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.
As the use of high-dose intravenous gamma globulin and available antimicrobials expands, the incidence of noninfectious common variable immunodeficiency-related disorders, such as ...granulomatous-lymphocytic interstitial lung disease (GLILD), is likely to increase. A thorough clinical evaluation using a multidisciplinary care model is recommended to achieve accurate diagnosis of GLILD. Enough progress has been made in the understanding of GLILD over the last decade that clinicians are now poised to conduct therapeutic trials. Data from large registries and population-based studies of GLILD are needed to better understand the disease trends and burden. This article focuses on the diagnosis and management of GLILD.
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.
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.
Background A recently published report from the United Kingdom suggested an association between sarcoidosis and pulmonary embolism (PE). We sought to examine whether this association was present ...among US decedents with sarcoidosis. Methods We used data from the National Center for Health Statistics to investigate the association between sarcoidosis and PE among US decedents from 1988 to 2007. Results From 1988 to 2007, there were 46,450,489 deaths in the United States and 23,679 decedents with sarcoidosis mentioned on their death certificates. Among these, 602 (2.54%) had PE mentioned on their death certificates, compared with only 1.13% of the background population ( P < .0001 for comparison). The association between sarcoidosis and PE was significant regardless of gender (OR, 2.07; 95% CI, 1.80-2.39; P < .0001 for men and OR, 1.76; 95% CI, 1.59-1.96; P ≤ .0001 for women) or race (OR, 1.57; 95% CI, 1.41-1.76; P < .0001 for blacks and OR, 1.87; 95% CI, 1.63-2.14; P < .0001 for whites). Among decedents with sarcoidosis, there was no difference in risk of PE between men and women (2.30% vs 2.54%, χ2 = 1.32, P = .25) or between blacks and whites (2.60% vs 2.23%, χ2 = 3.09, P = .08). The association between sarcoidosis and PE held regardless of age. Conclusions Using death certificate data from 1988 to 2007, we detected an association between sarcoidosis and PE regardless of gender, race, or age. Further investigation is needed to decipher the mechanisms of this apparent association.
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