Assessment of inflammatory activity in Takayasu's arteritis: performance of clinical scores and common biomarkers versus 18F-FDG PET/CT.

OBJECTIVES: There is no consensus on how to evaluate inflammatory activity in Takayasu's arteritis (TAK). Here we compare biochemical tests and three clinical scores, which evaluate inflammatory activity (IA) in TAK, versus quantitative 18F-FDG PET/CT as the gold standard. METHODS: This prospective study included patients with TA diagnosed according to the American College of Rheumatology (ACR) criteria. IA was assessed through laboratory tests, clinical scores of the National Institute of Health (NIH), Dabague-Reyes (DR) and the Indian Takayasu Clinical Activity Score 2010 (ITAS2010), and the result of these assessments was compared against 18F-FDG PET/CT Standardised Uptake Values (SUVmax). RESULTS: A total of 35 patients were studied, 86% were women. SUVmax had positive correlations with acute phase reactants and DR and NIH. Agreement of 18F-FDG PET/CT was significant with erythrocyte sedimentation rate (ESR) and DR score. Receiver Operating Characteristic (ROC) curve analysis showed diagnostic value for inflammatory activity in ESR, DR and NIH scores, which had higher specificity when they were estimated with new cut-off points for the Mexican population. CONCLUSIONS: ESR and other phase reactants have good sensitivity but low specificity to evaluate IA in TAK when compared against 18F-FDG PET/CT. Among all the clinical scores, DR had the best diagnostic value, with strong potential as a clinical tool to define the inflammatory status in TAK patients when the study image is not available. However, in complex TAK cases with doubtful diagnosis after assessment by clinical scores or laboratory, 18F-FDG PET/CT remains mandatory.

Vasculitis reumatoidea como primera manifestación de artritis reumatoide / Rheumatoid vasculitis as first manifestation of rheumatoid arthritis

Resumen La vasculitis reumatoidea es la complicación extraarticular más grave de la artritis reumatoide, con morbilidad y mortalidad altas. Se trata de un proceso inflamatorio que afecta principalmente los vasos sanguíneos de pequeño y mediano calibre, sus manifestaciones clínicas sobrevienen de forma heterogénea, la vasculitis cutánea y la neuropatía son las más comunes. Su incidencia ha disminuido en las últimas décadas debido a la administración temprana de fármacos modificadores de la enfermedad en la artritis reumatoide. A pesar de la administración de ciclofosfamida y la existencia de medicamentos biológicos, continúa siendo un reto lograr el control de la enfermedad. Comunicamos el caso de un paciente de 50 años de edad, sin antecedentes médicos de importancia, salvo tabaquismo, que inició con neuropatía secundaria a vasculitis con factor reumatoide y anticuerpos antipéptido cíclico citrulinado positivos. La revisión bibliográfica pone al día los conocimientos acerca de la enfermedad para considerarla diagnóstico diferencial.

Abstract The rheumatoid vasculitis is the most serious complication of rheumatoid arthritis, with high morbidity and mortality. It is an inflammatory process that affects small and medium vessels and that has heterogeneous manifestations, being the cutaneous lesions and neuropathy the most common. Its incidence has declined in the last decades because of the early use of disease modifying antirheumatic drugs. Despite the use of cyclophosphamide and the existence of biologic drugs, achieving the control of the disease continues to be a challenge. We present the case of a 50 year-old man, without relevant antecedents, only tabaco use, that presented with a vasculitic neuropathy, rheumatoid factor and cyclic citrullinated peptide antibody positive. This bibliographic review has the intention to update the knowledge of this entity and to be considered a differential diagnosis.

Enfermedad de Kikuchi-Fujimoto, manifestación junto con siliconosis / Kikuchi-Fujimoto disease in presentation with silicosis

Resumen La enfermedad de Kikuchi-Fujimoto, también llamada linfadenitis necrotizante histiocítica, es un padecimiento raro y benigno de causa desconocida; se distingue por linfadenopatía cervical y fiebre frecuentemente en mujeres jóvenes previamente sanas. Las manifestaciones clínicas, el antecedente de lupus eritemaso sistémico y el análisis histopatológico sugieren una respuesta inmunitaria celular mediada por células T e histocitos como parte de la fisiopatología. El diagnóstico de enfermedad de Kikuchi-Fujimoto requiere biopsia por escisión de los ganglios linfáticos afectados en los que se evidencia necrosis con infiltrados de histiocitos y característicamente ausencia de neutrófilos. No existe tratamiento efectivo contra la enfermedad de Kikuchi-Fujimoto, el cuadro se alivia de manera espontánea en uno a cuatro meses; ante síntomas persistentes y severos se administran glucocorticoides y antiinflamatorios no esteroides. La siliconosis es una condición en la que el silicón, componente de implantes mamarios, actúa como coadyuvante inmunogénico provocando una respuesta local y sistémica autoinmunitaria con síntomas inespecíficos que constituyen el síndrome autoinmunitario inducido por coadyuvantes. El tratamiento de este síndrome se basa en la eliminación del estímulo externo y en la mayoría de los casos se observa una respuesta favorable a largo plazo sin necesidad de iniciar tratamiento inmunomodulador. Se necesitan más casos para poder establecer a la siliconosis como una probable causa de enfermedad de Kikuchi-Fujimoto y conocer más a fondo la relación entre ambos padecimientos.

Abstract Kikuchi-Fujimoto disease, also called histiocytic necrotizing lympha-denitis, is a rare and benign condition of unknown etiology, characte-rized by cervical lymphadenopathy with fever that frequently occurs in previously young healthy women. The clinical manifestations, a history of systemic lupus erythematous and histopathologic analysis suggest an immune response of T cells and histiocytes as a part of the pathophysiology. The diagnosis of Kikuchi disease requires lymph node biopsy, in which there is evidence of necrosis with histiocyte infiltrates and characteristically absence of neutrophils. No effective treatment exists for Kikuchi disease, it is self-limited into 1-4 weeks; with severe and persistent symptoms, glucocorticoids and nonsteroidal anti-inflammatory drugs are prescribed. Silicosis is a condition in which silicone, a component of breast implants, acts as an immunogenic adjuvant, causing a local and systemic autoimmune response with non-specific symptomatology constituting the adjuvant-induced autoimmune syndrome (ASIA). The treatment of ASIA is based on the elimination of the external stimulus and in most cases a favorable long-term response is observed without initiating immunomodulatory treatment. More cases are needed in order to establish silicosis as a cause of Kikuchi-Fujimoto disease and to know more about the relationship between these conditions.

[Relation between autoimmune thyroid diseases and connective tissue diseases]. / Relación entre las enfermedades tiroideas autoinmunes y las del tejido conectivo.

The main physiological function of the immune system consists in the defense against infectious micro-organisms. Sometimes there is a loss of immunological tolerance with the consequence of ignorance of self-antibodies. Some thyroid diseases are related to autoimmune diseases associated with the most common exocrine glands between them. There are also the autoimmune thyroid organ specific diseases, such as Graves-Basedow and the Hashimoto thyroiditis. It has been shown that there is a higher prevalence of autoimmune thyroid diseases in patients with connective tissue diseases (systemic autoimmune) such as Sjögren syndrome, rheumatoid arthritis, systemic lupus erithmatosis and systemic myopathic diseases. In the same way a higher prevalence of antinuclear antibodies against antigens extracted from the nucleus in patients with a thyroid autoimmune disease has been identified. There is a high percentage of patients with subclinical thyroid diseases, and it is recommended for patients with connective tissue diseases with hypo- or hyperthyroidism to have thyroid globulin and peroxide antibodies measured.

Si bien la función fisiológica del sistema inmunitario consiste en la defensa contra los microorganismos infecciosos, en ocasiones hay pérdida de la autotolerancia, que deriva en el desconocimiento de los autoantígenos. Las tiroidopatías son las enfermedades autoinmunes más comunes relacionadas con las glándulas exocrinas, a su vez las tiroideas autoinmunes órgano-específicas más frecuentes son la enfermedad de Graves-Basedow y la tiroiditis de Hashimoto. Se ha observado que estas tienen una mayor prevalencia en pacientes con enfermedades del tejido conectivo (autoinmunes sistémicas) tales como el síndrome de Sjögren, la artritis reumatoide, el lupus eritematoso sistémico y las miopatías sistémicas. De igual forma, se ha identificado una mayor prevalencia de anticuerpos antinucleares y de anticuerpos contra antígenos extraídos del núcleo en pacientes con alguna enfermedad tiroidea autoinmune. Dado el alto porcentaje de pacientes con enfermedades tiroideas subclínicas, es recomendable que en los pacientes con enfermedades del tejido conectivo y datos de hipo o hipertiroidismo se solicite anticuerpos antitiroglobulina y antiperoxidasa.