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1.
Respir Res ; 25(1): 138, 2024 Mar 23.
Artículo en Inglés | MEDLINE | ID: mdl-38521926

RESUMEN

BACKGROUND: The prognostic and theragnostic role of histopathological subsets in systemic sclerosis interstitial lung disease (SSc-ILD) have been largely neglected due to the paucity of treatment options and the risks associated with surgical lung biopsy. The novel drugs for the treatment of ILDs and the availability of transbronchial cryobiopsy provide a new clinical scenario making lung biopsy more feasible and a pivotal guide for treatment. The aim of our study was to investigate the usefulness of lung biopsy in SSc ILD with a systematic literature review (SLR). METHODS: PubMed, Embase and Cochrane databases were searched up to June 30, 2023. Search terms included both database-specific controlled vocabulary terms and free-text terms relating to lung biopsy and SSc-ILD diagnostic and prognosis. The SLR was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA). Studies were selected according to the PEO (population, exposure, and outcomes) framework and Quality assessment of diagnostic accuracy studies (QUADAS) were reported. RESULTS: We selected 14 articles (comprising 364 SSc-ILD patients). The paucity and heterogeneity of the studies prevented a systematic analysis. Diffuse cutaneous SSc was present in 30-100% of cases. Female predominance was observed in all studies (ranging from 64 to 100%). Mean age ranged from 42 to 64 years. Mean FVC was 73.98 (+/-17.3), mean DLCO was 59.49 (+/-16.1). Anti-Scl70 antibodies positivity was detected in 33% of cases (range: 0-69.6). All patients underwent surgical lung biopsies, and multiple lobes were biopsied in a minority of studies (4/14). Poor HRCT-pathologic correlation was reported with HRCT-NSIP showing histopathologic UIP in up to 1/3 of cases. Limited data suggest that SSc-UIP patients may have a worse prognosis and response to immunosuppressive treatment compared to other histopathologic patterns. CONCLUSIONS: The data from this SLR clearly show the paucity and heterogeneity of the studies reporting lung biopsy in SSc ILD. Moreover, they highlight the need for further research to address whether the lung biopsy can be helpful to refine prognostic prediction and guide therapeutic choices.


Asunto(s)
Enfermedades Pulmonares Intersticiales , Esclerodermia Sistémica , Humanos , Femenino , Adulto , Persona de Mediana Edad , Masculino , Enfermedades Pulmonares Intersticiales/patología , Pulmón/patología , Esclerodermia Sistémica/diagnóstico , Esclerodermia Sistémica/complicaciones , Biopsia , Pronóstico
2.
Scand J Rheumatol ; : 1-5, 2024 Jun 04.
Artículo en Inglés | MEDLINE | ID: mdl-38832522

RESUMEN

OBJECTIVE: The efficacy of Janus kinase inhibitors (JAKi) in rheumatoid arthritis (RA) has been clearly shown. However, information on comparative drug retention rates (DRRs) of different JAKi is heterogeneous. The aim of this study was to compute and compare DRRs of different JAKi in a large cohort of RA patients. METHOD: Patients with RA treated with at least one JAKi and followed up at our centre were retrospectively identified. DRRs of each JAKi were computed at 24 months. The association of baseline features with drug persistence was tested. Variations in 28-joint Disease Activity Score-C-reactive protein (DAS28-CRP) and Clinical Disease Activity Index (CDAI) scores between baseline and 12 months were analysed. RESULTS: The study included 365 patients, with a total of 463 therapy courses. Tofacitinib was the most prescribed JAKi (33%), followed by baricitinib (25%), upadacitinib (24%), and filgotinib (21%). The mean treatment duration was 24 ± 17 months, with a maximum of 70 months. At 24 months, the overall DRR was 86%. DRRs were not significantly different across different JAKi. The only baseline predictor of treatment discontinuation was previous treatment with a biological disease-modifying anti-rheumatic drug (bDMARD) (hazard ratio 1.65, 95% confidence interval 1.08-2.53; p = 0.021). There were significant reductions in DAS28-CRP and CDAI 1 year after treatment start. CONCLUSIONS: In our large, monocentric cohort, the overall 24 month DRR for JAKi was greater than 80%. No significant differences in retention were found among different JAKi. Persistence was lower in patients who had previously been treated with other bDMARDs.

3.
Reumatismo ; 76(1)2024 Mar 22.
Artículo en Inglés | MEDLINE | ID: mdl-38523585

RESUMEN

OBJECTIVE: In the last decades, the number of foreigners in Tuscany has considerably increased with a multiethnic distribution. We reviewed the main rheumatic diseases in the foreign population resident in Tuscany and also reported the experience at the Rheumatology Division of the University Hospital of Careggi, Florence, in order to identify the areas of origin of these patients and the main rheumatic diseases observed in them. METHODS: The collaboration with the Tuscan Region provided data about foreign patients residing in Tuscany on January 1, 2021 (country of origin, chronic diseases). Moreover, we conducted a retrospective review of the clinical charts of our Rheumatologic Division from January 1, 2019, to December 31, 2020. RESULTS: In Tuscany, on January 1, 2021, there were 61,373 patients with chronic inflammatory rheumatic diseases, and 3994 of them (6.51%) were foreigners. Most patients were born in Europe (39.03%), followed by the Balkans (15%), South America (11.27%), and North Africa (10.31%). Inflammatory joint diseases, Sjögren syndrome, and systemic lupus erythematosus were the most frequent diseases. In the period 2019-2020, 511 foreign patients visited our Rheumatology Division and mainly originated from the Balkans (34.64%), South America (18%), and European countries (16.44%). In these patients, chronic inflammatory joint diseases and connective tissue diseases (systemic sclerosis, Sjögren syndrome, and systemic lupus erythematosus) were the most prevalent diseases. CONCLUSIONS: This study provides a picture of the rheumatic diseases affecting foreign patients residing in Tuscany that are in agreement with the epidemiological data previously provided.


Asunto(s)
Enfermedades Reumáticas , Migrantes , Humanos , Enfermedades del Tejido Conjuntivo , Lupus Eritematoso Sistémico , Enfermedades Reumáticas/epidemiología , Síndrome de Sjögren
4.
J Intern Med ; 289(6): 831-839, 2021 06.
Artículo en Inglés | MEDLINE | ID: mdl-33615623

RESUMEN

Recent evidence suggests that carpal tunnel syndrome (CTS) and brachial biceps tendon rupture (BBTR) represent red flags for ATTR cardiac amyloidosis (ATTR-CA). The prevalence of upper limb tenosynovial complications in conditions entering differential diagnosis with CA, such as HCM or Anderson-Fabry disease (AFD), and hence their predictive accuracy in this setting, still remains unresolved. OBJECTIVE: To investigate the prevalence of CTS and BBTR in a consecutive cohort of ATTR-CA patients, compared with patients with HCM or AFD and with individuals without cardiac disease history. PARTICIPANTS: Consecutive patients with a diagnosis of ATTR-CA, HCM and AFD were evaluated. A control group of consecutive patients was recruited among subjects hospitalized for noncardiac reasons and no cardiac disease history. The presence of BBTR, CTS or prior surgery related to these conditions was ascertained. RESULTS: 342 patients were prospectively enrolled, including 168 ATTR-CA (141 ATTRwt, 27 ATTRm), 81 with HCM/AFD (N = 72 and 9, respectively) and 93 controls. CTS was present in 75% ATTR-CA patients, compared with 13% and 10% of HCM/AFD and controls (P = 0.0001 for both comparisons). Bilateral CTS was present in 60% of ATTR-CA patients, while it was rare (2%) in the other groups. BBTR was present in 44% of ATTR-CA patients, 8% of controls and 1% in HCM/AFD. CONCLUSIONS: CTS and BBTR are fivefold more prevalent in ATTR-CA patients compared with cardiac patients with other hypertrophic phenotypes. Positive predictive accuracy for ATTR-CA is highest when involvement is bilateral. Upper limb assessment of patients with HCM phenotypes is a simple and effective way to raise suspicion of ATTR-CA.


Asunto(s)
Amiloidosis , Cardiomiopatía Hipertrófica , Síndrome del Túnel Carpiano , Enfermedad de Fabry , Amiloidosis/diagnóstico , Amiloidosis/epidemiología , Cardiomiopatía Hipertrófica/diagnóstico , Cardiomiopatía Hipertrófica/epidemiología , Síndrome del Túnel Carpiano/diagnóstico , Síndrome del Túnel Carpiano/epidemiología , Enfermedad de Fabry/diagnóstico , Enfermedad de Fabry/epidemiología , Humanos , Fenotipo
5.
Reumatismo ; 72(4): 228-246, 2021 Jan 18.
Artículo en Inglés | MEDLINE | ID: mdl-33677950

RESUMEN

The term pulmonary arterial hypertension (PAH) identifies a heterogeneous group of diseases characterized by a progressive increase in pulmonary arterial resistance (PVR), which causes a significant burden in terms of quality of life, right heart failure and premature death. The pathogenesis of PAH is not completely clear: the remodeling of the small pulmonary vessels is crucial, causing an increase in the resistance of the pulmonary circle. Its diagnosis is based on cardiac catheterization of the right heart. According to the present hemodynamic definition of pulmonary hypertension (PH) proposed by the Guidelines of the European Society of Cardiology/European Respiratory Society (ESC-ERS), the mean pulmonary arterial pressure (mPAP) values are ≥25 mmHg. In case of PAH, apart from an mPAP value ≥25 mmHg, patients must have a >3 Wood units increase in PVR and normal pressure values of the left heart. PH is a pathophysiological condition observed in more than 40 different diseases, while PAH is a primary disease of the pulmonary bloodstream potentially treatable with specific drugs. PAH is a severe complication of systemic sclerosis (SSc) affecting about 10% of the patients. Due to the devastating nature of SSc-PAH, there is a clear need to systematically adopt appropriate screening programs. In fact, despite awareness of the negative impact of SSc-PAH on quality of life and survival, as well as on the severity of lung function, at the moment standardized and shared guidelines and/or screening programs for the diagnosis and the subsequent early treatment of PAH in SSc are not available. The aim of the present paper is to highlight the lights and shadows of SSc-PAH, unraveling the unmet clinical needs on this topic with a proposal of clinical-diagnostic and therapeutic guidelines.


Asunto(s)
Hipertensión Pulmonar , Hipertensión Arterial Pulmonar , Esclerodermia Sistémica , Hemodinámica , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/terapia , Calidad de Vida
6.
Osteoporos Int ; 30(2): 391-402, 2019 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-30178159

RESUMEN

An innovative, non-ionizing technique to diagnose osteoporosis on lumbar spine and femoral neck was evaluated through a multicenter study involving 1914 women. The proposed method showed significant agreement with reference gold standard method and, therefore, a potential for early osteoporosis diagnoses and possibly improved patient management. INTRODUCTION: To assess precision (i.e., short term intra-operator precision) and diagnostic accuracy of an innovative non-ionizing technique, REMS (Radiofrequency Echographic Multi Spectrometry), in comparison with the clinical gold standard reference DXA (dual X-ray absorptiometry), through an observational multicenter clinical study. METHODS: In a multicenter cross-sectional observational study, a total of 1914 postmenopausal women (51-70 years) underwent spinal (n = 1553) and/or femoral (n = 1637) DXA, according to their medical prescription, and echographic scan of the same anatomical sites performed with the REMS approach. All the medical reports (DXA and REMS) were carefully checked to identify possible errors that could have caused inaccurate measurements: erroneous REMS reports were excluded, whereas erroneous DXA reports were re-analyzed where possible and otherwise excluded before assessing REMS accuracy. REMS precision was independently assessed. RESULTS: In the spinal group, quality assessment on medical reports produced the exclusion of 280 patients because of REMS errors and 78 patients because of DXA errors, whereas 296 DXA reports were re-analyzed and corrected. Analogously, in the femoral group there were 205 exclusions for REMS errors, 59 exclusions for DXA errors, and 217 re-analyzed DXA reports. In the resulting dataset (n = 1195 for spine, n = 1373 for femur) REMS outcome showed a good agreement with DXA: the average difference in bone mineral density (BMD, bias ± 2SD) was -0.004 ± 0.088 g/cm2 for spine and - 0.006 ± 0.076 g/cm2 for femur. Linear regression showed also that the two methods were well correlated: standard error of the estimate (SEE) was 5.3% for spine and 5.8% for femur. REMS precision, expressed as RMS-CV, was 0.38% for spine and 0.32% for femur. CONCLUSIONS: The REMS approach can be used for non-ionizing osteoporosis diagnosis directly on lumbar spine and femoral neck with a good level of accuracy and precision. However, a more rigorous operator training is needed to limit the erroneous acquisitions and to ensure the full clinical practicability.


Asunto(s)
Cuello Femoral/diagnóstico por imagen , Vértebras Lumbares/diagnóstico por imagen , Osteoporosis Posmenopáusica/diagnóstico por imagen , Absorciometría de Fotón/métodos , Anciano , Densidad Ósea/fisiología , Estudios Transversales , Femenino , Cuello Femoral/fisiopatología , Humanos , Vértebras Lumbares/fisiopatología , Persona de Mediana Edad , Osteoporosis Posmenopáusica/fisiopatología , Reproducibilidad de los Resultados , Ultrasonografía/métodos
7.
Scand J Rheumatol ; 48(1): 42-51, 2019 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-30039730

RESUMEN

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


Asunto(s)
Sistema de Registros , Esclerodermia Sistémica/epidemiología , Adulto , Distribución por Edad , Factores de Edad , Edad de Inicio , Estudios Transversales , Bases de Datos Factuales , Europa (Continente)/epidemiología , Femenino , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Prevalencia , Estudios Retrospectivos , Esclerodermia Sistémica/diagnóstico , Distribución por Sexo
8.
Scand J Rheumatol ; 47(4): 311-318, 2018 07.
Artículo en Inglés | MEDLINE | ID: mdl-29409385

RESUMEN

OBJECTIVES: Iloprost plays an important role in the treatment of Raynaud's phenomenon (RP), but has transient vasodilatory effects owing to its very short half-time. We aimed to evaluate short- and medium-term haemodynamic effects of iloprost by measuring dorsal finger microvessel blood flow using laser Doppler flowmetry (LDF), in patients with RP associated with systemic sclerosis (SSc). METHOD: In 24 consecutive SSc patients with RP (disease duration 10.5 ± 1.3 years), LDF with heating probes was used to measure blood flow in four fingers by occlusive and heating tests, at baseline, after 3 consecutive days of iloprost infusion, and at 24 h and 7 days after last iloprost infusion. Nailfold videocapillaroscopy (NVC) patterns of microvascular damage were investigated. Sixteen healthy controls were studied to compare baseline flows. RESULTS: Compared to controls, SSc patients showed significantly impaired axon reflex vasoregulation and nitric oxide responses at baseline (p = 0.001 and p = 0.03, respectively). After iloprost, a prompt but transient significant improvement in endothelial-dependent vasodilation (occlusive test) was seen only in SSc patients with an 'active' NVC pattern (p ≤ 0.05). The iloprost effects vanished within 7 days after the last infusion. No significant differences were found, in the whole study, between patients with and without digital ulcers. CONCLUSIONS: Microcirculatory blood flow increases following 3 days of iloprost infusion but fades shortly after treatment. Although iloprost is effective in reducing the severity of RP in SSc, the most suitable regimen and timing to obtain longer lasting vasodilatory benefits remain to be established.


Asunto(s)
Dedos/irrigación sanguínea , Iloprost/farmacología , Microcirculación/efectos de los fármacos , Flujo Sanguíneo Regional/efectos de los fármacos , Esclerodermia Sistémica/tratamiento farmacológico , Vasodilatación/efectos de los fármacos , Vasodilatadores/farmacología , Adulto , Anciano , Estudios de Casos y Controles , Femenino , Dedos/diagnóstico por imagen , Humanos , Iloprost/uso terapéutico , Infusiones Intravenosas , Flujometría por Láser-Doppler , Estudios Longitudinales , Masculino , Angioscopía Microscópica , Persona de Mediana Edad , Esclerodermia Sistémica/fisiopatología , Vasodilatadores/uso terapéutico
9.
Scand J Rheumatol ; 47(1): 62-70, 2018 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-28990485

RESUMEN

OBJECTIVES: Systemic sclerosis (SSc) is heterogenous. The objectives of this study were to evaluate the purpose, strengths and limitations of existing SSc subset criteria, and identify ideas among experts about subsets. METHODS: We conducted semi-structured interviews with randomly sampled international SSc experts. The interview transcripts underwent an iterative process with text deconstructed to single thought units until a saturated conceptual framework with coding was achieved and respondent occurrence tabulated. Serial cross-referential analyses of clusters were developed. RESULTS: Thirty experts from 13 countries were included; 67% were male, 63% were from Europe and 37% from North America; median experience of 22.5 years, with a median of 55 new SSc patients annually. Three thematic clusters regarding subsetting were identified: research and communication; management; and prognosis (prediction of internal organ involvement, survival). The strength of the limited/diffuse system was its ease of use, however 10% stated this system had marginal value. Shortcomings of the diffuse/limited classification were the risk of misclassification, predictions/generalizations did not always hold true, and that the elbow or knee threshold was arbitrary. Eighty-seven percent use more than 2 subsets including: SSc sine scleroderma, overlap conditions, antibody-determined subsets, speed of progression, and age of onset (juvenile, elderly). CONCLUSIONS: We have synthesized an international view of the construct of SSc subsets in the modern era. We found a number of factors underlying the construct of SSc subsets. Considerations for the next phase include rate of change and hierarchal clustering (e.g. limited/diffuse, then by antibodies).


Asunto(s)
Medición de Riesgo/métodos , Esclerodermia Sistémica/diagnóstico , Adulto , Estudios Transversales , Progresión de la Enfermedad , Femenino , Humanos , Masculino , Pronóstico
12.
Reumatismo ; 68(2): 109-11, 2016 Sep 09.
Artículo en Inglés | MEDLINE | ID: mdl-27608801

RESUMEN

Systemic sclerosis (SSc) is a connective tissue disease frequently associated with Raynaud's Phenomenon (RP). Among possible pharmacological treatments, phosphodiesterase 5 inhibitors are considered in cases of severe non -responsive RP. We present the case of a male SSc patient wh presented with critical finger ischemia and concomitant appearance of myocardial fibrosis after sudden interruption of sildenafil treatment.


Asunto(s)
Antirreumáticos/efectos adversos , Cardiomiopatías/patología , Dedos/irrigación sanguínea , Enfermedad de Raynaud/tratamiento farmacológico , Esclerodermia Sistémica/tratamiento farmacológico , Citrato de Sildenafil/efectos adversos , Síndrome de Abstinencia a Sustancias , Antirreumáticos/uso terapéutico , Cardiomiopatías/etiología , Humanos , Isquemia , Masculino , Persona de Mediana Edad , Miocardio/patología , Enfermedad de Raynaud/complicaciones , Enfermedad de Raynaud/patología , Factores de Riesgo , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/patología , Citrato de Sildenafil/uso terapéutico , Factores de Tiempo
14.
Reumatismo ; 67(4): 138-48, 2015 Dec 23.
Artículo en Inglés | MEDLINE | ID: mdl-27215179

RESUMEN

Diet and lifestyles modification are core aspects of the non-pharmacological management of gout, but a poor consistency with suggested guidelines is reported. This study aimed to investigate dietary and lifestyle habits of patients with gout followed in rheumatology settings. Data were retrieved from the baseline dataset of the KING study, a multicentre cohort study of patients with gout followed in rheumatology settings. Dietary habits were assessed with the Italian National Institute of Statistics (ISTAT) food-frequency questionnaire and compared with reported data about general population. The relative increase of exposure was estimated by standardized prevalence ratios adjusted for gender, age and geographical distribution. The study population included 446 patients, with a mean age of 63.9 years and a M/F ratio of 9:1. Compared to the Italian population, gouty patients showed a higher prevalence of obesity [1.82 (1.52-2.18)] and a higher consumption of wine [1.85 (1.48-2.32)] and beer [2.21 (1.68-2.90)], but a lower prevalence of smoking and a lower intake of liquor. They showed a lower intake of red meat [0.80 (0.71-0.91)], but a similar intake of other tested dietary factors. Gouty patients' lifestyle is still partially different from the recommended.


Asunto(s)
Conducta Alimentaria , Gota/complicaciones , Gota/prevención & control , Conocimientos, Actitudes y Práctica en Salud , Estilo de Vida , Obesidad/complicaciones , Obesidad/prevención & control , Reumatología , Animales , Cerveza/estadística & datos numéricos , Índice de Masa Corporal , Estudios de Cohortes , Femenino , Peces , Gota/epidemiología , Gota/etiología , Adhesión a Directriz , Humanos , Italia/epidemiología , Masculino , Carne/estadística & datos numéricos , Persona de Mediana Edad , Evaluación Nutricional , Obesidad/epidemiología , Obesidad/etiología , Prevalencia , Carne Roja/estadística & datos numéricos , Factores de Riesgo , Fumar/epidemiología , Encuestas y Cuestionarios , Vino/estadística & datos numéricos
15.
Ann Rheum Dis ; 73(5): 794-6, 2014 May.
Artículo en Inglés | MEDLINE | ID: mdl-24335919

RESUMEN

The use of biological agents in the treatment of rheumatic diseases has been widely associated with an increased risk of reactivation of several latent infections. National and international guidelines recommend screening for infectious diseases before starting these drugs. In Western countries screening is limited to latent tuberculosis infection, HIV and viral hepatitis. However, the increasing globalisation and the remarkable number of migrating and travelling people worldwide make this approach no longer adequate. The Italian and Spanish Societies of Rheumatology and Tropical Medicine wish to issue a warning about the need to improve awareness of doctors about the risk of reactivation of infectious tropical diseases in migrant or travelling patients who undergo biological therapy. Thus, the Italian and Spanish Societies are now planning to issue specific recommendations, based on a multidisciplinary contribution and a systematic review of the literature, for screening and follow-up of active and latent chronic infections in candidate patients for biological agents, taking into account the patient's area of origin and risk of infectious diseases.


Asunto(s)
Enfermedades Transmisibles/diagnóstico , Guías como Asunto , Tamizaje Masivo , Migrantes , Antirreumáticos/uso terapéutico , Humanos , Tuberculosis Latente , Enfermedades Reumáticas/tratamiento farmacológico
16.
Haemophilia ; 20(1): e32-9, 2014 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-24308756

RESUMEN

Haemophilic arthropathy (HA) is characterized by chronic proliferative synovitis leading to cartilage destruction and shares some pathological features with rheumatoid arthritis (RA). Apoptosis has been implicated in RA pathogenesis, and an agonistic anti-Fas monoclonal antibody (mAb) was found to induce RA fibroblast-like synoviocyte (FLS) apoptosis and suppress synovial hyperplasia in animal models of RA. The aim of this study was to evaluate the effect of anti-Fas mAb on HA-FLS. FLS were isolated from knee synovial biopsies from six HA patients, six RA patients and six healthy subjects. The expression of Fas in synovial biopsies was investigated by immunohistochemistry. FLS were stimulated with anti-Fas mAb at different concentrations, alone or in combination with tumour necrosis factor-α (TNF-α) and basic fibroblast growth factor (bFGF). Fas expression in FLS was assessed by Western blot. Cell viability was studied with the WST-1 assay. Active caspase-3 levels were measured using ELISA and Western blot. A strong Fas-immunoreactivity was observed in different cells of HA synovium, including FLS, inflammatory cells and endothelial cells. Fas antigen was constitutively overexpressed in cultured HA-FLS. Anti-Fas mAb had a significant cytotoxicity on HA-FLS in a dose-dependent manner, either alone or in combination with TNF-α and bFGF. These cytotoxic effects were due to the ability of anti-Fas to induce HA-FLS apoptosis, as shown by the increased active caspase-3 levels. Anti-Fas mAb exhibited a more pronounced pro-apoptotic effect on HA-FLS than RA-FLS. Fas antigen is highly expressed on HA-FLS and its stimulation by anti-Fas mAb may be an effective strategy to induce HA-FLS apoptosis.


Asunto(s)
Anticuerpos Monoclonales/farmacología , Apoptosis/efectos de los fármacos , Fibroblastos/efectos de los fármacos , Hemartrosis/etiología , Hemofilia A/complicaciones , Membrana Sinovial/efectos de los fármacos , Membrana Sinovial/patología , Adulto , Anticuerpos Monoclonales/inmunología , Anticuerpos Monoclonales de Origen Murino , Artritis Reumatoide/metabolismo , Caspasa 3/metabolismo , Supervivencia Celular/efectos de los fármacos , Fibroblastos/metabolismo , Hemartrosis/metabolismo , Hemartrosis/patología , Humanos , Inmunoglobulina M/inmunología , Inmunoglobulina M/farmacología , Masculino , Persona de Mediana Edad , Membrana Sinovial/metabolismo , Adulto Joven , Receptor fas/inmunología , Receptor fas/metabolismo
18.
Lupus ; 23(1): 57-68, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-24218395

RESUMEN

INTRODUCTION: There are few prospective data on bone mass and quality in patients with juvenile onset systemic lupus erythematosus (JSLE). There are also few studies analyzing bone mass and quality determinants by using at the same time dual-energy X-ray absorptiometry (DXA), peripheral quantitative computed tomography (pQCT) and quantitative ultrasound (QUS). OBJECTIVE: The objective of this paper is to evaluate cross-sectionally and longitudinally bone mass and quality determinants in adolescents and young adults with JSLE, and to identify the main predictors of reduced bone mineral density (BMD) and bone quality using these techniques. METHODS: Fifty-six patients with JSLE (mean age 18.5 ± 5.7 years) entered the study. In all subjects DXA scan at the lumbar spine, radius pQCT and phalangeal QUS were performed the same day. Of these, 46 patients (mean age 23.1 ± 6.2 years) were revaluated with a second DXA, pQCT and QUS. The data obtained were compared with 72 and 80 age- and sex- matched healthy controls. RESULTS: At the first evaluation, JSLE patients had a reduced spine BMAD SDS (p < 0.001), and significantly lower levels of TrabBMD (p < 0.0001), SSIp (p < 0.05), AD-SoS and QUS z-score (p < 0.005) but not reduced muscle CSA and CBA values. CortBMD and FatCSA were significantly increased (p < 0.0001). These data were confirmed at longitudinal evaluation regarding spine BMAD SDS (p < 0.001), TrabBMD (p < 0.0001), FatCSA (p < 0.005), AD-SoS (p < 0.001), and QUS z-score (p < 0.005) but not muscle CSA (p ≤ 0.05) and CBA (p < 0.0001). SSIp and CortBMD longitudinal evaluation showed that JSLE patients did not present significant differences in comparison to controls. CONCLUSIONS: Patients with JSLE have a low bone mass without catch-up growth over time, causing a reduction of peak bone mass with high risk of osteoporosis in early adulthood. To reduce the risk, close monitoring of BMD, better control of disease activity, physical activity and dietary intake of calcium and vitamin D are advocated to ameliorate the loss of bone mass. In patients with proved osteoporosis therapeutic approaches including bisphosphonates should be considered.


Asunto(s)
Densidad Ósea/fisiología , Huesos/diagnóstico por imagen , Huesos/patología , Lupus Eritematoso Sistémico/diagnóstico por imagen , Lupus Eritematoso Sistémico/fisiopatología , Absorciometría de Fotón/normas , Adolescente , Huesos/fisiopatología , Femenino , Humanos , Estudios Longitudinales , Lupus Eritematoso Sistémico/epidemiología , Masculino , Tomografía Computarizada por Rayos X/normas , Ultrasonografía , Adulto Joven
19.
Lupus ; 23(13): 1392-406, 2014 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-25074873

RESUMEN

BACKGROUND: Few prospective data have been published on the comparison of bone density and quality in homogeneous groups of patients with juvenile systemic lupus erythematosus (JSLE) and juvenile idiopathic arthritis (JIA). OBJECTIVE AND HYPOTHESIS: The objective of this study is to perform a longitudinal evaluation of the prevalence and the characteristics of bone mass and quality and to evaluate the differences on the bone parameters, using DXA, pQCT and QUS. POPULATION AND/OR METHODS: Forty-three JSLE patients (35 females, 8 males, median age 18.8, range 14.0-34.1 years) have been studied with DXA, pQCT and QUS scans and compared with 138 JIA patients (112 females, 26 males, median age 18.9, range 13.4-33.2 years), and 79 controls (59 females, 20 males; median age 19.3, range 13.5-36.5 years). Of these, 39 patients (32 females and 7 males, median age 20.3, range 16.6-36.8 years) with JSLE were followed longitudinally and compared with 131 patients (108 females, 23 males median age 20.7, range 15.8-37.1 years) with JIA and 63 controls (48 females, 15 males; median age 21.9, range 15.5-38.3 years). RESULTS: JSLE patients have a higher bone cortical density (CrtBMD) than controls and JIA patients (p < 0.005). However, JSLE and JIA patients have a significantly reduced bone trabecular density (TrbBMD) compared to controls (p < 0.0001), with no differences between JSLE and JIA. In addition, JIA patients show a significantly reduced muscle area (MuscleCSA) compared to JSLE and controls (p < 0.001). Conversely, fat area (FatCSA) is significantly increased both in JIA and JSLE patients when compared to controls (p < 0.001), with no differences between the JSLE and JIA groups. Analogous results are observed in the polar resistance to stress (SSIp). On longitudinal evaluation, contrary to CrtBMD, the difference between BMAD SDS, TrbBMD, MuscleCSA and FatCSA remains unchanged; in JSLE patients, SSIp is stable in comparison to JIA and controls without any difference between the two groups. CONCLUSIONS: The evaluation of bone density and structure parameters in JSLE patients highlights significant differences compared with JIA patients and controls. These data might indicate a different pathogenesis of bone damage in the two entities, and suggest a different diagnostic and therapeutic approach to improve the peak bone mass.


Asunto(s)
Artritis Juvenil/fisiopatología , Densidad Ósea , Huesos/diagnóstico por imagen , Lupus Eritematoso Sistémico/fisiopatología , Absorciometría de Fotón , Tejido Adiposo/diagnóstico por imagen , Adolescente , Adulto , Femenino , Humanos , Estudios Longitudinales , Masculino , Músculo Esquelético/diagnóstico por imagen , Ultrasonografía , Adulto Joven
20.
Clin Exp Rheumatol ; 32(6 Suppl 86): S-15-20, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-24850211

RESUMEN

OBJECTIVES: In systemic sclerosis (SSc), the frequent involvement of hand and face leads to their disability. We aimed to assess influence of hand and face disability on global disability and Health related Quality of life (HRQoL). METHODS: 119 SSc patients were assessed for global disability by HAQ, HRQoL, by SF36; hand disability by HAMIS, CHFDS, fist closure and hand opening measures; face disability by MHISS and mouth opening measure. RESULTS: Diffuse SSc (dSSc) patients present higher HAQ, lower Summary Physical Index (SPI) of SF36, major hand disability at hand (higher HAMIS, CHFDS, fist closure, lower hand opening) and face (lower mouth opening, higher MHISS) than lSSc patients (p<0.05). SPI of SF36 is negatively correlated with MHISS, CHFDS, HAMIS and positively correlated to mouth and hand opening (p<0.05). Summary Mental Index (SMI) of SF36 is negatively correlated with MHISS (p<0.05). HAQ is negatively correlated with mouth opening and positively correlated to MHISS, HAMIS, CHFDS (p<0.05). By hierarchical multiple linear regression, SPI of SF36 is significantly associated with total MHISS (B=-0.34; t=-3.78; p<0.001) and CHFDS (B=-0.27; t=-3.01; p=0.003), together, explaining 22% of SPI variance. SMI of SF36 is significantly associated only with MHISS total score (B=-0.22; t=-2.41; p=0.017), explaining 4% of its variance. HAQ is significantly associated with CHFDS score (B=0.61; t=7.90; p<0.001), explaining 36% of HAQ variance. CONCLUSIONS: dSSc patients present higher global and local disability, and lower HRQoL in SPI than lSSc patients. Local disabilities, assessed by CHDFS and MHISS, are independently related to global disability and HRQoL.


Asunto(s)
Dermatosis Facial/fisiopatología , Dermatosis de la Mano/fisiopatología , Calidad de Vida , Esclerodermia Difusa/fisiopatología , Esclerodermia Limitada/fisiopatología , Actividades Cotidianas , Anciano , Personas con Discapacidad , Dermatosis Facial/psicología , Femenino , Dermatosis de la Mano/psicología , Humanos , Masculino , Persona de Mediana Edad , Esclerodermia Difusa/psicología , Esclerodermia Limitada/psicología , Esclerodermia Sistémica/fisiopatología , Esclerodermia Sistémica/psicología , Encuestas y Cuestionarios
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