Validación al idioma español de Venezuela de los índices de actividad de enfermedad y el índice funcional para espondilitis anquilosante / Validity of a Venezuelan version of the bath ankylosing spondylitis functional index and bath ankylosing spondylitis disease activity index

Introducción: Las espondiloartritis (EsA) son enfermedades discapacitantes, con una prevalencia del 1,9% en la población general; los índices diseñados para su seguimiento deben ser válidos, confiables y adaptados transculturalmente para decidir el tratamiento y la vigilancia adecuada. El cambiar un adjetivo o un pronombre en un cuestionario autoadministrado puede ser la gran diferencia cuando se quiere investigar el estado actual de la enfermedad. Objetivos: Desarrollar una versión venezolana de la versión original en inglés de BASDAI/BASFI, para evaluar su confiabilidad y validez en pacientes venezolanos con EsA. Métodos: Se necesitaron lingüistas certificados para la traducción de una versión venezolana de BASDAI/BASFI. La evaluación de la confiabilidad y la validez se realizó mediante el cálculo de coeficientes de correlación, además de la correlación α de Cronbach, entre la puntuación de BASDAI y los parámetros clínicos (p. ej., ESR, CRP, índice de prueba de Schöber modificado, distancia occipucio-pared y recuento de entesis). Resultados: Cuarenta pacientes, 31 hombres (77,5%) y 9 mujeres (22,5%), con una edad promedio ± desviación estándar 35,9 ± 12,0 años. Duración de la enfermedad fue de 11,5 ± 9,5 años. El 45% presentaba EsA indiferenciada, el 27,5% espondilitis anquilosante y el 20,0% artritis psoriásica. La artritis reactiva, la espondilitis anquilosante juvenil y la artritis enteropática representaron el 2,5% cada una. La confiabilidad test-retest del BASDAI y del BASFI fueron ambas de 0,99 (p < 0,0001). La consistencia interna para el BASDAI fue de 0,88 (p = 0,002), coeficiente de correlación intraclase para consistencia interna: 0,9867 (p = 0,001). Consistencia interna para el BASFI: α de Cronbach: 0,7985 (p = 0,002), coeficiente de correlación intraclase para consistencia interna: 0,9055 (p = 0,001). La validez de constructo del BASDAI fue de 0,84 para estado de bienestar general del paciente y 0,84 para el recuento de entesis (p < 0,0001). Conclusión: La versión venezolana del BASDAI/BASFI podría utilizarse en la investigación clínica para evaluar el curso de la actividad de la enfermedad en pacientes venezolanos con EsA

Introduction: Spondyloarthropathies (SpA) are disabling diseases with a prevalence of 1.9% in the general population. The indices designed for monitoring the disease should be valid, reliable and cross-culturally adapted for decision-making concerning the appropriate treatment. Changing an adjective or pronoun in a self-administered questionnaire could be the big difference in condensing an idea in a few words and transmitting that concept to all those who share the same language. Objectives: To develop a Venezuelan version of the original English version of the BASDAI/BASFI and to evaluate its reliability and validity in Venezuelan patients with SpA. Methods: Certified linguists were needed for the translation of a Venezuelan version of the BASDAI/BASFI. The evaluation of reliability and validity was performed by calculating correlation coefficients in addition to Cronbach's alpha correlation between the BASDAI score and the clinical parameters (for example: erythrocyte sedimentation rate, C-reactive protein, modified Schöber test, occiput-to-wall distance and enthesis count). Results: We studied 40 patients including 31 men (77.5%) and 9 women (22.5%). The mean age was 35.9 years ± standard deviation (SD) 12.01 and the disease duration was 11.5 years (± SD 9.5). The most common diagnoses were undifferentiated spondyloarthritis (45%), ankylosing spondylitis (27.5%) and psoriatic arthritis (20%). The incidences of reactive arthritis, ankylosing spondylitis and juvenile Reiter's syndrome were 2.5% each. The test-retest reliability of the BASDAI and BASFI was high (R = 0.99 and 0.99, respectively; P<.0001). The internal consistency for the BASDAI was high (Cronbach's alpha = 0.88; P=.002) and the intraclass correlation coefficient for internal consistency: 0.9867 (P=.001). Internal consistency for the BASFI: Cronbach's alpha = 0.7985 (P=.002), intraclass correlation coefficient for internal consistency: 0.9055 (P=.001). Construct validity of the BASDAI was high for general well-being of the patient (R = 0.84) and for enthesis count (R = 0.84). Low back pain showed moderate correlation with BASDAI (R = 0.69; P<.0001) and the erythrocyte sedimentation rate showed a low correlation (R = 0.39683; P=.0112). Conclusion: The Venezuelan version of the BASDAI/BASFI could be used in clinical research to assess and evaluate the course of disease activity in Venezuelan SpA patients

Validity of a Venezuelan version of the Bath Ankylosing Spondylitis Functional Index and Bath Ankylosing Spondylitis Disease Activity Index. / Validación al idioma español de Venezuela de los índices de actividad de enfermedad y el índice funcional para espondilitis anquilosante.

INTRODUCTION: Spondyloarthropathies (SpA) are disabling diseases with a prevalence of 1.9% in the general population. The indices designed for monitoring the disease should be valid, reliable and cross-culturally adapted for decision-making concerning the appropriate treatment. Changing an adjective or pronoun in a self-administered questionnaire could be the big difference in condensing an idea in a few words and transmitting that concept to all those who share the same language. OBJECTIVES: To develop a Venezuelan version of the original English version of the BASDAI/BASFI and to evaluate its reliability and validity in Venezuelan patients with SpA. METHODS: Certified linguists were needed for the translation of a Venezuelan version of the BASDAI/BASFI. The evaluation of reliability and validity was performed by calculating correlation coefficients in addition to Cronbach's alpha correlation between the BASDAI score and the clinical parameters (for example: erythrocyte sedimentation rate, C-reactive protein, modified Schöber test, occiput-to-wall distance and enthesis count). RESULTS: We studied 40 patients including 31 men (77.5%) and 9 women (22.5%). The mean age was 35.9 years ± standard deviation (SD) 12.01 and the disease duration was 11.5 years (± SD 9.5). The most common diagnoses were undifferentiated spondyloarthritis (45%), ankylosing spondylitis (27.5%) and psoriatic arthritis (20%). The incidences of reactive arthritis, ankylosing spondylitis and juvenile Reiter's syndrome were 2.5% each. The test-retest reliability of the BASDAI and BASFI was high (R = 0.99 and 0.99, respectively; P<.0001). The internal consistency for the BASDAI was high (Cronbach's alpha = 0.88; P=.002) and the intraclass correlation coefficient for internal consistency: 0.9867 (P=.001). Internal consistency for the BASFI: Cronbach's alpha = 0.7985 (P=.002), intraclass correlation coefficient for internal consistency: 0.9055 (P=.001). Construct validity of the BASDAI was high for general well-being of the patient (R = 0.84) and for enthesis count (R = 0.84). Low back pain showed moderate correlation with BASDAI (R = 0.69; P<.0001) and the erythrocyte sedimentation rate showed a low correlation (R = 0.39683; P=.0112). CONCLUSION: The Venezuelan version of the BASDAI/BASFI could be used in clinical research to assess and evaluate the course of disease activity in Venezuelan SpA patients.

Epicardial fat thickness as cardiovascular risk factor and therapeutic target in patients with rheumatoid arthritis treated with biological and nonbiological therapies.

Rheumatoid arthritis (RA) is a chronic inflammatory disease associated with high cardiovascular morbidity and mortality. Epicardial adipose tissue (EAT) thickness may act as a therapeutic target during treatments with drugs modulating the adipose tissue. We evaluate EAT thickness in RA patients treated with biological and nonbiological disease-modifying antirheumatic drugs (DMARDs). A cross-sectional study was conducted with a cohort of 34 female RA patients and 16 controls matched for age and body mass index (BMI). Plasma glucose, basal insulin, plasma lipids, and high-sensitivity C-reactive protein (hs-CRP) were assessed. EAT thickness and left ventricular mass (LVM) were measured by echocardiography. No significant differences in waist circumference (WC), blood pressure, fasting blood glucose, basal insulin, and lipid parameters were found between the groups. The control group showed lower concentrations (P = 0.033) of hs-CRP and LVM (P = 0.0001) than those of the two RA groups. Patients treated with TNF-α inhibitors showed significantly lower EAT thickness than those treated with nonbiological DMARDs (8.56 ± 1.90 mm versus 9.71 ± 1.45 mm; P = 0.04). Women with no RA revealed reduced EAT thickness (5.39 ± 1.52 mm) as compared to all RA patients (P = 0.001). Results suggest that RA patients have greater EAT thickness than controls regardless of BMI and WC.

Clinical and magnetic resonance imaging manifestations of neurosarcoidosis.

OBJECTIVES: To describe clinical and neuroimaging manifestations of neurosarcoidosis in a cohort of 21 patients. PATIENTS AND METHODS: We reviewed records of 21 patients with sarcoidosis and central nervous system (CNS) manifestations referred to Cooper University Hospital, with emphasis on neuroimaging findings and associated clinical and laboratory evidence of sarcoidosis. Nineteen patients were categorized as having "definite," "probable," or "possible" neurosarcoidosis, while 1 had associated CNS vasculitis and another had Hodgkins lymphoma with cauda equina syndrome. RESULTS: The most common manifestations included myelopathy, cranial neuropathies, and encephalopathy. In 6 patients, CNS biopsy showed sterile, noncaseating granuloma (NCG), while in the remainder, the diagnosis was established through a combination of clinical, radiographic, and laboratory findings. Notably, 10 patients developed acute neurological emergencies, including seizures, spinal cord compression, and increased intracranial pressure. Findings on magnetic resonance imaging (MRI) included a variety of manifestations, including isolated mass lesion, diffuse intraparenchymal inflammatory lesions in the brain and spinal cord, leptomeningeal enhancement, hydrocephalus, and intracranial hemorrhage. CONCLUSIONS: Sarcoidosis is associated with diverse neurological manifestations and neuroimaging findings. The diagnosis of isolated CNS sarcoidosis requires a biopsy to document the presence of sterile NCG and to exclude neoplasms and other granulomatous diseases. When a biopsy of the CNS is not possible, a diagnosis of neurosarcoidosis can reasonably be supported in many patients by MRI findings and exclusion of other disorders. RELEVANCE: Optimum management of patients with neurosarcoidosis relies on the ability of clinicians to recognize the broad spectrum of clinical and neuroimaging manifestations of the disorder.

Effect of knee osteoarthritis on the perception of quality of life in Venezuelan patients.

OBJECTIVE: To measure the perception of quality of life in Venezuelan patients with knee osteoarthritis and to identify those variables that may influence it. METHODS: A multicenter, cross-sectional study of 126 mestizo patients with knee osteoarthritis recruited from 8 rheumatology centers in Venezuela. We used a Spanish-translated version of the Arthritis Impact Measurement Scales (AIMS), as adapted in Venezuela. One-way analysis of variance was used to compare the AIMS mean total score among subgroups of knee pain, anatomic stage, and socioeconomic status (SES); a post-hoc test was performed to identify significant intragroup differences. Pearson's correlation coefficient was used to examine correlations between age, body mass index (BMI), disease duration, knee pain, and AIMS score. Associations between radiologic stage, SES, and AIMS scores were examined using Spearman's rank correlation. Multiple regression analysis was used to estimate predictor factors of AIMS scores. RESULTS: A significant correlation was found between total AIMS scores and knee pain, age, and socioeconomic status, but not with BMI, disease duration, or anatomic stage. Patients with severe knee pain differed from those with mild and moderate pain, and the highest AIMS mean total score was seen in patients within the severe knee pain subset. Patients in the highest socioeconomic levels differed from those within lowest categories. Patients classified as being at the levels of relative and critical poverty showed the highest AIMS scores. Multiple regression analysis showed that knee pain was the only variable that exerted an independent effect on the quality of life in our patients. CONCLUSION: The perception of quality of life is negatively affected by increasing levels of joint pain, old age, and low socioeconomic status in Venezuelan patients with knee osteoarthritis. Our study supports the need for an early and vigorous approach to treat pain in this group of patients.

Rheumatoid nodulosis: is it a different subset of rheumatoid arthritis?

Rheumatoid nodulosis is an entity that describes a particular variant of polyarthritis associated with early manifestations of palindromic rheumatism, radiologic subchondral bone cysts, and subcutaneous rheumatoid nodules. This study describes the clinical, radiologic, histologic, crystallographic, and laboratory findings, as well as the outcome in a group of 16 patients with rheumatoid nodulosis that were followed for a period of 1-12 years. Six of these patients had an aggressive course and developed classic erosive polyarticular rheumatoid arthritis, while the others continued having episodic arthritis without erosive disease. Seven patients had cholesterol crystals in olecranon bursae containing nodules. Second-line drugs used to control the articular manifestations did not improve the nodulosis, erosive, or cystic subchondral bone changes. Rheumatoid nodulosis mimics several other rheumatic diseases, and in about 40%, classic erosive rheumatoid arthritis develops. The presence of cholesterol crystals in rheumatoid nodules or affected bursae can increase the confusion with other crystal-induced arthritis, in particular, tophaceous monosodium urate gout or xanthomatosis.

Oxalate crystal deposition disease.

In addition to monosodium urate, calcium pyrophosphate dihydrate, and apatite crystals, oxalate crystals are less often found in synovial fluids in association with acute or chronic arthritis. Oxalate crystal deposition disease is seen in patients with primary hyperoxaluria types 1 and 2 (PH1 and 2) and in patients with end-stage renal disease managed with long-term dialysis. Oxalate crystal deposits are found mainly in kidneys, bone, skin, and vessels, and less often inside the joints. Musculoskeletal and systemic manifestations of oxalate crystal deposition disease may be confused with those observed with the other most common types of crystal deposition diseases. Clinical and radiographic features include calcium oxalate osteopathy, acute and chronic arthropathy with chondrocalcinosis, synovial calcification, and miliary skin calcium oxalate deposits and vascular calcifications that affect mainly the hands and feet. Systemic life-threatening cardiovascular, neurologic, and hematologic manifestations are rare. Genomic DNA studies have identified those genetic defects of PH1 and PH2 that allow a precise early diagnosis. Kidney transplantation has poor outcome as a result of graft oxalosis. Combined liver and kidney transplantation is the treatment of choice in patients with PH1 and advanced renal failure. Pre-emptive isolated liver transplantation is the preferred treatment in patients who develop the disease during infancy with progressive manifestations of oxalosis. These novel findings in the understanding of the molecular and enzymatic aspects of primary hyperoxalurias have provided a more rational basis for the management and prevention of oxalate crystal deposition disease. This information may lead to a better understanding and effective management of other common calcium-containing crystal deposition diseases.

Pathologic fracture of the femoral neck in a female soccer player.

We report a case of a healthy, young, female soccer player who developed progressive pain in her right hip. A bone cystic lesion was found in the right femoral neck and proximal femur. The lesion was considered a benign bone cyst and the patient was treated with injections of autologous bone marrow and grafting into the femoral neck. However, the cystic lesion did not heal. Subsequently, the patient fell and developed a fracture of the femoral neck that required internal fixation. The bone biopsy showed characteristic histologic features of fibrous dysplasia. A bone scan showed other areas of suspected dysplasia. Pamidronate therapy was given, and a reduction of the increased uptake was seen on bone scans. Fibrous dysplasia must be considered in the differential diagnosis of any cystic bone lesion.

Anticuerpos antifosfolípidos en malaria / Antibody antiphopholipids in malaria

Los anticuerpos antifosfolípidos (AFL) son anticuerpos que se unen a los fosfolípidos con carga negativa, y se han asociado a procesos trombóticos en enfermedades inmunes como el lupos eritematoso sistématico, pero también se han reportado elevaciones transitorias en procesos infecciosos agudos, entre éstos la malaria complicada. En este estudio se escogieron 29 pacientes con malaria dividiéndose en dos grupos: "A" malaria complicada y grupo "B" malaria no complicada. Se deterninó la presencia de AFL, a través del VDRL, el anticoagulante lúpico y los anticuerpos anticardiolipinas (ACL) tipo IgG e IgM, además se realizaron pruebas generales de laboratorio, perfil de coagulación, Rx de tórax y gasometría arterial. El sexo predominante en el grupo "B" fue el masculino (69 por ciento), no existiendo diferencia en el grupo "A". El grupo etario predominante en ambas series fue el encontrado en el rango de 20-40 años (69,85 por ciento). El plasmodium falciparum fue la especie principalmente reportada (63,6-92,3 por ciento). La anemia se observó más en los pacientes del "A" con diferencia significativa en los valores de Hb y Hcto para ambos grupos (p<0,002 y p<0,001). El contaje leucocitario fue menor en el "A" (p<0,05), el tiempo de protombina alargado en el "A" (p<0.06). El contaje plaquetario, fibrinógeno, PTT, TT no mostraron diferencia significativa entre ambos grupos. El VDRL y el anticuagulante lúpico fueron negativos para todos. Los títulos de ACL-IgG y ACL-IgM fueron mayores en el grupo "A" (p<0,001) y (p<0,0001). Se observa que los títulos de ACL en especial el isotipo IgM se elevan en los pacientes con malaria complicada, desconociéndose sus implicaciones patológicas o si por el contrario ejercen un efecto protector al bloquear los exoantígenos liberados en la ruptura del esquizonte y así impedir la liberación de citocinas (factor de necrosis tumoral) involucradas en patogenia de la enfermedad