ABSTRACT Objective Lupus nephritis is one of the most severe complications of systemic lupus erythematosus and it has been estimated that can occur in up to 60% of patients. Direct costs of lupus nephritis have not been studied in developing countries. This study aimed to describe lupus nephritis direct costs in Colombia. Methods Administrative data from two Colombian health maintenance organizations for 2014 and 2015 was obtained. An algorithm based on the International Statistical Classification of Diseases and Related Health Problems 10th revision codes was developed to identify patients with lupus nephritis and lupus nephritis under study. Results The average annual per-patient, all-claims, all-cause direct cost for lupus nephritis was US$ 12,624, 7.5 times higher than the average lupus patient without lupus nephritis. For lupus nephritis cases under study, estimated direct cost was US$ 3,664, 2 times higher than average lupus patient in Colombia. Difference in lupus nephritis patients is mainly accounted for the cost and frequency of procedures, exceeding by a factor of 5 the cost for durable medical equipment and the cost for drugs, respectively. Conclusion Lupus patients who progress to lupus nephritis stage increased seven-fold the average annual per-patient, all-claims, and all-cause direct cost for the Colombian health system.

Resumen Descripción del caso: Paciente de cinco años de sexo femenino con telangiectasia hemorrágica hereditaria. Hallazgos Clínicos: Deterioro de la función cardiopulmonar con mayores requerimientos de oxígeno secundario a shunt pulmonar arteriovenoso, epistaxis. Tratamiento y resultado: La paciente fue tratada con el anticuerpo monoclonal bevacizumab, que inhibe el factor de crecimiento endotelial vascular, con buen resultado clínico. Relevancia clínica: La telangiectasia hemorrágica hereditaria es un trastorno autosómico dominante caracterizado por malformaciones arteriovenosas en diferentes órganos, lo que hace que sus presentaciones clínicas varíen. Las opciones terapéuticas sistémicas para la enfermedad generalizada son limitadas. El anticuerpo monoclonal bevacizumab, parece ser una buena opción en este trastorno. Aunque se ha reportado como exitoso en la población adulta, su uso en población pediátrica aún no ha sido reportado. Aquí se informa el uso de bevacizumab en una paciente de 5 años de edad con telangiectasia hemorrágica hereditaria, mostrando beneficios clínicos y buen resultado.

Abstract Case description: Five-year-old female patient with hereditary hemorrhagic telangiectasia. Clinical Findings: Deterioration of cardiopulmonary function with higher oxygen requirements secondary to pulmonary arteriovenous shunts, epistaxis. Treatment and Outcome: The patient was treated with the monoclonal antibody bevacizumab, which inhibits the vascular endothelial growth factor, with good clinical outcome. Clinical Relevance: Hereditary hemorrhagic telangiectasia is an autosomal dominant disorder characterized by arteriovenous malformations in different organs, making its clinical presentations varied. Systemic therapeutic options for a generalized disease are limited. The monoclonal antibody bevacizumab, seems to be a good option in this disorder. Although reported as successful in adult population, its use in pediatric population has not yet been reported. Here we report the use of bevacizumab in a 5-year-old female patient with hereditary hemorrhagic telangiectasia, showing clinical benefits and good outcome.

Los tumores primarios del corazón son raros; 75% de éstos son benignos y cerca de la mitad de los benignos son mixomas que, en la mayoría de los casos, se encuentran en las cavidades izquierdas. Las manifestaciones clínicas de los mixomas dependen del sitio de localización del tumor. Sin embargo, se afirma que la isquemia cerebral es la manifestación clínica inicial en un tercio de los mixomas atriales. Se presenta el caso de un paciente de género masculino, de 65 años de edad, en quien la primera manifestación clínica de mixoma atrial fue un evento cerebral vascular isquémico.

Primary heart tumors are rare; 75% of them are benign and almost half of the benign ones are mixomas that in most cases are located in the left cavities. Clinical manifestations of myxomas depend on its localization site. Nevertheless, it is accepted that brain ischemia is the initial clinical manifestation in a third of atrial myxomas. The case of a 65 years old male patient in whom the first clinical manifestation of an atrial myxoma was an ischemic cerebrovascular event, is presented.

En el presente estudio se examinó la especificidad y la sensibilidad de los anticuerpos antipéptidos citrulinados cíclicos (CCP) en pacientes latinoamericanas con artritis reumatoidea (AR), así como su relación con la actividad de la enfermedad, manifestaciones extraarticulares (MEA), síntesis de citocinas (IL-4, IL-10, IL-12, TNF-<FONT FACE=Symbol>µ</FONT> e IFN-gamma ) y factor reumatoideo (FR) IgM e IgA, y con el polimorfismo del HLA-DRB1. Se examinaron 79 pacientes con AR (69 con AR establecida y 10 con AR temprana sin previo tratamiento), 56 pacientes con espondilitis anquilosante (EA), 25 con lupus eritematoso sistémico (LES), 50 con síndrome de Sjögren primario (SSp) y diez individuos sanos. De las 69 pacientes con AR establecida, 36 fueron reevaluadas 2 años después. La actividad de la AR se examinó según los criterios del Colegio Americano de Reumatología. Los anticuerpos anti-CCP2, el FR y los niveles de citocinas se determinaron mediante inmunoensayo, y la genotipificación del HLA se llevó a cabo por reacción en cadena de la polimerasa utilizando mezclas de iniciadores específicos. Los anticuerpos anti-CCP se observaron en 96% de los pacientes con AR en la primera evaluación y en 86% en la segunda ( p=0,12), sin modificación significativa en los valores (131±58,7 vs. 130,6±67,1 UI). Su sensibilidad y especificidad global fue de 94% y 92%, respectivamente, pero cuando sólo se consideraron los niveles altos (>60 UI) fueron de 84% y 95%, respectivamente. La razón de probabilidades (RP) positiva fue de 12 y la RP negativa de 0,06. El valor predictivo (VP) positivo fue de 87% y el VP negativo de 96%. Los anticuerpos anti-CCP se observaron en 12% de los pacientes con LES y con SSp, en 2% de los de EA y en 10% de los controles sanos. En los pacientes con AR no se asociaron con la actividad de la enfermedad, MEA y alelos del HLA-DRB1. Tampoco se observaron correlaciones significativas entre sus valores y los niveles de citocinas. En conclusión, los anticuerpos anti-CCP tienen un interés diagnóstico para la AR en nuestra población, pero su utilidad en el seguimiento clínico es limitada y su síntesis es independiente del HLA-DRB1 y no se correlacionan con niveles de citocinas Th1/Th2.

The specificity and sensitivity of anti-cyclic citrullinated peptide antibodies (anti-CCP) was examined in Latin-American patients with rheumatoid arthritis (RA). The variables considered included: 1) relation with the activity of disease, 2) extra-articular manifestations (EAM), 3) synthesis of cytokines (IL-4, IL-10, IL-12, TNF-alpha , and IFN-gamma ) and IgM and IgA rheumatoid factor (RF), and 4) the association with HLA-DRB1 polymorphism. Seventy-nine RA patients were assessed (69 with established RA, and 10 with recent-onset RA not receiving any treatment), 56 with ankylosing spondylitis (AS), 25 with systemic lupus erythematosus (SLE), 50 with primary Sjögren’s syndrome (pSS), and 10 healthy individuals. Of the 69 patients with established RA, 36 were reexamined 2 years later. The activity of the RA was measured by criteria adopted by the American College of Rheumatology. Anti-CCP2, RF and cytokines levels were determined by ELISA. HLA genotypes were established by first, PCR sequence amplification using sequence-specific primers and then, complete sequencing of the product. Anti-CCP antibodies were observed in 96% of patients with RA during the first evaluation and in 86% at the second evaluation ( p=0.12). No significant change in antibody titre was observed between the two evaluations (131±58.7 and 130.6±67.1 IU, respectively). The overall sensitivity and specificity was 94% and 92%, respectively; however, at titres >60 IU, the values were 84% and 95%, respectively. The anti-CCP likelihood ratio positive test was 12 and the likelihood ratio negative test was 0.06. The positive predictive value was 87%, and the negative predictive value was 96%. Anti-CCP antibodies were observed in 12% of SLE and pSS patients, in 2% of AS patients, and in 10% of healthy controls. In RA patients, these antibodies were not associated with the activity of disease, EAM or HLA-DRB1 alleles; no significant correlation was observed between antibody titre and cytokines level. Although anti-CCP antibodies have potential as a diagnostic tool for RA, they are not useful for monitoring clinical activity or predicting the clinical course of disease. Antibody synthesis is HLA-DRB1 independent and not correlated with Th1/Th2 cytokines.