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1.
Presse Med ; 48(10): e284-e291, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31653544

RESUMO

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


Assuntos
Esclerodermia Limitada , Escleroderma Sistêmico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Calcinose/epidemiologia , Centrômero/imunologia , Estudos Transversais , DNA Topoisomerases Tipo I/imunologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Retrospectivos , Esclerodermia Limitada/complicações , Esclerodermia Limitada/diagnóstico , Esclerodermia Limitada/imunologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/imunologia , Tunísia , Adulto Jovem
2.
BMC Neurol ; 19(1): 234, 2019 Oct 14.
Artigo em Inglês | MEDLINE | ID: mdl-31607267

RESUMO

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


Assuntos
Mielite Transversa/etiologia , Neuromielite Óptica/complicações , Escleroderma Sistêmico/complicações , Adulto , Autoanticorpos/sangue , Feminino , Humanos , Fatores Imunológicos/uso terapêutico , Imagem por Ressonância Magnética , Mielite Transversa/tratamento farmacológico , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/tratamento farmacológico , Rituximab/uso terapêutico , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/tratamento farmacológico , Síndrome
3.
Wiad Lek ; 72(9 cz 1): 1683-1686, 2019.
Artigo em Polonês | MEDLINE | ID: mdl-31586983

RESUMO

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


Assuntos
Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Idoso , Anticorpos Antinucleares/sangue , Humanos , Hipertensão Pulmonar/complicações , Pessoa de Meia-Idade
4.
Lakartidningen ; 1162019 Sep 26.
Artigo em Sueco | MEDLINE | ID: mdl-31573670

RESUMO

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


Assuntos
Escleroderma Sistêmico , Autoanticorpos/imunologia , Humanos , Atenção Primária à Saúde , Doenças Raras/complicações , Doenças Raras/diagnóstico , Doenças Raras/patologia , Doenças Raras/terapia , Doença de Raynaud/etiologia , Encaminhamento e Consulta , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/terapia
5.
Autoimmun Rev ; 18(11): 102394, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31520797

RESUMO

OBJECTIVES: This study was designed to propose a simple "Fast Track algorithm" for capillaroscopists of any level of experience to differentiate "scleroderma patterns" from "non-scleroderma patterns" on capillaroscopy and to assess its inter-rater reliability. METHODS: Based on existing definitions to categorise capillaroscopic images as "scleroderma patterns" and taking into account the real life variability of capillaroscopic images described standardly according to the European League Against Rheumatism (EULAR) Study Group on Microcirculation in Rheumatic Diseases, a fast track decision tree, the "Fast Track algorithm" was created by the principal expert (VS) to facilitate swift categorisation of an image as "non-scleroderma pattern (category 1)" or "scleroderma pattern (category 2)". Mean inter-rater reliability between all raters (experts/attendees) of the 8th EULAR course on capillaroscopy in Rheumatic Diseases (Genoa, 2018) and, as external validation, of the 8th European Scleroderma Trials and Research group (EUSTAR) course on systemic sclerosis (SSc) (Nijmegen, 2019) versus the principal expert, as well as reliability between the rater pairs themselves was assessed by mean Cohen's and Light's kappa coefficients. RESULTS: Mean Cohen's kappa was 1/0.96 (95% CI 0.95-0.98) for the 6 experts/135 attendees of the 8th EULAR capillaroscopy course and 1/0.94 (95% CI 0.92-0.96) for the 3 experts/85 attendees of the 8th EUSTAR SSc course. Light's kappa was 1/0.92 at the 8th EULAR capillaroscopy course, and 1/0.87 at the 8th EUSTAR SSc course. CONCLUSION: For the first time, a clinical expert based fast track decision algorithm has been developed to differentiate a "non-scleroderma" from a "scleroderma pattern" on capillaroscopic images, demonstrating excellent reliability when applied by capillaroscopists with varying levels of expertise versus the principal expert and corroborated with external validation.


Assuntos
Algoritmos , Esclerodermia Localizada/diagnóstico , Escleroderma Sistêmico/diagnóstico , Humanos , Angioscopia Microscópica/métodos , Reprodutibilidade dos Testes
9.
Hautarzt ; 70(9): 723-741, 2019 Sep.
Artigo em Alemão | MEDLINE | ID: mdl-31384958

RESUMO

Systemic sclerosis is a rare rheumatologic disease that is characterised by skin and organ fibrosis as well as vascular changes and the occurrence of specific autoantibodies. It has a high morbidity and mortality while its manifestations show significant heterogeneity in patients. Thus, diagnosis and follow-up of patients with systemic sclerosis has to be extensive, the more so because treatment must be adapted to organ manifestations. Although specific therapies for gastrointestinal, pulmonary or vascular complications exist, patients respond only partly to these and new therapeutic approaches are still needed.


Assuntos
Escleroderma Sistêmico , Autoanticorpos , Fibrose , Humanos , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/terapia , Pele
10.
Medicine (Baltimore) ; 98(29): e16423, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31335694

RESUMO

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


Assuntos
Doenças Pulmonares Intersticiais , Pulmão/diagnóstico por imagem , Escleroderma Sistêmico , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Algoritmos , Diagnóstico Diferencial , Diagnóstico Precoce , Feminino , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Masculino , Pessoa de Meia-Idade , Doses de Radiação , Intensificação de Imagem Radiográfica/métodos , Interpretação de Imagem Radiográfica Assistida por Computador/métodos , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico
12.
Iran J Kidney Dis ; 13(3): 207-210, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-31209194

RESUMO

Known as systemic sclerosis (SSc), this autoimmune rheumatic disease has vast pathogenesis on many organs, including kidneys. It can lead to the point where the patient's survival relies entirely on dialysis. This report has basically focused on scleroderma renal crisis (SRC), which is the most serious renal manifestation of SSc, characterized by renal failure and sudden onset of hypertension. A 44-year-old man was hospitalized with hypertension, headache, vertigo, nausea, rhinorrhea, reflux, dysphagia, dyspnea (Fc II), visual impairment, mechanical arthralgia, and edema (+3) accompanied by a rare skin lesion. Raynaud's phenomenon was also remarkable in fingers and toes. According to signs and symptoms, SSc diagnosed and the proper treatment was applied. It is of great importance that in the case of malignant hypertension in patients with scleroderma, renal crisis always be kept in mind.


Assuntos
Lesão Renal Aguda/etiologia , Hipertensão/etiologia , Doença de Raynaud/etiologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Lesão Renal Aguda/fisiopatologia , Adulto , Edema , Humanos , Hipertensão/fisiopatologia , Masculino , Imagem de Perfusão do Miocárdio , Doença de Raynaud/fisiopatologia
13.
Rev Port Cardiol ; 38(4): 299-303, 2019 04.
Artigo em Inglês, Português | MEDLINE | ID: mdl-31203919

RESUMO

INTRODUCTION: Systemic sclerosis (SSc) is a systemic autoimmune disease involving multiple organs. We present a rare case of SSc in which clinical manifestations of cardiac fibrosis occurred early in the disease course. CASE REPORT: We report the case of a 40-year-old Caucasian man, previously diagnosed with SSc, who presented with decompensated heart failure. Transthoracic echocardiography was remarkable for severe right ventricular systolic dysfunction, abnormal ventricular septal motion, severe functional tricuspid regurgitation and normal pulmonary artery systolic pressure. Left ventricular ejection fraction was 45%. Right heart catheterization revealed no signs of pulmonary hypertension. Cardiac magnetic resonance (CMR) showed diffuse myocardial infiltration, later confirmed as myocardial fibrosis by endomyocardial biopsy. CONCLUSIONS: Myocardial fibrosis is an important cause of early heart failure in SSc patients and is associated with poor prognosis. Echocardiography and CMR help establish the diagnosis and enable an appropriate therapeutic strategy to be developed in such cases.


Assuntos
Fibrose Endomiocárdica/etiologia , Ventrículos do Coração/diagnóstico por imagem , Miocárdio/patologia , Escleroderma Sistêmico/complicações , Adulto , Biópsia , Progressão da Doença , Ecocardiografia , Fibrose Endomiocárdica/diagnóstico , Fibrose Endomiocárdica/fisiopatologia , Ventrículos do Coração/fisiopatologia , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Escleroderma Sistêmico/diagnóstico , Fatores de Tempo , Função Ventricular Esquerda/fisiologia
14.
Z Rheumatol ; 78(5): 439-457, 2019 Jun.
Artigo em Alemão | MEDLINE | ID: mdl-31089800

RESUMO

Systemic sclerosis is a rare rheumatologic disease that is characterised by skin and organ fibrosis as well as vascular changes and the occurrence of specific autoantibodies. It has a high morbidity and mortality while its manifestations show significant heterogeneity in patients. Thus, diagnosis and follow-up of patients with systemic sclerosis has to be extensive, the more so because treatment must adapted to organ manifestations. Although specific therapies for gastrointestinal, pulmonary or vascular complications exist, patients respond only partly to these and new therapeutic approaches are still needed.


Assuntos
Escleroderma Sistêmico , Autoanticorpos , Fibrose , Humanos , Imunossupressores/uso terapêutico , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/tratamento farmacológico , Escleroderma Sistêmico/imunologia , Pele
15.
Int J Rheum Dis ; 22(6): 1029-1035, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30989785

RESUMO

AIM: Autonomic dysfunction (AD) is an early feature of systemic sclerosis (SSc). A regular endothelial function is a prerequisite for normal response of the myocardial blood flow (MBF) to cold pressure test (CPT). The aim of the study was to evaluate the relation between MBF and AD at rest and after CPT in asymptomatic SSc patients. METHODS: Twenty SSc patients and 10 age-, sex- and body mass index-matched healthy controls underwent cardiac magnetic resonance at rest and after CPT. All subjects underwent 24 hours ambulatory 3-channel electrocardiogram Holter to evaluate AD by heart rate variability. RESULTS: We did not observe any significant difference in MBF (mL/g/min) at rest and after CPT between SSc patients and healthy controls. Delta of MBF (difference between MBF after CPT and rest MBF) was lower (P = 0.039) in SSc patients than healthy controls (0.28 [0.04-0.40] vs 0.33 [0.24-0.54]). The low frequency/high frequency (LF/HF) was higher (P = 0.002) in SSc patients than healthy controls (3 [1.7-6] vs 1.8 [1.1-2.8]). The high frequencies (HF), modulated mainly by paraympathetic system, was lower (P = 0.003) in SSc patients than healthy controls (30 [16-42] vs 36.5 [24-44]). Sympathetic hyperactivity, due to reduction of parasympathetic activity (HF), is present in SSc patients. A negative correlation was observed between Delta of MBF and LF/HF (r = -0.572, P = 0.0031). CONCLUSION: AD, characterized by sympathovagal imbalance due to a reduced parasympathetic tone with high LF/HF ratio, could be responsible for the reduced myocardial vasodilatory response after CPT.


Assuntos
Doenças do Sistema Nervoso Autônomo/etiologia , Sistema Nervoso Autônomo/fisiopatologia , Circulação Coronária , Cardiopatias/diagnóstico por imagem , Coração/diagnóstico por imagem , Coração/inervação , Imagem Cinética por Ressonância Magnética , Imagem de Perfusão do Miocárdio/métodos , Escleroderma Sistêmico/complicações , Adulto , Doenças do Sistema Nervoso Autônomo/diagnóstico , Doenças do Sistema Nervoso Autônomo/fisiopatologia , Estudos de Casos e Controles , Feminino , Cardiopatias/etiologia , Cardiopatias/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Fatores de Risco , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/fisiopatologia , Vasodilatação , Adulto Jovem
16.
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
17.
Ann Rheum Dis ; 78(6): 807-816, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30928903

RESUMO

OBJECTIVE: We sought to develop the first Damage Index (DI) in systemic sclerosis (SSc). METHODS: The conceptual definition of 'damage' in SSc was determined through consensus by a working group of the Scleroderma Clinical Trials Consortium (SCTC). Systematic literature review and consultation with patient partners and non-rheumatologist experts produced a list of potential items for inclusion in the DI. These steps were used to reduce the items: (1) Expert members of the SCTC (n=331) were invited to rate the appropriateness of each item for inclusion, using a web-based survey. Items with >60% consensus were retained; (2) Using a prospectively acquired Australian cohort data set of 1568 patients, the univariable relationships between the remaining items and the endpoints of mortality and morbidity (Physical Component Summary score of the Short Form 36) were analysed, and items with p<0.10 were retained; (3) using multivariable regression analysis, coefficients were used to determine a weighted score for each item. The DI was externally validated in a Canadian cohort. RESULTS: Ninety-three (28.1%) complete survey responses were analysed; 58 of 83 items were retained. The univariable relationships with death and/or morbidity endpoints were statistically significant for 22 items, with one additional item forced into the multivariable model by experts due to clinical importance, to create a 23-item weighted SCTC DI (SCTC-DI). The SCTC-DI was predictive of morbidity and mortality in the external cohort. CONCLUSIONS: Through the combined use of consensus and data-driven methods, a 23-item SCTC-DI was developed and retrospectively validated.


Assuntos
Escleroderma Sistêmico/diagnóstico , Índice de Gravidade de Doença , Austrália/epidemiologia , Estudos de Coortes , Interpretação Estatística de Dados , Humanos , Morbidade , Curva ROC , Estudos Retrospectivos , Escleroderma Sistêmico/mortalidade
18.
PLoS One ; 14(3): e0214202, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30913258

RESUMO

Systemic sclerosis (SSc) is a heterogeneous autoimmune disorder associated with vascular dysfunction and fibrotic changes in the skin, vasculature and internal organs. Although serologic abnormalities are an important diagnostic tool for SSc, little is known about whether autoantibodies precede clinical diagnosis. Here we investigated the presence of autoantibodies before SSc diagnosis and assessed whether certain autoantibodies might associate with the future onset of scleroderma renal crisis (SRC), a potentially fatal complication of the disease. Using the Department of Defense Serum Repository, autoantibodies were analyzed from archived, prospectively collected, longitudinal serum samples from sixteen individuals with SRC (SSc/SRC) and thirty cases of SSc without SRC (SSc/no SRC), matched for age, sex, and race. Seventy five percent (12/16) of the SSc/SRC and 40% (12/30) of the SSc/no SRC were seropositive for at least one autoantibody prior to clinical diagnosis (up to 27.1 years earlier, mean = -7.4 years). Although both disease groups demonstrated a heterogeneous immunoreactivity profile against the autoantigen panel, the SSc/SRC subjects showed two enriched clusters with one featuring elevated levels of autoantibodies against Ro52 and/or Ro60 and another with high levels of immunoreactivity against the RNA polymerase complex. Consistent with larger spectrum of immunoreactivity and the elevated levels of autoantibodies in SSc/SRC, the total response against the autoantigen panel from the last time point of the seropositive subjects revealed that the SSc/SRC cohort harbored higher antibody levels (p = 0.02) compared to SSc/no SRC. Overall, our findings demonstrate that relevant seropositive autoantibodies often precede the clinical diagnosis of SSc/no SRC and SSc/SRC.


Assuntos
Autoanticorpos , Ribonucleoproteínas/imunologia , Escleroderma Sistêmico , Adulto , Autoanticorpos/sangue , Autoanticorpos/imunologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/imunologia
19.
Clin Rheumatol ; 38(7): 1917-1923, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30840212

RESUMO

OBJECTIVES: The Scleroderma Health Assessment Questionnaire (SHAQ) is a functional scale which consists of five scleroderma-specific items (overall disease severity, Raynaud's phenomenon, digital ulcers, respiratory and intestinal involvement) in addition to Health Assessment Questionnaire Disability Index (HAQ-DI). The objective of this study was to perform an adaptation and validation of a Turkish version of the SHAQ. METHOD: We validated psychometric properties of the scale with 70 consecutive systemic sclerosis (SSc) patients, who fulfilled the 2013 ACR/EULAR classification criteria for SSc. We evaluated test-retest reliability with the intraclass correlation coefficient (ICC), discriminant validity by stratifying patients according to organ involvements and disease subtypes, and convergent validity by testing the correlation between SHAQ and related components of Short Form 36 version 2 (SF-36v2). Internal consistency of the questionnaire was evaluated by Cronbach's alpha coefficient. RESULTS: The SHAQ-global, visual analogue scales (VAS) of pulmonary, digital ulcer, and Raynaud's phenomenon were significantly correlated with the physical component score of the SF-36v2 (r = - 0.274, r = - 0.295, r = - 0.326, r = - 0.308, p < 0.05, respectively) for the convergent validity. The instruments could not discriminate between disease subtypes, except the digital ulcer VAS which was significantly higher in patients with dcSSc (1.00 ± 0.93 vs 0.55 ± 0.88, p = 0.026) for the discriminant validity. The HAQ-DI, SHAQ-global, digital ulcer VAS, and pulmonary VAS showed moderate correlation with an increase in the number of the organs involved (r = 0.319, r = 0.329, r = 0.341, r = 0.278, p < 0.05, respectively). We demonstrated high reproducibility for HAQ-DI (ICC = 0.962, 95% confidence interval = 0.934-0.978) and the other items of SHAQ. The overall internal consistency of the SHAQ was satisfactory (Cronbach's alpha = 0.953). CONCLUSIONS: The Turkish version of the SHAQ met the requirements of validity and reproducibility.


Assuntos
Escleroderma Sistêmico/diagnóstico , Inquéritos e Questionários , Adulto , Idoso , Características Culturais , Avaliação da Deficiência , Feminino , Humanos , Linguagem , Masculino , Pessoa de Meia-Idade , Medição da Dor , Psicometria , Qualidade de Vida , Reprodutibilidade dos Testes , Escleroderma Sistêmico/psicologia , Índice de Gravidade de Doença , Turquia , Escala Visual Analógica
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