Integrating Clinics, Laboratory, and Imaging for the Diagnosis of Common Variable Immunodeficiency-Related Granulomatous-Lymphocytic Interstitial Lung Disease.

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.

Artralgias migratorias: manifestación inicial de toxicidad sistémica asociada al tratamiento crónico con nitrofurantoína / Migratory arthralgia as the initial sign of systemic toxicity associated with chronic nitrofurantoin treatment

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[Impact of baseline and induced dyspnea on the quality of life of patients with COPD]. / Importancia de la disnea basal e inducida en la calidad de vida de los pacientes con EPOC.

OBJECTIVE: Dyspnea is the main symptom of chronic obstructive pulmonary disease (COPD) and as such is an important determinant of health-related quality of life. It is, however, weakly correlated to severity of obstruction and there is little information available on how it exercises its effect on health-related quality of life. The aims of this study were to identify the determinants of baseline dyspnea and to ascertain how that factor influences the health-related quality of life of patients with COPD. PATIENTS AND METHODS: A total of 101 patients with COPD were studied. Tests included full lung function assessment, the bronchial provocation test (n=70), and the 6-minute walk test. The following variables were measured: Baseline dyspnea, bronchoconstriction-induced dyspnea, exertional dyspnea, health-related quality of life, and levels of anxiety and depression. RESULTS: Determinants of baseline dyspnea were anxiety (explained variance, 17%), maximal inspiratory pressure (4%), and PaO2 (4%). In patients with mild to moderate COPD (forced expiratory volume in 1 second, >50% of predicted), the main determinant of health-related quality of life was anxiety (explained variance, 43%). Other determinants were the number of meters walked in the 6-minute-walk test, age, and Baseline dyspnea (variance explained by both factors, 26%). Baseline dyspnea and bronchoconstriction-induced dyspnea were both identified as independent determinants of health-related quality of life (on the activity and impact subscales of the St George's Respiratory Questionnaire, respectively). The main determinant of health-related quality of life in patients with severe COPD (forced expiratory volume in 1 second, < or =50% of predicted) was baseline dyspnea. Finally, the main determinants of anxiety were exertional dyspnea (variance, 42%) and baseline dyspnea (6%). CONCLUSIONS: Anxiety is the main determinant of health-related quality of life in patients with COPD, and it is triggered mainly by baseline dyspnea and exertional dyspnea.

Determinants of dyspnea in patients with different grades of stable asthma.

Dyspnea is a main feature of symptomatology in asthma, and its perception does not necessarily correlates well with airway obstruction. The aim of this study was twofold: (1) to identify factors determining the subjective degree of dyspnea in patients with different grades of stable bronchial asthma and (2) to compare various clinical methods existing for grading dyspnea. The investigation comprised 153 outpatients with stable asthma. The parameters studied were the following: demographic characteristic of subjects, baseline dyspnea score by means of three clinical instruments (baseline dyspnea index [BDI], Medical Research Council [MRC] scale, and modified Borg scale), asthma severity, standard measures of physiologic lung function, anxiety, depression, subconscious illness attention, and asthma-related quality of life (HRQOL). The dyspnea scores were all significantly interrelated (r=0.77-0.85, p<0.001). The three clinical scales for grading dyspnea were significantly correlated with the same parameters: airflow obstruction, lung hyperinflation, emotional factors, HRQOL, age, age at asthma onset, asthma duration, female gender, clinical severity, and lower economical, and educational levels. Multiple regression analysis showed that independent factors determining clinical dyspnea scores were: age, airway obstruction, and emotional status. Moreover, in patients with severe asthma, lung hyperinflation helped to explain the individual dyspnea score. These data suggest that clinical methods are appropriate for evaluating the impact of dyspnea on daily activities of asthmatic patients. BDI, MRC, and Borg clinical dyspnea scales showed similarly information in subjects with asthma. Independently of asthma severity, older age, airway obstruction, and psychological disturbance were associated with higher degree of dyspnea. However, if subjects had severe airway obstruction, lung hyperinflation was a major determinant of baseline dyspnea score.

Gender differences in health-related quality of life among patients with asthma.

This study has a twofold objective: 1) to explore to what extent suffering from asthma affects the HRQL of men and women differently at several stages of disease severity and 2) to analyze whether the informed poorer HRQL of asthmatic women is related to their higher scores on instruments measuring emotionally disordered symptoms. One hundred fifty-one outpatient asthmatics (84 women and 67 men) completed the Spanish versions of the Asthma Quality of Life questionnaire (AQL), as well as anxiety and depression inventories. A full history, physical examination, and pulmonary function test were performed on all subjects. Patients were classified into one of four asthma severity categories following the criteria of the Global Initiative on Asthma (GINA). There were no gender differences in sociodemographic variables, asthma duration, GINA, FEV1 or dyspnea. However, women showed a poorer HRQL than men, as well as high degrees of anxiety and depression. When these data were reanalyzed taking into account the four groups of asthma severity, women only reported a poorer HRQL than men at the intermittent asthma level. The gender differences on depression and anxiety scores were maintained at the three lower severity levels, but not at the most severe asthma degree. When depression and anxiety scores were partialed out, the AQL scores maintained significant relationships with asthma severity, dyspnea, and FEV1, both in women and men. Therefore, only in men were there also relationships among AQL and sociodemographic data. The best predictor of the women's HRQL was the dyspnea score, whereas in men it was the asthma severity (GINA).