Frecuencia de eventos cardiovasculares en pacientes con lupus eritematoso sistémico / Frequency of cardiovascular events in patients with systemic lupus erythematosus

Resumen Introducción: el progreso en los tratamientos para el lupus eritematoso sistémico (LES) resultó en una disminución de la mortalidad; sin embargo, la enfermedad cardiovascular y las complicaciones infecciosas aún son las principales causas de muerte. La evidencia apoya la participación del sistema inmunológico en la generación de la placa aterosclerótica, así como su conexión con las enfermedades autoinmunes. Objetivos: describir la frecuencia de eventos cardiovasculares (ECV) en el Registro de Lupus Eritematoso Sistémico de la Sociedad Argentina de Reumatología (RELESSAR) transversal, así como sus principales factores de riesgo asociados. Materiales y métodos: estudio descriptivo y transversal para el cual se tomaron los pacientes ingresados en el registro RELESSAR transversal. Se describieron las variables sociodemográficas y clínicas, las comorbilidades, score de actividad y daño. ECV se definió como la presencia de al menos una de las siguientes patologías: enfermedad arterial periférica, cardiopatía isquémica o accidente cerebrovascular. El evento clasificado para el análisis fue aquel posterior al diagnóstico del LES. Se conformaron dos grupos macheados por edad y sexo 1:2. Resultados: 1515 pacientes mayores de 18 años participaron del registro. Se describieron 80 pacientes con ECV (5,3%). En este análisis se incluyeron 240 pacientes conformando dos grupos. La edad media fue de 47,8 (14,4) y 47,6 (14,2) en el grupo con y sin ECV respectivamente. Los pacientes con ECV tuvieron mayor duración del LES en meses, mayor índice de Charlson, mayor SLICC (Systemic Lupus International Collaborating Clinics/American College of Rheumatology), mayor frecuencia de manifestaciones neurológicas, síndrome antifosfolípido, hospitalizaciones y uso de ciclofosfamida. Las únicas variables asociadas en el análisis multivariado fueron el índice de Charlson (p=0,004) y el SLICC (p<0,001). Conclusiones: los ECV influyen significativamente en nuestros pacientes, y se asocian a mayor posibilidad de daño irreversible y comorbilidades.

Abstract Introduction: progress in treatments for systemic lupus erythematosus (SLE) has resulted in a decrease in mortality; however, cardiovascular and infectious diseases remain the leading causes of death. Evidence supports the involvement of the immune system in the generation of atherosclerotic plaque, as well as its connection to autoimmune diseases. Objectives: to describe the frequency of cardiovascular disease (CVD) in the cross-sectional RELESSAR registry, as well as its associated variables. Materials and methods: a descriptive and cross-sectional study was performed using patients admitted to the cross-sectional RELESSAR registry. Sociodemographic variables, clinical variables, comorbidities, activity and damage scores were described. CVD was defined as at least one of the following: peripheral arterial disease, ischemic heart disease, or cerebrovascular accident. All patients with at least one CVD were included in our analysis (heart attack, central nervous system vascular disease, and peripheral arteries atherosclerotic disease). The event classified for the analysis was that after the diagnosis of SLE. SLE diagnosis was previous to CVD. Two groups matched by age and sex, 1:2 were formed. Results: a total of 1515 patients older than 18 years participated in the registry. Eighty patients with CVD (5.3%) were described in the registry. Two-hundred and forty patients were included, according to two groups. The mean age was 47.8 (SD 14.4) and 47.6 (SD 14.2) in patients with and without CVD, respectively. Patients with CVD had a longer duration of SLE in months, a higher Charlson index, a higher SLICC, increased frequency of neurological manifestations, antiphospholipid syndrome, hospitalizations, and use of cyclophosphamide. The associated variables in the multivariate were the Charlson Index (p=0.004) and the SLICC (p<0.001). Conclusions: CVDs have a significant influence on our patients, being associated with a greater possibility of damage and comorbidities.

Behçet disease in Latin American countries: A systematic literature review of demographic and clinical features, and HLA-B*51 allele frequency.

OBJECTIVE: To describe the demographic and clinical features, as well as the frequency of the HLA-B*51 allele in Behçet disease (BD) patients in Latin American countries. METHODS: A systematic literature review of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines was conducted without performing a meta-analysis. We included observational studies (cross-sectional or cohort) of BD patients fulfilling the International Study Group for BD classification criteria and reported the demographic, clinical, and laboratory features of the disease in adult patients. RESULTS: Twelve studies were included in the SLR. Information from 532 patients across 5 Latin American countries was included for the analysis. Mean age at disease diagnosis was 33 years, 58.3% were female and 41.7% male; most patients were non-Caucasian. The most common clinical manifestations were recurrent oral ulcers and genital ulcers, followed by skin, eye, joint, neurological, gastrointestinal, vascular, and cardiac involvement. The prevalence of BD was described in 2 studies, 1 conducted in Brazil that reported a prevalence of .3/100,000 inhabitants, and another in Colombia with a prevalence of 1.1/100,000 inhabitants. The frequency of HLA-B*51 allele in BD patients was 38%, 30.1%, and 9% in Argentina, Brazil, and Mexico, respectively. CONCLUSIONS: The prevalence of BD in the Latin American countries seems to be low, as well as the frequency of HLA-B*51 allele. However, the strength of association between HLA-B*51 and BD remains high in our population. The key clinical features of BD are like those reported in countries/regions where BD is endemic.

Significance of HLA-B[*]51 allele expression in Crohn's disease: a case-control study.

OBJECTIVES: Crohn's disease (CD) and Behçet's disease (BD) are two autoinflammatory diseases that share clinical and pathogenic features. Furthermore, when BD involves the gastrointestinal tract, it is extremely difficult to distinguish endoscopic lesions from CD lesions. HLA-B*51 allele expression is highly associated with BD diagnosis. In this study we analysed HLA-B*51 status in 70 Argentine patients with confirmed CD diagnosis and compared it to our previous Argentine BD cohort, with the aim of finding similarities or differences between these two diseases regarding HLA-B*51 status. METHODS: This is a multi-centre case-control study, including 70 patients with confirmed CD diagnosis, who underwent HLA-B*51 allele status testing; the results were compared to our previous BD cohort of 34 patients. RESULTS: Among patients with CD, 12.85% were positive for the HLA-B*51 allele, compared with 38.24% patients with BD (OR=0.238; 95% CI=0.089-0.637; p=0.004). CONCLUSIONS: Our finding suggests that determination of HLA-B*51 allele status may contribute to the differential diagnosis between CD and BD.

Recomendaciones de la Sociedad Argentina de Reumatología en el manejo de la arteritis de células gigantes / Recommendations of the Argentine Society of Rheumatology in the management of giant cell arteritis

La arteritis de células gigantes (ACG) es una vasculitis sistémica que afecta a personas adultas; compromete vasos arteriales de mediano y gran calibre, con potenciales complicaciones de gravedad, como la ceguera, y es considerada una emergencia médica. El objetivo de estas guías fue desarrollar las primeras recomendaciones argentinas para su tratamiento, basadas en la revisión de la literatura mediante metodología GRADE. Un panel de expertos en vasculitis elaboró las preguntas en formato PICO (población, intervención, comparador y outcomes), y luego un panel de expertos en metodología efectuó la revisión de la bibliografía con la extracción de la evidencia para cada una de las preguntas. Se realizó un focus group de pacientes para conocer sus preferencias y experiencias. Finalmente, con la información recabada, el panel de expertos en vasculitis procedió a la votación de las recomendaciones que a continuación se presentan.

Giant cell arteritis (GCA) is a systemic vasculitis affecting adult patients and involving large and medium vessels. Potential serious complications as blindness may occur and it is considered a medical emergency. The objective of elaborating this guideline was to develop first Argentinian GCA treatment recommendations using GRADE methodology. An expert panel generated clinically meaningful questions addressing aspects of the treatment of GCA in the Population, Intervention, Comparator and Outcome (PICO) format and then a group of methodology experts reviewed and extracted data from literature summarizing available evidence. A patient's focus group discussion took place gathering information on their preferences and experiences. Finally, the vasculitis expert panel, with all the information obtained, voted recommendations here presented.

Guías Argentinas de Vasculitis Suplemento. Tablas arteritis de células gigantes / Argentine Vasculitis Guidelines Supplement. Giant cell arteritis tables

La arteritis de células gigantes (ACG) es una vasculitis sistémica que afecta a personas adultas; compromete vasos arteriales de mediano y gran calibre, con potenciales complicaciones de gravedad, como la ceguera, y es considerada una emergencia médica. El objetivo de estas guías fue desarrollar las primeras recomendaciones argentinas para su tratamiento, basadas en la revisión de la literatura mediante metodología GRADE. Un panel de expertos en vasculitis elaboró las preguntas en formato PICO (población, intervención, comparador y outcomes), y luego un panel de expertos en metodología efectuó la revisión de la bibliografía con la extracción de la evidencia para cada una de las preguntas. Se realizó un focus group de pacientes para conocer sus preferencias y experiencias. Finalmente, con la información recabada, el panel de expertos en vasculitis procedió a la votación de las recomendaciones que a continuación se presentan.

Multicenter lupus register from Argentina, the RELESSAR database: Influence of ethnicity on disease phenotype.

OBJECTIVE: The objective is to describe the main characteristics of patients with systemic lupus erythematosus (SLE) in Argentina and to examine the influence of ethnicity on the expression of the disease. PATIENTS AND METHODS: RELESSAR is a multicentre register carried out by 106 researchers from 67 rheumatologic Argentine centres. It is a cross-sectional study of SLE (1982/1997 ACR) patients. RELESSAR electronic database includes demographic, cumulative SLE manifestations, SELENA-SLEDAI, SLICC-SDI, Katz's severity and Charlson's comorbidity indexes and treatment patterns. RESULTS: We included 1,610 patients, 91.7% were female with a median age at diagnosis of 28.1 ± 12.8; 96.2% met ≥4 ACR 1982/97 criteria. Frequent manifestations were arthritis (83.5%), malar rash (79.5%), photosensitivity (75.3%), haematological (63.8%) and renal disease (47.4%), antinuclear antibodies (96%), anti-dsDNA (66.5%) and anti-Smith antibodies (29%). The mean Selena-SLEDAI score at last visit was 3.18 (SD 4.3) and mean SDI was 1 (SD 1.3). The accumulated treatments most frequently used were antimalarials (90.4%), corticosteroids (90%), azathioprine (31.8%), intravenous cyclophosphamide (30.2%), mycophenolate mofetil or mycophenolic acid (24.5%), methotrexate (19.3%), belimumab 5.3% and rituximab 5.1%. Refractory lupus was diagnosed in 9.3% of the cases. The main causes of death were lupus activity (25.0%), activity and concomitant infections (25.0%), infections (18.2%), vascular disease (13.6%) and cancer (4.5%). Mortality was associated with higher SLEDAI, Katz, damage indexes and comorbidities. Of the 1610 patients included, 44.6% were Caucasian, 44.5% Mestizo, 8.1% Amerindian and 1.2% Afro-Latin American. Mestizo patients had higher male representation, low socioeconomic status, more inadequate medical coverage, fewer formal years of education and shorter disease duration. Polyadenopathies and Raynaud's phenomenon were more frequent in Caucasians. In the logistic regression analysis higher damage index (OR 1.28, CI 95% 1.02-1.61, p = 0.03) remained associated to mestizo ethnicity. CONCLUSIONS: This study represents the largest number of adult patients with SLE studied in Argentina. Caucasian patients were differentiated by having Raynaud's phenomenon and polyadenopathy more frequently, while patients of Mestizo origin had higher damage indexes.

Lupus en Argentina. Pacientes no respondedores al tratamiento estándar y belimumab como posible opción. Datos del registro RELESSAR / Lupus in Argentina. Non-responders to standard treatment and belimumab as a possible option. RELESSAR record data

Introducción: el lupus es una enfermedad compleja y varias veces de difícil abordaje. Alcanzar la remisión es uno de los objetivos, incorporando opciones terapéuticas. Objetivos: describir las características generales de los pacientes según el estado de la enfermedad y el uso de belimumab. Materiales y métodos: estudio de corte transversal, registro RELESSAR. Se definió el estado de la enfermedad como: remisión: SLEDAI=0 y sin corticoides; baja actividad de la enfermedad: SLEDAI >0 y ≤4 y sin corticoides; control no óptimo: SLEDAI >4 y cualquier dosis de corticoides. Resultados: se incluyeron 1.277 pacientes, 23,4% en remisión, 12,6% en baja actividad y 63,8% con control no óptimo. En este último grupo eran más jóvenes y con menor duración de la enfermedad; presentaban mayores índices de actividad y cronicidad, y mayor empleo de inmunosupresores. Solo el 22,3% de los pacientes con criterio potencial de uso de belimumab (lupus eritematoso sistémico activo a pesar del tratamiento estándar) lo recibía en ese momento. Las variables asociadas a hospitalizaciones fueron: terapia con corticoides, ciclofosfamida y mayor SLICC. Conclusiones: se refleja la complejidad del manejo de estos pacientes y se visualizan aspectos estructurales como la desigualdad. El uso del belimumab resultaría beneficioso en los pacientes seleccionados.

Introduction: lupus is a complex disease and often difficult to approach. Achieving remission is one of the objectives, incorporating therapeutic options. Objectives: to describe the characteristics of the patients and the use of belimumab, according to the status of the disease. Materials and methods: cross-sectional study. Patients of the RELESSAR registry. Stratification: Remission: SLEDAI=0 and without corticosteroids. Low disease activity SLEDAI> 0 and ≤4 and without corticosteroids and non-optimal control: SLEDAI> 4 and any dose of corticosteroids. Results: a total of 1,277 patients were included, 23.4% in remission, 12.6% in low disease activity and 63.8% in non-optimal control. The last group was younger and had a shorter duration of the disease. They had higher activity and chronicity indices and greater use of immunosuppressants. Only 22.3% of the patients with potential criteria for the use of belimumab (activity disease despite standard treatment) were receiving it. The variables associated with hospitalizations were: corticosteroids, cyclophosphamide and higher SLICC. Those associated with severe infection: mycophenolate mofetil, azathioprine, corticosteroids, and higher SLICC. Conclusions: the complexity of the management of these patients is reflected, visualizing structural aspects such as inequality. The use of belimumab could be beneficial in selected patients.

Lupus en Argentina. Pacientes no respondedores al tratamiento estándar y belimumab como posible opción. Datos del registro RELESSAR / Lupus in Argentina. Non-responders to standard treatment and belimumab as a possible option. RELESSAR record data

Introducción: el lupus es una enfermedad compleja y varias veces de difícil abordaje. Alcanzar la remisión es uno de los objetivos, incorporando opciones terapéuticas. Objetivos: describir las características generales de los pacientes según el estado de la enfermedad y el uso de belimumab. Materiales y métodos: estudio de corte transversal, registro RELESSAR. Se definió el estado de la enfermedad como: remisión: SLEDAI=0 y sin corticoides; baja actividad de la enfermedad: SLEDAI >0 y ≤4 y sin corticoides; control no óptimo: SLEDAI >4 y cualquier dosis de corticoides. Resultados: se incluyeron 1.277 pacientes, 23,4% en remisión, 12,6% en baja actividad y 63,8% con control no óptimo. En este último grupo eran más jóvenes y con menor duración de la enfermedad; presentaban mayores índices de actividad y cronicidad, y mayor empleo de inmunosupresores. Solo el 22,3% de los pacientes con criterio potencial de uso de belimumab (lupus eritematoso sistémico activo a pesar del tratamiento estándar) lo recibía en ese momento. Las variables asociadas a hospitalizaciones fueron: terapia con corticoides, ciclofosfamida y mayor SLICC. Conclusiones: se refleja la complejidad del manejo de estos pacientes y se visualizan aspectos estructurales como la desigualdad. El uso del belimumab resultaría beneficioso en los pacientes seleccionados.

Introduction: lupus is a complex disease and often difficult to approach. Achieving remission is one of the objectives, incorporating therapeutic options. Objectives: to describe the characteristics of the patients and the use of belimumab, according to the status of the disease. Materials and methods: cross-sectional study. Patients of the RELESSAR registry. Stratification: Remission: SLEDAI=0 and without corticosteroids. Low disease activity SLEDAI> 0 and ≤4 and without corticosteroids and non-optimal control: SLEDAI> 4 and any dose of corticosteroids. Results: a total of 1,277 patients were included, 23.4% in remission, 12.6% in low disease activity and 63.8% in non-optimal control. The last group was younger and had a shorter duration of the disease. They had higher activity and chronicity indices and greater use of immunosuppressants. Only 22.3% of the patients with potential criteria for the use of belimumab (activity disease despite standard treatment) were receiving it. The variables associated with hospitalizations were: corticosteroids, cyclophosphamide and higher SLICC. Those associated with severe infection: mycophenolate mofetil, azathioprine, corticosteroids, and higher SLICC. Conclusions: the complexity of the management of these patients is reflected, visualizing structural aspects such as inequality. The use of belimumab could be beneficial in selected patients.

The HLA-B*51 Allele is strongly associated with Behçet Disease in an Argentinean population.

OBJECTIVE: To assess the association between the HLA-B*51 allele and Behçet Disease (BD) in Argentinean patients. METHODS: We enrolled 34 consecutive Argentinean patients with definitive diagnosis of BD between October 2016 and March 2017. None of the patients had the HLA-B*51 allele determined at study entry. Unrelated controls (n=240) were randomly obtained from the national cadaveric donor database. Demographic and clinical features of the patients were recorded by attending physicians through a questionnaire. RESULTS: Mean age of cases was 42 years old. Nineteen (55.8%) were male, and the mean age at diagnosis was 35 years old; twenty (58.8%) were Mestizos, 8 (23.5%) were Caucasian, and 6 (17.6%) were Amerindians. Thirteen (38.2%) of 34 cases were HLA-B*51 allele positive; 11 were heterozygous and 2 homozygous for the allele. Thirty-four (14.2%) of 240 controls were positive for the HLA-B*51 allele. The association between BD and HLA-B*51 allele was greater than that of control group (OR=3.75; p=0.0012). CONCLUSIONS: The HLA-B*51 allele is strongly associated with BD in Argentinean patients. Our finding is consistent with previous studies indicating that the HLA-B*51 allele is an important susceptibility gene in BD regardless the geographical region and ethnicity.

Cat scratch disease during etanercept therapy in a rheumatoid arthritis patient. / Enfermedad por arañazo de gato en paciente con artritis reumatoidea tratado con etanercept.

Cat scratch disease (CSD) is an infectious disorder caused by Bartonella henselae and characterized by fever and granulomatous lymphadenopathy. Immunosuppression is a risk factor for the development of atypical forms of the disease. We report the case of a 52-year-old woman who presented with fever and bilateral inguinal lymph node enlargement. She did not have apparent contact with animals. The patient was receiving etanercept therapy for rheumatoid arthritis. Lymph node biopsy demonstrated granulomatous lymphadenitis. She was successfully managed by discontinuing etanercept and by treatment with minocycline. She developed clinical remission and typical seroconversion. Infection with Bartonella should be considered in the differential diagnosis in rheumatoid arthritis patients with lymphadenopathy of unknown origin.

La hiperostosis esquelética idiopática difusa (DISH) como expresión de un proceso sistémico / Diffuse idiopathic skeletal hiperostosis (DISH) as an expression of a systemic process

La Hiperostosis Esquelética Idiopática Difusa es una condición caracterizada por la calcificación y/u osificación de los tejidos blandos, principalmente entesis, ligamentos y cápsulas articulares. En 1950, Forestier y Rotés-Querol publicaron una serie denominándola "hiperostosis anquilosante vertebral senil" y la distinguieron como entidad nosológica separada de la espondiloartrosis y la espondilitis anquilosante

Rituximab in the treatment of eosinophilic granulomatosis with polyangiitis.

BACKGROUND: The general consensus is that for patients with EGPA with poor prognosis, intensive therapy with both GC and CF is indicated. The maintenance of remission is made with GC and AZA. A considerable number of patients with EGPA are refractory to first line therapy, experience dose-limiting side effects or relapse. In clinical trials, RTX was effective for the treatment of ANCA-associated vasculitis. However, patients with a diagnosis of EGPA were not included. OBJECTIVE: to review and analyze the published literature regarding the use of RTX in the treatment of EGPA. METHODS: The literature search was performed in MEDLINE and LILACS from 1965 and 1986 respectively until february 2014. RESULTS: 27 patients were included. RTX treatment was due to refractory disease (n=20), relapse (n=5) and with newly diagnosed (n=2). The affected organs were the lungs, peripheral nervous system, kidney and the eyes. Sixteen patients had clinical remission and 8 patients had clinical response. CONCLUSIONS: RTX was effective and well tolerated for the treatment of EGPA.

Association of diffuse idiopathic skeletal hyperostosis and aortic valve sclerosis.

The principal objective of this investigation was to analyze the association between diffuse idiopathic skeletal hyperostosis (DISH) and the presence of aortic valve sclerosis (AVS). For this study we used results from 1000 consecutive outpatients (473 males), older than 50 years of age (average 67.6 years), that had been examined with Doppler echocardiogram and anterior and lateral chest radiographs. Overall, 195 patients (19.5%) were diagnosed with DISH and 283 (28.3%) with AVS. DISH was more prevalent than AVS in males (66.7% vs. 42.6%, p< 0.0001) and in older patients (73.6 ± 9 years vs. 66.1 ± 9 years, p < 0.0001). Furthermore, 55.4% of patients with dorsal DISH presented aortic sclerosis calcification vs. 21.7% of patients free of DISH (OR = 4.47; 95% CI = 3.22-6.21). The adjusted odds ratio (OR) was calculated by sex and age resulting in 3.04 (95% CI = 2.12-4.36; p < .0001). A statistically significant association was found between DISH and AVS in accordance to age and sex. The biological plausibility of this association is based on similar risk factors, pathogenic mechanisms and vascular complications.

Association of diffuse idiopathic skeletal hyperostosis and aortic valve sclerosis / Asociación de hiperostosis esquelética idiopática difusa y esclerosis valvular aórtica

The principal objective of this investigation was to analyze the association between diffuse idiopathic skeletal hyperostosis (DISH) and the presence of aortic valve sclerosis (AVS). For this study we used results from 1000 consecutive outpatients (473 males), older than 50 years of age (average 67.6 years), that had been examined with Doppler echocardiogram and anterior and lateral chest radiographs. Overall, 195 patients (19.5%) were diagnosed with DISH and 283 (28.3%) with AVS. DISH was more prevalent than AVS in males (66.7% vs. 42.6%, p< 0.0001) and in older patients (73.6 ± 9 years vs. 66.1 ± 9 years, p < 0.0001). Furthermore, 55.4% of patients with dorsal DISH presented aortic sclerosis calcification vs. 21.7% of patients free of DISH (OR = 4.47; 95% CI = 3.22-6.21). The adjusted odds ratio (OR) was calculated by sex and age resulting in 3.04 (95% CI = 2.12-4.36; p < .0001). A statistically significant association was found between DISH and AVS in accordance to age and sex. The biological plausibility of this association is based on similar risk factors, pathogenic mechanisms and vascular complications.

El objetivo principal fue analizar la asociación entre la hiperostosis esquelética idiopática difusa (DISH) y la presencia de esclerosis valvular aórtica (AVS). Se evaluaron los resultados de 1000 pacientes ambulatorios consecutivos (473 varones), mayores de 50 años (promedio, 67.6 años), que habían sido examinados con un ecocardiograma Doppler y radiología torácica anterior y lateral. Globalmente, 195 pacientes (19.5%) tuvieron diagnóstico de DISH y 283 (28.3%) de AVS. DISH fue más prevalente que AVS en varones (66.7% vs. 42.6%, p < 0.0001) y en pacientes de mayor edad (73.6 ± 9 años vs. 66.1 ± 9 años, p < 0.0001). Además, 55.4% de los pacientes con DISH dorsal presentaron AVS vs. 21.7% de los pacientes sin DISH (OR = 4.47; 95% CI = 3.22-6.21). El odds ratio (OR) ajustado por sexo y edad fue 3.04 (95% CI = 2.12-4.36; p < 0.0001). Se encontró una asociación estadísticamente significativa entre DISH y AVS, que se mantuvo después de ajustar por sexo y edad. La plausibilidad biológica de esta asociación se basa en los factores de riesgo, mecanismos patogénicos y complicaciones vasculares compartidos.

Carta al Comité de Redacción / Letter to the Drafting Committee

He leído con atención el artículo del Dr. Alfredo Pinzón titulado: "Babinsky o Babinski" (1) publicado en su prestigiosa revista, al cual accedí a través de Internet. Coincido profundamente con los datos aportados por el Dr. Pinzón, siendo un tema de preocupación no sólo por la existencia de un simple error ortográfico, sino por otra serie de implicancias desarrolladas previamente. Nuestro trabajo "¿Qué Babinsky? Babinski" (2), dio origen a la respuesta del Dr. Fernando A. Navarro: "Ni Babinsky ni Babinski: ¡Babinski!" (3). El Dr. Navarro además, es autor del "Diccionario crítico de dudas inglés-español de medicina" (4), imprescindible para el manejo de la literatura médica de los profesionales hispanoparlantes, donde el tema de Babinski es nuevamente citado. Atendiendo al título de la sección, Lapsus Medicus, y siendo el lapsus (Del lat. lapsus, resbalón) una f.lta o equivocación cometida por descuido (Diccionario de la Real Academia Española), me parece correcto completar este aporte

4G/5G plasminogen activator inhibitor-1 and -308 A/G tumor necrosis factor-α promoter gene polymorphisms in Argentinean lupus patients: focus on lupus nephritis.

We investigated the relationship between the 4G/5G plasminogen activator inhibitor (PAI-1) and -308 A/G tumor necrosis factor-α (TNF-α) polymorphisms and the clinical and biochemical features of systemic lupus erythematosus (SLE) in an Argentinean patient cohort. A total of 402 patients were studied, including 179 SLE patients and 223 healthy individuals. PCR-RLFP was used to determine the genotypes of the 4G/5G PAI-1 and -308 A/G TNF-α polymorphisms. SLE patients with lupus nephritis (LN) (n = 86) were compared with patients without LN (n = 93). Additionally, LN patients were divided into proliferative LN and non-proliferative LN groups according to the results of the renal biopsies. No significant differences were noted in the genotype distributions or allele frequencies of these TNF-α and PAI-1 polymorphisms between SLE patients and controls. There were higher numbers of criteria for SLE, more lupus flares and higher damage scores in LN patients, but there were similar frequencies of anti-phospholipid antibody (APA) positivity and anti-phospholipid syndrome. No significant difference was noted for any studied variable between the proliferative LN and non-proliferative LN groups except for the presence of APA. We found no significant differences in the TNF-α and PAI-1 genotype distributions or allele frequencies between groups. We found that the -308 A/G TNF-α and 4G/5G PAI-1 polymorphisms are not associated with susceptibility to SLE in an Argentinean population. We also did not find any association between the presence of any specific allele or genotype and the development of LN in SLE patients. Finally, no association was noted between either of the two polymorphisms and the severity of renal disease.

Causas de admisión hospitalaria y factores pronósticos en pacientes con vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilo / Causes of hospitalization and prognosis in with antineutrophil cytoplamic autoantibodies associated to vasculitis

Antecedentes: Existen pocos estudios descriptivos sobre las causas de internación de los pacientes con vasculitis asociadas a ANCA (VAA), todos son retrospectivos y realizados en unidades de cuidados intensivos. Objetivo: Investigar la epidemiología, los hallazgos clínicos y la evolución de los pacientes con VAA durante su internación, e identificar los factores pronósticos asociados a mortalidad. Materiales y métodos: Se incluyeron los pacientes con diagnóstico de VAA internados en el Hospital Juan A. Fernández y la Clínica San Camilo (Ciudad Autónoma de Buenos Aires, Argentina), entre el 1 de enero de 2011 y el 31 de diciembre de 2013. Resultados: Treinta y cuatro pacientes fueron incluidos, 18 (53%) de sexo femenino. Edad media de 60 ± 12 años. En 9 (26%) pacientes el diagnóstico de VAA se realizó en la internación. La Poliangeítis Microscópica (MPA) fue la vasculitis más común (50%). Dieciocho (53%) pacientes se internaron por actividad clínica de la vasculitis. Órganos/sistemas afectados: pulmón (n=9), riñón (n=6), otorrinolaringológicas (n=5), sistema nervioso periférico (n=5) y piel (n=2). Ocho (23,5%) pacientes fueron admitidos por complicaciones infecciosas y ocho por otras causas. Fallecieron 8 (23,5%) pacientes, 3 debido a actividad de la vasculitis, 4 debido a complicaciones infecciosas y 1 por falla multiorgánica (2º infusión pamidronato). Los que ingresaron a UCI tuvieron mayor mortalidad (p=0,001); el sexo (p=0,69), la edad (p=0,15), el diagnóstico “de novo” de vasculitis (p=0,4), el BVAS y VDI no mostraron diferencias entre los sobrevivientes y fallecidos. La mortalidad de los pacientes que ingresaron por actividad de la vasculitis comparado con los que ingresaron por complicaciones infecciosas fue similar (p=0,6). Conclusiones: La causa más frecuente de internación en pacientes con VAA fue la actividad de la enfermedad, seguida por las causas infecciosas.

Background: Few retrospective studies have described the clinical course of patients with ANCA-asocciated vasculitis (AAV) admitted to the hospital, all of them in intensive care units (ICU).Objective: To study the epidemiology, clinical features and outcome of patients with AAV admitted to the hospital, and to identify the prognostic factors associated with mortality.Methods: Patients with AAV admitted to the Juan A. Fernández Hos-pital and San Camilo Clinic (Buenos Aires City, Argentina) betweenJanuary 2011 and December 2013 were included. Results: Thirty four patients [18 (53%) female] with an average 60 ±12 years old were included. AAV was diagnosed in 9 (26%) patientsin the hospital. Microscopic Polyangiitis was the most common AAV. Eighteen (53%) patients were admitted due to active vasculitis. Lung(n=9), kidney (n=6), ear-nose-throat (n=5), peripheral nervous system (n=5) and skin (n=2) were the organs/systems involved. Other reasons for admission were: infection and metabolic conditions [8(23.5%) patients each]. Eight (23.5%) patients died, 3 due to active vasculitis, 4 due to infection and 1 patient due to multiorgan failure after pamidronate treatment. Mortality was significantly higher for patients who were admitted in ICU (p=0.001); gender (p=0.69), age(p=0.15), new diagnosis of AAV (p=0.4), BVAS and VDI showed no significant differences between survival and dead patients. The mortality was similar (p=0.6) between the patients with active vasculitis and the patients with infections. Conclusion: The main reason for hospitalization in AAV patients was active vasculitis followed by infection. Mortality rate was high and the main causes were infections regardless the diagnosis at admission.

Clinical features and outcomes of 37 Argentinean patients with severe granulomatosis with polyangiitis (wegener granulomatosis).

BACKGROUND: Most epidemiologic studies involving severe granulomatosis with polyangiitis (SGPA) patients have investigated populations from the northern hemisphere, whereas few studies have been conducted in South America. None of the South American studies have differentiated between localized GPA and SGPA. PURPOSE: The present study was designed to describe a cohort of Argentinean patients who were diagnosed with SGPA and to compare this cohort with previously well-described cohorts. METHODS: We performed a retrospective study that included 37 consecutive SGPA patients who were seen at 2 tertiary centers in Buenos Aires. RESULTS: Nineteen patients (51.3%) were male, and 18 patients (49.7%) were female. The mean age at the onset of symptoms was 48.5 ± 12.01 years. Antineutrophil cytoplasmic antibody (ANCA) was detected in 34 patients (91.89%): 32 patients (86.48%) had a cytoplasmic staining pattern, whereas 2 patients (5.40%) had a perinuclear pattern. Three patients were ANCA-negative. Twenty-four patients (64%) achieved remission, and 7 patients (19%) had response as defined by at least 50% reduction in the disease activity score. Nineteen relapses were observed in 12 patients, and 2 of the relapses were fatal. Overall, there were 14 deaths (37.83%). CONCLUSIONS: The present series demonstrated that Argentinean patients have similar demographics, clinical manifestations, and outcomes as the cohorts from the northern hemisphere. There was less granulomatous organ involvement (ear/nose/throat, lung granulomas) in the present cohort compared with other series.

Lesión destructiva de la línea media, a propósito de un caso / Midline destructive lesion. Case report

Introducción: El síndrome destructivo de la línea media es una entidad caracterizada por un conjunto de signos y síntomas secundarios a la afección, generalmente destructiva, de localización medio facial. Objetivos: Presentación de caso clínico y revisión bibliográfica. Reporte de caso: Varón de 38 años procedente de Chaco con diagnóstico de Leishmaniasis mucocutánea que cursó internación por una lesión destructiva de la línea media que luego de múltiples planteos diagnósticos y estudios complementarios se llega al diagnóstico final de vasculitis granulomatosa con poliangeitis (granulomatosis de Wegener).Discusión Estos pacientes representan un desafío diagnóstico en medicina interna, debido a las múltiples etiologías capaces de desarrollarlo, con manifestaciones clínicas similares pero con pronóstico y tratamiento diferente.

Introduction:Midline destructive syndrome is an entity characterized by a group of signs and symptoms, secondary to the condition, usually destructive and located in the middle of the face.Objectives: Clinical case report and literature review.Case Report: 38 year old male patient, from Chaco, with mucocutaneous Leishmaniasis diagnosis, who was hospitalized due to a midline destructive lesion. After multiple diagnostic options and complementary studies, the final diagnosis ofgranulomatosis with polyangiitis vasculitis (Wegener's granulomatosis) is reached. Discussion: These patients pose a diagnostic challenge in internal medicine, due to the multiple etiology factors that can be responsible of its development, with similar clinical symptoms but with a different treatment and prognosis.

Síndrome pulmo-renal en poliangeitis con granulomatosis (Granulomatosis de Wegener): estudio de 13 casos / Pulmonary renal syndrome in granulomatosis with polyangiitis (Wegener’s Granulomatosis): a study of 13 cases

Las vasculitis ANCA asociadas son la causa más frecuente de síndrome pulmo-renal (SPR); dentro de este grupo se halla la poliangeitis con granulomatosis (GPA). El objetivo de este trabajo fue describir las características clínicas y de laboratorio de pacientes con SPR y GPA, y comparar su sobrevida con un grupo de GPA graves sin SPR. Se revisaron retrospectivamente las historias clínicas de 37 casos de GPA pertenecientes a dos centros terciarios de la Ciudad de Buenos Aires. Se incluyeron para el análisis 13 casos con GPA/SPR; 7 fueron de sexo femenino, la media de edad al diagnóstico fue de 48 años. La media de seguimiento fue 4.66 años. El Birmingham Vasculitis Activity Score (BVAS) inicial fue de 31.13 ± 7.99 vs 18.19 ± 4.45 en el grupo de GPA sin SPR. Doce casos fueron ANCA-c positivos. El tratamiento consistió inicialmente en esteroides y ciclofosfamida; en los casos cuyo compromiso renal fue severo (creatinina plasmática 5mg/dll) se realizó plasmaféresis y diálisis. Se obtuvo la remisión de 8 casos; 2 recayeron durante el seguimiento. Se observaron 6 muertes; 2 fueron debidas a la actividad de la enfermedad, 3 debidas a infecciones y 1 a un accidente cerebrovascular. Todos fallecieron dentro de los 12 meses posteriores al diagnóstico. La mortalidad a los 30 días fue 38.46% en el grupo de GPA/SPR vs. 4.16% en el grupo de GPA sin SPR. El SPR es una de las formas más graves de la GPA y conlleva una elevada morbimortalidad. El diagnóstico debe ser rápido, excluyéndose otras posibles etiologías para poder instaurar un adecuado tratamiento que pueda modificar la mortalidad temprana que se observa en este síndrome. La determinación precoz de los ANCAs en el contexto clínico adecuado sería una herramienta de gran utilidad diagnóstica.