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1.
ARP Rheumatol ; 3(2): 84-94, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38956991

RESUMEN

OBJECTIVE: To develop evidence-based recommendations for the non-pharmacological and pharmacological management of Raynaud's phenomenon (RP) and digital ulcers (DUs) in patients with systemic sclerosis and other immune-mediated connective tissue diseases (CTDs). METHODS: A task force comprising 21 rheumatologists, two surgeons (vascular and plastic), two nurses, and one patient representative was established. Following a systematic literature review performed to inform the recommendations, statements were formulated and discussed during two meetings (one online and one in-person). Levels of evidence, grades of recommendation (GoR), and level of agreement (LoA) were determined. RESULTS: Five overarching principles and 13 recommendations were developed. GoR ranged from A to D. The mean ± standard difference (SD) LoA with the overarching principles and recommendations ranged from 7.8±2.1 to 9.8±0.4. Briefly, the management of RP and DUs in patients with CTDs should be coordinated by a multidisciplinary team and based on shared decisions with patients. Nifedipine should be used as first-line therapy for RP and/or DUs. Sildenafil, tadalafil, and/or iloprost IV are second-line options for severe and/or refractory patients with RP and/or DUs. Sildenafil, tadalafil and/or Iloprost IV, should be prescribed for healing and prevention (also including bosentan) of DUs. In patients with RP and/or DUs, non-pharmacological interventions might be considered as add-ons, but there is limited quality and quantity of scientific evidence supporting their use. CONCLUSIONS: These recommendations will inform rheumatologists, specialist nurses, other healthcare professionals, and patients about a comprehensive and personalized management of RP and DUs. A research agenda was developed to address unmet needs, particularly for non-pharmacologic interventions.


Asunto(s)
Enfermedades del Tejido Conjuntivo , Dedos , Enfermedad de Raynaud , Esclerodermia Sistémica , Úlcera Cutánea , Humanos , Enfermedades del Tejido Conjuntivo/complicaciones , Enfermedades del Tejido Conjuntivo/terapia , Dedos/irrigación sanguínea , Dedos/patología , Portugal , Enfermedad de Raynaud/terapia , Enfermedad de Raynaud/etiología , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/terapia , Úlcera Cutánea/terapia , Úlcera Cutánea/etiología
2.
Artículo en Inglés | MEDLINE | ID: mdl-37998293

RESUMEN

The Health Assessment Questionnaire Disability Index (HAQ-DI) was completed with five visual analog scales to assess systemic sclerosis (SSc) called Scleroderma HAQ (SHAQ). We performed a validation of the European Portuguese version of SHAQ for patients with SSc. Patients with different forms of SSc from five Hospital Centers were invited. The reliability of the Portuguese SHAQ was evaluated by internal consistency and by test-retest reliability. Content validity was checked by two rheumatologists and by a panel of patients. Construct validity was assessed by structural validity and by known-groups hypothesis tests. Criterion validity was addressed with selected dimensions from the UCLA GIT 2.0, the SF-36v2, and the EuroQoL EQ-5D-5L. A total of 102 SSc patients agreed to participate, 31 of which answered to the retest. HAQ-DI demonstrated high internal consistency reliability (α = 0.866) and SHAQ also showed high test-retest reliability (ICC 0.61-0.95). We evidenced the unidimensionality of all VASs. HAQ-DI scores were worse in males, patients older than 65 years, and individuals with a diffuse form of SSc. Criterion validity was mainly evidenced through the correlation between the HAQ-DI and SF-36v2 physical summary measure (r = -0.688) and EQ-5D-5L index score (r = -0.723). Likewise, the SHAQ overall disease severity VAS was also correlated with SF-36v2 physical summary measure (r = -0.628). Mental score correlations were smaller. With the exception of the Raynaud's VAS, all the other VASs correlated well with similar clinical variables. This paper provides evidence to demonstrate how reliable and valid the European Portuguese version of SHAQ is, to be used in SSc patients to assess the clinical severity under the perspective of patients.


Asunto(s)
Esclerodermia Sistémica , Masculino , Humanos , Reproducibilidad de los Resultados , Portugal , Encuestas y Cuestionarios , Índice de Severidad de la Enfermedad , Esclerodermia Sistémica/diagnóstico , Calidad de Vida , Evaluación de la Discapacidad
3.
Clin Rheumatol ; 42(8): 2125-2134, 2023 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-37154983

RESUMEN

INTRODUCTION/OBJECTIVES: The study aims to define the clinical and subclinical calcinosis prevalence, the sensitivity of radiographed site and clinical method for its diagnosis, and the phenotype of Portuguese systemic sclerosis (SSc) patients with calcinosis. METHOD: A cross-sectional multicenter study was conducted with SSc patients fulfilling Leroy/Medsger 2001 or ACR/EULAR 2013 classification criteria, registered in the Reuma.pt. Calcinosis was assessed through clinical examination and radiographs of hands, elbows, knees, and feet. Independent parametric or non-parametric tests, multivariate logistic regression, and sensitivity calculation of radiographed site and clinical method for calcinosis detection were performed. RESULTS: We included 226 patients. Clinical calcinosis was described in 63 (28.1%) and radiological calcinosis in 91 (40.3%) patients, of which 37 (40.7%) were subclinical. The most sensitive location to detect calcinosis was the hand (74.7%). Sensitivity of the clinical method was 58.2%. Calcinosis patients were more often female (p = 0.008) and older (p < 0.001) and had more frequently longer disease duration (p < 0.001), limited SSc (p = 0.017), telangiectasia (p = 0.039), digital ulcers (p = 0.001), esophageal (p < 0.001) and intestinal (p = 0.003) involvements, osteoporosis (p = 0.028), and late capillaroscopic pattern (p < 0.001). In multivariate analysis, digital ulcers (OR 2.63, 95% CI 1.02-6.78, p = 0.045) predicted overall calcinosis, esophageal involvement (OR 3.52, 95% CI 1.28-9.67, p = 0.015) and osteoporosis (OR 4.1, 95% CI 1.2-14.2, p = 0.027) predicted hand calcinosis, and late capillaroscopic pattern (OR 7.6, 95% CI 1.7-34.9, p = 0.009) predicted knee calcinosis. Anti-nuclear antibody positivity was associated with less knee calcinosis (OR 0.021, 95% CI 0.001-0477, p = 0.015). CONCLUSIONS: Subclinical calcinosis high prevalence suggests that calcinosis is underdiagnosed and radiographic screening might be relevant. Multifactorial pathogenesis may explain calcinosis predictors' variability. Key Points • Prevalence of subclinical calcinosis in SSc patients is substantial. • Hand radiographs are more sensitive to detect calcinosis than other locations or clinical method. • Digital ulcers were associated with overall calcinosis, esophageal involvement and osteoporosis were associated with hand calcinosis, and late sclerodermic pattern in nailfold capillaroscopy was associated with knee calcinosis. • Anti-nuclear antibody positivity may be a protective factor for knee calcinosis.


Asunto(s)
Calcinosis , Osteoporosis , Esclerodermia Sistémica , Femenino , Humanos , Estudios Transversales , Portugal , Calcinosis/complicaciones , Calcinosis/diagnóstico por imagen , Osteoporosis/complicaciones
4.
Artículo en Inglés | MEDLINE | ID: mdl-36674306

RESUMEN

(1) Background: The UCLA GIT 2.0 questionnaire has been recognized as a feasible and reliable instrument to assess gastrointestinal (GI) symptoms in systemic sclerosis (SSc) patients and their impact on quality of life. The aim of this study was to create and validate UCLA GIT 2.0 for Portuguese patients with SSc. (2) Methods: A multi-center study was conducted enrolling SSc patients. UCLA GIT 2.0 was validated in Portuguese using reliability (internal consistency, item -total correlation, and reproducibility) and validity (content, construct, and criterion) tests. Criterion tests included EQ-5D and SF-36v2. Social-demographic and clinical data were collected. (3) Results: 102 SSc patients were included, 82.4% of them female, and with a mean sample age of 57.0 ± 12.5 years old. The limited form of SSc was present in 62% of the patients and 56.9% had fewer than five years of disease duration. Almost 60% presented with SSc-GI involvement with a negative impact on quality of life. The means for SF-36v2 were 39.3 ± 10.3 in the physical component summary and 47.5 ± 12.1 in the mental component summary. Total GI score, reported as mild in 57.8% of the patients, was highly reliable (ICC = 0.912) and the Cronbach's alpha was 0.954. There was a high correlation between the total GI score and EQ-5D-5L and SF-36v2 scores. (4) Conclusion: The Portuguese version of UCLA GIT 2.0 showed good psychometric properties and can be used in research and clinical practice.


Asunto(s)
Enfermedades Gastrointestinales , Esclerodermia Sistémica , Humanos , Femenino , Adulto , Persona de Mediana Edad , Anciano , Calidad de Vida , Reproducibilidad de los Resultados , Portugal , Índice de Severidad de la Enfermedad , Enfermedades Gastrointestinales/diagnóstico , Enfermedades Gastrointestinales/epidemiología , Esclerodermia Sistémica/diagnóstico , Psicometría , Encuestas y Cuestionarios
5.
Reumatol Clin (Engl Ed) ; 18(8): 493-494, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-36210142

RESUMEN

We describe a case of a 57-year-old white woman treated for rheumatoid arthritis (RA) with tofacitinib 10mg daily (started one year ago) and prednisolone 5mg daily. She presented to the emergency department with a tight squeezing chest pain and shortness of breath for 7h and the clinical evaluation revealed regional systolic dysfunction of the left ventricle, mimicking a myocardial infarction, in the absence of angiographic evidence of obstructive coronary artery disease or acute plaque rupture. All changes were transient and resolved completely within 4 days. The diagnosis of Takotsubo cardiomyopathy (TKM) was established. This is, as far as we know, the first report of a case of TKM in a RA patient taking tofacitinib. Although the association has not been previously described and the precise cause cannot be identified in this patient, the association with tofacitinib should be considered given the etiopathogenic rationale and the absence of any other identifiable cause.


Asunto(s)
Artritis Reumatoide , Cardiomiopatía de Takotsubo , Artritis Reumatoide/complicaciones , Artritis Reumatoide/tratamiento farmacológico , Femenino , Humanos , Persona de Mediana Edad , Piperidinas/efectos adversos , Prednisolona , Pirimidinas , Cardiomiopatía de Takotsubo/inducido químicamente , Cardiomiopatía de Takotsubo/diagnóstico
6.
ARP Rheumatol ; 1(2): 179-180, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35810378

RESUMEN

Patients with Systemic Sclerosis (SSc) seem to have higher prevalence of low bone mineral density (BMD) and spine fracture risk. We performed a transversal study including patients with the diagnosis of SSc to determine, by conventional densitometry and using the fracture risk assessment tool (FRAX), the prevalence of low BMD and the fracture risk, respectively, in a SSc Portuguese cohort and its potential determinants. Ninety-seven patients were included; 88.7% females (n=86) with median age of 62 years [56, 70]. Low BMD was present in 45 patients (46.4%). BMD in the femoral neck (FN) presented a weak positive correlation with body mass index (BMI) and the risk for major fracture with and without BMD presented a positive correlation with spine fractures. No correlations were found between BMD-FN and disease manifestations. Our results showed that low BMD is prevalent in SSc patients and may be associated with low BMI. FRAX appears to be an useful instrument as it correlates with spine fracture risk and with BMD. This is the first study in Portugal evaluating prevalence of low BMD and fracture risk in a Portuguese SSc cohort.


Asunto(s)
Densidad Ósea , Enfermedades Óseas Metabólicas , Fracturas Óseas , Osteoporosis , Esclerodermia Sistémica , Fracturas de la Columna Vertebral , Anciano , Enfermedades Óseas Metabólicas/complicaciones , Femenino , Fracturas Óseas/complicaciones , Humanos , Masculino , Persona de Mediana Edad , Osteoporosis/epidemiología , Portugal/epidemiología , Factores de Riesgo , Esclerodermia Sistémica/complicaciones , Fracturas de la Columna Vertebral/diagnóstico por imagen
8.
J Clin Rheumatol ; 28(1): e49-e55, 2022 Jan 01.
Artículo en Inglés | MEDLINE | ID: mdl-32956158

RESUMEN

OBJECTIVES: To evaluate potential predictors of subsequent fracture and increased mortality in a population 65 years or older who suffered a proximal femur fragility fracture. METHODS: This was a longitudinal study that included patients with a proximal femur fragility fracture, referred from the Orthopedics Inpatient Department to the Rheumatology Department's Fracture Liaison Service, from March 2015 to March 2017. RESULTS: Five hundred twenty-two patients were included, with a median age (IQR) of 84 years (interquartile range [IQR], 11 years), 79.7% (n = 416) female. Nine percent (n = 47) suffered a new fracture, with a median time to event of 298 days (IQR, 331 days). Cumulative probability without refracture at 12 months was 93% (95% confidence interval [CI], 90.2%-95.0%); 22.8% (n = 119) patients died, with median time to death of 126 days (IQR, 336 days). Cumulative survival probability at 12 months was 81.7 (95% CI, 77.9-84.8). Neurologic disease (hazard ratio [HR], 2.30; 95% CI, 0.97-5.50; p = 0.06) and chronic obstructive pulmonary disease (HR, 3.61; 95% CI, 1.20-10.9; p = 0.022) were both predictors of refracture. Age older than 80 years (HR, 1.54; 95% CI, 0.99-2.38; p = 0.052), higher degree of dependence (HR, 1.24;95% CI, 1.09-1.42; p = 0.001), male sex (HR, 1.55; 95% CI, 1.03-2.33; p = 0.034), femoral neck fracture (HR, 0.45; 95% CI, 0.24-0.88; p = 0.018), Charlson score (HR, 2.08; 95% CI, 1.17-3.69; p = 0.012), heart failure (HR, 2.44; 95% CI, 1.06-5.63; p = 0.037), hip bone mass density (HR, 3.99; 95% CI, 1.19-13.4; p = 0.025), hip T score (HR, 0.64; 95% CI, 0.44-0.93; p = 0.021), and ß-crosslaps (HR, 1.98; 95% CI, 1.02-3.84; p = 0.042) all predicted a higher mortality. CONCLUSIONS: Neurologic disease and chronic obstructive pulmonary disease may increase the risk of subsequent fracture after a hip fracture. Male sex, age, autonomy degree, femur bone mass density/T score, fracture type, Charlson score, diabetes mellitus, heart failure, and ß-crosslaps had significant impact on survival. The authors highlight ß-crosslaps as a potential serological marker of increased mortality in clinical practice.


Asunto(s)
Fracturas del Fémur , Fracturas de Cadera , Anciano de 80 o más Años , Niño , Femenino , Fracturas del Fémur/diagnóstico , Fracturas del Fémur/epidemiología , Fémur , Fracturas de Cadera/diagnóstico , Fracturas de Cadera/epidemiología , Humanos , Estudios Longitudinales , Masculino , Factores de Riesgo
9.
Acta Reumatol Port ; 46(3): 266-271, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34628460

RESUMEN

Tumor necrosis factor alpha inhibitors (TNFi) are basilar treatments in a number of inflammatory rheumatic conditions and autoimmune phenomena such as de novo neuroinflammatory events were already described in these populations under TNFi. We conducted a single-center retrospective study in a cohort of rheumatic patients treated with TNFi to characterize neurological demyelinating/inflammatory disease in these patients. We report 3 cases (n= 744): all of them had spondyloarthritis, the onset of neurological manifestations occurred between 37 and 58 years old and all of them initially presented with an optic neuritis. The neurological symptoms emerged between 13 and 26 months after starting TNFi. All patients discontinued treatment with TNFi, but one resumed therapy with symptomatic worsening, having to interrupt treatment again. All patients, latter on, fulfilled multiple sclerosis (MS) McDonald criteria 1 and were diagnosed with relapsing-remitting MS. Our study support the prior view of a risk, disease-dependent or agent-dependent, although a causal relationship is yet to be enlightened.


Asunto(s)
Antirreumáticos , Esclerosis Múltiple , Espondiloartritis , Adulto , Antirreumáticos/uso terapéutico , Estudios de Cohortes , Humanos , Persona de Mediana Edad , Esclerosis Múltiple/tratamiento farmacológico , Estudios Retrospectivos , Espondiloartritis/tratamiento farmacológico , Factor de Necrosis Tumoral alfa/uso terapéutico
10.
Acta Reumatol Port ; 46(3): 283-285, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34667140

RESUMEN

INTRODUCTION: Long-term survivors of cystic fibrosis (CF) have a dramatic increase in the risk of osteoporosis and incident fracture. The objective of this work is to characterize a CF related bone disease in a Portuguese cohort of CF patients. METHODS: We performed a cross-sectional, observational study on a cohort of CF adult patients. Clinical status, laboratory parametres, nutrition, lung function tests, genetics and bone mineral density (BMD) data were collected from a CF reference centre. RESULTS: Of 30 patients, 53.3% were males (n=16). Median age was 32.5 (27.0; 32,5) and median body mass index (BMI) was 22,04 (19,85; 24,55), with 4 patients (13.3%) being underweight (BMI<18.5 kg/m²). Four patients (13.3 %) were diagnosed with osteoporosis and 15 patients (50%) has low BMD. Among them, 2 (6.7%) had fragility fractures. A moderate correlation was found between the lumbar spine (LS) BMD and BMI and, as expected, femural neck BMD and LS BMD has moderate to strong correlations with BMD Z scores. CONCLUSION: Despite the young middle age we found a high prevalence of low BMD and osteoporosis in patients with CF. Early recognition and treatment are the most effective strategies for reducing the morbidity due to osteoporosis in these patients.


Asunto(s)
Fibrosis Quística , Osteoporosis , Adulto , Densidad Ósea , Estudios Transversales , Fibrosis Quística/complicaciones , Humanos , Masculino , Persona de Mediana Edad , Osteoporosis/epidemiología , Osteoporosis/etiología , Portugal/epidemiología , Adulto Joven
11.
Artículo en Inglés, Español | MEDLINE | ID: mdl-34417133

RESUMEN

We describe a case of a 57-year-old white woman treated for rheumatoid arthritis (RA) with tofacitinib 10mg daily (started one year ago) and prednisolone 5mg daily. She presented to the emergency department with a tight squeezing chest pain and shortness of breath for 7h and the clinical evaluation revealed regional systolic dysfunction of the left ventricle, mimicking a myocardial infarction, in the absence of angiographic evidence of obstructive coronary artery disease or acute plaque rupture. All changes were transient and resolved completely within 4 days. The diagnosis of Takotsubo cardiomyopathy (TKM) was established. This is, as far as we know, the first report of a case of TKM in a RA patient taking tofacitinib. Although the association has not been previously described and the precise cause cannot be identified in this patient, the association with tofacitinib should be considered given the etiopathogenic rationale and the absence of any other identifiable cause.

12.
Acta Reumatol Port ; 46(1): 69-71, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33820901

RESUMEN

Hypertrophic pachymeningitis (HP) is a rare clinical entity which uncommonly occurs in rheumatoid arthritis (RA) patients. We describe the case of a rheumatoid factor and anti-citrullinated protein antibody positive RA male that was diagnosed with HP and multiple mononeuropathy. Although histological evaluation was not performed, after excluding infectious and neoplastic causes, it was possible to determine a probable inflammatory etiology. He was treated with glucocorticoids and rituximab with a good clinical evolution. This case represented a challenging differential diagnosis but the need for an accurate diagnosis should not delay the introduction of an effective therapy for a potentially lethal disease.


Asunto(s)
Artritis Reumatoide , Meningitis , Artritis Reumatoide/complicaciones , Sistema Nervioso Central , Glucocorticoides , Humanos , Masculino , Meningitis/etiología , Rituximab
13.
J Immunol Res ; 2018: 5954897, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30148175

RESUMEN

PURPOSE: Rheumatoid arthritis (RA) is an often debilitating autoinflammatory disease. Patients with rheumatoid arthritis are often troubled by co-occurring depression or other psychological manifestations. RA patients have a variety of treatment options available, including biologicals that inhibit cytokines or immune cells. If these cytokines influence the psychological symptoms, then the use of cytokine inhibitors should modulate these symptoms. METHODS: A cohort of 209 individuals was recruited. This group included 82 RA patients, 22 healthy subjects, 32 depressed control subjects, and 73 subjects with systemic lupus erythematosus. Of the RA patients, 51% were on a biological therapeutic. ELISA was used to measure cytokine levels. A variety of psychological assessments were used to evaluate depression, anxiety, sleep, fatigue, and relationship status. Clinical values were obtained from medical records. RESULTS: IL-10 concentration was associated with depressive symptoms in the RA patients, healthy controls, and the lupus patients. In the patients with primary depression, depressive symptoms were associated with IL-6 and TNF-alpha. In RA patients, Tocilizumab use was associated with decreased depressive symptoms. 14 RA patients who were not using biologicals began using them by a one-month follow-up. In these patients, there was no significant change to any value except for fatigue. CONCLUSIONS: A variety of both biological and social factors influences depressive symptoms in RA. IL-10 and IL-6 are likely to be involved, since IL-10 concentration was associated with depression and Tocilizumab decreased depressive symptoms in the RA patients. The roles of these cytokines are different in RA and lupus, as high IL-10 in RA is associated with increased depressive symptoms, but high IL-10 in the lupus patients is associated with decreased depression. IL-6 was also associated with depressive symptoms in the patients with primary depression. These results strongly indicate that disease activity, including cytokine levels, has a strong impact on depressive symptoms.


Asunto(s)
Artritis Reumatoide/inmunología , Productos Biológicos/uso terapéutico , Depresión/inmunología , Inmunoterapia/métodos , Interleucina-10/metabolismo , Interleucina-6/metabolismo , Factor de Necrosis Tumoral alfa/metabolismo , Adulto , Anciano , Anticuerpos Monoclonales Humanizados/uso terapéutico , Artritis Reumatoide/psicología , Artritis Reumatoide/terapia , Depresión/psicología , Depresión/terapia , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Receptores de Interleucina-6/inmunología , Resultado del Tratamiento
14.
Medicine (Baltimore) ; 97(28): e11376, 2018 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-29995777

RESUMEN

Depression and anxiety cause severe loss of quality of life for patients with systemic lupus erythematosus. The causes and factors that contribute to these psychological manifestations in lupus are difficult to disentangle. This study compared clinical, psychological, and demographic factors between lupus patients, depressed patients, and rheumatoid arthritis patients to discover lupus-specific contributors to depression. Lupus-specific manifestations of depression were also investigated.Physiological, clinical, and psychosocial data were collected from 77 patients. ELISA was used to measure cytokine levels. Univariate and Multivariate analyses were used to compare the patient populations and identify correlations between key physical and psychological indicators.The prevalence of depression in the SLE cohort was 6 times greater than the healthy control subjects. Pain, IL-6, and Pittsburgh Sleep Quality index values were all significantly higher in SLE patients compared with the healthy control group (P < .001, P = .038, and P = .005, respectively). Anxiety levels were significantly higher in SLE patients compared to healthy and RA control patients (P = .020 and .011, respectively). Serum IL-10 concentrations, relationship assessment scale, and fatigue severity scale values were found to be correlated with depression among the SLE patients (P = .036, P = .007, and P = .001, respectively). Relationship assessment and fatigue severity scale scores were found to be the best indicators of depression for the SLE patients (P = .042 and .028, respectively).Fatigue Severity, relationship satisfaction, and IL-10 concentrations are indicators of depression in lupus patients. Despite also suffering from the pain and disability that accompanies chronic autoimmune disease, the rheumatoid arthritis patients had less anxiety and better relationship scores.


Asunto(s)
Ansiedad/etiología , Depresión/etiología , Lupus Eritematoso Sistémico/psicología , Adulto , Artritis Reumatoide/complicaciones , Artritis Reumatoide/psicología , Escolaridad , Femenino , Humanos , Interleucina-10/sangre , Lupus Eritematoso Sistémico/sangre , Lupus Eritematoso Sistémico/complicaciones , Masculino , Matrimonio , Persona de Mediana Edad , Dolor/etiología , Factores de Riesgo , Sueño
16.
Arthritis Care Res (Hoboken) ; 67(8): 1180-5, 2015 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-25581417

RESUMEN

OBJECTIVE: The new Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria aimed to improve the performance of systemic lupus erythematosus (SLE) classification over the American College of Rheumatology (ACR) 1997 criteria. However, the SLICC 2012 criteria need further external validation. Our objective was to compare the sensitivity for SLE classification between the ACR 1997 and the SLICC 2012 criteria sets in a real-life, multicenter, international SLE population. METHODS: We conducted a cross-sectional observational study of patients with a clinical diagnosis of SLE followed at the participating rheumatology centers and registered in the Portuguese and Spanish national registries. The sensitivity of the 2 classification sets was compared using McNemar's test. The sensitivity of ACR 1997 and SLICC 2012 was further examined in 5 subgroups, defined according to disease duration. RESULTS: We included 2,055 SLE patients (female 91.4%, white 93.5%, mean ± SD age at disease onset 33.1 ± 14.4 years, mean ± SD age at SLE diagnosis 35.3 ± 14.7 years, and mean ± SD age at the time of the study 47.4 ± 14.6 years) from 17 centers. The sensitivity for SLE classification was higher with the SLICC 2012 than with the ACR 1997 (93.2% versus 85.6%; P < 0.0001). Of 296 patients not fulfilling the ACR 1997, 62.8% could be classified with the SLICC 2012. The subgroup of patients with ≤5 years since disease onset presented the largest difference in sensitivity between the SLICC 2012 and the ACR 1997 (89.3% versus 76.0%; P < 0.0001); this difference diminished with longer disease duration, and it was no longer significant for patients with >20 years of disease duration. CONCLUSION: The SLICC 2012 criteria were more sensitive than the ACR 1997 criteria in real-life clinical practice in SLE. The SLICC 2012 criteria may allow patients to be classified as having SLE earlier in the disease course.


Asunto(s)
Lupus Eritematoso Sistémico/clasificación , Reumatología/normas , Adulto , Estudios de Cohortes , Estudios Transversales , Femenino , Humanos , Masculino , Estados Unidos
17.
Acta Reumatol Port ; 38(1): 44-8, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-24131911

RESUMEN

Although frequent in juvenile dermatomyositis, calcinosis is a rare finding in adult dermatomyositis. It has been associated with disease activity and delayed treatment. It is more common in later phases of the disease, in sites under chronic stress and trauma. Calcinosis has been associated with inflammation but information about its pathogeny continues to evolve as we learn more about the underlying processes. Being uncommon, there is no standard therapy and management is guided by case studies and series. Different treatments have been used in an attempt to clear calcinosis lesions and prevent its recurrence but none has been clearly effective. The authors present the case of a 25 year-old female diagnosed with dermatomyositis who developed calcinosis universalis after stopping therapy. Immunossupressive therapy was reinitiated and therapy aiming at reduction of calcinosis was sequentially tried using: colchicine, hydroxide magnesium, diltiazem, alendronate, probenecid and pamidronate. After receiving intravenous pamidronate, calcinosis lesions decreased and the patient regained full range of movement and quality of life. No recurrence has occurred after eight years of follow-up.


Asunto(s)
Calcinosis/etiología , Dermatomiositis/complicaciones , Enfermedades de la Piel/etiología , Adulto , Calcinosis/tratamiento farmacológico , Femenino , Humanos , Enfermedades de la Piel/tratamiento farmacológico
18.
Rheumatol Int ; 33(6): 1601-4, 2013 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-21526358

RESUMEN

Lipoma arborescens is a benign tumor, but it may be a reactive process to other disorders, and its clinical, analytical, radiological and ultrasound presentation may be redundant to any synovial tumor. Despite the characteristic feature on magnetic resonance imaging (MRI), the correct differential diagnosis in atypical presentation, and the need for timely removal of the lesion to prevent joint damage, forces, ultimately, to invasive procedures. The clinical case reported here, fourth described in English language publications on the polyarticular form, also presented other specificities related to one of the swellings, in the knee. Because of its atypical location in the popliteal fossa, recurrent episodes of joint effusion, personal history of knee trauma, pulmonary tuberculosis, and family history of rheumatoid arthritis required particular attention. This process was hampered by the refusal of knee (and ankle) surgery by the patient. He accepted surgical removal of the swellings of the wrists, for aesthetical reasons, with pathologic confirmation of the diagnosis, and clinical success in that location. MRI of the knee showed the typical image of lipoma arborescens, but also other changes that compromise the prognosis.


Asunto(s)
Artropatías/patología , Lipoma/patología , Membrana Sinovial/patología , Humanos , Artropatías/diagnóstico , Articulación de la Rodilla/patología , Lipoma/diagnóstico , Masculino , Persona de Mediana Edad , Articulación de la Muñeca/patología
19.
Acta Reumatol Port ; 36(2): 102-9, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21841729

RESUMEN

Vasculitic neuropathy corresponds to the occurrence of vasculitis at the level of vasa nervorum, resulting in ischemic damage of the peripheral nerve and axonal degeneration. Vasculitic neuropathy commonly occurs in association with systemic diseases and may be the initial manifestation or arise in the course of established disease. Although rare, vasculitis can be confined to the peripheral nervous system - non-systemic vasculitic neuropathy. This paper aims to review the classification, diagnosis and treatment of vasculitic neuropathy.


Asunto(s)
Enfermedades del Sistema Nervioso Periférico/complicaciones , Vasculitis/complicaciones , Humanos , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Enfermedades del Sistema Nervioso Periférico/etiología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Enfermedades del Sistema Nervioso Periférico/terapia , Vasculitis/diagnóstico , Vasculitis/etiología , Vasculitis/fisiopatología , Vasculitis/terapia
20.
Acta Reumatol Port ; 35(3): 352-7, 2010.
Artículo en Portugués | MEDLINE | ID: mdl-20975639

RESUMEN

OBJECTIVES: Osteoporosis is a «silent epidemic¼, resulting from a combination of many possible causal and risk factors, which prevalence depend on particular features of the population. The authors propose to characterize some of these elements, with special attention to vitamin D (vitD) levels, in a Portuguese population at risk, selected on the basis of a history of prior fragility fracture. METHODS: Participants were selected from the computerized list of discharges from all hospitalized patients in a Central Hospital in Oporto, between the dates 1/1/2002 and 31/12/2004, with the diagnosis of low-impact fracture of the wrist. They were invited to a reassessment of their risk of fracture, after 5 to 7 years from that event. For each one, a protocol was completed, focusing on clinical, epidemiological, laboratorial, radiological and densitometric parameters. RESULTS: In this population, as expected, there was a high rate of osteoporosis risk factors, which may explain the also high rate of subsequent osteoporotic fractures. Among others, early menopause, low intake of calcium and protein, levels of vitD generally inadequate and low frequency of osteopo-rosis treatment were noticed. DISCUSSION: The widespread insufficiency of vitD, described in other populations, mostly from the Northern countries, was also found here, supporting the suspicion that also in Portugal low vitD levels could be prevalent, despite the supposed regular sun exposure. These and other observations in this series, to be proven in directed studies, should, meanwhile, raise the levels of awareness of the community, but particularly health professionals and policymakers of public health, in a matter with a strong impact on individual and collective health in the country.


Asunto(s)
Fracturas Osteoporóticas/sangre , Vitamina D/sangre , Adulto , Anciano , Anciano de 80 o más Años , Estudios Transversales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Portugal
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