RESUMEN
Background: Granulomatous-lymphocytic interstitial lung disease (GLILD) is a distinct clinic-radio-pathological interstitial lung disease (ILD) that develops in 9% to 30% of patients with common variable immunodeficiency (CVID). Often related to extrapulmonary dysimmune disorders, it is associated with long-term lung damage and poorer clinical outcomes. The aim of this study was to explore the potential use of the integration between clinical parameters, laboratory variables, and developed CT scan scoring systems to improve the diagnostic accuracy of non-invasive tools. Methods: A retrospective cross-sectional study of 50 CVID patients was conducted in a referral unit of primary immune deficiencies. Clinical variables including demographics and comorbidities; analytical parameters including immunoglobulin levels, lipid metabolism, and lymphocyte subpopulations; and radiological and lung function test parameters were collected. Baumann's GLILD score system was externally validated by two observers in high-resolution CT (HRCT) scans. We developed an exploratory predictive model by elastic net and Bayesian regression, assessed its discriminative capacity, and internally validated it using bootstrap resampling. Results: Lymphadenopathies (adjusted OR 9.42), splenomegaly (adjusted OR 6.25), Baumann's GLILD score (adjusted OR 1.56), and CD8+ cell count (adjusted OR 0.9) were included in the model. The larger range of values of the validated Baumann's GLILD HRCT scoring system gives it greater predictability. Cohen's κ statistic was 0.832 (95% CI 0.70-0.90), showing high concordance between both observers. The combined model showed a very good discrimination capacity with an internally validated area under the curve (AUC) of 0.969. Conclusion: Models integrating clinics, laboratory, and CT scan scoring methods may improve the accuracy of non-invasive diagnosis of GLILD and might even preclude aggressive diagnostic tools such as lung biopsy in selected patients.
Asunto(s)
Inmunodeficiencia Variable Común , Enfermedades Pulmonares Intersticiales , Teorema de Bayes , Inmunodeficiencia Variable Común/complicaciones , Inmunodeficiencia Variable Común/diagnóstico por imagen , Estudios Transversales , Humanos , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/etiología , Estudios RetrospectivosRESUMEN
La necrólisis epidérmica tóxica es una patología de escasa ocurrencia, que se acompaña de mortalidad elevada y se caracteriza por lesiones cutáneas vesículo ampollosas que con frecuencia afecta la mucosa respiratoria, digestiva y ocular. Se presenta un caso de la enfermedad atendido en el Hospital Universitario de Santander en octubre del año 2007 desencadenado por el tratamiento con fenitoína ácido valproico como terapia anticonvulsiva, con desenlace fatal y al cual se le practicó autopsia médico científica. Se pretende con este trabajo presentar una revisión del tema y analizar los factores relacionados con esta grave condición clínica...
Toxic epidermal necrolysis is an infrequent pathology, accompanied of high mortality risk in concordance with extension of comprised skin characterized for blisters. Frequently these are present in respiratory, digestive and ocular mucous. An autopsy case is presented occurred in Hospital Universitario de Santander in October 2007 of toxic epidermal necrolysis secondary to treatment with phenytoin associated with valproic acid as anticonvulsivant therapy for messial hippocampal sclerosis. It pretends with this work show a review and to analyze related factors with this condition...