Ocular cicatricial pemphigoid: is there an association with autoimmune diseases?

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.

Cobertura de medicamentos de alto costo para enfermedades huérfanas en Argentina. Aspectos conceptuales, metodológicos y de aplicabilidad para informar la toma de decisiones / Coverage of high-cost drugs for orphan diseases in Argentina. Conceptual, methodological and applicability aspects to inform decision making

INTRODUCCIÓN El tratamiento de las enfermedades poco frecuentes (EPOF) involucra en parte medicamentos caracterizados por tener un costo de adquisición muy elevado, por lo que suelen representar un impacto económico elevado para los sistemas sanitarios y muchas veces para los mismos pacientes. Este estudio propone herramientas con el fin de asistir a priorizar y justificar políticas que puedan hacer frente a estos dilemas. METODOS se llevaron a cabo diferentes componentes de análisis específicos con el fin de describir el estado de situación local e internacional en relación a la cobertura de estos medicamentos; desarrollar una metodología de impacto presupuestario en salud que sea de utilidad para los financiadores en estos medicamentos, junto con el análisis de un caso; y proponer una serie de criterios que deberían ser considerados a nivel local en la toma de decisión sobre la cobertura de estos medicamentos. RESULTADOS La fragmentación del sistema de salud argentino dificulta el acceso oportuno a los tratamientos de las EPOF. Por otro lado, es notorio el avance en esta temática en países de altos ingresos, que demostraron presentar sólidos criterios al momento de definir la financiación, cobertura y/o reembolso de estos medicamentos. Los hallazgos obtenidos de análisis locales demuestran que la incorporación de medicamentos para el tratamiento de enfermedades huérfanas avanza sobre los presupuestos de salud en los últimos años. Se recomiendan entonces criterios específicos con relación al proceso de favorecer el acceso de la población a estas tecnologías, consideradas de alto valor social. DISCUSIÓN Los hallazgos de este estudio contribuyen a avanzar en el análisis y posibles soluciones de los problemas que surgen en lagestión de las EPOF en el marco de un sistema de salud fragmentado

Complement levels and risk of organ involvement in patients with systemic lupus erythematosus.

OBJECTIVE: Complement plays a major role in SLE. Complement participation has been linked to disease activity and damage. Our objective was to estimate the association of complement behaviour with clinical manifestations, visceral injury and mortality in patients with SLE. METHODS: Complement determinations (C3 and C4 levels) were analysed in patients with SLE (fulfilling American College of Rheumatology (ACR) or Systemic Lupus International Collaborating Clinics (SLICC)criteria) seen at a university hospital between 2000 and 2013. Patients were grouped in those with permanent C3 and/or C4 low values (low complement group), those with C3 and C4 constant normal values (normal complement group) and those with fluctuant values (periods of normal and periods of low values: fluctuant group). Clinical characteristics and mortality were analysed and compared between groups. RESULTS: 270 patients with SLE were included (242 females, 89.6%), mean age at diagnosis was 34.2 years (SD 15.8). 75 patients had fluctuant levels of complement, 79 patients had persistent low complement levels and 116 had normal complement levels. Lupus glomerulonephritis was more frequent in patients with fluctuant levels (75%, 56% and 49%, respectively, p=0002). The normal complement group had less frequency of haematological involvement and anti-double stranded DNA (dsDNA) antibodies. At the end of the follow-up, 53% of the patients had damage (SLICC/ACR ≥1). In a Cox proportional hazard model age at diagnosis, neurological impairment, thrombocytopaenia and corticosteroids were associated with more damage, while hydroxychloroquine was a protective factor. There were no differences between complements groups on accumulated damage. Ten-year survival rate was 93%, 93.5% and 92% for the normal complement group, the persistently low group and the fluctuant group, respectively. CONCLUSIONS: Patients with constant normal complement had lower prevalence of haematological involvement and anti-dsDNA, while patients with fluctuant complement had higher renal impairment. Neither the persistent low complement nor the fluctuant complement groups had increased mortality and/or visceral damage.

Incidence and Prevalence of Rheumatoid Arthritis in a Health Management Organization in Argentina: A 15-year Study.

OBJECTIVE: To estimate incidence and prevalence rates of rheumatoid arthritis (RA) in the city of Buenos Aires (CABA), Argentina, using data from a university hospital-based health management organization. METHODS: Global, age-specific, and sex-specific incidence and prevalence rates were calculated for members of the Hospital Italiano Medical Care Program (HIMCP), age ≥ 18 years. Incidence study followed members with continuous affiliation ≥ 1 year from January 2000 to January 2015 until he/she voluntarily left the HIMCP, RA was diagnosed, death, or study finalization. Cases from the Rheumatology Section database, electronic medical records, laboratory database, and pharmacy database were filtered with the 2010 American College of Rheumatology/European League Against Rheumatism criteria. Prevalence was calculated on January 1, 2015, and standardized for CABA. Capture-recapture (C-RC) analysis estimated true population sizes. RESULTS: In the study period, incidence rates (cases per 100,000 person-yrs) were 18.5 (95% CI 16.7-20.4) overall, 25.2 (95% CI 22.4-28.0) for women, and 8.8 (95% CI 6.8-10.8) for men. Prevalence rates (percentage of RA cases in the sample population) were 0.329 (95% CI 0.298-0.359) overall, 0.464 (95% CI 0.417-0.510) for women, and 0.123 (95% CI 0.093-0.152) for men. Standardized CABA prevalence rate was 0.300 (95% CI 0.292-0.307). C-RC adjusted rates were almost the same as unadjusted rates. CONCLUSION: This study's incidence and prevalence rates are in the lower range of the rates found around the world. Our female to male prevalence ratio was 4:1. Our peak incidence age was in the sixth and seventh decades for both sexes.