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Interstitial lung disease (ILD) is a common pulmonary manifestation of rheumatoid arthritis. There is lack of clarity around predictors of mortality and disease behaviour over time in these patients.We identified rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients evaluated at National Jewish Health (Denver, CO, USA) from 1995 to 2013 whose baseline high-resolution computed tomography (HRCT) scans showed either a nonspecific interstitial pneumonia (NSIP) or a "definite" or "possible" usual interstitial pneumonia (UIP) pattern. We used univariate, multivariate and longitudinal analytical methods to identify clinical predictors of mortality and to model disease behaviour over time.The cohort included 137 subjects; 108 had UIP on HRCT (RA-UIP) and 29 had NSIP on HRCT (RA-NSIP). Those with RA-UIP had a shorter survival time than those with RA-NSIP (log rank p=0.02). In a model controlling for age, sex, smoking and HRCT pattern, a lower baseline % predicted forced vital capacity (FVC % pred) (HR 1.46; p<0.0001) and a 10% decline in FVC % pred from baseline to any time during follow up (HR 2.57; p<0.0001) were independently associated with an increased risk of death.Data from this study suggest that in RA-ILD, disease progression and survival differ between subgroups defined by HRCT pattern; however, when controlling for potentially influential variables, pulmonary physiology, but not HRCT pattern, independently predicts mortality. @ERSpublications In rheumatoid-arthritis associated interstitial lung disease, physiology, and not HRCT pattern, predicts mortality

In the ICU, pulmonary edema frequently occurs after blood transfusion. The association between infusion of plasma and the development of suspected or possible TRALI may have important implications with regards to etiology and prevention of this syndrome.

Objective Small series suggest mycophenolate mofetil (MMF) is well tolerated and may be an effective therapy for connective tissue disease-associated interstitial lung disease (CTD-ILD). We examined the tolerability and longitudinal changes in pulmonary physiology in a large and diverse cohort of patients with CTD-ILD treated with MMF. Methods We identified consecutive patients evaluated at our center between January 2008 and January 2011 and prescribed MMF for CTD-ILD. We assessed safety and tolerability of MMF and used longitudinal data analyses to examine changes in pulmonary physiology over time, before and after initiation of MMF. Results We identified 125 subjects treated with MMF for a median 897 days. MMF was discontinued in 13 subjects. MMF was associated with significant improvements in estimated percentage of predicted forced vital capacity (FVC%) from MMF initiation to 52, 104, and 156 weeks (4.9% ± 1.9%, p = 0.01; 6.1% ± 1.8%, p = 0.0008; and 7.3% ± 2.6%, p = 0.004, respectively); and in estimated percentage predicted diffusing capacity (DLCO%) from MMF initiation to 52 and 104 weeks (6.3% ± 2.8%, p = 0.02; 7.1% ± 2.8%, p = 0.01). In the subgroup without usual interstitial pneumonia (UIP)-pattern injury, MMF significantly improved FVC% and DLCO%, and in the subgroup with UIP-pattern injury, MMF was associated with stability in FVC% and DLCO%. Conclusion In a large diverse cohort of CTD-ILD, MMF was well tolerated and had a low rate of discontinuation. Treatment with MMF was associated with either stable or improved pulmonary physiology over a median 2.5 years of followup. MMF appears to be a promising therapy for the spectrum of CTD-ILD.

Rationale: It has been nearly 20 years since sarcoidosis mortality was examined at the population level in the United States. Objectives: To examine mortality rates and underlying causes of death among United States decedents with sarcoidosis from [1988][1989][1990][1991][1992][1993][1994][1995][1996][1997][1998][1999][2000][2001][2002][2003][2004][2005][2006][2007]. Methods: We used data from the National Center for Health Statistics to (1) calculate age-adjusted sarcoidosis-associated mortality rates; (2) examine how those rates differ by age, sex, and race and ethnicity; and (3) determine underlying causes of death among sarcoidosis decedents. Measurements and Main Results: From 1988 deaths in the United States and 23,679 decedents with sarcoidosis mentioned on their death certificates. Over this time, the age-adjusted, sarcoidosis-related mortality rate increased 50.5% in women and 30.1% in men. The greatest absolute increase in death rates was among non-Hispanic black females. Regardless of sex or race, mortality rates climbed most in decedents 55 years or older. The most common cause of death was sarcoidosis itself. Younger sarcoidosis decedents with pulmonary fibrosis were more likely to be black than white, and younger sarcoidosis decedents were more likely than similarly aged decedents in the general population to have a cardiac cause contribute to death. Conclusions: From 1988-2007, sarcoidosis-related mortality rates increased significantly, particularly in non-Hispanic black females aged 55 years or older. The underlying cause of death in most patients with sarcoidosis was the disease itself. Among young sarcoidosis decedents, those with pulmonary fibrosis or a cardiac cause contributing to death were more likely to be black than white.Keywords: sarcoidosis; mortality; epidemiology Sarcoidosis is a multisystem, granulomatous disease of unknown cause (1) that most commonly affects young adults, particularly females (2). Sarcoidosis affects the thorax (lungs or mediastinal lymph nodes) in more than 90% of patients (3). Worldwide, the highest annual incidence of sarcoidosis (5-40 cases per 100,000 population) has consistently been observed in northern European countries (4). In the United States, the adjusted annual incidence of sarcoidosis is over three times greater in blacks than whites (35.5 cases vs. 10.9 cases per 100,000 population) (4). In the Black Women's Health Study, a study that enrolled a cohort of 59,000 black United States women, investigators calculated an annual incidence rate of 71 per 100,000 (5). Black Americans with sarcoidosis are more likely than other racial groups in the United States to suffer extrathoracic organ (e.g., skin or eye) involvement and a chronic disease course (3, 6).For most patients, sarcoidosis is a benign disorder; many never develop clinically significant disease, and spontaneous remission is not infrequent (6, 7). However, for a significant minority of patients, sarcoidosis is a chronic, debilitating, and even life-threatening condition: investigators have r...

H ypersensitivity pneumonitis (HP) is an interstitial lung disease (ILD) characterized pathologically by varying degrees of inflammation and/or fi brosis that result from the inhalation of an antigen to which a person has been previously sensitized. The clinical expression of HP can vary and includes acute, sub acute, and chronic forms. Symptom onset that coincides with antigen exposure provides the fi rst diagnostic clue; however, despite an exhaustive search, a recognizable, temporal relationship (between exposure and symptoms) is frequently absent, particularly in patients who present with the chronic form of HP.Chronic HP, characterized clinically by ventilatory restriction and, most commonly, upper-zonepredominant fi brosis on high-resolution CT (HRCT) scan, is irreversible and often progressive. It is believed that continued exposure to an inciting antigen (IA) perpetuates progression and the development of fi brosis in HP, but this belief hinges more on scientific rationale than systematic research. In this study, we hypothesized that among patients with histologic and 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 stratifi ed on identifi cation of an IA exposure. A Cox proportional hazards (PH) model was used to identify independent predictors in time-todeath analysis. Results: Of 142 patients, 67 (47%) had an identifi ed IA, and 75 (53%) had an unidentifi ed IA. Compared with survivors, patients who died (n 5 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 fi brosis, and were less likely to have an identifi able 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 fi brosis (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 fi brosis, mean FVC%, mean diffusing capacity of the lung for carbon monoxide (%), and history of smoking, survival was longer for patients with an identifi ed IA exposure than those with an unidentifi ed IA exposure (median, 8.75 years vs 4.88 years; P 5 .047). Conclusions: Among patients with chronic HP, when adjusting for a number of potentially infl uential predictors, including the presence of fi brosis, the inability to identify an IA was independently associated with shortened survival. CHEST 2013; 144(5):1644-1651Abbreviations: D lco 5 diffusing capacity of the lung f...

Background: Limited data exist regarding the population-based epidemiology of idiopathic pulmonary fi brosis (IPF). The objective of the study was to describe the trends in the incidence, prevalence, and clinical course of IPF in the community. Methods:We conducted a population-based study of adult patients with IPF in Olmsted County, Minnesota, from 1997 to 2005. Two methods were used to identify IPF cases, as defi ned by the 2002 American Thoracic Society/European Respiratory Society consensus statement: (1) usual interstitial pneumonia (UIP) on a surgical lung biopsy specimen or a defi nite UIP pattern on a high-resolution CT image (narrow criteria) and (2) UIP on a surgical lung biopsy specimen or a defi nite or possible UIP pattern on CT image (broad criteria). Results: Of 596 patients screened for the possibility of pulmonary disease or pulmonary fi brosis over 9 years of follow-up, 47 cases had IPF. Of these, 24 met the narrow criteria. The age-and sex-adjusted incidence was 8.8/100,000 and 17.4/100,000 person-years, for narrow and broad criteria, respectively. The age-adjusted incidence was higher in men than in women, and among patients aged 70-79 years. During the study period, the incidence of IPF decreased ( P , .001). On December 31, 2005, the age-and sex-adjusted prevalence was 27.9/100,000 and 63/100,000 persons by narrow and broad criteria, respectively. Thirty-seven patients experienced a total of 53 respiratory exacerbations (26 IPF related, 27 non-IPF related), and 34 (72%) patients died. The primary cause of death was IPF related in 16 (47%) patients. Median survival for narrow-criteria and broad-criteria incidence cases was 3.5 and 4.4 years, respectively. Conclusions: The incidence of IPF in Olmsted County decreased over the study period. Nonprimary IPF respiratory exacerbations are as frequent as primary IPF respiratory exacerbations and an important cause of death.CHEST 2010; 137( 1 ):129-137Abbreviations: ATS/ERS 5 American Thoracic Society and European Respiratory Society; HRCT 5 high-resolution computed tomography; IPF 5 idiopathic pulmonary fi brosis; REP 5 Rochester Epidemiology Project; UIP 5 usual interstitial pneumonia

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