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1.
Am J Gastroenterol ; 116(3): 517-521, 2021 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-33657040

RESUMO

INTRODUCTION: Systemic sclerosis or scleroderma (SSc) is a chronic autoimmune disease that renders the esophagus prone to significant gastroesophageal reflux due to impaired esophageal clearance and reduced lower esophageal sphincter pressure. The reported prevalence of Barrett's esophagus (BE) in women with SSc varies from 2% to 37% and is derived from older studies with small sample sizes. We aimed to assess the prevalence of BE in a large cohort of women with SSc. METHODS: Women with SSc referred from the Mayo Clinic Arizona Rheumatology Clinic who completed esophagogastroduodenoscopy between 2002 and 2020 were included. Demographic and high-resolution manometry data were evaluated. The diagnosis of scleroderma was confirmed by an expert rheumatologist. The BE diagnosis was confirmed by an expert gastrointestinal pathologist. RESULTS: There were 235 women with SSc who underwent EGD. High-resolution manometry (HRM) was completed in 172 patients. Women with SSc with BE were significantly more likely to have scleroderma esophagus (absent contractility with hypotensive lower esophageal sphincter) on HRM than women with SSc without BE (P = 0.018). There were 30 patients with SSc (12.8%) with histologically proven BE. Dysplasia was found in 13 (43.3%): 4 with indefinite, 7 with low grade, and 2 with adenocarcinoma. The incidence of any dysplasia was 5.3% per year (0.9% per year for adenocarcinoma). DISCUSSION: This the largest study on prevalence of BE in women with SSc, yielding a prevalence of 12.8%. Women with SSc with BE were significantly more likely to have absent contractility with hypotensive lower esophageal sphincter findings on HRM. The high prevalence and incidence of dysplasia found suggest that women with SSc should be included in the screening recommendations for BE.


Assuntos
Transtornos de Deglutição/epidemiologia , Escleroderma Sistêmico/epidemiologia , Adulto , Idoso , Esôfago de Barrett , Comorbidade , Feminino , Humanos , Incidência , Manometria , Prevalência
2.
JAMA Netw Open ; 3(12): e2029917, 2020 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-33315114

RESUMO

Importance: Patients with autoimmune disease and lung cancer pose a multidisciplinary treatment challenge, particularly with the advent of immunotherapy. However, the association between autoimmune disease and lung cancer survival is largely unknown. Objective: To determine the association between autoimmune disease and lung cancer survival. Design, Setting, and Participants: Retrospective cohort study between 2003 and 2019 at a single academic medical center (Northwestern University). A query of the Northwestern Medicine Enterprise Data Warehouse identified 349 patients with lung cancer and several autoimmune diseases. Types of lung cancers included small cell, adenocarcinoma, squamous cell carcinoma, non-small cell not otherwise specified, and large cell carcinoma. Autoimmune diseases included rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, mixed connective tissue disease, myositis, and Sjögren syndrome. Inclusion criteria were biopsy-confirmed lung cancer, autoimmune diagnosis confirmed by a rheumatologist, and death or an encounter listed in the electronic medical record within 2 years of study end. A control group of patients with biopsy-proven lung cancer but without autoimmune disease was identified. Data analysis was conducted from March to July 2020. Exposure: Presence of autoimmune disease. Main Outcomes and Measures: Overall survival and progression-free survival in patients with autoimmune disease. The hypothesis was that patients with autoimmune disease would have worse progression-free survival and overall survival compared with patients in the control group. Results: Of the original 349 patients, 177 met inclusion criteria. Mean (SD) age at lung cancer diagnosis was 67.0 (10.0) years and 136 (76.8%) were women. Most common autoimmune diseases were rheumatoid arthritis (97 [54.8%]), systemic sclerosis (43 [24.3%]), and systemic lupus erythematous (15 [8.5%]). Most common lung cancers were adenocarcinoma (99 [55.9%]), squamous cell carcinoma (29 [16.4%]), and small cell lung cancer (17 [9.6%]). A total of 219 patients (mean [SD] age at diagnosis, 65.9 [4.1] years; 173 [79.0%]) were identified as having lung cancer without autoimmune disease and included in the control cohort. Compared with patients in the control group, patients with autoimmune disease experienced no difference in overall survival (log-rank P = .69). A total of 126 patients (69.5%) with autoimmune disease received standard of care vs 213 patients (97.3%) in the control group (P < .001). No individual autoimmune disease was associated with worse prognosis, even among patients with underlying interstitial lung disease. Conclusions and Relevance: Compared with institutional controls, patients with autoimmune disease experienced no difference in survival despite the fact that fewer patients in this group received standard-of-care treatment. No individual autoimmune disease was associated with worse prognosis. Future multicenter prospective trials are needed to further evaluate autoimmune disease and lung cancer survival.


Assuntos
Neoplasias Pulmonares , Pulmão/patologia , Idoso , Artrite Reumatoide/epidemiologia , Artrite Reumatoide/fisiopatologia , Artrite Reumatoide/terapia , Autoimunidade , Biópsia/métodos , Biópsia/estatística & dados numéricos , Comorbidade , Registros Eletrônicos de Saúde/estatística & dados numéricos , Feminino , Humanos , Pesquisa Interdisciplinar , Neoplasias Pulmonares/imunologia , Neoplasias Pulmonares/mortalidade , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/terapia , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Estadiamento de Neoplasias , Noroeste dos Estados Unidos/epidemiologia , Prognóstico , Estudos Retrospectivos , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/fisiopatologia , Escleroderma Sistêmico/terapia , Padrão de Cuidado/organização & administração , Padrão de Cuidado/estatística & dados numéricos , Análise de Sobrevida
3.
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
4.
Clin Rheumatol ; 39(7): 2043-2047, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32514674

RESUMO

COVID-19 is a world health emergency which may inevitably affect the management of a complex autoimmune disease such as systemic sclerosis (SSc). Several SSc patients are frail and, in this pandemic, need a careful protection. The COVID-19 infection might complicate the clinical scenario of interstitial lung disease (ILD) in SSc because it determines a severe pneumonia characterized by radiological features similar to SSc-ILD. The striking CT similarities between the 2 diseases make it difficult to distinguish a worsening of SSc-ILD from COVID-19-ILD superinfection. Moreover, other aspects, like isolation during lock down, may cause a significant psychological stress which will pile up on the already difficult contact with the patients for a routine check-up. Moreover, the drug shortage is a real problem in these times. For these reasons, the rheumatologist in daily clinical practice should carefully differentiate the possible COVID-19 infection in order to optimize the patient management. Therefore, the challenge in everyday life will be to achieve in due time the differential diagnosis as well as the long-term psychological impact.Key Points• SSc patients should be encouraged to continue their chronic therapy; in case of immunosuppressive therapy it must be discontinued for safety in case of COVID-19 infection.• Psychological support must be guaranteed to every SSc patients.• COVID-19 pneuminia is hard to distinguish from an interstitial lung disease due to SSc lung involvment.• Data sharing is fundamental for an optimal managment of SSc patients during COVID-19 pandemia.


Assuntos
Infecções por Coronavirus , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais , Pandemias , Pneumonia Viral/diagnóstico , Escleroderma Sistêmico , Betacoronavirus/isolamento & purificação , Comorbidade , Infecções por Coronavirus/complicações , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/fisiopatologia , Infecções por Coronavirus/terapia , Diagnóstico Diferencial , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Administração dos Cuidados ao Paciente/métodos , Pneumonia Viral/complicações , Pneumonia Viral/epidemiologia , Pneumonia Viral/fisiopatologia , Pneumonia Viral/terapia , Angústia Psicológica , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/psicologia , Escleroderma Sistêmico/terapia , Isolamento Social/psicologia , Tomografia Computadorizada por Raios X/métodos
5.
Clin Rheumatol ; 39(7): 2025-2029, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32406001

RESUMO

The coronavirus disease 2019 (COVID-19), the result of an infection with the new virus, SARS-CoV-2, is rapidly spreading worldwide. It is largely unknown whether the occurrence of COVID-19 in patients with rheumatic immune diseases has some specific manifestations, or makes them more prone to rapidly progress into severe COVID-19. In this case report, we describe the clinical features of 5 rheumatic immune disease patients with the concomitant presence of COVID-19. Amongst these patients, 4 had rheumatoid arthritis (RA) and 1 had systemic sclerosis (SSc). Two patients had a history of close contact with a COVID-19 patient. The age of the patients ranged between 51 and 79 years. Fever (80%), cough (80%), dyspnea (40%), and fatigue (20%) were the most common presenting symptoms. Laboratory investigations revealed leukopenia and lymphopenia in 2 patients. In all the patients, chest computerized tomography (CT) revealed patchy ground glass opacities in the lungs. During the hospital stay, the condition of two patients remained the same (i.e., mild COVID-19), two patients progressed to the severe COVID-19, and one patient worsened to the critically ill COVID-19. These patients were treated with antiviral agents for COVID-19, antibiotics for secondary bacterial infections, and immunomodulatory agents for rheumatic immune diseases. All the patients responded well, were cured of COVID-19, and subsequently discharged.


Assuntos
Antivirais/uso terapêutico , Artrite Reumatoide , Infecções por Coronavirus , Imunomodulação , Pandemias , Pneumonia Viral , Escleroderma Sistêmico , Idoso , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/epidemiologia , Artrite Reumatoide/terapia , Betacoronavirus/isolamento & purificação , Contagem de Células Sanguíneas/métodos , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/terapia , Estado Terminal/terapia , Progressão da Doença , Feminino , Humanos , Pulmão/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Pneumonia Viral/diagnóstico , Pneumonia Viral/epidemiologia , Pneumonia Viral/imunologia , Pneumonia Viral/terapia , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/terapia , Avaliação de Sintomas/métodos , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
6.
Ann Rheum Dis ; 79(6): 724-726, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32349982

RESUMO

Due to the frequent presence of interstitial lung disease and widespread use of immunosuppressive treatment, systemic sclerosis (SSc) patients may be considered at risk for a more severe disease course and higher mortality when they develop Severe Acute Respiratory Syndrome - Coronavirus - 2 (SARS-CoV-2) virus infection. Therefore, with World Scleroderma Foundation endorsement, experts from different specialties including rheumatology, virology and clinical immunology gathered virtually to answer to the main practical clinical questions regarding SARS-CoV-2 infection coming from both patients and physicians. This preliminary advice is aligned with other national and international recommendations, adapted for SSc patients.


Assuntos
Infecções por Coronavirus/tratamento farmacológico , Infecções por Coronavirus/fisiopatologia , Pneumonia Viral/tratamento farmacológico , Pneumonia Viral/fisiopatologia , Escleroderma Sistêmico/terapia , Escleroderma Sistêmico/virologia , Anticorpos Monoclonais Humanizados/uso terapêutico , Betacoronavirus , Infecções por Coronavirus/epidemiologia , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/virologia , Pandemias , Pneumonia Viral/epidemiologia , Pneumonia Viral/virologia , /virologia , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/imunologia
8.
Clin Rheumatol ; 39(7): 2063-2065, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32462423

RESUMO

COVID-19 outbreak has quickly spread worldwide, causing a high pressure on the health-care system. In Italy, from March 8, 2020, all the deferrable clinical activities have been suspended to increase the health care offer for COVID-19 patients. The hospital organization has been modified also in order to assure non-COVID-19 patients assistance. The Scleroderma Unit of ASST Pini-CTO Hospital, in Milan, in the region mostly hit by SARS-CoV-2 in Italy, follows more than 600 patients affected by systemic sclerosis (SSc). Patients with SSc need a close follow-up with a regular screening of organ involvement and frequent intravenous treatments. All SSc patients have been educated about ministerial directives to limit COVID-19 spread. The organization of our Scleroderma Unit has been quickly rethought to assure SSc patients assistance in safety for them and for health-care workers during urgent visits or infusion therapies. Using electronic way of communication with frequent virtual contact and guarantying home deliveries of some therapies, we allowed a continuity of care also outside the Hospital.


Assuntos
Infecções por Coronavirus , Procedimentos Clínicos , Unidades Hospitalares/organização & administração , Controle de Infecções/organização & administração , Pandemias , Pneumonia Viral , Escleroderma Sistêmico , Betacoronavirus/isolamento & purificação , Comorbidade , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/prevenção & controle , Procedimentos Clínicos/organização & administração , Procedimentos Clínicos/tendências , Gerenciamento Clínico , Humanos , Itália/epidemiologia , Inovação Organizacional , Pandemias/prevenção & controle , Pneumonia Viral/epidemiologia , Pneumonia Viral/prevenção & controle , Reumatologia/métodos , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/terapia
9.
Clin Exp Dermatol ; 45(6): 673-678, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32472964

RESUMO

Morphoea, also known as localized scleroderma, is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Paediatric-onset disease is not uncommon and is associated with frequent relapses. The disease has complex pathogenetic mechanisms and multiple clinical subtypes, and affects children of all ages. Recent research has focused on elucidating the disease pathophysiology and identifying measures of disease activity. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Relevant studies, including randomized trials, reviews of standard current guidelines and original research articles, were selected, and results were analysed before being summarized. In the first of this two-part review, we provide a bird's-eye view of the current literature concerning the epidemiology, aetiopathogenesis and clinical classification of paediatric morphoea; in Part 2, we review the diagnosis, markers of disease activity, management and natural history.


Assuntos
Esclerodermia Localizada , Criança , Humanos , Esclerodermia Localizada/classificação , Esclerodermia Localizada/epidemiologia , Esclerodermia Localizada/etiologia , Escleroderma Sistêmico/classificação , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/etiologia
10.
Ann Biol Clin (Paris) ; 78(2): 126-133, 2020 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-32175889

RESUMO

AIM: To describe the autoantibody profile in a cohort of Algerian patients with systemic sclerosis (SSc) and to determine clinical associations between SSc-related autoantibodies, disease subtypes and specific clinical features. METHODS: Consecutive Algerian patients with SSc were included in the present study. In addition to clinical characterization, all subjects underwent autoantibody testing using indirect immunofluorescence, immunoenzymatic, and line immunoblot assays. RESULTS: A total of 150 patients were included in this study, 103 (68.7%) had limited cutaneous SSc (lcSSc), 42 (28%) had diffuse cutaneous SSc (dcSSc) and 5 (3.3%) had sine cutaneous scleroderma. One hundred thirty-five (90.0%) patients were positive for SSc-related autoantibodies, including 63 (42%) with more than one autoantibody. The two most frequent autoantibodies were anti-topoisomerase I (ATA) (76; 50.7%) and anti-SSA/Ro (49; 32.7%). Only 23 (15.3%) patients were positive for anticentromere; 9 (6%) were positive for anti RNA polymerase III; 5 (3.3%) for anti-U3 RNP; 3 (2%) for anti Th/To; 25 (16.7%) for anti-U1 RNP; 11 (7.3%) for anti-PM/Scl and 4 (2.7%) for anti-Ku. Anti-topoisomerase I was associated with dcSSc (p <0.0001), interstitial lung disease (ILD) (p <0.0001) and digital ulcers (p <0.0001). Anti-U3 RNP was associated with pulmonary arterial hypertension (PAH) (p=0.031). CONCLUSION: Notable similarities and differences in the prevalence of SSc-related autoantibodies were found in our population when compared to other ethnic groups. ATA and anti-U3 RNP may be a reliable biomarker for ILD and PAH. Further studies should be conducted to better understand the ethnic influence on disease expression and autoantibody production.


Assuntos
Autoanticorpos/sangue , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/epidemiologia , Adulto , Argélia/epidemiologia , Autoanticorpos/análise , Biomarcadores/análise , Biomarcadores/sangue , Estudos de Coortes , Feminino , Imunofluorescência , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Prognóstico , Escleroderma Sistêmico/diagnóstico
11.
Ann Rheum Dis ; 79(5): 626-634, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32161055

RESUMO

OBJECTIVES: To evaluate initial combination therapy with ambrisentan plus tadalafil (COMB) compared with monotherapy of either agent (MONO), and the utility of baseline characteristics and risk stratification in predicting outcomes, in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) and the systemic sclerosis (SSc)-pulmonary arterial hypertension (PAH) subpopulation. METHODS: This post hoc analysis of the Ambrisentan and Tadalafil in Patients with Pulmonary Arterial Hypertension (AMBITION) study included patients with CTD-PAH from the modified intention-to-treat population. Time to clinical failure (TtCF) was assessed by baseline characteristics, treatment assignment and risk group (low, intermediate and high) at baseline and week 16. TtCF was compared between groups using Kaplan-Meier curves and Cox proportional hazards regression modelling. RESULTS: The analysis included 216 patients (COMB, n=117; MONO, n=99). The risk of clinical failure was lower with COMB versus MONO (risk reduction: CTD-PAH 51.7%, SSc-PAH 53.7%), particularly in patients with haemodynamic parameters characteristic of typical PAH without features of left heart disease and/or restrictive lung disease at baseline. The risk of clinical failure was lower with COMB versus MONO in the baseline low-risk group (HR not calculated due to no events in COMB), baseline intermediate-risk group (HR 0.519, 95% CI 0.297 to 0.905) and in the week 16 low-risk group (HR 0.069, 95% CI 0.009 to 0.548). CONCLUSIONS: The benefit of COMB over MONO was demonstrated in patients with CTD-PAH, particularly in those with typical PAH haemodynamic characteristics at baseline. COMB is appropriate for patients categorised as low risk and intermediate risk at baseline and low risk at follow-up. TRIAL REGISTRATION NUMBER: NCT01178073.


Assuntos
Fenilpropionatos/administração & dosagem , Hipertensão Arterial Pulmonar/tratamento farmacológico , Hipertensão Arterial Pulmonar/epidemiologia , Piridazinas/administração & dosagem , Escleroderma Sistêmico/tratamento farmacológico , Escleroderma Sistêmico/epidemiologia , Tadalafila/administração & dosagem , Adulto , Comorbidade , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/tratamento farmacológico , Doenças do Tecido Conjuntivo/epidemiologia , Relação Dose-Resposta a Droga , Método Duplo-Cego , Esquema de Medicação , Quimioterapia Combinada , Feminino , Seguimentos , Humanos , Análise de Intenção de Tratamento , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Hipertensão Arterial Pulmonar/diagnóstico , Medição de Risco , Escleroderma Sistêmico/diagnóstico , Resultado do Tratamento , Vasodilatadores/administração & dosagem
12.
Isr Med Assoc J ; 22(2): 104-110, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32043328

RESUMO

BACKGROUND: Autologous hematological stem cell transplantation (HSCT) is a novel therapy for systemic sclerosis (SSc) that has been validated in three randomized controlled trials. OBJECTIVES: To report the first Israeli experience with HSCT for progressive SSc and review the current literature. METHODS: Five SSc patients who were evaluated in our department and were treated by HSCT were included. Medical records were evaluated retrospectively. Demographic, clinical, and laboratory data were recorded. Continuous data are presented as the mean ± standard deviation. Categorical variables are presented as frequencies and percentages. RESULTS: Five SSc patients were treated with HSCT. Four patients were adults (mean age 53 ± 12 years) and one was a 12-year-old pediatric patient. All patients were female. HSCT was initiated 1.4 ± 0.8 years after diagnosis. Two patients were RNA POLIII positive, two were anti-topoisomerase 1 positive, and one only antinuclear antibodies positive. All patients had skin and lung involvement. The mean modified Rodnan Skin Score was 29 ± 4.7 before HSCT, which improved to 10.4 ± 9.6 after HSCT. The forced vital capacity improved from 68 ± 13% to 90 ± 28%. Diffusing capacity of the lungs for carbon monoxide increased by 6%. Among severe adverse events were cyclophosphamide-related congestive heart failure, antithymocyte globulin-related capillary leak syndrome, and scleroderma renal crisis. All symptoms completely resolved with treatment without sequela. No treatment related mortality was recorded. CONCLUSIONS: HSCT is an important step in the treatment of progressive SSc in Israel. Careful patient selection reduces treatment related morbidity and mortality.


Assuntos
Ciclofosfamida , Transplante de Células-Tronco Hematopoéticas , Escleroderma Sistêmico , Adulto , Autoanticorpos/sangue , Autoanticorpos/classificação , Criança , Ciclofosfamida/administração & dosagem , Ciclofosfamida/efeitos adversos , Feminino , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Israel/epidemiologia , Pulmão/patologia , Monitorização Fisiológica/métodos , Avaliação de Processos e Resultados em Cuidados de Saúde , Testes de Função Respiratória/métodos , Estudos Retrospectivos , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/imunologia , Escleroderma Sistêmico/terapia , Pele/patologia , Transplante Autólogo
13.
Occup Environ Med ; 77(2): 64-69, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31848232

RESUMO

OBJECTIVES: Increased risk has been suggested for autoimmune rheumatic diseases following solvent exposure. The evidence for specific solvents is limited, and little is known about exposure-response relations. Styrene is an aromatic, organic solvent and the objective of this study was to analyse the association between occupational styrene exposure and autoimmune rheumatic diseases in men and women. METHODS: We followed 72 212 styrene-exposed workers of the Danish reinforced plastics industry from 1979 to 2012. We modelled full work history of styrene exposure from employment history, survey data and historical styrene exposure measurements. We identified cases in the national patient registry and investigated gender-specific exposure-response relations by cumulative styrene exposure for different exposure time windows adjusting for age, calendar year and educational level. RESULTS: During 1 515 126 person-years of follow-up, we identified 718 cases of an autoimmune rheumatic disease, of which 73% were rheumatoid arthritis. When adjusting for potential confounders and comparing the highest with the lowest styrene exposure tertile, we observed a statistically non-significantly increased risk of systemic sclerosis among women (incidence rate ratio (IRR)=2.50; 95% CI 0.50 to 12.50) and men (IRR=1.86; 95 % CI 0.50 to 7.00), based on 9 and 22 cases, respectively. Results were inconsistent for the other autoimmune rheumatic diseases examined. CONCLUSION: This study suggests an association between occupational styrene exposure and systemic sclerosis in men as well as in women but based on few cases. This is a new finding and has to be replicated before conclusions can be drawn.


Assuntos
Doenças Autoimunes/etiologia , Doenças Profissionais/etiologia , Exposição Ocupacional/efeitos adversos , Doenças Reumáticas/etiologia , Escleroderma Sistêmico/etiologia , Solventes/efeitos adversos , Estireno/efeitos adversos , Adulto , Idoso , Artrite Reumatoide/epidemiologia , Artrite Reumatoide/etiologia , Doenças Autoimunes/epidemiologia , Dinamarca/epidemiologia , Humanos , Indústria Manufatureira , Pessoa de Meia-Idade , Doenças Profissionais/epidemiologia , Plásticos , Sistema de Registros , Doenças Reumáticas/epidemiologia , Escleroderma Sistêmico/epidemiologia , Fatores Sexuais
14.
Rheumatology (Oxford) ; 59(6): 1315-1324, 2020 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-31586421

RESUMO

OBJECTIVES: Urinary tract involvement is a seldom-reported manifestation of SSc that could compromise patients' quality of life. This study compares lower urinary tract symptoms (LUTS) in SSc patients and in healthy subjects and their association with clinical and diagnostic parameters. METHODS: LUTS were assessed through self-reported questionnaires in 42 SSc patients and 50 matched healthy subjects. Statistical analyses were performed to explore LUTS in the two populations and their association with SSc variables, including nailfold videocapillaroscopy patterns, SSc-related antibodies and DXA parameters. RESULTS: SSc patients showed significantly higher prevalence and severity of urinary incontinence (UI) and overactive bladder (OAB) than healthy controls (P < 0.005, P < 0.01). SSc was a strong predictor of LUTS, independent of demographic data, comorbidities and treatments (odds ratio 5.57, 95% CI 1.64-18.88). In SSc patients OAB positively correlated with sarcopenia (P < 0.001), and both OAB and UI significantly correlated with reduced BMD (P < 0.05, P = 0.001). UI positively correlated with Scl70 antibodies (P < 0.05) and ciclosporin treatment (P = 0.001) and negatively with RNA polymerase III antibodies (P < 0.05); OAB positively correlated with calcinosis (P < 0.005) and negatively with methotrexate treatment (P < 0.05). Nailfold videocapillaroscopy 'active' and 'late' patterns were predominant among SSc patients presenting urinary symptoms, although no statistical correlation was found. CONCLUSION: For the first time urinary tract involvement was found to be significantly higher in SSc patients than in healthy matched controls. In addition, sarcopenia, bone damage and calcinosis appeared significantly correlated with LUTS, suggesting a possible interplay.


Assuntos
Sintomas do Trato Urinário Inferior/epidemiologia , Escleroderma Sistêmico/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Comorbidade , Feminino , Humanos , Sintomas do Trato Urinário Inferior/diagnóstico , Sintomas do Trato Urinário Inferior/fisiopatologia , Masculino , Angioscopia Microscópica , Pessoa de Meia-Idade , Prevalência , Qualidade de Vida , Índice de Gravidade de Doença , Inquéritos e Questionários , Adulto Jovem
16.
Clin Rheumatol ; 39(1): 85-92, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31444650

RESUMO

OBJECTIVE: To assess whether systemic sclerosis (SSc) is associated with total knee arthroplasty (TKA) outcomes. METHODS: We used the 1998-2014 US National Inpatient Sample. We conducted multivariable-adjusted logistic regression analyses to examine the association of a diagnosis of SSc with post-TKA in-hospital complications (implant infection, revision, transfusion, mortality) and healthcare utilization (hospital charges, hospital stay, non-home vs. home discharge). Odds ratios (OR) and 95 % confidence intervals (CI) were calculated. RESULTS: Our cohort included 8,123,388 people without SSc and 3894 people with SSc. In multivariable-adjusted analyses, compared to people without SSc, people with SSc had higher odds of transfusion, hospital stay > 3 days and non-home discharge with higher OR of 1.42 (95 % CI, 1.20, 1.69), 1.29 (95 % CI, 1.11, 1.49), and 1.29 (95 % CI, 1.11, 1.49), respectively. No differences were seen in revision, 0.68 (95 % CI, 0.10, 4.69) or hospital charges above the median, 1.01 (95 % CI, 0.70, 1.46). Differences in implant infection or mortality were not estimable, since none of the patients with SSc had implant infection or died. Sensitivity analyses that adjusted the main analysis additionally for hospital-level variables confirmed study findings with minimal or no attenuation of OR. CONCLUSION: SSc was associated with higher risk of transfusion and increased healthcare utilization after TKA. Future studies should examine if interventions can address modifiable factors to further optimize these outcomes.Key Points• Systemic sclerosis was independently associated with higher healthcare utilization after TKA.• The adjusted odds of transfusion was higher in people with systemic sclerosis compared to those without systemic sclerosis who underwent TKA.


Assuntos
Artroplastia do Joelho , Transfusão de Sangue/estatística & dados numéricos , Osteoartrite do Joelho/cirurgia , Aceitação pelo Paciente de Cuidados de Saúde/estatística & dados numéricos , Escleroderma Sistêmico/epidemiologia , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Comorbidade , Feminino , Preços Hospitalares , Humanos , Tempo de Internação/estatística & dados numéricos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Osteoartrite do Joelho/epidemiologia , Alta do Paciente/estatística & dados numéricos , Complicações Pós-Operatórias/epidemiologia , Infecções Relacionadas à Prótese/epidemiologia , Fatores de Risco , Estados Unidos
17.
Clin Rheumatol ; 39(1): 19-26, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31087225

RESUMO

INTRODUCTION/OBJECTIVES: To compare the risk of different bleeding outcomes between patients with systemic sclerosis (SSc) and the general population free of SSc. METHODS: Using UK electronic primary care data (2000-2012), 1314 patients with SSc and a matched SSc-free comparison cohort (n = 19,992) were followed until December 2013 to identify bleeding, confirmed following manual review of patient records including free text comments. Incidence rates were calculated and Cox regression used to estimate adjusted hazard ratios (HRs; SSc cohort vs. matched general population cohort) adjusted for confounders. RESULTS: One hundred and twenty-seven bleeding events occurred in the SSc cohort and 1762 in the general population cohort; incidence rates per 1000 person-years for the SSc cohort and general population cohort were 0.5 versus 0.3 for hemorrhagic stroke, 4.1 versus 3.3 for gastrointestinal bleeding, 2.5 versus 1.7 for pulmonary hemorrhage, 8.4 versus 7.5 for urogenital bleeding, and 15.5 versus 12.9 for any of the aforementioned bleedings. Adjusted HRs (95% confidence intervals) were 1.21 (1.00-1.46) for any bleeding, 1.51 (0.54-4.21) for hemorrhagic stroke, 1.50 (0.96-2.35) for pulmonary hemorrhage, 1.08 (0.75-1.54) for gastrointestinal bleeds, and 1.28 (1.00-1.64) for urogenital bleeds. HRs were more often higher in SSc patients with organ involvement than without organ involvement and in those with diffuse cutaneous SSc. CONCLUSION: Our results are consistent with a moderately increased risk of bleeding in SSc patients. Further evidence from large SSc patient cohorts is needed to confirm this finding.Key Points• The risk of experiencing a major bleed may be higher among patients with SSc than the general population.• Further large and well-designed studies are needed to corroborate our findings.


Assuntos
Hemorragia/epidemiologia , Escleroderma Sistêmico/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Estudos de Coortes , Bases de Dados Factuais , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Reino Unido/epidemiologia , Adulto Jovem
18.
Z Rheumatol ; 79(7): 718-724, 2020 Sep.
Artigo em Alemão | MEDLINE | ID: mdl-31848701

RESUMO

BACKGROUND: Scleroderma or systemic sclerosis (SSc) is a rare autoimmune rheumatic connective tissue disease. The clinical picture is manifold and symptoms can vary greatly between different patients. All manifestations are possible ranging from isolated skin involvement up to systemic disease with multiple organ manifestations. Due to this inhomogeneous clinical picture, it often takes years until the correct diagnosis is made and adequate treatment is started. METHODS: Patients with the main or secondary diagnosis of systemic sclerosis (M34) between 2002 and 2017 were retrospectively recorded from the patient databases of the ACURA clinic for acute rheumatology in Bad Kreuznach and the data were evaluated. Of special interest were pulmonary parameters over the course of time. Furthermore, standardized questionnaires were distributed to general practitioners in Rhineland-Palatinate via the Association of Statutory Health Insurance Physicians as well as to patients admitted to the hospital (2016-2017). RESULTS: A total of 135 patients could be evaluated. For women the median age of onset was 52 years (interquartile range, IQR 44-64 years) and for men the median age of onset was 49 years (IQR 38-54 years). Lung involvement was detected in 54% of the cases. Including the individual time to diagnosis, there was a significant worsening of the diffusing capacity for carbon monoxide (73% vs. 56%, p = 0.046) between earlier (<4 months) and later (4-18 months) diagnoses, which also persisted in the follow-up (74% vs. 53%) despite adequate treatment. CONCLUSION: A rapid diagnosis within 3 months of the onset of Raynaud's phenomenon seems to play a key role in the preservation of lung function.


Assuntos
Doenças do Tecido Conjuntivo , Doença de Raynaud , Escleroderma Sistêmico , Adulto , Comorbidade , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Raynaud/diagnóstico , Doença de Raynaud/epidemiologia , Estudos Retrospectivos , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia
19.
Proc Natl Acad Sci U S A ; 117(1): 552-562, 2020 01 07.
Artigo em Inglês | MEDLINE | ID: mdl-31871193

RESUMO

Systemic sclerosis (SSc) is a clinically heterogeneous autoimmune disease characterized by mutually exclusive autoantibodies directed against distinct nuclear antigens. We examined HLA associations in SSc and its autoantibody subsets in a large, newly recruited African American (AA) cohort and among European Americans (EA). In the AA population, the African ancestry-predominant HLA-DRB1*08:04 and HLA-DRB1*11:02 alleles were associated with overall SSc risk, and the HLA-DRB1*08:04 allele was strongly associated with the severe antifibrillarin (AFA) antibody subset of SSc (odds ratio = 7.4). These African ancestry-predominant alleles may help explain the increased frequency and severity of SSc among the AA population. In the EA population, the HLA-DPB1*13:01 and HLA-DRB1*07:01 alleles were more strongly associated with antitopoisomerase (ATA) and anticentromere antibody-positive subsets of SSc, respectively, than with overall SSc risk, emphasizing the importance of HLA in defining autoantibody subtypes. The association of the HLA-DPB1*13:01 allele with the ATA+ subset of SSc in both AA and EA patients demonstrated a transancestry effect. A direct correlation between SSc prevalence and HLA-DPB1*13:01 allele frequency in multiple populations was observed (r = 0.98, P = 3 × 10-6). Conditional analysis in the autoantibody subsets of SSc revealed several associated amino acid residues, mostly in the peptide-binding groove of the class II HLA molecules. Using HLA α/ß allelic heterodimers, we bioinformatically predicted immunodominant peptides of topoisomerase 1, fibrillarin, and centromere protein A and discovered that they are homologous to viral protein sequences from the Mimiviridae and Phycodnaviridae families. Taken together, these data suggest a possible link between HLA alleles, autoantibodies, and environmental triggers in the pathogenesis of SSc.


Assuntos
Autoanticorpos/imunologia , Autoantígenos/genética , Antígenos HLA/genética , Mimetismo Molecular/imunologia , Escleroderma Sistêmico/genética , Afro-Americanos/genética , Alelos , Sequência de Aminoácidos/genética , Antígenos Virais/genética , Antígenos Virais/imunologia , Autoantígenos/imunologia , Biologia Computacional , Conjuntos de Dados como Assunto , Grupo com Ancestrais do Continente Europeu/genética , Feminino , Predisposição Genética para Doença , Antígenos HLA/imunologia , Humanos , Masculino , Mimiviridae/imunologia , Phycodnaviridae/imunologia , Estrutura Secundária de Proteína/genética , Medição de Risco , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/imunologia , Homologia de Sequência de Aminoácidos
20.
Clin Rev Allergy Immunol ; 58(1): 40-51, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30607749

RESUMO

Systemic sclerosis is an autoimmune disease characterized by fibrosis of skin and internal organs, vasculopathy, and dysregulation of immune system. A diagnostically important feature of immunological abnormalities in systemic sclerosis is the presence of circulating antinuclear antibodies, which may be detected in 90-95% of patients with either of the four main laboratory methods: immunofluorescence, enzyme-linked immunosorbent assay, immunodiffusion, and immunoblotting. There are several antinuclear antibodies specific for systemic sclerosis. These include antibodies against topoisomerase (anti-TOPO I), kinetochore proteins (ACA), RNA polymerase enzyme (anti-RNAP III), ribonuclear proteins (anti-U11/U12 RNP, anti-U1 RNP, anti-U3 RNP) and nucleolar antigens (anti-Th/To, anti-NOR 90, anti-Ku, antiRuvBL1/2, and anti-PM/Scl). Autoantibodies specific for systemic sclerosis have been linked to distinct clinical features. Therefore, detecting a particular antibody type is important in predicting a possible organ involvement and prognosis and may have an impact on monitoring and treatment.


Assuntos
Anticorpos Antinucleares/imunologia , Autoimunidade , Suscetibilidade a Doenças , Escleroderma Sistêmico/etiologia , Autoanticorpos/imunologia , Autoantígenos/imunologia , Biomarcadores , Diagnóstico Diferencial , Humanos , Técnicas de Diagnóstico Molecular/métodos , Técnicas de Diagnóstico Molecular/normas , Prevalência , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/metabolismo
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