Background The cornerstone of hypersensitivity pneumonitis (HP) management is having patients avoid the inciting antigen (IA). Often, despite an exhaustive search, an IA cannot be found. The ...objective of this study was to examine whether identifying the IA impacts survival in patients with chronic HP. Methods We used the Kaplan-Meier method to display, and the log-rank test to compare, survival curves of patients with well-characterized chronic HP stratified on identification of an IA exposure. A Cox proportional hazards (PH) model was used to identify independent predictors in time-to-death analysis. Results Of 142 patients, 67 (47%) had an identified IA, and 75 (53%) had an unidentified IA. Compared with survivors, patients who died (n = 80, 56%) were older, more likely to have smoked, had lower total lung capacity % predicted and FVC % predicted, had higher severity of dyspnea, were more likely to have pulmonary fibrosis, and were less likely to have an identifiable IA. In a Cox PH model, the inability to identify an IA (hazard ratio HR, 1.76; 95% CI, 1.01–3.07), older age (HR, 1.04; 95% CI, 1.01–1.07), the presences of pulmonary fibrosis (HR, 2.43; 95% CI, 1.36–4.35), a lower FVC% (HR, 1.36; 95% CI, 1.10–1.68), and a history of smoking (HR, 2.01; 95% C1, 1.15–3.50) were independent predictors of shorter survival. After adjusting for mean age, presence of fibrosis, mean FVC%, mean diffusing capacity of the lung for carbon monoxide (%), and history of smoking, survival was longer for patients with an identified IA exposure than those with an unidentified IA exposure (median, 8.75 years vs 4.88 years; P = .047). Conclusions Among patients with chronic HP, when adjusting for a number of potentially influential predictors, including the presence of fibrosis, the inability to identify an IA was independently associated with shortened survival.
Abstract Purpose Interstitial lung disease is a common extra-articular manifestation of rheumatoid arthritis (RA-ILD) and is associated with significant morbidity and mortality. However, limited data ...exist regarding predictors of mortality. We sought to examine the prognostic value of the high-resolution computed tomography (HRCT) patterns in patients with RA-ILD. Materials and methods RA-ILD patients with HRCT patterns of usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP) were identified among a longitudinal cohort of individuals evaluated at National Jewish Health. A total of 158 subjects were included in the study. For each subject, the earliest available HRCT was reviewed independently by two expert thoracic radiologists blinded to clinical data. HRCT patterns were classified as demonstrating definite UIP, possible UIP, or NSIP. Kaplan-Meier curves were generated and survival was compared among the three patterns using a log rank test for trend. Results One hundred subjects (63%) had HRCT findings classified as definite UIP, 23 (15%) as possible UIP and 35 (22%) as NSIP. No difference in survival was seen between subjects with definite UIP versus those with possible UIP. The combined group of subjects with either definite- or possible UIP had significantly worse survival than those with NSIP (log-rank p = 0.03). Conclusions In patients with RA-ILD, patients with either definite UIP or possible UIP have equally poor survival when compared to those with an NSIP pattern.
BACKGROUND The usual interstitial pneumonia (UIP) pattern of lung injury may occur in the setting of connective tissue disease (CTD), but it is most commonly found in the absence of a known cause, in ...the clinical context of idiopathic pulmonary fibrosis (IPF). Our objective was to observe and compare longitudinal changes in pulmonary function and survival between patients with biopsy-proven UIP found in the clinical context of either CTD or IPF. METHODS We used longitudinal data analytic models to compare groups (IPF n = 321 and CTD-UIP n = 56) on % predicted FVC (FVC %) or % predicted diffusing capacity of the lung for carbon monoxide (Dlco%), and we used both unadjusted and multivariable techniques to compare survival between these groups. RESULTS There were no significant differences between groups in longitudinal changes in FVC % or Dlco% up to diagnosis, or from diagnosis to 10 years beyond (over which time, the mean decrease in FVC % per year 95% CI was 4.1 3.4, 4.9 for IPF and 3.5 1.8, 5.1 for CTD-UIP, P = .49 for difference; and the mean decrease in Dlco% per year was 4.7 4.0, 5.3 for IPF and 4.3 3.0, 5.6 for CTD-UIP, P = .60 for difference). Despite the lack of differences in pulmonary function, subjects with IPF had worse survival in unadjusted (log-rank P = .003) and certain multivariable analyses. CONCLUSIONS Despite no significant differences in changes in pulmonary function over time, patients with CTD-UIP (at least those with certain classifiable CTDs) live longer than patients with IPF—an observation that we suspect is due to an increased rate of mortal acute exacerbations in patients with IPF.
In 2000, the American Thoracic Society and European Respiratory Society published the first consensus statement providing guidelines on the diagnosis and treatment of idiopathic pulmonary fibrosis ...(IPF). This statement presented, for the first time, diagnostic criteria for IPF and recommendations for treatment. Results from several studies have reshaped the thinking on IPF, and as a result, the guidelines have been recently revised using an evidence-based approach. Meanwhile, several epidemiologic studies have yielded data that identify potential risk factors and that better define the societal burden of IPF. This article summarizes the approach to diagnosing IPF and reviews epidemiologic data on IPF.
Eosinophilic lung diseases Fernández Pérez, Evans R; Olson, Amy L; Frankel, Stephen K
The Medical clinics of North America,
11/2011, Letnik:
95, Številka:
6
Journal Article
Recenzirano
Accurate diagnosis of eosinophilic lung diseases is essential to optimizing patient outcomes, but remains challenging. Signs and symptoms frequently overlap among the disorders, and because these ...disorders are infrequent, expertise is difficult to acquire. Still, these disorders are not rare, and most clinicians periodically encounter patients with one or more of the eosinophilic lung diseases and need to understand how to recognize, diagnose, and manage these diseases. This review focuses on the clinical features, general diagnostic workup, and management of the eosinophilic lung diseases.
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