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1.
Osteoporos Int ; 30(8): 1679-1691, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31030240

RESUMO

Radius and tibia bone microarchitecture, analyzed through a high-resolution peripheral quantitative computed tomography, were significantly impaired in female patients with diffuse systemic sclerosis compared with healthy controls. Acroosteolysis, quality of life-grip strength, hand disability, and disease duration were significantly associated with this bone deterioration. INTRODUCTION: The effect of diffuse systemic sclerosis (dSSc) on the bone is not completely understood. The objective of this study was to analyze the volumetric bone mineral density (vBMD), microarchitecture, and biomechanical parameters at the distal radius and tibia using high-resolution peripheral quantitative computed tomography (HR-pQCT, XtremeCT) in female patients with dSSc and identify clinical and laboratory variables associated with these parameters. METHODS: Thirty-eight women with dSSc and 76 healthy controls were submitted to HR-pQCT at the distal radius and tibia. Clinical and laboratory findings, bone mineral density(BMD), nailfold capillaroscopy (NC), total passive range of motion(ROM), and quality of life (health assessment questionnaire-HAQ) were associated with HR-pQCT (Scanco Medical AG, Brüttisellen, Switzerland) parameters. Multiple linear regression models adjusted for clinical and laboratory variables, ROM and HAQ, were performed. RESULTS: Density, microarchitecture, and biomechanical parameters at the distal radius and tibia were significantly impaired in dSSc patients compared with healthy controls (p < 0.001). Multiple linear regression models showed that lower trabecular density (Tb.vBMD) (radius R2 = 0.561, p = 0.002; and tibia R2 = 0.533, p = 0.005), and lower trabecular number (Tb.N) (tibia R2 = 0.533, p = 0.005) were significantly associated with acroosteolysis. Higher trabecular separation (Tb.Sp) was associated with disease duration and higher HAQ-grip strength (radius R2 = 0.489, p = 0.013), while cortical density (Ct.vBMD) was associated with ROM (radius R2 = 0.294, p = 0.002). CONCLUSION: Bone microarchitecture in patients with dSSc, analyzed through HR-pQCT, showed impairment of trabecular and cortical bone at distal radius and tibia. Variables associated with hand involvement (acroosteolysis, quality of life-grip strength, and ROM) and disease duration may be considered prognostic factors of this bone impairment.


Assuntos
Densidade Óssea/fisiologia , Rádio (Anatomia)/fisiopatologia , Esclerodermia Difusa/fisiopatologia , Tíbia/fisiopatologia , Acro-Osteólise/etiologia , Acro-Osteólise/fisiopatologia , Adolescente , Adulto , Antropometria/métodos , Fenômenos Biomecânicos/fisiologia , Estudos de Casos e Controles , Feminino , Articulações dos Dedos/fisiopatologia , Força da Mão/fisiologia , Humanos , Angioscopia Microscópica , Pessoa de Meia-Idade , Qualidade de Vida , Rádio (Anatomia)/diagnóstico por imagem , Amplitude de Movimento Articular/fisiologia , Esclerodermia Difusa/complicações , Esclerodermia Difusa/diagnóstico por imagem , Tíbia/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto Jovem
2.
Ann Rheum Dis ; 78(5): 648-656, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30852552

RESUMO

OBJECTIVES: To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). METHODS: We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, valid mRSS at 12±3 months after baseline and ≥1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ≥25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. RESULTS: Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ≥10% (53.6% vs 34.4%; p<0.001) and all-cause death (15.4% vs 7.3%; p=0.003) than non-progressors. These significant associations were also found in subgroup analyses of patients with either low baseline mRSS (≤22/51) or short disease duration (≤15 months). In multivariable analyses, skin progression within 1 year was independently associated with FVC decline ≥10% (HR 1.79, 95% CI 1.20 to 2.65) and all-cause death (HR 2.58, 95% CI 1.31 to 5.09). CONCLUSIONS: Progressive skin fibrosis within 1 year is associated with decline in lung function and worse survival in dcSSc during follow-up. These results confirm mRSS as a surrogate marker in dcSSc, which will be helpful for cohort enrichment in future trials and risk stratification in clinical practice.


Assuntos
Esclerodermia Difusa/mortalidade , Esclerodermia Difusa/patologia , Dermatopatias/mortalidade , Dermatopatias/fisiopatologia , Pele/patologia , Adulto , Estudos de Coortes , Bases de Dados Factuais , Progressão da Doença , Europa (Continente) , Feminino , Fibrose , Humanos , Estimativa de Kaplan-Meier , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Esclerodermia Difusa/complicações , Índice de Gravidade de Doença , Dermatopatias/etiologia , Análise de Sobrevida , Fatores de Tempo
4.
Am J Dermatopathol ; 41(2): 135-136, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30235169

RESUMO

We report a case of a 65-year-old man who developed an asymptomatic bluish spot that affected the flank and left lumbar region with the onset 10 years prior. He had a history of diffuse systemic sclerosis with anti-Scl-70-positive antibodies. The appearance of the skin lesion coincided with the onset of his disease. The skin biopsy was consistent with the diagnosis of acquired dermal melanocytosis. The relationship between the appearance of acquired pigmented macules and spots and systemic sclerosis has been known for years, although it is an infrequent finding.


Assuntos
Melanose/complicações , Esclerodermia Difusa/complicações , Idoso , Grupo com Ancestrais do Continente Europeu , Humanos , Masculino
5.
Arq Gastroenterol ; 55Suppl 1(Suppl 1): 47-51, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30304292

RESUMO

BACKGROUND: Scleroderma or progressive systemic sclerosis is characterized by a chronic inflammatory process with proliferation of fibrous connective tissue and excessive deposition of collagen and extracellular matrix in the skin, smooth muscle, and viscera. The smooth muscle most involved in scleroderma is that of the esophagus, and dysphagia is the most commonly reported symptom. However, the internal anal sphincter may also be impaired by degeneration and fibrosis, leading to concomitant anal incontinence in scleroderma patients. These patients may neglect to complain about it, except when actively questioned. OBJECTIVE: To assess anorectal function and anatomy of female scleroderma patients with symptoms of anal incontinence through Cleveland Clinic Florida Fecal Incontinence Score (CCFIS), anorectal manometry and endoanal ultrasound at the outpatient clinic of colorectal and anal physiology, Clinics Hospital, University of São Paulo Medical School (HC-FMUSP). METHODS: Female scleroderma patients were prospectively assessed and questioned as to symptoms of anal incontinence. The anorectal manometry and endoanal ultrasound results were correlated with clinical data and symptoms. RESULTS: In total, 13 women were evaluated. Their mean age was 55.77 years (±16.14; 27-72 years) and their mean disease duration was 10.23 years (±6.23; 2-23 years). All had symptoms of fecal incontinence ranging from 1 to 15. Seven (53.8%) patients had fecal incontinence score no higher than 7; three (23.1%) between 8 and 13; and three (23.1%) 14 or higher, corresponding to mild, moderate, and severe incontinence, respectively. Ten (76.92%) patients had hypotonia of the internal anal sphincter. Three-dimensional endoanal ultrasound showed tapering associated with muscle atrophy of the internal sphincter in six cases and previous muscle defects in three cases. CONCLUSION: A functional and anatomical impairment of the sphincter is an important factor to assess in patients with progressive systemic sclerosis and it should not be underestimated.


Assuntos
Endossonografia/métodos , Incontinência Fecal/diagnóstico por imagem , Distúrbios do Assoalho Pélvico/diagnóstico por imagem , Esclerodermia Difusa/complicações , Adulto , Idoso , Incontinência Fecal/etiologia , Incontinência Fecal/fisiopatologia , Feminino , Humanos , Imagem Tridimensional , Manometria , Pessoa de Meia-Idade , Distúrbios do Assoalho Pélvico/etiologia , Distúrbios do Assoalho Pélvico/fisiopatologia , Estudos Prospectivos , Esclerodermia Difusa/fisiopatologia , Índice de Gravidade de Doença
6.
Clin Exp Rheumatol ; 36 Suppl 113(4): 68-75, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30277860

RESUMO

OBJECTIVES: To describe differences in clinical presentation between men and women in a large group of patients with early (<3 years' duration) systemic sclerosis (SSc) according to disease subsets. METHODS: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research database (EUSTAR) was performed. Patients fulfilling preliminary ACR 1980 classification criteria for SSc, with less than 3 years from the first non-Raynaud's symptom at first entry, were selected. A group of patients with less than 3 years from the first SSc symptom, including Raynaud's phenomenon, was also analysed. SSc related variables, including antibodies, SSc subsets, disease activity and organ involvement were included. Descriptive and bivariate analyses were performed. RESULTS: A total of 1,027 patients were included, 90% Caucasian, 80% women, and 40% with diffuse cutaneous disease. In early stages of SSc, men showed more frequently than women active disease, diffuse cutaneous subset, anti-Scl-70 antibodies, elevated acute phase reactants, muscular and pulmonary involvement. Differences between men and women were confirmed in the limited, but not in the diffuse SSc subset. The results were similar when 650 patients with less than three years from the first SSc symptom, including Raynaud's phenomenon, were analysed. CONCLUSIONS: In early stages of SSc, men present signs and symptoms of more severe disease. In the limited disease subset, men might appear with clinical features and organ involvement similar to those of the diffuse subgroup. In clinical practice, the identification of such differences might help to select the appropriate management for each particular patient.


Assuntos
Disparidades nos Níveis de Saúde , Esclerodermia Difusa/diagnóstico , Esclerodermia Limitada/diagnóstico , Proteínas da Fase Aguda/análise , Autoanticorpos/sangue , Biomarcadores/sangue , Estudos Transversais , Bases de Dados Factuais , Progressão da Doença , Feminino , Humanos , Pneumopatias/diagnóstico , Pneumopatias/etiologia , Masculino , Proteínas Nucleares/imunologia , Prognóstico , Doença de Raynaud/diagnóstico , Doença de Raynaud/etiologia , Fatores de Risco , Esclerodermia Difusa/sangue , Esclerodermia Difusa/complicações , Esclerodermia Difusa/imunologia , Esclerodermia Limitada/sangue , Esclerodermia Limitada/complicações , Esclerodermia Limitada/imunologia , Índice de Gravidade de Doença , Fatores Sexuais
7.
Rheumatol Int ; 38(12): 2279-2288, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30206672

RESUMO

The objective of this study is to investigate the impact of skin sclerosis burden on an internal organ involvement over a 1-year period, as measured by time-adjusted accrual-modified Rodnan skin score (TA-mRSS), and to evaluate association between TA-mRSS patterns and laboratory tests in patients with systemic sclerosis (SSc). This prospective study was conducted at Siriraj Hospital (Bangkok, Thailand) during the November 2013-November 2016. SSc patients by ACR/EULAR 2013 or ACR 1980 criteria were eligible. TA-mRSS was classified as low, intermediate, or high, and then compared between groups. Correlation between the arithmetic mean of laboratory tests and TA-mRSS was assessed by multiple linear regression analysis. A total of 118 patients, with 81.4% women, median (IQR) age 49.8 (43.8, 55.1) years, disease duration from onset of non-Raynaud symptoms to first visit of 3.3 (1, 6.8) years, 78% dcSSc, and 75.3% anti-Scl-70 positivity, were analyzed. TA-mRSS over 1 year ranged from 0 to 37.44. The high skin sclerosis burden group had a median TA-mRSS > 7.26 (> 67th percentile). Patients with high TA-mRSS were dcSSc, high initial and average mRSS, and had tendon friction rub, digital ischemic complications, usual interstitial pneumonia, diastolic dysfunction, gastrointestinal dysmotility, and low serum albumin. In multiple linear regression analysis, the arithmetic mean of hemoglobin (B = - 1.007, 95% CI - 1.779 to - 0.236), erythrocyte sedimentation rate (B = - 0.078, 95% CI - 0.126 to - 0.029), serum glutamic oxaloacetic transaminase (B = 0.073, 95% CI 0.026-0.12), creatine phosphokinase (B = 0.012, 95% CI 0.003-0.021), and albumin (B = - 4.117, 95% CI - 6.958 to - 1.276) were associated with TA-mRSS. This study found a higher cumulative course of mRSS over a 1-year period to be significantly associated with severe internal organ involvement.


Assuntos
Esclerodermia Difusa/complicações , Esclerodermia Limitada/complicações , Pele/patologia , Adulto , Biomarcadores/sangue , Progressão da Doença , Feminino , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Indução de Remissão , Esclerodermia Difusa/sangue , Esclerodermia Difusa/tratamento farmacológico , Esclerodermia Difusa/patologia , Esclerodermia Limitada/sangue , Esclerodermia Limitada/tratamento farmacológico , Esclerodermia Limitada/patologia , Índice de Gravidade de Doença , Síndrome , Tailândia , Fatores de Tempo , Resultado do Tratamento
8.
Clin Nephrol ; 90(6): 413-418, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30106367

RESUMO

AIMS: Overlap syndrome of ANCA-associated vasculitis (AAV) and scleroderma (SSc) is rare with conflicting data on renal outcomes. We describe the clinical characteristics and treatment outcome of ANCA-associated glomerulonephritis (AAG) in SSc patients followed at a single center. MATERIALS AND METHODS: We conducted a retrospective study of 3,570 patients in our SSc database to identify SSc patients who subsequently developed AAV with renal involvement. Patient demographics, serology, renal function, renal histology, and treatment outcomes were assessed. RESULTS: Of the 3,570 patients, we identified 7 patients who developed acute glomerulonephritis, and all were ANCA positive. The mean age at SSc diagnosis was 47 years, 4 patients were female, and 6 had diffuse SSc. Anti-nuclear antibody (ANA) was positive in all. Mean time of onset of AAV from time of diagnosis of SSc was 6 years, and all were myeloperoxidase (MPO) positive. Patients presented with hematuria, proteinuria, with or without rise in serum creatinine, and all patients had biopsy-proven crescentic glomerulonephritis. One patient required dialysis at presentation. Five patients were treated with cyclophosphamide and steroids, and 2 were treated with rituximab and steroids. Of the 7 patients, 4 did not receive maintenance immunosuppression. Three patients died, and 1 of them experienced relapse with fulminant alveolar hemorrhage. CONCLUSION: AAG in SSc is rare, with disease manifestation and course similar to that of AAV. This case series demonstrates that disease remission can be achieved with standard induction therapy. Vasculitis relapse can occur, and similar to idiopathic AAV, maintenance immunosuppression should be initiated to maintain remission.
.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Glomerulonefrite/etiologia , Glomerulonefrite/terapia , Esclerodermia Difusa/complicações , Adulto , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/sangue , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Anticorpos Anticitoplasma de Neutrófilos/sangue , Creatinina/sangue , Ciclofosfamida/uso terapêutico , Feminino , Glomerulonefrite/sangue , Glomerulonefrite/patologia , Hematúria/etiologia , Humanos , Fatores Imunológicos/uso terapêutico , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Peroxidase/sangue , Proteinúria/etiologia , Recidiva , Diálise Renal , Estudos Retrospectivos , Rituximab/uso terapêutico , Esclerodermia Difusa/sangue , Esteroides/uso terapêutico , Resultado do Tratamento , Adulto Jovem
9.
Rheumatology (Oxford) ; 57(12): 2106-2113, 2018 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-30053212

RESUMO

Objectives: SSc is characterized by fibrotic changes in the skin and lung, and the mainstay of treatment has been CYC. B cell involvement suggests that rituximab (RTX) may also be of therapeutic benefit. The aim of the study was to compare the efficacy and safety of RTX compared with CYC in retarding the progression of interstitial lung disease and skin manifestations of primary SSc. Methods: We randomly assigned 60 patients of dcSSc, age 18-60 years with skin and lung involvement, to monthly pulses of CYC 500 mg/m2 or RTX 1000 mg × 2 doses at 0, 15 days. Primary outcomes were forced vital capacity (FVC) percent predicted at 6 months. Secondary outcomes were: absolute change in litres (FVC-l) at 6 months; modified Rodnan skin scores at 6 months, 6-min walk test, Medsgers score and new onset or worsening of existing pulmonary hypertension by echocardiographic criteria. Results: The FVC [%mean (s.d.)] in the RTX group improved from 61.30 (11.28) to 67.52 (13.59), while in the CYC group it declined from 59.25 (12.96) to 58.06 (11.23) at 6 months (P = 0.003). The change of FVC was 1.51 (0.45) l to 1.65 (0.47) l in the RTX group, compared with 1.42 (0.49) to 1.42 (0.46) l in the CYC group. The mRSS changed from 21.77 (9.86) to 12.10 (10.14) in the RTX group and 23.83 (9.28) to 18.33 (7.69) in the CYC group after 6 months. Serious adverse events were more common in the CYC group. Conclusion: RTX is a safe and effective alternative to CYC in the primary therapy of skin and lung manifestations of scleroderma. Trial registration: Clinical Trials Registry - India, www.ctri.nic.in, CTRI/2017/07/009152.


Assuntos
Antirreumáticos/uso terapêutico , Ciclofosfamida/uso terapêutico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Rituximab/uso terapêutico , Esclerodermia Difusa/tratamento farmacológico , Adulto , Progressão da Doença , Feminino , Humanos , Pulmão/patologia , Doenças Pulmonares Intersticiais/etiologia , Masculino , Esclerodermia Difusa/complicações , Esclerodermia Difusa/patologia , Índice de Gravidade de Doença , Pele/patologia , Resultado do Tratamento , Capacidade Vital/efeitos dos fármacos , Teste de Caminhada
11.
Ann Rheum Dis ; 77(8): 1179-1186, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29678941

RESUMO

OBJECTIVES: Recent studies demonstrate autoantibodies are powerful tools to interrogate molecular events linking cancer and the development of autoimmunity in scleroderma. Investigating cancer risk in these biologically relevant subsets may provide an opportunity to develop personalised cancer screening guidelines. In this study, we examined cancer risk in distinct serologic and phenotypic scleroderma subsets and compared estimates with the general population. METHODS: Patients in the Johns Hopkins Scleroderma Center observational cohort were studied. Overall and site-specific cancer incidence was calculated in distinct autoantibody and scleroderma phenotypic subsets, and compared with the Surveillance, Epidemiology and End Results registry, a representative sample of the US population. RESULTS: 2383 patients with scleroderma contributing 37 686 person-years were studied. 205 patients (8.6%) had a diagnosis of cancer. Within 3 years of scleroderma onset, cancer risk was increased in patients with RNA polymerase III autoantibodies (antipol; standardised incidence ratio (SIR) 2.84, 95% CI 1.89 to 4.10) and those lacking centromere, topoisomerase-1 and pol antibodies (SIR 1.83, 95% CI 1.10 to 2.86). Among antipol-positive patients, cancer-specific risk may vary by scleroderma subtype; those with diffuse scleroderma had an increased breast cancer risk, whereas those with limited scleroderma had high lung cancer risk. In contrast, patients with anticentromere antibodies had a lower risk of cancer during follow-up (SIR 0.59, 95% CI 0.44 to 0.76). CONCLUSIONS: Autoantibody specificity and disease subtype are biologically meaningful filters that may inform cancer risk stratification in patients with scleroderma. Future research testing the value of targeted cancer screening strategies in patients with scleroderma is needed.


Assuntos
Autoanticorpos/sangue , Neoplasias/etiologia , Esclerodermia Difusa/complicações , Esclerodermia Localizada/complicações , Adulto , Anticorpos Antinucleares/sangue , Neoplasias da Mama/epidemiologia , Neoplasias da Mama/etiologia , Neoplasias da Mama/imunologia , Feminino , Humanos , Incidência , Neoplasias Pulmonares/epidemiologia , Neoplasias Pulmonares/etiologia , Neoplasias Pulmonares/imunologia , Masculino , Pessoa de Meia-Idade , Neoplasias/epidemiologia , Neoplasias/imunologia , Fenótipo , Sistema de Registros , Medição de Risco/métodos , Esclerodermia Difusa/epidemiologia , Esclerodermia Difusa/imunologia , Esclerodermia Localizada/epidemiologia , Esclerodermia Localizada/imunologia , Estados Unidos/epidemiologia
12.
Clin Exp Rheumatol ; 36 Suppl 113(4): 102-108, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29652651

RESUMO

OBJECTIVES: To identify prognostic factors among serum biomarkers and endothelial vasodilator function findings in patients with systemic sclerosis (SSc). METHODS: This is a clinical observational study. We assessed 60 consecutive SSc patients (44 limited cutaneous-type, 16 diffuse cutaneous-type). Circulating growth differentiation factor-15 (GDF-15), placenta growth factor (PlGF), endostatin, vascular endothelial growth factor (VEGF), and pentraxin 3 (PTX3) were measured by ELISA. Peripheral endothelial function was measured by forearm blood dilatation response to brachial artery occlusion using noninvasive plethysmography (EndoPAT2000), which is associated with nitric-oxide-dependent vasodilatation and yields a reactive hyperemia index (RHI). We evaluated whether abnormalities in these values were associated with type of SSc - namely, diffuse cutaneous SSc (dcSSc) or limited cutaneous SSc (lcSSc) - or organ involvement including interstitial lung disease (ILD), digital ulcer (DU) and estimated right ventricular systolic pressure (RVSP) by echocardiography >30 mmHg. RESULTS: SSc patients showed significantly elevated serum GDF-15, PlGF, endostatin and VEGF but not PTX3 compared with controls. GDF-15 and PlGF were high in dcSSc patients. EndoPAT-RHI was low, and incidence of RVSP >30 mmHg was high in dcSSc. Multivariate analysis revealed that elevated GDF-15 was highly predictive of dcSSc, ILD or RVSP >30 mmHg. PlGF for DU was also found. Conversely, a low EndoPAT-RHI value was predictive of the presence of dcSSc, ILD or DU. CONCLUSIONS: This is the first study to inclusively investigate the relationships among biomarkers, EndoPAT-RHI and organ involvement in patients with SSc. Our data suggest a complex pathological progression of SSc through fibrotic impairment and microvascular damage.


Assuntos
Artéria Braquial/fisiopatologia , Endostatinas/sangue , Fator 15 de Diferenciação de Crescimento/sangue , Fator de Crescimento Placentário/sangue , Esclerodermia Difusa/diagnóstico , Esclerodermia Limitada/diagnóstico , Fator A de Crescimento do Endotélio Vascular/sangue , Vasodilatação , Idoso , Biomarcadores/sangue , Progressão da Doença , Feminino , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Fatores de Risco , Esclerodermia Difusa/sangue , Esclerodermia Difusa/complicações , Esclerodermia Difusa/fisiopatologia , Esclerodermia Limitada/sangue , Esclerodermia Limitada/complicações , Esclerodermia Limitada/fisiopatologia , Úlcera Cutânea/diagnóstico , Úlcera Cutânea/etiologia , Disfunção Ventricular Direita/diagnóstico , Disfunção Ventricular Direita/etiologia
16.
Rheumatology (Oxford) ; 57(2): 370-381, 2018 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-29207002

RESUMO

Objectives: Our aim was to describe the burden of early dcSSc in terms of disability, fatigue and pain in the European Scleroderma Observational Study cohort, and to explore associated clinical features. Methods: Patients completed questionnaires at study entry, 12 and 24 months, including the HAQ disability index (HAQ-DI), the Cochin Hand Function Scale (CHFS), the Functional Assessment of Chronic Illness Therapy-fatigue and the Short Form 36 (SF36). Associates examined included the modified Rodnan skin score (mRSS), current digital ulcers and internal organ involvement. Correlations between 12-month changes were also examined. Results: The 326 patients recruited (median disease duration 11.9 months) displayed high levels of disability [mean (s.d.) HAQ-DI 1.1 (0.83)], with 'grip' and 'activity' being most affected. Of the 18 activities assessed in the CHFS, those involving fine finger movements were most affected. High HAQ-DI and CHFS scores were both associated with high mRSS (ρ = 0.34, P < 0.0001 and ρ = 0.35, P < 0.0001, respectively). HAQ-DI was higher in patients with digital ulcers (P = 0.004), pulmonary fibrosis (P = 0.005), cardiac (P = 0.005) and muscle involvement (P = 0.002). As anticipated, HAQ-DI, CHFS, the Functional Assessment of Chronic Illness Therapy and SF36 scores were all highly correlated, in particular the HAQ-DI with the CHFS (ρ = 0.84, P < 0.0001). Worsening HAQ-DI over 12 months was strongly associated with increasing mRSS (ρ = 0.40, P < 0.0001), decreasing hand function (ρ = 0.57, P < 0.0001) and increasing fatigue (ρ = -0.53, P < 0.0001). Conclusion: The European Scleroderma Observational Study highlights the burden of disability in early dcSSc, with high levels of disability and fatigue, associating with the degree of skin thickening (mRSS). Impaired hand function is a major contributor to overall disability.


Assuntos
Avaliação da Deficiência , Fadiga/fisiopatologia , Dor/fisiopatologia , Esclerodermia Difusa/fisiopatologia , Índice de Gravidade de Doença , Adulto , Efeitos Psicossociais da Doença , Europa (Continente) , Fadiga/etiologia , Feminino , Dedos , Força da Mão , Inquéritos Epidemiológicos , Humanos , Masculino , Dor/etiologia , Estudos Prospectivos , Esclerodermia Difusa/complicações , Úlcera Cutânea/etiologia , Úlcera Cutânea/fisiopatologia
18.
J Rheumatol ; 44(10): 1453-1457, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28668810

RESUMO

OBJECTIVE: Systemic sclerosis (SSc) is a fibrotic immune-mediated disease of unknown etiology. Among its clinical manifestations, pulmonary involvement is the leading cause of mortality in patients with SSc. However, the genetic factors involved in lung complication are not well defined. We aimed to review the association of the MIF gene, which encodes a cytokine implicated in idiopathic pulmonary hypertension among other diseases, with the susceptibility and clinical expression of SSc, in addition to testing the association of this polymorphism with SSc-related pulmonary involvement. METHODS: A total of 4392 patients with SSc and 16,591 unaffected controls from 6 cohorts of European origin were genotyped for the MIF promoter variant rs755622. An inverse variance method was used to metaanalyze the data. RESULTS: A statistically significant increase of the MIF rs755622*C allele frequency compared with controls was observed in the subgroups of patients with diffuse cutaneous SSc (dcSSc) and with pulmonary arterial hypertension (PAH) independently (dcSSc: p = 3.20E-2, OR 1.13; PAH: p = 2.19E-02, OR 1.32). However, our data revealed a stronger effect size with the subset of patients with SSc showing both clinical manifestations (dcSSc with PAH: p = 6.91E-3, OR 2.05). CONCLUSION: We reviewed the association of the MIF rs755622*C allele with SSc and described a phenotype-specific association of this variant with the susceptibility to develop PAH in patients with dcSSc.


Assuntos
Predisposição Genética para Doença , Hipertensão Pulmonar/genética , Oxirredutases Intramoleculares/genética , Fatores Inibidores da Migração de Macrófagos/genética , Polimorfismo de Nucleotídeo Único , Regiões Promotoras Genéticas , Esclerodermia Difusa/genética , Alelos , Frequência do Gene , Estudos de Associação Genética , Genótipo , Humanos , Hipertensão Pulmonar/etiologia , Esclerodermia Difusa/complicações
19.
BMC Cardiovasc Disord ; 17(1): 187, 2017 07 17.
Artigo em Inglês | MEDLINE | ID: mdl-28716007

RESUMO

BACKGROUND: Systemic sclerosis (SSc) is an autoimmune disease characterized by microvascular abnormalities, inflammation and fibrosis. We hypothesized that myocarditis may be diagnosed in asymptomatic SSc, undergoing routine cardio-vascular magnetic resonance (CMR) for fibrosis assessment, using the Lake Louise criteria: T2 ratio, early (EGE) and late gadolinium enhanced (LGE) images. METHODS: Eighty-two asymptomatic SSc, diagnosed according to American College of Rheumatology criteria, aged 43 ± 5 yrs., 62 with diffuse (dSSc) and 20 with localized (lSSc) systemic sclerosis were evaluated by CMR, performed at 1.5 T scanner, according to Lake Louise criteria. RESULTS: CMR documented normal biventricular function in all SSc. However, 7/62 (11.2%) with dSSc and 2/20 (10%) with lSSc, had CMR signs of myocarditis according to Lake Louise criteria, without any clinical cardiac symptom. In these 9 patients, T2 ratio, EGE ratio and LGE (positive in all 9 SSc) were 2.8 ± 0.5%, 8 ± 3% and 5 ± 3% of LV mass, respectively. No correlation between CMR and blood inflammatory indices (C-reactive protein and erythrocyte sedimentation rate), cardiac troponin T, disease characteristics or type of SSc was identified. A repeat CMR at 6 months, after treatment with prednisone and azathioprine, showed normalisation of the acute inflammation CMR indices. CONCLUSIONS: Silent myocarditis may be diagnosed using the Lake Louise paper criteria in SSc patients without cardiac symptoms, has no correlation with blood inflammatory indices, cardiac troponin or disease characteristics. CMR is a promising tool to diagnose silent myocarditis in SSc and monitor the response to immunosuppressive treatment.


Assuntos
Imagem por Ressonância Magnética , Miocardite/diagnóstico por imagem , Esclerodermia Difusa/complicações , Esclerodermia Limitada/complicações , Adulto , Doenças Assintomáticas , Biomarcadores/sangue , Sedimentação Sanguínea , Proteína C-Reativa/metabolismo , Meios de Contraste/administração & dosagem , Feminino , Fibrose , Gadolínio DTPA/administração & dosagem , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Miocardite/tratamento farmacológico , Miocardite/etiologia , Miocardite/fisiopatologia , Miocárdio/patologia , Valor Preditivo dos Testes , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/tratamento farmacológico , Esclerodermia Limitada/diagnóstico , Esclerodermia Limitada/tratamento farmacológico , Fatores de Tempo , Resultado do Tratamento , Troponina T/sangue , Função Ventricular Esquerda , Função Ventricular Direita
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