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Introduction Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by recurrent thrombosis that can affect the arterial and venous circulation. Objectives To analyze the immunological and pharmacological differences, as well as the clinical outcomes of a cohort of patients with primary antiphospholipid syndrome and secondary antiphospholipid syndrome. Materials and methods A retrospective cohort study was conducted that included 352 records of patients diagnosed with APS and treated between 2014 and 2018. A description is presented of the sociodemographic, clinical, and immunological profile of the population. A bivariate analysis performed using the chi-squared test to determine differences between groups with primary APS and secondary APS, and finally a multivariate analysis to search for associations with thrombotic clinical outcomes in patients with APS. Results The mean age was 42.4 ± 14 years, and 84.6% were females. Two-thirds (67.6%) of the patients had a diagnosis of primary APS, and 32.4% of secondary APS, of which 84% were associated with systemic lupus erythematosus (SLE). Among the thrombotic events, the most frequent were deep vein thrombosis (17.3%) and stroke (9.9%). Obstetric events were frequent, with a prevalence of 39.4% for miscarriages. No differences were found in the sociodemographic or immunoserological profile when comparing the group of primary vs. secondary APS. Thrombotic events were more frequent in the primary APS group, although only pulmonary embolism reached statistical significance. There were no differences between the two groups as regards obstetric events, such as miscarriages. Women were found to be 5 times more likely to have a stroke and 3 times more to have deep vein thrombosis. The anti-B2GPI type IgM increased the probability of presenting miscarriages about 3 times in women with APS. Conclusion The study contains one of the largest Colombian cohorts with APS reported so far, and although it is both clinically and sociodemographically similar to other cohorts, there is a higher prevalence of primary APS. There was a lower frequency of thrombotic complications compared to other cohorts. Patients with primary APS had a tendency to develop thrombosis, as has already been reported in the literature.

RESUMEN Introducción: El síndrome antifosfolípido (SAF) es una enfermedad autoinmune sistémica, caracterizada por trombosis recurrente, que puede afectar la circulación arterial y venosa. Objetivo: Analizar las diferencias inmunológicas y farmacológicas, así como los desenlaces clínicos de una cohorte de pacientes con SAF primario y secundario. Materiales y métodos: Estudio de corte transversal que incluyó 352 pacientes con diagnóstico de SAF atendidos entre los arios 2014 y 2018. Se analizaron variables sociodemográficas, clínicas e inmunológicas y se realizó un análisis univariado y un análisis bivariado mediante la prueba chi-cuadrado para determinar diferencias entre los pacientes con SAF primario y SAF secundario. Finalmente, se hizo un análisis multivariado para buscar asociaciones con los desenlaces clínicos trombóticos en los pacientes con SAF. Resultados: La edad promedio de la población fue de 42,4 ± 14 años; el 84,6% correspondió a sexo femenino. El 67,6% de los pacientes tenía diagnóstico de SAF primario y un 32,4% de SAF secundario, siendo el lupus eritematoso sistémico (LES) la enfermedad asociada en un 84%. Dentro de los eventos trombóticos, el más frecuente fue la trombosis venosa profunda (17,3%), seguida por el ataque cerebrovascular (9,9%). En los eventos obstétricos existió una prevalencia del 39,4% para abortos. No se encontraron diferencias en el perfil sociodemográfico ni en el perfil inmunoserológico entre los pacientes con diagnóstico de SAF primario y aquellos con SAF secundario. Los eventos trombóticos tuvieron mayor frecuencia en el grupo de SAF primario, pero solo la tromboembolia pulmonar alcanzó significación estadística. Eventos obstétricos como los abortos no fueron diferentes entre ambos grupos. Dentro de los factores asociados a los eventos trombóticos, se encontró que el sexo femenino tiene una probabilidad 5 veces mayor de accidente cerebrovascular y 3 veces mayor de trombosis venosa profunda. Los anti- β2GPI tipo IgM aumentaron alrededor de 3 veces la probabilidad de presentar abortos en mujeres con SAF. Conclusión: Se presenta una de las cohortes colombianas más grandes de pacientes con SAF reportadas hasta el momento en la literatura. La población es comparable clínica y sociodemográficamente con lo encontrado en otros estudios, aunque la prevalencia de SAF primario fue mayor y las complicaciones trombóticas fueron menores. La tromboembolia pulmonar fue significativamente mayor en el grupo de SAF primario. ABSTRACT Introduction: Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by recurrent thrombosis that can affect the arterial and venous circulation. Objective: To analyze the immunological and pharmacological differences, as well as the clinical outcomes of a cohort of patients with primary APS and secondary APS. Materials and methods: A retrospective cohort study was conducted that included 352 records of patients diagnosed with APS and treated between 2014 and 2018. A description is presented of the sociodemographic, clinical, and immunological profile of the population. A bivariate analysis performed using the chi-squared test to determine differences between groups with primary APS and secondary APS, and finally a multivariate analysis to search for associations with thrombotic clinical outcomes in patients with APS. Results: The mean age was 42.4 ± 14 years, and 84.6% were females. Two-thirds (67.6%) of the patients had a diagnosis of primary APS, and 32.4% of secondary APS, of which 84% were associated with systemic lupus erythematosus (SLE). Among the thrombotic events, the most frequent were deep vein thrombosis (17.3%) and stroke (9.9%). Obstetric events were frequent, with a prevalence of 39.4% for miscarriages. No differences were found in the sociodemographic or immunoserological profile when comparing the group of primary vs. secondary APS. Thrombotic events were more frequent in the primary APS group, although only pulmonary embolism reached statistical significance. There were no differences between the two groups as regards obstetric events, such as miscarriages. Women were found to be 5 times more likely to have a stroke and 3 times more to have deep vein thrombosis. The anti-β2GPI type IgM increased the probability of presenting miscarriages about 3 times in women with APS. Conclusion: The study contains one of the largest Colombian cohorts with APS reported so far, and although it is both clinically and sociodemographically similar to other cohorts, there is a higher prevalence of primary APS. There was a lower frequency of thrombotic complications compared to other cohorts. Patients with primary APS had a tendency to develop thrombosis, as has already been reported in the literature.

Sarcoidosis is a chronic granulomatous disease that has been linked to exposure to certain environmental antigens, including previous contact with infectious agents, and a variety of other organic and inorganic particles. There is an acute form of presentation of this disease that courses with stereotypical clinical findings known as Lofgren's syndrome. A case is presented of a female patient with all the characteristic features of this syndrome.

Resumen El dano irreversible de organo es predictor de morbilidad, mortalidad, mayor acumulo de dano y mala calidad de vida en los pacientes con lupus eritematoso sistemico. Objetivos Caracterizar el dano y los factores que mejor lo explican, en una poblacion de pacientes colombianos con lupus eritematoso sistemico. Metodos Estudio retrospectivo de seguimiento a una cohorte. El dano se midio con el SLICC/ACR (indice de Systemic Lupus International Collaborating Clinics y del American College of Rheumatology) y la actividad de la enfermedad por SELENA SLEDAI. La caracterizacion del dano se hizo mediante estadistica descriptiva, los factores asociados con el desenlace se evaluaron con Chi2 de Pearson o Fisher, t de Student o U de Mann-Whitney; la proporcion de pacientes que acumularon dano se evaluo con el test de Friedman y el puntaje acumulado con el test de Wilcoxon. La determinacion de los factores asociados independientemente con el desenlace se hizo con una regresion logistica. Resultados Se incluyeron 161 pacientes con diagnostico de novo y seguimiento minimo de un ano; el 28,9% sufrio dano. Los dominios mas representados fueron el neuropsiquiatrico, renal y vascular. Los anticuerpos antifosfolipido, las dosis promedio de prednisolona mayores a 12,5 mg/dia y presentar 2 o mas recaidas se asociaron independientemente al dano organico. Conclusiones Los anticuerpos antifosfolipido, la dosis de esteroides y la frecuencia de recaidas se asocian al dano organico en una poblacion colombiana de pacientes con lupus eritematoso sistemico.

La sarcoidosis es una enfermedad granulomatosa cronica relacionada frecuentemente con antigenos ambientales e infecciones. Sin embargo, no se ha logrado identificar una causa clara en todos los escenarios. Por su parte, la amiloidosis secundaria se caracteriza por el deposito de proteina amiloide AA en los diferentes tejidos, la cual se asocia a procesos inflamatorios cronicos. Es supremamente infrecuente coincidir con estas dos enfermedades ya que no existe una relacion de causalidad directa. A continuacion presentamos un caso de un paciente con hallazgos de esta rara asociacion. DOI: http://dx.doi.org/10.21615/cesmedicina.32.1.7

Organ damage predicts mortality, increased accrual of detriment, and poor quality of life in systemic lupus erythematosus patients. The objective of this study is to determine the damage-free survival and its predictive factors in a population of Colombian subjects. The method used in this study is the retrospective follow-up of a cohort; damage was measured with SLICC/ACR index. Predictors of impairment were assessed by logistic regression and survival analysis. One hundred sixty-one individuals were included; 28.9% suffered damage, primarily neuropsychiatric, renal, and vascular. Arterial hypertension, antiphospholipid antibodies, prednisone dose, and number of relapses were all predictors of detriment. Onset after age 50 and daily prednisone dose higher than 7.5 mg determined earlier occurrence of damage.

Monckeberg's sclerosis is a rare and low prevalence disease of unknown cause in which small and medium size arteries suffer calcification of the middle layer, leading to a reduction in caliber. Clinically, the disease manifests by the appearance of arterial type ulcers on the skin of upper and lower extremities. Its diagnosis is difficult, and requires histopathological studies. Some rheumatic conditions, such as polyarteritis nodosa and giant cell arteritis may mimic this process. Treatment of the disease is not well established. Although the use of calcium chelators such as sodium thiosulfate has been proposed, the few available studies have failed to show significant results.

RESUMEN El daño irreversible de órgano es predictor de morbilidad, mortalidad, mayor acúmulo de daño y mala calidad de vida en los pacientes con lupus eritematoso sistémico. Objetivos: Caracterizar el daño y los factores que mejor lo explican, en una población de pacientes colombianos con lupus eritematoso sistémico. Métodos: Estudio retrospectivo de seguimiento a una cohorte. El daño se midió con el SLICC/ACR (índice de Systemic Lupus International Collaborating Clinics y del American College of Rheumatology) y la actividad de la enfermedad por SELENA SLEDAI. La caracterización del daño se hizo mediante estadística descriptiva, los factores asociados con el desenlace se evaluaron con Chi2 de Pearson o Fisher, t de Student o U de Mann-Whitney; la proporción de pacientes que acumularon daño se evaluó con el test de Friedman y el puntaje acumulado con el test de Wilcoxon. La determinación de los factores asociados independientemente con el desenlace se hizo con una regresión logística. Resultados: Se incluyeron 161 pacientes con diagnóstico de novo y seguimiento mínimo de un año; el 28,9% sufrió daño. Los dominios más representados fueron el neuropsiquiátrico, renal y vascular. Los anticuerpos antifosfolípido, las dosis promedio de prednisolona mayores a 12,5 mg/día y presentar 2 o más recaídas se asociaron independientemente al daño orgánico. Conclusiones: Los anticuerpos antifosfolípido, la dosis de esteroides y la frecuencia de recaídas se asocian al daño orgánico en una población colombiana de pacientes con lupus eritematoso sistémico. ABSTRACT Irreversible organ damage is a predictive factor of morbidity, mortality, increased accumulation of damage, and poor quality of life in patients with systemic lupus erythematosus. Objectives: To describe the damage, and the factors that best explain it, in a population of Colombian patients. Methods: A retrospective follow-up study of a patient cohort. The damage was measured using the Systemic Lupus International Collaborating Clinics (SLICC) and the American College of Rheumatology ACR index, and disease activity by SELENA SLEDAI. Descriptive statistics were used to describe the damage. The factors associated with the outcome were evaluated with Pearson's or Fisher's Chi2, Student's t or Mann-Whitney's U. The proportion of patients that accumulated damage was evaluated with the Friedman test, and the cumulative score with the Wilcoxon test. The determination of the factors independently associated with the outcome was performed using logistic regression. Results: A total of 161 patients with recent diagnosis, and followed for one year or more, were included, 28.9% of whom had suffered damage. The most represented domains were neuropsychiatric, renal and vascular. Anti-phospholipid antibodies, mean doses of prednisolone greater than 12.5 mg/day, and suffering two or more relapses were independently associated with organ damage. Conclusions: Anti-phospholipid antibodies, steroid doses and frequency of relapses are associated with organ damage in a Colombian population of patients with SLE.

Systemic lupus erythematosus (SLE), a complex and heterogeneous autoimmune disease, represents a significant challenge for both diagnosis and treatment. Patients with SLE in Latin America face special problems that should be considered when therapeutic guidelines are developed. The objective of the study is to develop clinical practice guidelines for Latin American patients with lupus. Two independent teams (rheumatologists with experience in lupus management and methodologists) had an initial meeting in Panama City, Panama, in April 2016. They selected a list of questions for the clinical problems most commonly seen in Latin American patients with SLE. These were addressed with the best available evidence and summarised in a standardised format following the Grading of Recommendations Assessment, Development and Evaluation approach. All preliminary findings were discussed in a second face-to-face meeting in Washington, DC, in November 2016. As a result, nine organ/system sections are presented with the main findings; an 'overarching' treatment approach was added. Special emphasis was made on regional implementation issues. Best pharmacologic options were examined for musculoskeletal, mucocutaneous, kidney, cardiac, pulmonary, neuropsychiatric, haematological manifestations and the antiphospholipid syndrome. The roles of main therapeutic options (ie, glucocorticoids, antimalarials, immunosuppressant agents, therapeutic plasma exchange, belimumab, rituximab, abatacept, low-dose aspirin and anticoagulants) were summarised in each section. In all cases, benefits and harms, certainty of the evidence, values and preferences, feasibility, acceptability and equity issues were considered to produce a recommendation with special focus on ethnic and socioeconomic aspects. Guidelines for Latin American patients with lupus have been developed and could be used in similar settings. Fil: Pons Estel, Bernardo A.. Centro Regional de Enfermedades Autoinmunes y Reumáticas; Argentina Fil: Bonfa, Eloisa. Universidade de Sao Paulo; Brasil Fil: Soriano, Enrique R.. Instituto Universitario Hospital Italiano de Buenos Aires. Rectorado.; Argentina Fil: Cardiel, Mario H.. Centro de Investigación Clínica de Morelia; México Fil: Izcovich, Ariel. Hospital Alemán; Argentina Fil: Popoff, Federico. Hospital Aleman; Argentina Fil: Criniti, Juan M.. Hospital Alemán; Argentina Fil: Vásquez, Gloria. Universidad de Antioquia; Colombia Fil: Massardo, Loreto. Universidad San Sebastián; Chile Fil: Duarte, Margarita. Hospital de Clínicas; Paraguay Fil: Barile Fabris, Leonor A.. Hospital Angeles del Pedregal; México Fil: García, Mercedes A.. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina Fil: Amigo, Mary Carmen. Centro Médico Abc; México Fil: Espada, Graciela. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez"; Argentina Fil: Catoggio, Luis J.. Hospital Italiano. Instituto Universitario. Escuela de Medicina; Argentina Fil: Sato, Emilia Inoue. Universidade Federal de Sao Paulo; Brasil Fil: Levy, Roger A.. Universidade do Estado de Rio do Janeiro; Brasil Fil: Acevedo Vásquez, Eduardo M.. Universidad Nacional Mayor de San Marcos; Perú Fil: Chacón Díaz, Rosa. Policlínica Méndez Gimón; Venezuela Fil: Galarza Maldonado, Claudio M.. Corporación Médica Monte Sinaí; Ecuador Fil: Iglesias Gamarra, Antonio J.. Universidad Nacional de Colombia; Colombia Fil: Molina, José Fernando. Centro Integral de Reumatología; Colombia Fil: Neira, Oscar. Universidad de Chile; Chile Fil: Silva, Clóvis A.. Universidade de Sao Paulo; Brasil Fil: Vargas Peña, Andrea. Hospital Pasteur Montevideo; Uruguay Fil: Gómez Puerta, José A.. Hospital Clinic Barcelona; España Fil: Scolnik, Marina. Instituto Universitario Hospital Italiano de Buenos Aires. Rectorado.; Argentina Fil: Pons Estel, Guillermo J.. Centro Regional de Enfermedades Autoinmunes y Reumáticas; Argentina. Hospital Provincial de Rosario; Argentina Fil: Ugolini Lopes, Michelle R.. Universidade de Sao Paulo; Brasil Fil: Savio, Verónica. Instituto Universitario Hospital Italiano de Buenos Aires. Rectorado.; Argentina Fil: Drenkard, Cristina. University of Emory; Estados Unidos Fil: Alvarellos, Alejandro J.. Hospital Privado Universitario de Córdoba; Argentina Fil: Ugarte Gil, Manuel F.. Universidad Cientifica del Sur; Perú. Hospital Nacional Guillermo Almenara Irigoyen; Perú Fil: Babini, Alejandra. Instituto Universitario Hospital Italiano de Buenos Aires. Rectorado.; Argentina Fil: Cavalcanti, André. Universidade Federal de Pernambuco; Brasil Fil: Cardoso Linhares, Fernanda Athayde. Hospital Pasteur Montevideo; Uruguay Fil: Haye Salinas, Maria Jezabel. Hospital Privado Universitario de Córdoba; Argentina Fil: Fuentes Silva, Yurilis J.. Universidad de Oriente - Núcleo Bolívar; Venezuela Fil: Montandon De Oliveira E Silva, Ana Carolina. Universidade Federal de Goiás; Brasil Fil: Eraso Garnica, Ruth M.. Universidad de Antioquia; Colombia Fil: Herrera Uribe, Sebastián. Hospital General de Medellin Luz Castro de Gutiérrez; Colombia Fil: Gómez Martín, DIana. Instituto Nacional de la Nutrición Salvador Zubiran; México Fil: Robaina Sevrini, Ricardo. Universidad de la República; Uruguay Fil: Quintana, Rosana M.. Hospital Provincial de Rosario; Argentina. Centro Regional de Enfermedades Autoinmunes y Reumáticas; Argentina Fil: Gordon, Sergio. Hospital Interzonal General de Agudos Dr Oscar Alende. Unidad de Reumatología y Enfermedades Autoinmunes Sistémicas; Argentina Fil: Fragoso Loyo, Hilda. Instituto Nacional de la Nutrición Salvador Zubiran; México Fil: Rosario, Violeta. Hospital Docente Padre Billini; República Dominicana Fil: Saurit, Verónica. Hospital Privado Universitario de Córdoba; Argentina Fil: Appenzeller, Simone. Universidade Estadual de Campinas; Brasil Fil: Dos Reis Neto, Edgard Torres. Universidade Federal de Sao Paulo; Brasil Fil: Cieza, Jorge. Hospital Nacional Edgardo Rebagliati Martins; Perú Fil: González Naranjo, Luis A.. Universidad de Antioquia; Colombia Fil: González Bello, Yelitza C.. Ceibac; México Fil: Collado, María Victoria. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; Argentina Fil: Sarano, Judith. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; Argentina Fil: Retamozo, Maria Soledad. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; Argentina Fil: Sattler, María E.. Provincia de Buenos Aires. Ministerio de Salud. Hospital Interzonal de Agudos "Eva Perón"; Argentina Fil: Gamboa Cárdenas, Rocio V.. Hospital Nacional Guillermo Almenara Irigoyen; Perú Fil: Cairoli, Ernesto. Universidad de la República; Uruguay Fil: Conti, Silvana M.. Hospital Provincial de Rosario; Argentina Fil: Amezcua Guerra, Luis M.. Instituto Nacional de Cardiologia Ignacio Chavez; México Fil: Silveira, Luis H.. Instituto Nacional de Cardiologia Ignacio Chavez; México Fil: Borba, Eduardo F.. Universidade de Sao Paulo; Brasil Fil: Pera, Mariana A.. Hospital Interzonal General de Agudos General San Martín; Argentina Fil: Alba Moreyra, Paula B.. Universidad Nacional de Córdoba. Facultad de Medicina; Argentina Fil: Arturi, Valeria. Hospital Interzonal General de Agudos General San Martín; Argentina Fil: Berbotto, Guillermo A.. Provincia de Buenos Aires. Ministerio de Salud. Hospital Interzonal de Agudos "Eva Perón"; Argentina Fil: Gerling, Cristian. Hospital Interzonal General de Agudos Dr Oscar Alende. Unidad de Reumatología y Enfermedades Autoinmunes Sistémicas; Argentina Fil: Gobbi, Carla Andrea. Universidad Nacional de Córdoba. Facultad de Medicina; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina Fil: Gervasoni, Viviana L.. Hospital Provincial de Rosario; Argentina Fil: Scherbarth, Hugo R.. Hospital Interzonal General de Agudos Dr Oscar Alende. Unidad de Reumatología y Enfermedades Autoinmunes Sistémicas; Argentina Fil: Brenol, João C. Tavares. Hospital de Clinicas de Porto Alegre; Brasil Fil: Cavalcanti, Fernando. Universidade Federal de Pernambuco; Brasil Fil: Costallat, Lilian T. Lavras. Universidade Estadual de Campinas; Brasil Fil: Da Silva, Nilzio A.. Universidade Federal de Goiás; Brasil Fil: Monticielo, Odirlei A.. Hospital de Clinicas de Porto Alegre; Brasil Fil: Seguro, Luciana Parente Costa. Universidade de Sao Paulo; Brasil Fil: Xavier, Ricardo M.. Hospital de Clinicas de Porto Alegre; Brasil Fil: Llanos, Carolina. Universidad Católica de Chile; Chile Fil: Montúfar Guardado, Rubén A.. Instituto Salvadoreño de la Seguridad Social; El Salvador Fil: Garcia De La Torre, Ignacio. Hospital General de Occidente; México Fil: Pineda, Carlos. Instituto Nacional de Rehabilitación; México Fil: Portela Hernández, Margarita. Umae Hospital de Especialidades Centro Medico Nacional Siglo Xxi; México Fil: Danza, Alvaro. Hospital Pasteur Montevideo; Uruguay Fil: Guibert Toledano, Marlene. Medical-surgical Research Center; Cuba Fil: Reyes, Gil Llerena. Medical-surgical Research Center; Cuba Fil: Acosta Colman, Maria Isabel. Hospital de Clínicas; Paraguay Fil: Aquino, Alicia M.. Hospital de Clínicas; Paraguay Fil: Mora Trujillo, Claudia S.. Hospital Nacional Edgardo Rebagliati Martins; Perú Fil: Muñoz Louis, Roberto. Hospital Docente Padre Billini; República Dominicana Fil: García Valladares, Ignacio. Centro de Estudios de Investigación Básica y Clínica; México Fil: Orozco, María Celeste. Instituto de Rehabilitación Psicofísica; Argentina Fil: Burgos, Paula I.. Pontificia Universidad Católica de Chile; Chile Fil: Betancur, Graciela V.. Instituto de Rehabilitación Psicofísica; Argentina Fil: Alarcón, Graciela S.. Universidad Peruana Cayetano Heredia; Perú. University of Alabama at Birmingahm; Estados Unidos

Resumen Las vasculitis sistemicas idiopaticas se caracterizan por inflamacion y necrosis de las paredes de los vasos de origen desconocido 1 ; se han propuesto medicamentos e infecciones como posibles disparadores de estas enfermedades 2 . A continuacion se presenta el caso de un paciente con diagnostico de granulomatosis con poliangeitis, con anticuerpos contra el citoplasma de neutrofilos tanto antiproteinasa 3 como antimieloperoxidasa y con el hallazgo poco usual de granuloma renal.