Vasculitis cutáneas: etiología y características clínicas en pacientes adultos en un centro de tercer nivel / Cutaneous vasculitis: etiology and clinical characteristics in adult patients at a third-level center

Introducción: Diversas entidades clínicas, como enfermedades autoinmunes, infecciones, neoplasias y fármacos pueden manifestarse con lesiones vasculíticas en la piel. Debido a la heterogeneidad de las causas, suelen representar un desafío diagnóstico. El objetivo de este estudio es describir la etiología de las vasculitis cutáneas (VC) y evaluar las características clínicas, histológicas y de laboratorio halladas en estos pacientes. Material y métodos: Se realizó un estudio retrospectivo con revisión de historias clínicas de pacientes mayores de 16 años con VC por diagnóstico clínico y/o histológico evaluados en el período 2010-2018. Resultados: Se incluyeron 74 pacientes. El 69% son mujeres con una edad media al diagnóstico de 41 años (DE 16.5, rango 16-75). Las causas más frecuentes asociadas a las VC fueron las enfermedades autoinmunes (EAI) en un 50% de los pacientes evaluados. En el 29.7% de los casos no pudo identificarse una causa subyacente. En el 2.7% de los casos se asoció a neoplasias, otro 2.7% a fármacos y un 12% a otras etiologías. El 76% de los pacientes presentaron formas clínicas no severas, predominando la púrpura palpable en el 65% de los casos. Entre los pacientes biopsiados, el 76% fueron vasculitis leucocitoclásticas (VLC). Como manifestaciones extracutáneas asociadas, predominó el compromiso articular (43,2%). En las vasculitis asociadas a EAI, el 33 % presentó compromiso renal, en tanto que éste no se observó en ninguno de los pacientes con vasculitis idiopáticas. El 78% de los pacientes recibieron glucocorticoides sistémicos. Conclusión: En nuestro centro, la etiología más común de VC fue la asociada a EAI. La mayoría de los pacientes eran mujeres. Clínicamente predominaron las manifestaciones cutáneas no severas y la VLC fue el hallazgo más frecuente en las biopsias.

Background: Various clinical entities, such as autoimmune diseases, infections, neoplasms and drugs can manifest with vasculitic lesions on the skin. Due to the heterogeneity of causes, they often represent a diagnostic challenge. The aim of this study is to describe the etiology of cutaneous vasculitis (CV) and to assess the clinical, histological and laboratory characteristics found in these patients. Material and methods: A retrospective study was carried out with a review of the medical records of patients over 16 years old with CV by clinical and/or histological diagnosis evaluated in the period 2010-2018. Results: 74 patients were included. 69% are women with a mean age at diagnosis of 41 years (SD 16.5, range 16-75). The most frequent causes associated with CVs were autoimmune diseases (AID) in 50% of the patients evaluated. In 29.7% of the cases, an underlying cause could not be identified. In 2.7% of the cases it was associated with neoplasms, another 2.7% with drugs, and 12% with other etiologies. 76% of the patients presented non-severe clinical forms, with palpable purpura predominant in 65% of the cases. Among the biopsied patients, 76% were leukocytoclastic vasculitis (LCV). As associated extracutaneous manifestations, joint involvement predominated (43.2%). In vasculitis associated with AID, 33% presented renal involvement, while this was not observed in any of the patients with idiopathic vasculitis. 78% of the patients received systemic glucocorticoids. Conclusion: In our center, the most common etiology of CV was associated with AID. Most of the patients were women. Clinically, non-severe skin manifestations predominated and VLC was the most frequent finding in biopsies.

Validation of the adjusted global antiphospholipid syndrome score in systemic lupus erythematosus patients in Argentina.

INTRODUCTION: Assessment of risk both for pregnancy morbidity and thrombosis in the presence of anti-phospholipid antibodies (aPL) is still a challenge in Systemic Lupus Erythematosus (SLE) patients. The Global Antiphospholipid Syndrome Score (GAPSS) takes into account the aPL profile (criteria and non-criteria aPL), the conventional cardiovascular risk factors and the autoimmune antibody profile. An adjusted model of the score (aGAPSS) excluding anti-phosphatidylserine/Prothrombin (aPS/PT), suggests that the score is able to stratify patients for their rate of events making it widely applicable in daily clinical practice. OBJECTIVE: To validate the aGAPSS in a multicentric cohort of SLE patients in Argentina. PATIENTS AND METHODS: consecutive SLE patients with and with andwithout thrombotic events from seven Rheumatologist centers were included. Traditional cardiovascular risk factors, aPL antibodies and medications received (aspirin, hydroxychloroquine and anticoagulation) were collected. The score aGAPSS was calculated for each patient at the last visit by adding together the points corresponding to the risk factors: 1 for hypertension, 3 for dyslipidemia, 4 for LA and B2GPI (IgM or IgG) antibodies and 5 for aCL (IgM or IgG) antibodies. The discriminative ability of the aGAPSS was calculated by measuring the area under the receiver operating characteristic curve (AUC). Multivariate logistic regression analysis was performed to examine the impact of multiple cardiovascular risk factors and laboratory parameters on the occurrence of thrombosis. RESULTS: Two hundred and ninety-six SLE patients were included. One-hundred and twenty-one patients (40.9%) presented thrombotic and/or pregnancy complications. Median aGAPSS was significantly higher in patients who experienced an event (thrombosis and/or pregnancy morbidity) compared with those without [4 (IQR 1-9) versus 1 (IQR 0-5); p < 0.001]. The best cut off point for the diagnosis of thrombosis and/or pregnancy complications was aGAPSS ≥4. Multivariate logistic regression analysis showed that aCL antibodies [OR 2.1 (95% CI 1.16-3.90); p = 0.015] were an independent risk factors for thrombotic events. CONCLUSIONS: This score is a simple tool, easy to apply to SLE patients in daily practice. The use of the aGAPSS could change the non-pharmacologic and pharmacologic treatment in higher risk patients to improve their survival.

Lesiones destructivas de la línea media: diagnóstico diferencial con Vasculitis Asociada a ANCA / Destructive lesions of midline: differential diagnosis with ANCA-associated Vasculitis

Presentamos los casos clínicos de tres pacientes adultos jóvenes de origen boliviano, que fueron hospitalizados en salas de clínica médica de un hospital de tercer nivel por manifestaciones de la vía aérea superior y lesiones de las estructuras de la línea media. Reumatología evaluó la posibilidad del diagnóstico de Vasculitis asociada a ANCA, la cual es un diagnóstico diferencial de la entidad conocida como "lesión destructiva de la línea media". En todos los casos se arribó al diagnóstico definitivo de Linfoma T luego de un exhaustivo estudio histopatológico.

We present clinical cases of three Bolivian young adults who were hospitalized in the medical clinic rooms of a third level hospital for upper airway manifestation and lesions of the midline structures. Rheumatology service evaluated the diagnosis of ANCA associated Vasculitis, which is a differential diagnosis of the entity known as midline destructive lesion. In all cases, the final diagnosis of T lymphoma was reached after an exhaustive histopathological study.

Outcomes in biopsy-proven lupus nephritis: evaluation of 190 white patients from a single center.

We describe the natural history of lupus nephritis (LN) in a historical cohort of 190 white patients with the diagnosis of biopsy-proven LN followed in a single reference center.We evaluated 670 patients with systemic lupus erythematosus (SLE) consecutively followed in our department from 1970 until 2006. All patients fulfilled the 1997 revised criteria for the classification of SLE. White patients (Spanish-born) with biopsy-proven LN were selected as the study population.The cohort included 190 patients (170 female patients and 20 male) with a mean age at LN diagnosis of 31 years. Renal biopsy revealed type I LN in 8 (4%) patients, type II in 33 (17%), type III in 46 (24%), type IV in 72 (38%), type V in 28 (15%), and type VI in 3 (2%) patients. Induction remission was achieved in 85% of patients with types I and II, 78% with type III, 70% with type IV, and 32% of patients with type V. After a mean follow-up of 2391 patient-years, 62 (33%) patients developed chronic renal failure and 18 (9%) evolved to end-stage renal disease. Adjusted multivariate Cox regression analysis identified male sex (hazard ratio [HR], 4.33) and elevated creatinine at LN diagnosis (HR, 5.18) as independent variables for renal failure. Survival was 92% at 10 years of follow-up, 80% after 20 years, and 72% after 30 years.Our results suggest that biopsy-proven LN in white patients has an excellent prognosis. Ethnicity should be considered a key factor when evaluating the prognosis and therapeutic response to different agents in patients with LN.

Factores de riesgo cardiovascular asociados a trombosis en lupues eritematoso sistémico / Cardiovascular risk factors associated with thrombosis in systemic lupus erythematosus

Objetivo: Determinar la prevalencia de factores de riesgo cardiovascular en un grupo de pacientes con lupus eritematoso sistémico (LES) y su asociación con eventos trombóticos arteriales y/o venosos. Pacientes y métodos: Se estudiaron los siguientes factores de riesgo cardiovascular en cien pacientes con diagnóstico de LES...

Toxocarosis neurológica: descripción de un caso clínico / Neurologic toxocariasis: description of clinical case

La toxocarosis es una parasitosis producida por los nematodos Toxocara canis y Toxocara felis cuyos huéspedes naturales son los cánidos y félidos domésticos, respectivamente. La infección humana ocurre de manera accidental por la ingesta de huevos larvados presentes en la tierra, arena, agua o alimentos contaminados. Abarca una diversidad de presentaciones clínicas que sumadas a la inespecifidad de la sintomatología y a la dificultad para acceder a un diagnóstico de laboratorio preciso, lleva a que la mayoría de los profesionales no piensen en esta helmintiasis. EL presente trabajo describe un caso clínico de toxocarosis neurológica, diagnóstica, tratamiento y epidemiología. Paciente femenino de 42 años de edad, con historia de contacto íntimo con cachorros y perras preñadas que desarrolló compromisos neurológicos (paraparesia y vejiga neurógena espástica) asociada a leucocitosis con eosinofilia intensa y persiste (11.766 eosinófilos/mm3), hepatomegalia y serología positiva a Toxocara canis determinada por Elisa y confirmada por Western-blot. Tratamiento: albendazol 400 mg/ día cada 12 hs por vía oral durante 7 días y metilprednisona 80 mg/ día por vía oral durante 3 semanas seguidas de una disminución paulatina de la dosis. El estudio epidemiológico consistió en diagnóstico serológico a convivientes de la paciente, análisis parasitológicos a las heces de su perro y a muestras de tierra procedentes de su domicilio, resultando en todos casos positivos para Toxocara canis