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PURPOSE: To assess the prevalence of autoimmune diseases (ADs) associated with ocular cicatricial pemphigoid (OCP) and analyze clinical, laboratory, and treatment associations between these entities. METHODS: A multicentre cross-sectional study of patients with an OCP diagnosis. The population was divided into two groups according to their association with other ADs or not. Clinical, laboratory and treatment variables were described and compared between groups. A multivariable logistic regression analysis was performed to identify variables that could suggest the association between OCP and ADs. RESULTS: Eighty-eight patients were recruited, with a mean age at diagnosis of 64.3 years (SD 11.9). Biopsy was performed in 86.8% of the patients. There was a median delay of 2 years from the onset of symptoms to diagnosis. Extraocular involvement was evidenced in 11.5%. The group associated with ADs included 24 patients (27.3%). The most prevalent diagnosis was Sjögren´s syndrome. Hypergammaglobulinemia was associated with ADs and OCP, adjusted for age, sex, smoking, skin and mucosal involvement, and erythrocyte sedimentation rate (OR 8.7; 95%CI 1.6-46.8; p = 0.012). CONCLUSIONS: Due to OCP's autoimmune nature, it could coexist with other ADs. This study observed that more than a quarter of the population presented with this association, and hypergammaglobulinemia could suggest it.

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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.

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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.

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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.

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Introduction: Giant cell arteritis (GCA) is the most frequent systemic vasculitis in patients older than 50 years. The diagnosis is based on the clinical history, laboratory findings and imaging studies associated with a temporal artery biopsy (TAB). However, the biopsy result could be inconclusive in up to 40% of the cases. The aim of this study was to review the current management of the patients with clinical suspect of GCA in the university hospital CEMIC in Buenos Aires, Argentina, and correlate the disease behavior with the TAB result. Methods: Retrospective study that reviewed consecutive patients to whom a TAB was made in an 11-year period (2005-2016). Clinical and pathology reports were reviewed. Descriptive statistics were performed. Quantitative variables were described as mean (S.D.) or median [range or inter- quartile range (IQR)] and qualitative variables as number (%). To compare the characteristics of the groups, bivariate analyzes were performed using contingency tables and logistic regression models if necessary. Results: Sixty three patients were included, 68% women. The mean age was 72 years old (SD 8.4). Seventeen biopsies (26.9%) were positive for GCA. The average post fixation length was 1.68 cm (SD 1.2). Patients were divided into 3 groups taking into account the result of the TAB, the ACR criteria and the imaging studies. We could not identify predictors of biopsy positivity. The group of patients with GCA and negative TAB showed a higher percentage of patients with abnormal temporal artery at physical examination. Conclusion: The TAB positive percentage (26.9%) was similar to the reported in other series as well as the post fixation length. We could not identify predictors of biopsy positivity.

Introducción: La arteritis de células gigantes (ACG) es la vasculitis sistémica primaria más frecuente en pacientes mayores de 50 años. El diagnóstico de ACG se basa en la evaluación clínica, de laboratorio y estudios por imágenes, asociados a una biopsia. Sin embargo, el resultado de la biopsia puede no ser concluyente en más del 40% de los casos. El objetivo de este estudio fue revisar el manejo de los pacientes con sospecha de ACG en el hospital universitario CEMIC en Buenos Aires, Argentina, y correlacionar el comportamiento de la enfermedad con el resultado de la biopsia de arteria temporal (BAT). Métodos: Estudio retrospectivo que analizó pacientes consecutivos a los cuales se les realizó BAT en un período de 11 años (2005-2016). La información recolectada se obtuvo a partir de las historias clínicas y de los informes de anatomía patológica. Se realizó estadística descriptiva. Para las variables cuantitativas se estimaron medias y sus respectivos desvíos estándar o medianas y percentil 25-75, y para las variables cualitativas, la cantidad y el porcentaje. Para comparar las características de los grupos se realizaron análisis bivariados mediante tablas de contingencia y modelos de regresión logística de ser necesario. Resultados: Sesenta y tres pacientes fueron incluidos, 68% mujeres, con una edad media de 72 años (DS 8,4). Diecisiete biopsias (26,9%) fueron positivas. La longitud media posfijación fue de 1,68 cm (DS 1,2). La población se dividió en 3 grupos según la BAT, los criterios ACR y los estudios por imágenes. No se identificaron factores predictores de positividad de la BAT. El grupo con ACG y BAT negativa presentó mayor porcentaje de pacientes con arteria temporal anormal al examen físico. Conclusión: El porcentaje de positividad de las biopsias (26,9%) fue similar al reportado por otras series, así como la longitud de la biopsia luego de la fijación (1,68 cm). No identificamos factores predictores de positividad de la BAT.

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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.

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Central nervous system demyelinating processes such as multiple sclerosis and acute disseminated encephalomyelitis constitute a group of diseases not completely understood in their physiopathology. Environmental and toxic insults are thought to play a role in priming autoimmunity. The aim of the present report is to describe a case of acute demyelinating disease with fatal outcome occurring 15 days after oral exposure to herbal extracts.