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 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.
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.
Background Lymphangiogenesis responds to tissue injury as a key component of normal wound healing. The development of fibrosis in the idiopathic interstitial pneumonias may result from abnormal wound ...healing in response to injury. We hypothesize that increased lymphatic vessel (LV) length, a marker of lymphangiogenesis, is associated with parenchymal components of the fibroblast reticulum (organizing collagen, fibrotic collagen, and fibroblast foci), and its extent correlates with disease severity. Methods We assessed stereologically the parenchymal structure of fibrotic lungs and its associated lymphatic network, which was highlighted immunohistochemically in age-matched samples of usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP) with FVC < 80%, COPD with a Global Initiative for Obstructive Lung Disease stage 0, and normal control lungs. Results LV length density, as opposed to vessel volume density, was found to be associated with organizing and fibrotic collagen density ( P < .0001). Length density of LVs and the volume density of organizing and fibrotic collagen were significantly associated with severity of both % FVC ( P < .001) and diffusing capacity of the lung for carbon monoxide ( P < .001). Conclusions Severity of disease in UIP and NSIP is associated with increased LV length and is strongly associated with components of the fibroblast reticulum, namely organizing and fibrotic collagen, which supports a pathogenic role of LVs in these two diseases. Furthermore, the absence of definable differences between UIP and NSIP suggests that LVs are a unifying mechanism for the development of fibrosis in these fibrotic lung diseases.
This is a case of a 55-year-old man with Roux-en-Y gastric bypass surgery 15 years prior who presented with acute pancreatitis and developed distributive shock, bacteremia, acute respiratory distress ...syndrome, anuric acute renal failure, and a distended abdomen with increasing ascitic fluid on imaging. An elevated bladder pressure, lactic acidosis, and anuria raised concern for abdominal compartment syndrome. Paracentesis was done and four liters of bilious ascitic fluid were drained. Intra-abdominal pressure was measured and improved from 27 cmH2O to 13 cmH2O with paracentesis. Mean arterial pressure and urine output also improved. The patient developed recurrent loculated intra-abdominal fluid collections, though ultrasound, CT scans with and without contrast, MRCP, ERCP, upper GI fluoroscopy, and small bowel enteroscopy failed to reveal a source of the bilious output. Ultimately, a gastrostomy tube was placed and delivery of contrast material through the tube revealed active extravasation from the remnant stomach. This case underscores the importance of considering post-surgical leak regardless of how remotely a Roux-en-Y surgery took place, confirms the importance of pursuing early gastrostomy tube placement and contrast administration when post-Roux-en-Y gastric remnant leaks are suspected, and demonstrates the role of paracentesis in critically ill patients with abdominal compartment syndrome.
PDF
