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1.
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 , Testes de Função Respiratória/métodos , Estudos Retrospectivos , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/imunologia , Escleroderma Sistêmico/terapia , Pele/patologia , Transplante Autólogo
2.
Br J Hosp Med (Lond) ; 80(9): 530-536, 2019 Sep 02.
Artigo em Inglês | MEDLINE | ID: mdl-31498665

RESUMO

Systemic sclerosis is a complex autoimmune connective tissue disease which carries a significant burden of disease-related morbidity including potentially life-threatening complications. Systemic sclerosis can affect all the major organs and therefore, although the disease is uncommon, many hospital-based specialists are involved in patient care. Vascular disease (e.g. Raynaud's phenomenon) is an almost universal symptom in patients with systemic sclerosis and is often the earliest manifestation of the disease. Systemic sclerosis not uncommonly can overlap with other rheumatological conditions (e.g. rheumatoid arthritis and myositis). During the past few decades there have been major advances in understanding the pathogenesis of systemic sclerosis and these are driving advances in treatment. There are now a number of effective treatments to manage many of the different organ-based complications. Autologous haemopoietic stem cell transplantation is a potential treatment option in highly selected patients. This review updates the clinician about epidemiology, pathogenesis, differential diagnosis, the wide clinical spectrum of disease, and current and emerging treatments for systemic sclerosis.


Assuntos
Escleroderma Sistêmico/fisiopatologia , Humanos , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/terapia
3.
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
4.
J Dtsch Dermatol Ges ; 17(7): 716-728, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31364293

RESUMO

Systemic scleroderma/systemic sclerosis (SSc) is an inflammatory connective tissue disease clinically characterized by two major subtypes: limited and diffuse SSc. While both conditions present with Raynaud's phenomenon (paroxysmal digital ischemia), diffuse SSc is associated with rapid disease progression and early - prognostically relevant - involvement of internal organs. Treatment is challenging. In addition to general lifestyle modifications, measures include treatments aimed at improving circulation as well as immunosuppressive and immunomodulatory drugs. However, these agents are effective only in terms of slowing disease progression.


Assuntos
Doença de Raynaud/terapia , Escleroderma Sistêmico/terapia , Progressão da Doença , Humanos , Imunossupressores , Estilo de Vida , Doença de Raynaud/complicações , Escleroderma Sistêmico/classificação , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/epidemiologia
5.
Int J Rheum Dis ; 22(6): 1041-1045, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30938067

RESUMO

AIM: Low levels of vitamin D (25OHD) have been found to associated with digital ulcers (DUs) in systemic sclerosis (SSc), although only cross-sectional studies have been performed. We aimed to investigate if variations in vitamin D serum levels over time affect DU in SSc. METHODS: This is a retrospective study on 65 patients. 25OHD was measured in 2011 and 2016 and its variations correlated with DU. RESULTS: The mean age of our cohort was 58 (SD 12) years with a mean disease duration of 9.5 (5.3) years. Most of our patients had a limited SSc (69.2%). At baseline 50.8% and 41.5% after 5 years had 25OHD <30 ng/mL. Patients receiving supplementation (8750 IU/wk) at baseline numbered 39 (60.0%) and 45 (69.2%) at the end of follow up. Nevertheless, 31 (47.7%) had a decrease of 25OHD in 5 years. In univariate analysis, patients with incident DU had a decrease in 25OHD as compared to patients with no incident DU (-17.4 [37.0] vs 13.0 [89.5], P = 0.018). No differences in 25OHD variations were found for other disease characteristics. In multivariate analysis correcting for previous DU and modified Rodnan Skin Score at baseline, patients with a decrease in 25OHD had an increased risk of developing DU (odds ratio 16.6; 95% CI 1.7-164.5, P = 0.017). CONCLUSIONS: A decrease in 25OHD is associated with the risk of developing DUs. In addition, vitamin D supplementation with the doses currently recommended may be insufficient in SSc. Further studies in wider cohorts are needed to confirm these results.


Assuntos
Escleroderma Sistêmico/sangue , Úlcera Cutânea/sangue , Deficiência de Vitamina D/sangue , Vitamina D/análogos & derivados , Idoso , Biomarcadores/sangue , Suplementos Nutricionais , Feminino , Humanos , Incidência , Itália/epidemiologia , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/tratamento farmacológico , Escleroderma Sistêmico/epidemiologia , Úlcera Cutânea/diagnóstico , Úlcera Cutânea/epidemiologia , Úlcera Cutânea/prevenção & controle , Fatores de Tempo , Vitamina D/sangue , Deficiência de Vitamina D/diagnóstico , Deficiência de Vitamina D/tratamento farmacológico , Deficiência de Vitamina D/epidemiologia
6.
Arthritis Rheumatol ; 71(9): 1553-1570, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-30969034

RESUMO

OBJECTIVE: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. METHODS: A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty-four clinical and serologic variables were used for clustering. RESULTS: Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. CONCLUSION: Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis.


Assuntos
Fenótipo , Esclerodermia Difusa/epidemiologia , Esclerodermia Limitada/epidemiologia , Escleroderma Sistêmico/epidemiologia , Adulto , Idoso , Autoanticorpos/sangue , Análise por Conglomerados , Bases de Dados Factuais , Europa (Continente)/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , Esclerodermia Difusa/sangue , Esclerodermia Difusa/patologia , Esclerodermia Limitada/sangue , Esclerodermia Limitada/patologia , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/patologia , Índice de Gravidade de Doença
7.
Int J Rheum Dis ; 22(6): 1016-1022, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30924296

RESUMO

AIM: This study aimed to evaluate the association between myocardial abnormalities and left ventricular (LV) geometry as assessed using cardiac magnetic resonance imaging (CMRI) in systemic sclerosis (SSc) patients without cardiac symptoms. METHODS: SSc patients without cardiac symptoms or cardiovascular risk factors underwent contrast CMRI. CMRI were assessed for structural and functional LV parameters and myocardial fibrosis based on myocardial late gadolinium enhancement (LGE). The correlation between brain natriuretic peptide (BNP) levels and LGE status was evaluated. RESULTS: Among 49 patients, 27 (55%) showed LGE positivity. The most common identified LGE pattern was a linear pattern. LGE was not consistent with coronary artery distribution. There was no difference in ejection fraction between those with and without LGE. LV morphological changes were observed in 29% of SSc patients. An abnormal LV structure was detected in 44% and 14% of patients in the LGE+ and LGE- groups, respectively. The BNP levels were higher by 57% in the LGE+ group than in the LGE-group. Receiver operating characteristic analysis showed that BNP levels reliably detected myocardial abnormalities (area under the curve, 0.72; 95% confidence interval 0.58-0.88). CONCLUSIONS: Myocardial abnormalities were common in SSc patients without cardiac symptoms. We suggest that LV morphological changes may have resulted from myocardial abnormalities. BNP may be useful as a screening tool for the detection of myocardial abnormalities in SSc patients.


Assuntos
Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Peptídeo Natriurético Encefálico/sangue , Escleroderma Sistêmico/epidemiologia , Função Ventricular Esquerda , Remodelação Ventricular , Doenças Assintomáticas , Biomarcadores/sangue , Feminino , Fibrose , Humanos , Hipertrofia Ventricular Esquerda/sangue , Hipertrofia Ventricular Esquerda/epidemiologia , Hipertrofia Ventricular Esquerda/fisiopatologia , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Valor Preditivo dos Testes , Prevalência , Prognóstico , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Escleroderma Sistêmico/diagnóstico
8.
Int J Rheum Dis ; 22(6): 1023-1028, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30834657

RESUMO

AIM: We investigated the association between systemic sclerosis (SSc) and perioperative cardiovascular risk for inpatient non-cardiac surgical procedures. METHODS: We used data from the National Inpatient Sample (NIS) for the year 2014 to identify patients undergoing inpatient non-cardiac surgery. SSc and major adverse cardiovascular events (MACE) were defined by International Classification of Diseases 9th Revision diagnosis codes. Univariate and multivariate analyses were performed. We adjusted for demographic information, socioeconomic status, cardiac comorbidities, cardiovascular risk factors and procedural category. Two models were used with different categorization strategies for surgical procedures. RESULTS: A total of 8 156 379 hospitalizations for non-cardiac surgeries were included, 4385 of which had a diagnosis of SSc. Patients with SSc were older, more likely to be female and Caucasian and with higher cardiac and systemic comorbidity burden. In univariate analysis, SSc was associated with higher risk of perioperative MACE (odds ratio [OR] = 2.9; P < 0.001) and all-cause death (P = 3.07; P < 0.001). Multivariate analysis yielded conflicting results regarding the association between SSc and perioperative MACE (Model 1: OR = 1.42; P = 0.146; Model 2: OR = 1.59; P = 0.048). Subsequent analysis showed that only perioperative myocardial infarction (Model 1 OR = 1.85; P = 0.048; Model 2 OR = 1.94; P = 0.031) was independently associated with SSc. CONCLUSION: We did not find consistent association between SSc and perioperative MACE in non-cardiac surgical procedures. SSc may be associated with perioperative myocardial infarction.


Assuntos
Doenças Cardiovasculares/epidemiologia , Escleroderma Sistêmico/epidemiologia , Procedimentos Cirúrgicos Operatórios/efeitos adversos , Doenças Cardiovasculares/diagnóstico , Estudos Transversais , Bases de Dados Factuais , Feminino , Humanos , Pacientes Internados , Masculino , Pessoa de Meia-Idade , Infarto do Miocárdio/epidemiologia , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Escleroderma Sistêmico/diagnóstico , Fatores de Tempo , Resultado do Tratamento , Estados Unidos/epidemiologia
9.
Int J Rheum Dis ; 22(4): 695-699, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30729669

RESUMO

BACKGROUND: Small intestinal bacterial overgrowth (SIBO) results in nutrient malabsorption and malnutrition, thereby increasing the morbidity and mortality in systemic sclerosis (SSc) patients. OBJECTIVES: To evaluate the prevalence and associated factors of SIBO in SSc patients. METHOD: A cross-sectional study was conducted between July 2015 and January 2016 in SSc patients over 18, using the glucose H2 /CH4 breath test to evaluate SIBO. RESULTS: Eighty-nine SSc patients (30 male and 59 female) underwent the glucose H2 /CH4 breath test. The mean age was 54.4. Twelve participants were positive for the glucose H2 /CH4 breath test, yielding a SIBO prevalence of 13.5% (95% CI 7.2-22.4) among SSc patients. A multivariate analysis revealed that duration of disease >5 years was significantly associated with SIBO (adjusted odds ratio 9.38; 95% CI 1.09-80.47). CONCLUSION: The prevalence of SIBO, using the glucose H2 /CH4 breath test, is not common among Thai SSc patients. However, a positive result was associated with longer duration of disease.


Assuntos
Bactérias/crescimento & desenvolvimento , Síndrome da Alça Cega/epidemiologia , Microbioma Gastrointestinal , Intestino Delgado/microbiologia , Escleroderma Sistêmico/epidemiologia , Adulto , Idoso , Síndrome da Alça Cega/diagnóstico , Síndrome da Alça Cega/microbiologia , Testes Respiratórios , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Medição de Risco , Fatores de Risco , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/microbiologia , Tailândia/epidemiologia , Fatores de Tempo
10.
Medicine (Baltimore) ; 98(6): e14342, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30732161

RESUMO

BACKGROUND: Pulmonary artery systolic pressure (PASP) is an important parameter for detecting pulmonary arterial hypertension (PAH). The difference between rest PASP and post-exercise PASP (ΔPASP) may play a role in predicting and screening resting PAH. The aim of this study is to analyze ΔPASP in systemic sclerosis (SSc) patients with PAH or non-PAH and suggest a cutoff value of ΔPASP for detection of PAH. METHODS: PubMed, Embase, and Web of Science were searched for relevant publications up to July 7, 2018. Characteristics of control, no PAH, exercise-induced PAH (EIPH) and PAH subgroups in SSc patients were extracted. R 3.5.0 with the "meta" package was used to conduct this meta-analysis. RESULTS: Twelve articles involving 1279 patients were included in this study. The subgroups meta-analysis showed pooled mean ΔPASP in different subgroups: control group (8.6 mmHg, 95% CI: 6.9-10.5), no PAH group (12.2 mmHg, 95% CI: 11.2-13.2), EIPH group (26.0 mmHg, 95% CI: 24.2-27.7) and PAH group (36.2 mmHg, 95% CI: 29.7-42.7). CONCLUSION: Combining the results of our study with the previous studies, an abnormal increase in PASP after exercise could indicate the development of PAH in SSc patients. In addition, if ΔPASP>29 mmHg, a high suspicion of PAH should be raised.


Assuntos
Exercício/fisiologia , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Artéria Pulmonar/fisiopatologia , Escleroderma Sistêmico/epidemiologia , Pressão Sanguínea/fisiologia , Humanos , Hipertensão Pulmonar/fisiopatologia , Descanso/fisiologia , Escleroderma Sistêmico/fisiopatologia
11.
Expert Rev Gastroenterol Hepatol ; 13(3): 213-227, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30791766

RESUMO

INTRODUCTION: Systemic sclerosis (SSc) is a multisystem connective tissue disease, characterized by chronic inflammation and vascular changes that result in esophageal smooth muscle atrophy and fibrosis. Subsequent progressive loss of peristalsis in the distal esophagus and loss of lower esophageal sphincter function lead to problems with the protective barrier and exposure of sensitive tissues to the gastroduodenal contents, a disorder called reflux disease. Areas covered: Depending on the range, nature and symptoms of the disease, the term 'reflux disease' may refer to gastroesophageal reflux, laryngopharyngeal reflux, microaspiration into the airways and silent reflux. Despite the links between these visceral complications, this connection remains controversial. This is due to a lack of complete understanding, the asymptomatic nature of the disease and the limited diagnostic accuracy of tests, which can delay diagnosis. Such delays are problematic, given that the early detection of GERD in SSc patients, the timing of assessment, the treatment of the organs involved are critical aspects of patient prognosis and disease outcome. Expert commentary: This review summarizes the most recent knowledge about the pathophysiology, diagnosis and prospective treatment of GERD in SSc patients and highlights how innovative technologies applied through an integrative, interdisciplinary approach may soon lead to effective treatment strategies.


Assuntos
Esôfago/fisiopatologia , Refluxo Gastroesofágico , Refluxo Laringofaríngeo , Aspiração Respiratória , Sistema Respiratório/fisiopatologia , Escleroderma Sistêmico , Refluxo Gastroesofágico/diagnóstico , Refluxo Gastroesofágico/epidemiologia , Refluxo Gastroesofágico/fisiopatologia , Refluxo Gastroesofágico/terapia , Humanos , Refluxo Laringofaríngeo/diagnóstico , Refluxo Laringofaríngeo/epidemiologia , Refluxo Laringofaríngeo/fisiopatologia , Refluxo Laringofaríngeo/terapia , Laringe/fisiopatologia , Aspiração Respiratória/diagnóstico , Aspiração Respiratória/epidemiologia , Aspiração Respiratória/fisiopatologia , Fatores de Risco , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/fisiopatologia , Escleroderma Sistêmico/terapia , Resultado do Tratamento
12.
Respir Res ; 20(1): 13, 2019 Jan 18.
Artigo em Inglês | MEDLINE | ID: mdl-30658650

RESUMO

BACKGROUND: Systemic sclerosis (SSc) is a rare connective tissue disease with a heterogeneous clinical course. Interstitial lung disease (ILD) is a common manifestation of SSc and a leading cause of death. MAIN BODY: All patients newly diagnosed with SSc should receive a comprehensive clinical evaluation, including assessment of respiratory symptoms, a high-resolution computed tomography (HRCT) scan of the chest, and pulmonary function tests. ILD can develop in any patient with SSc, including those with pulmonary hypertension, but the risk is increased in those with diffuse (rather than limited) cutaneous SSc, those with anti-Scl-70/anti-topoisomerase I antibody, and in the absence of anti-centromere antibody. While it can occur at any time, the risk of developing ILD is greatest early in the course of SSc, so patients should be monitored closely in the first few years after diagnosis. An increased extent of lung fibrosis on HRCT and a low forced vital capacity (FVC) are predictors of early mortality. While not all patients will require treatment, current approaches to the treatment of progressive SSc-ILD focus on immunosuppressant therapies, including cyclophosphamide and mycophenolate mofetil. In patients with severe and/or rapidly progressive disease, both haematopoietic stem cell transplantation (HSCT) and lung transplantation have been successfully used. A number of medications, including the two drugs approved for the treatment of idiopathic pulmonary fibrosis (IPF), are under active investigation as potential new therapies for SSc-ILD. CONCLUSIONS: Physicians managing patients with SSc should maintain a high level of suspicion and regularly monitor for ILD, particularly in the first few years after diagnosis.


Assuntos
Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/terapia , Escleroderma Sistêmico/fisiopatologia , Escleroderma Sistêmico/terapia , Anti-Inflamatórios/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/métodos , Transplante de Células-Tronco Hematopoéticas/tendências , Humanos , Doenças Pulmonares Intersticiais/epidemiologia , Transplante de Pulmão/métodos , Transplante de Pulmão/tendências , Ensaios Clínicos Controlados Aleatórios como Assunto/métodos , Escleroderma Sistêmico/epidemiologia
13.
Arthritis Rheumatol ; 71(2): 182-195, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30604506
14.
J Eur Acad Dermatol Venereol ; 33(2): 405-409, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29888406

RESUMO

BACKGROUND: Survival can be threatened in certain forms of systemic sclerosis (SSc) so clear prognostic factors are needed. OBJECTIVES: The aim of this meta-analysis was to assess the association between the presence of digital ulcers (DUs) and mortality in SSc. METHODS: We performed a systematic review and meta-analysis in the Pubmed and Scopus databases from the earliest records to May 2017. Two research strategies were performed: « systemic sclerosis ¼ and « digital ulcers ¼ (strategy A); « systemic sclerosis ¼ and « mortality ¼ (strategy B). The primary outcome was the mortality associated with the presence of DUs in patients with SSc. RESULTS: The literature search identified 1473 citations. Fifty-nine studies were examined for full text. Ten articles were included for the meta-analysis. SSc patients with DUs had an increased pooled mortality risk: RR = 1.53 (IC 95%: [1.23-1.90]). CONCLUSIONS: This meta-analysis revealed a higher mortality in SSc patients with associated DUs. Having DUs may be a predictive factor of developing organ involvement such as pulmonary or cardiovascular events that could be associated with poor survival. It suggests that early screening of DUs in SSc patients is important to identify patients most at risk of poor survival.


Assuntos
Causas de Morte , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/patologia , Úlcera Cutânea/epidemiologia , Úlcera Cutânea/patologia , Comorbidade , Intervalos de Confiança , Feminino , Dedos , Humanos , Masculino , Modelos de Riscos Proporcionais , Medição de Risco , Escleroderma Sistêmico/fisiopatologia , Índice de Gravidade de Doença , Úlcera Cutânea/fisiopatologia , Análise de Sobrevida
15.
Arthritis Care Res (Hoboken) ; 71(1): 142-154, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-29648677

RESUMO

OBJECTIVE: To determine excess productivity losses and costs of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and Sjögren's syndrome (SS) at the population level. METHODS: Administrative databases from the province of British Columbia, Canada, were used to establish population-based cohorts of SLE, SSc, and SS, and matched comparison cohorts were selected from the general population. Random samples from these cohorts were surveyed about time absent from paid and unpaid work and working at reduced levels/efficiency (presenteeism), using validated labor questionnaires. We estimated excess productivity losses and costs of each diagnosis (over and above nonsystemic autoimmune rheumatic diseases [non-SARDs]), using 2-part models and work disability rates (not employed due to health). RESULTS: Surveys were completed by 167 SLE, 42 SSc, and 90 SS patients, and by 375 non-SARDs (comparison group) participants. Altogether, predicted excess hours of paid and unpaid work loss were 3.5, 3.2, and 3.4 hours per week for SLE, SSc, and SS patients, respectively. Excess costs were $86, $69, and $84 (calculated as 2015 Canadian dollars) per week, or $4,494, $3,582, and $4,357 per person annually, respectively. Costs for productivity losses from paid work stemmed mainly from presenteeism (SLE = 69% of costs, SSc = 67%, SS = 64%, and non-SARDs = 53%), not from absenteeism. However, many working-age patients were not employed at all, due to health (SLE = 36%, SSc = 32%, SS = 30%, and non-SARDs = 18%), and the majority of total productivity costs were from unpaid work loss (SLE = 73% of costs, SSc = 74%, SS = 60%, and non-SARDs = 47%). Adjusted excess costs from these unpaid production losses were $127, $100, and $82 per week, respectively, among SLE, SSc, and SS patients. CONCLUSION: In this population-based sample of prevalent SLE, SSc, and SS, lost productivity costs were substantial, mainly from presenteeism and unpaid work impairments.


Assuntos
Análise Custo-Benefício/métodos , Eficiência , Lúpus Eritematoso Sistêmico/economia , Vigilância da População , Escleroderma Sistêmico/economia , Síndrome de Sjogren/economia , Adulto , Idoso , Colúmbia Britânica/epidemiologia , Estudos de Coortes , Feminino , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/epidemiologia , Masculino , Pessoa de Meia-Idade , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/epidemiologia
16.
Scand J Rheumatol ; 48(1): 42-51, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30039730

RESUMO

OBJECTIVE: The aim of this study was to analyse differences in clinical presentation in patients with early (< 3 years' duration) systemic sclerosis (SSc), comparing three age groups according to disease subsets. METHOD: Cross-sectional analysis of the prospective EULAR Scleroderma Trials and Research database (EUSTAR) was performed. Patients fulfilling preliminary American College of Rheumatology 1980 classification criteria for SSc, with < 3 years from the first non-Raynaud's SSc symptom at first entry, were selected. Patients with < 3 years from the first SSc symptom, including Raynaud's phenomenon, were also analysed. SSc-related variables, including antibodies, SSc subsets, and organ involvement, were examined. Age was categorized into ≤ 30, 31-59, and ≥ 60 years. We performed descriptive and bivariate analyses. RESULTS: The study included 1027 patients: 90% Caucasian, 80% women, and 40% with diffuse disease. In early stages of SSc, younger patients had significantly more anti-Scl-70 antibodies and diffuse disease. With increasing age, we observed more elevation of estimated pulmonary systolic pressure on echocardiography (5%, 13%, and 30%, respectively, in the three age groups), cardiac conduction blocks (6%, 6%, and 15%), and left ventricular diastolic dysfunction (4%, 12%, and 27%). The results were similar for 650 patients with < 3 years from first SSc symptom, including Raynaud's. CONCLUSION: In early stages of SSc, older patients showed data indicating more severe disease with greater cardiac involvement. The diffuse subset was more frequent in the younger subgroup. The identification of such differences may help in selecting appropriate management for individual patients in clinical practice.


Assuntos
Sistema de Registros , Escleroderma Sistêmico/epidemiologia , Adulto , Distribuição por Idade , Fatores Etários , Idade de Início , Estudos Transversais , Bases de Dados Factuais , Europa (Continente)/epidemiologia , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Retrospectivos , Escleroderma Sistêmico/diagnóstico , Distribuição por Sexo
17.
J Am Acad Dermatol ; 80(2): 478-484, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30092330

RESUMO

BACKGROUND: Skin pigmentation disorders in systemic sclerosis (SSc) have been sparsely described in the literature. Nevertheless, they could be a diagnostic and/or severity marker. OBJECTIVES: To assess the association between pigmentation disorders and systemic involvement in patients with SSc. METHODS: A total of 5 patterns of skin pigmentation disorders were defined: diffuse hyperpigmentation; hyperpigmentation of sun-exposed areas; hypopigmentation of the head, neck, and/or upper part of the chest; acral hypopigmentation; and diffuse hypopigmentation. RESULTS: A total of 239 patients were included; 88 patients (36.8%) had skin pigmentation disorders as follows: diffuse hyperpigmentation and hyperpigmentation of sun-exposed areas in 38.6% (n = 34) and 27.3% (n = 24) of patients, respectively; hypopigmentation of the face, neck, and/or chest in 10.2% of patients (n = 9); diffuse hypopigmentation in 12.5% (n = 11); and acral hypopigmentation in 17% (n = 15). Diffuse hyperpigmentation was associated with diffuse SSc (P = .001), increased modified Rodnan skin score (P = .001), and shorter duration of Raynaud phenomenon (P = .002) in univariate analysis but not in multivariate analysis. Moreover, diffuse hyperpigmentation was associated with digital ulcers (P = .005), as confirmed by multivariate analysis (odds ratio, 2.96; 95% confidence interval, 1.28-6.89). LIMITATIONS: This was a single-center retrospective study of a cohort of patients with SSc. CONCLUSION: Screening for skin pigmentation disorders could be useful in the management of patients with SSc to identify those with a high risk of development of digital ulcers, which is a symptom of vascular involvement in SSc.


Assuntos
Dedos/patologia , Hiperpigmentação/epidemiologia , Escleroderma Sistêmico/epidemiologia , Úlcera Cutânea/epidemiologia , Adulto , Distribuição por Idade , Idoso , Estudos de Coortes , Comorbidade , Feminino , França , Humanos , Hiperpigmentação/diagnóstico , Hiperpigmentação/terapia , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prevalência , Doença de Raynaud/fisiopatologia , Estudos Retrospectivos , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/terapia , Índice de Gravidade de Doença , Distribuição por Sexo , Úlcera Cutânea/diagnóstico , Úlcera Cutânea/terapia , Estatísticas não Paramétricas
19.
J Affect Disord ; 243: 427-431, 2019 01 15.
Artigo em Inglês | MEDLINE | ID: mdl-30268959

RESUMO

BACKGROUND: Systemic sclerosis (SSc) can clinically present with psychological symptoms, including pain, depression, and distress about disfigurement, physical and social functioning. The existing small studies have reported a prevalence of depression ranging from 36% to 65% among SSc patients, likely reflecting the disease impact on the patient's self-image and function. AIM OF THE STUDY: To investigate the association between SSc and depression using big data analysis methods. METHODS: We designed a nation-wide epidemiological survey relying on a large database of 2500 SSc patients and explored the relationship between SSc and depression, but also the impact of depression on the survival of SSc patients. Chi-squared and t-tests were used for univariate analysis and a logistic regression model was used for multivariate analysis. RESULTS: The proportion rate of depression among SSc patients was significantly higher than controls (16.2% vs 10.9%), and this proportion was even higher in female SSc patients and of low socioeconomic status. At the multivariate logistic regression, SSc was found to be an independent risk factor for depression with an OR of 1.55 (95%CI 1.29-1.88, p < 0.0001). No significant association was found between SSc-specific autoantibodies (anti-centromere, anti-Scl-70, anti-RNA polymerase III and anti-RNP) status and the risk of depression. Depression was not found to have a significant impact on the survival of SSc patients with an HR of 1.06 (0.80-1.42). CONCLUSIONS: This study provides further support for the high prevalence of depression in SSc patients and therefore, SSc patients may benefit from a screening approach and a broad supportive care program.


Assuntos
Big Data , Depressão/epidemiologia , Escleroderma Sistêmico/epidemiologia , Idoso , Autoanticorpos/imunologia , Comorbidade , Feminino , Humanos , Israel/epidemiologia , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Prevalência , Fatores de Risco , Escleroderma Sistêmico/imunologia
20.
Clin Rev Allergy Immunol ; 56(3): 293-307, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28849549

RESUMO

Autoimmune diseases affect up to 10% of the world's population and, as a whole, they are far more common in females, although differences exist according to the single disease and also in different age groups. In childhood-onset autoimmune diseases, the sex bias is generally less evident than in adults, probably for the different hormonal milieau, being estrogens strongly implicated in the development of autoimmunity. Still, some rheumatic conditions, such as juvenile idiopathic arthritis (JIA), show a strong predilection for girls (F:M = 3-6.6:1), and differences may coexist between males and females regarding disease outcome. For example, chronic anterior uveitis associated with JIA affects more commonly girls but boys tend to have a more severe course. Systemic lupus erythematosus predominantly affects girls and women (F:M = 3-5:1 in children, F:M = 10-15:1 in adults). Behςet's disease has been reported to be more prevalent in adult males (F:M = 1:1-4); in children, there are no differences. The sex ratio is equal in children and adults for Henoch-Schönlein purpura (F:M = 1:1). A higher male-to-female ratio exists for Kawasaki disease (F:M = 1:1.1-1.6 in children, F:M = 1:1,5 in adults). Juvenile dermatomyositis (F:M = 2-5:1), systemic sclerosis (F:M = 4:1 in children, F:M = 6:1 in adults), and Takayasu arteritis (F:M = 2:1 in children, F:M = 7-9:1 in adults) are more common in girls and women then in boys and men. There is no gender bias for acute rheumatic fever in children, while in adults, the F:M ratio is 2:1. Given that estrogen levels are not different between genders during childhood, pediatric rheumatic diseases could represent good models to study other mechanisms related to the development of autoimmunity. Recently, the levels of miRNA expression, and their variation according to sex chromosomes, have been linked to the development of autoimmune diseases, with different impact among sexes. This review will focus not only on the sex bias reported in the more common rheumatic conditions of childhood, focusing on differences in incidence, but also on outcome and trying to depict the mechanisms underlying those differences.


Assuntos
Artrite Juvenil/epidemiologia , Dermatomiosite/epidemiologia , Lúpus Eritematoso Sistêmico/epidemiologia , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Púrpura de Schoenlein-Henoch/epidemiologia , Febre Reumática/epidemiologia , Escleroderma Sistêmico/epidemiologia , Arterite de Takayasu/epidemiologia , Adolescente , Adulto , Artrite Juvenil/fisiopatologia , Autoimunidade , Criança , Pré-Escolar , Dermatomiosite/fisiopatologia , Exposição Ambiental/efeitos adversos , Feminino , Predisposição Genética para Doença , Humanos , Incidência , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Síndrome de Linfonodos Mucocutâneos/fisiopatologia , Prevalência , Púrpura de Schoenlein-Henoch/fisiopatologia , Febre Reumática/fisiopatologia , Escleroderma Sistêmico/fisiopatologia , Fatores Sexuais , Arterite de Takayasu/fisiopatologia
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