Clinical risk factors in patients with interstitial lung disease associated with anti-MDA5 autoantibodies.

INTRODUCTION: The anti-MDA5-associated autoimmune disease represents a poorly understood entity. The study's objectives were to describe a cohort of interstitial lung disease (ILD) patients who were positive for anti-MDA5 autoantibody and identify clinical risk factors associated with survival. METHODS: This single-center cohort study included ILD patients positive for anti-MDA5 autoantibody. Baseline clinical features were registered, and survival analysis was performed to identify risk factors associated with worse survival. RESULTS: Fifty-three ILD-MDA5 positive patients were included; twelve died during follow-up due to rapidly progressive interstitial lung disease (RP-ILD). Dermatological signs of anti-MDA5 (Gottron papules, Gottron sign, palmar papules, V-neck sign, facial dermatomyositis rashes, and skin ulcers) were strongly associated with death secondary to RP-ILD (HR: 3.7, 95% CI: 1.02-13.35). Patients with dermatological signs were younger, had higher anti-MDA5 autoantibodies titers, more frequent inflammatory patterns in HRCT evaluation, and less fibrosis extent in HRCT. CONCLUSION: Dermatological manifestation in ILD patients to anti-MDA5 autoantibodies are associated with RP-ILD and short-term fatal outcomes. Dermatological signs may identify a subgroup of ILD-positive to anti-MDA5 patients with a high risk of RP-ILD.

An Open-label Study With Pirfenidone on Chronic Hypersensitivity Pneumonitis.

BACKGROUND: Chronic hypersensitivity pneumonitis (cHP) represents a severe lung disease often evolving to fibrosis with the subsequent destruction of the lung parenchyma. There are no approved therapies with confirmed efficacy to deal with this disease. METHODS: We performed an open-label, proof of concept study, to evaluate the efficacy and safety of pirfenidone added to immunosuppressive drugs on the treatment of cHP. We included 22 patients assigned to two groups: Group 1, nine patients that received prednisone plus azathioprine and Group 2, thirteen patients, received prednisone plus azathioprine and pirfenidone (ClinicalTrials.gov identifier NCT02496182). There were no significant imbalances in clinically relevant baseline characteristics between two study groups. RESULTS: After 1 year of treatment, inclusion of pirfenidone was not associated with improved forced vital capacity (primary end-point). A not significant tendency to show higher improvement of diffusion capacity of the lung for carbon monoxide (DLCO) was observed in the group receiving pirfenidone (p=0.06). Likewise, a significant improvement in the total score on the SGRQ was found in the group 2 (p=0.02) without differences in other two questionnaires related to quality of life (ATAQ-IPF and EQ-5D-3L). HRCT showed a decrease of the ground glass attenuation without changes in the fibrotic lesions and without differences between both groups. CONCLUSIONS: These findings suggest that the addition of pirfenidone to the anti-inflammatory treatment in patients with chronic HP may improve the outcome with acceptable safety profile. However, prospective randomized double-blind, placebo-controlled trials in largest cohorts are needed to validate its efficacy.

Pulmonary Involvement in Systemic Vasculitis.

PURPOSE OF REVIEW: The purpose of this study is to describe the most relevant advances concerning lung involvement in the ANCA-associated vasculitides (excluding eosinophilic granulomatosis with polyangiitis which may have different disease mechanisms). Focus is on pathophysiology, recent important imagenological procedures, treatment, and outcome. RECENT FINDINGS: Emerging information exists on potential newly investigated diagnostic procedures (v.g. transbronchial cryobiopsies), detailed tomographic abnormalities, the potential favorable role of rituximab and the still uncertain one of plasma exchange in the treatment, and the increasing description of interstitial lung disease. Survival is reduced in case of both, diffuse alveolar hemorrhage and diffuse parenchymal disease. There is the need to expand the knowledge concerning better long-term treatment options with specific regimes, and to incorporate other measures regarding integral treatment in patients afflicted with lung involvement these maladies, as the outcome seems adverse in this scenario.

Valor pronóstico de la PCT y proteína C reactiva como marcadores de gravedad en neumonía adquirida en la comunidad en adultos mayores / Prognostic value of PCT and CRP as markers of severity in community acquired pneumonia in older adults

Introducción: La neumonía adquirida en la comunidad (NAC) es una de las principales causas de mortalidad en el mundo. La proteína C reactiva puede identificar a pacientes críticamente enfermos. La procalcitonina (PCT) ha sido referida como un marcador sensible de gravedad de la infección bacteriana y sepsis. Metodología: Se realizó un estudio cohorte prospectivo en el servicio de geriatría del Centro Médico ISSEMYM, Metepec, Estado de México, con todos los pacientes que ingresaron a hospitalización con diagnóstico de NAC entre mayo 2012 a marzo 2013. Se midieron PCR, PCT y laboratorios de rutina. Para la comparación de variables continuas se utilizó la T de Student ó U de Mann Whitney según su distribución. Para la comparación de variables cate-góricas se utilizo la prueba de X2. Para el análisis de supervivencia se utilizó el estimador de Kaplan-Meier. Para establecer el riesgo de mortalidad se empleó el modelo de regresión de COX obteniendo el Hazard Ratio. Para la correlación entre los niveles séricos de PCT y PCR se utilizó el coeficiente de correlación de Spearman. Resultados: Se registraron los datos de un total de 82 pacientes. La supervivencia media cuando PCT > de 0.5 ng/dl fue de 17 días (IC 95%, 11 a 23 días) versus 26 días (IC 95%, 17 a 35 días) para PCT < de 0.5 ng/dl (p < 0.01). Conclusiones: El nivel sérico de PCT mayor a 0.5 ng/dl mostró ser un marcador pronóstico en pacientes geriátricos con neumonía...(AU)