , Tomografia Computadorizada por Raios X
8.
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
9.
Medicine (Baltimore) ; 99(41): e22582, 2020 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-33031308

RESUMO

RATIONALE: Systemic sclerosis (SSc) is a serious multisystem connective tissue disease. When SSc is accompanied by systemic lupus erythematosus (SLE), called SSc-SLE overlap syndrome. SSc associated thrombotic microangiopathy (SSc-TMA) can lead to scleroderma renal crisis, it mainly manifests hypertension or even malignant hypertension, acute kidney injury, and higher mortality. The case of SSc-SLE overlap syndrome combined with SSc-TMA has rarely been reported. PATIENT CONCERNS: We report the case of an elderly male with SSc-SLE overlap syndrome combined with scleroderma renal crisis and SSc-TMA. DIAGNOSES: The patient has typical of SSc on the face and hands, combined with pulmonary artery hypertension, interstitial lung disease, heart failure and malignant hypertension, as well as SLE, lupus nephritis class V, and TMA, which were definitively diagnosed by clinical laboratory examination and renal histopathology. INTERVENTIONS: The patient was treated with prednisone, cyclophosphamid, renin-angiotensin system inhibitors, diuretics, and acetylcysteine. OUTCOMES: The patient died suddenly of heart failure on the 35th day after discharge. LESSONS: The occurrence of TMA leads to the deterioration of the prognosis of SSC-SLE overlap syndrome. The diagnosis of SSC-TMA in SSc-SLE overlap syndrome depends on clinical laboratory examination and renal histopathology.


Assuntos
Lúpus Eritematoso Sistêmico/complicações , Esclerodermia Localizada/complicações , Escleroderma Sistêmico/complicações , Microangiopatias Trombóticas/complicações , Humanos , Masculino , Pessoa de Meia-Idade
10.
Medicine (Baltimore) ; 99(33): e21734, 2020 Aug 14.
Artigo em Inglês | MEDLINE | ID: mdl-32872058

RESUMO

To compare clinical characteristics and identify long-term outcomes of Chinese patients with systemic sclerosis (SSc) with and without muscle involvement.We retrospectively investigated the medical records, laboratory results, and computed tomography images of 204 consecutive SSc patients. Kaplan-Meier analysis was performed to determine survival rates. Patients were allocated into groups with and without myopathy.The prevalence of myopathy was 21.6%. The myopathy group was more likely to develop diffuse cutaneous involvement (90.9% vs 56%, P = .006), interstitial lung disease (90% vs 56%, P < .001), digestive system involvement (56.7% vs 29.3%, P = .001), pulmonary hypertension (29.5% vs 10.5%, P = .004), and pericardial effusion (25% vs. 10%, P = .019). Patients with myopathy had lower single-breath diffusing capacity of the lung for carbon oxide (46.5 ±â€Š11.1 vs 57.1 ±â€Š13.4, P < .001).Further, the myopathy group has similar results in interstitial lung disease associated higher resolution computed tomography score (186.8 ±â€Š64.5 vs 152.3 ±â€Š45.5, P = .037), Valentini score for disease activity (3.4 ±â€Š0.9 vs 2.0 ±â€Š0.9, P < .001) and modified Rodnan total skin score (19.4 ±â€Š6.1 vs 15.1 ±â€Š7.7, P = .002), compared with non-myopathy group. Kaplan-Meier survival analysis revealed decreased overall survival rate of the myopathy group (P = .028).SSc Patients with myopathy had more severe clinical manifestations and higher disease activity compared with those without, which affected survival rates and indicated worse prognosis.


Assuntos
Doenças Musculares/mortalidade , Escleroderma Sistêmico/mortalidade , Adulto , Idoso , China/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Musculares/etiologia , Estudos Retrospectivos , Escleroderma Sistêmico/complicações
11.
RMD Open ; 6(2)2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32878994

RESUMO

OBJECTIVES: Patients with inflammatory rheumatic diseases (IRD) infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may be at risk to develop a severe course of COVID-19. The influence of immunomodulating drugs on the course of COVID-19 is unknown. To gather knowledge about SARS-CoV-2 infections in patients with IRD, we established a registry shortly after the beginning of the pandemic in Germany. METHODS: Using an online questionnaire (www.COVID19-rheuma.de), a nationwide database was launched on 30 March 2020, with appropriate ethical and data protection approval to collect data of patients with IRD infected with SARS-CoV-2. In this registry, key clinical and epidemiological parameters-for example, diagnosis of IRD, antirheumatic therapies, comorbidities and course of the infection-are documented. RESULTS: Until 25 April 2020, data from 104 patients with IRD infected with SARS-CoV-2 were reported (40 males; 63 females; 1 diverse). Most of them (45%) were diagnosed with rheumatoid arthritis, 59% had one or more comorbidities and 42% were treated with biological disease-modifying antirheumatic drugs. Hospitalisation was reported in 32% of the patients. Two-thirds of the patients already recovered. Unfortunately, 6 patients had a fatal course. CONCLUSIONS: In a short time, a national registry for SARS-CoV2-infected patients with IRD was established. Within 4 weeks, 104 cases were documented. The registry enables to generate data rapidly in this emerging situation and to gain a better understanding of the course of SARS-CoV2-infection in patients with IRD, with a distinct focus on their immunomodulatory therapies. This knowledge is valuable for timely information of physicians and patients with IRD, and shall also serve for the development of guidance for the management of patients with IRD during this pandemic.


Assuntos
Antirreumáticos/uso terapêutico , Produtos Biológicos/uso terapêutico , Infecções por Coronavirus/fisiopatologia , Pneumonia Viral/fisiopatologia , Sistema de Registros , Doenças Reumáticas/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Artrite Psoriásica/complicações , Artrite Psoriásica/tratamento farmacológico , Artrite Reumatoide/complicações , Artrite Reumatoide/tratamento farmacológico , Betacoronavirus , Infecções por Coronavirus/complicações , Infecções por Coronavirus/mortalidade , Feminino , Alemanha , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/tratamento farmacológico , Hospitalização , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/complicações , Pneumonia Viral/mortalidade , Polimialgia Reumática/complicações , Polimialgia Reumática/tratamento farmacológico , Prognóstico , Doenças Reumáticas/complicações , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/tratamento farmacológico , Índice de Gravidade de Doença , Espondilite Anquilosante/complicações , Espondilite Anquilosante/tratamento farmacológico , Adulto Jovem
14.
J Vis Exp ; (160)2020 06 16.
Artigo em Inglês | MEDLINE | ID: mdl-32744514

RESUMO

Early diagnosis of systemic sclerosis-related interstitial lung disease (SSc-ILD) is important to enable treatment to be administered with minimal delay. However, diagnosing SSc-ILD is challenging because key symptoms are non-specific. High-resolution computed tomography (HRCT) of the chest is recognized as a sensitive imaging method for diagnosing and assessing SSc-ILD. Exposure of patients to ionizing radiation may be considered as a limitation, although methodological steps may be taken to moderate this. We present practical recommendations for performing HRCT scans and interpreting the results. Key features of SSc-ILD on HRCT include a non-specific interstitial pneumonia (NSIP) pattern with peripheral ground-glass opacities and extensive traction bronchiectasis. Despite similarities between SSc-ILD and idiopathic pulmonary fibrosis (IPF), HRCT can be used to differentiate between these conditions: in SSc-ILD compared with IPF, there is a greater proportion of ground-glass opacity and fibrosis is less coarse. A dilated, air-filled esophagus with diameter >10 mm, suggestive of esophageal dysmotility is commonly seen in SSc-ILD. Pulmonary artery size greater than the adjacent ascending aorta suggests coexistent pulmonary hypertension. Nodules must be monitored due to the increased risk of lung cancer. A large extent of disease on HRCT (≥20%) or a high fibrosis score suggests an increased risk of mortality. HRCT is central to diagnosing SSc-ILD, and serial assessments can be helpful in monitoring disease progression or treatment response.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico por imagem , Escleroderma Sistêmico/diagnóstico por imagem , Fibrose , Humanos , Pulmão/patologia , Doenças Pulmonares Intersticiais/etiologia , Escleroderma Sistêmico/complicações , Tomografia Computadorizada por Raios X
15.
J Comput Assist Tomogr ; 44(5): 667-672, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32842066

RESUMO

OBJECTIVE: The aim of the study was to compare the prevalence of diffuse pulmonary ossification (DPO) in idiopathic pulmonary fibrosis (IPF), systemic sclerosis (SSc)-related interstitial lung disease (ILD), and chronic hypersensitivity pneumonitis (HP). METHODS: High-resolution computed tomography (HRCT) from 71 IPF, 67 SSc-ILD, and 75 HP cases were independently evaluated by 2 thoracic radiologists blinded to patient data. Studies were assessed for the presence of DPO, HRCT scanning pattern, stigmata of granulomatous infection, and honeycombing. RESULTS: The prevalence of DPO was significantly higher in cases of IPF and SSc compared with HP, although there was no significant difference in prevalence between the IPF and SSc groups, even when accounting for the presence of prior granulomatous infection. Interobserver agreement for the presence of DPO was substantial. CONCLUSIONS: Although prevalence DPO on HRCT varies between some forms of ILD, the use of DPO to influence characterization of ILD should be considered with caution.


Assuntos
Fibrose Pulmonar Idiopática/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Ossificação Heterotópica/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Doença Crônica , Feminino , Humanos , Fibrose Pulmonar Idiopática/patologia , Pulmão/diagnóstico por imagem , Pulmão/patologia , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/patologia , Masculino , Pessoa de Meia-Idade , Ossificação Heterotópica/patologia , Escleroderma Sistêmico/complicações , Adulto Jovem
16.
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
17.
Methodist Debakey Cardiovasc J ; 16(2): 162-166, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32670478

RESUMO

Cardiac involvement of infiltrative disease, such as systemic sclerosis, carries significant morbidity and mortality. All parts of the heart may be affected, although the conduction system is less commonly involved. We report a rare case of systemic sclerosis causing third-degree atrioventricular block and the first known reported case to use cardiac magnetic resonance imaging (CMR) as a diagnostic and prognostic instrument. It is not known whether using cardiac CMR in systemic sclerosis with cardiac involvement could lead to earlier intervention with escalation of medical therapy or earlier referral for transplant evaluation.


Assuntos
Bloqueio Atrioventricular/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Imagem por Ressonância Magnética , Escleroderma Sistêmico/complicações , Adulto , Bloqueio Atrioventricular/etiologia , Bloqueio Atrioventricular/fisiopatologia , Bloqueio Atrioventricular/terapia , Estimulação Cardíaca Artificial , Cardiomiopatias/etiologia , Cardiomiopatias/fisiopatologia , Cardiomiopatias/terapia , Progressão da Doença , Evolução Fatal , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Masculino , Valor Preditivo dos Testes , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/tratamento farmacológico , Resultado do Tratamento
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