Identification and Prognosis of Patients With Interstitial Pneumonia With Autoimmune Features.

BACKGROUND/OBJECTIVE: Patients classified as interstitial pneumonia with autoimmune features (IPAF) have interstitial lung disease (ILD) and features of autoimmunity but do not fulfill criteria for connective tissue diseases (CTDs). Our goal was to identify patients classifiable as IPAF, CTD-ILD, and idiopathic pulmonary fibrosis (IPF) from a preexisting pulmonary cohort and evaluate the prognosis of patients with IPAF. METHODS: We reviewed the medical records of 456 patients from a single-center pulmonary ILD cohort whose diagnoses were previously established by a multidisciplinary panel that did not include rheumatologists. We reclassified patients as IPAF, CTD-ILD, or IPF. We compared transplant-free survival using Kaplan-Meier methods and identified prognostic factors using Cox models. RESULTS: We identified 60 patients with IPAF, 113 with CTD-ILD, and 126 with IPF. Transplant-free survival of IPAF was not statistically significantly different from that of CTD-ILD or IPF. Among IPAF patients, male sex (hazard ratio, 4.58 [1.77-11.87]) was independently associated with worse transplant-free survival. During follow-up, only 10% of IPAF patients were diagnosed with CTD-ILD, most commonly antisynthetase syndrome. CONCLUSION: Despite similar clinical characteristics, most patients with IPAF did not progress to CTD-ILD; those who did often developed antisynthetase syndrome, highlighting the critical importance of comprehensive myositis autoantibody testing in this population. As in other types of ILD, male sex may portend a worse prognosis in IPAF. The routine engagement of rheumatologists in the multidisciplinary evaluation of ILD will help ensure the accurate classification of these patients and help clarify prognostic factors.

Fellowship Education in Interstitial Lung Disease. A National Survey of Program Directors and Trainees.

Background: Whether graduating pulmonary and critical care medicine (PCCM) fellows feel adequately trained in interstitial lung disease (ILD) remains unknown. In addition, there are no published data describing the current approach to educating trainees about ILD. Objective: To characterize the present state of ILD training during fellowship and to determine graduating PCCM fellows' perceived abilities to diagnose and manage ILD. Methods: We surveyed PCCM fellowship program directors nationwide and compared their perceptions of graduating fellows' abilities to diagnose, provide initial management to, and offer longitudinal care to patients with ILD using a series of unpaired t tests. We also inquired about existing practices for educating fellows about ILD. We then surveyed graduating PCCM fellows from 19 different preselected programs to assess comfort level with ILD in comparison with other core clinical domains. Results: Program director respondents (n = 74, 40% response rate) rated graduating fellows' abilities to establish specific ILD diagnoses and to provide initial management similarly (4.3 ± 0.8 on five-point Likert scale), whereas the ability to provide longitudinal expert care was rated significantly lower (3.8 ± 0.9, P = 0.001). Most respondents (n = 52, 70.3%) reported having dedicated outpatient ILD specialists with whom fellows could rotate, but only half required this rotation. In addition, very few (n = 17, 23.0%) reported that a majority of patients with suspected or newly diagnosed ILD were scheduled in fellow clinics, many of whom received subsequent longitudinal care from dedicated ILD specialists. Among 71 third-year fellow respondents, confidence in managing ILD was rated poorly (3.2 ± 1.0 on a five-point Likert scale) in contrast to more common diseases like chronic obstructive pulmonary disease (4.4 ± 0.7, P < 0.001) and asthma (4.2 ± 0.8, P < 0.001). Conclusion: Trainee exposure to ILD in both clinical and educational settings varied across PCCM fellowships nationwide. Fellows nearing graduation were significantly less confident in their ability to manage ILD compared with other more common pulmonary diseases.

Exposure to Ambient Particulate Matter Is Associated With Accelerated Functional Decline in Idiopathic Pulmonary Fibrosis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF), a progressive disease with an unknown pathogenesis, may be due in part to an abnormal response to injurious stimuli by alveolar epithelial cells. Air pollution and particulate inhalation of matter evoke a wide variety of pulmonary and systemic inflammatory diseases. We therefore hypothesized that increased average ambient particulate matter (PM) concentrations would be associated with an accelerated rate of decline in FVC in IPF. METHODS: We identified a cohort of subjects seen at a single university referral center from 2007 to 2013. Average concentrations of particulate matter < 10 and < 2.5 µg/m3 (PM10 and PM2.5, respectively) were assigned to each patient based on geocoded residential addresses. A linear multivariable mixed-effects model determined the association between the rate of decline in FVC and average PM concentration, controlling for baseline FVC at first measurement and other covariates. RESULTS: One hundred thirty-five subjects were included in the final analysis after exclusion of subjects missing repeated spirometry measurements and those for whom exposure data were not available. There was a significant association between PM10 levels and the rate of decline in FVC during the study period, with each µg/m3 increase in PM10 corresponding with an additional 46 cc/y decline in FVC (P = .008). CONCLUSIONS: Ambient air pollution, as measured by average PM10 concentration, is associated with an increase in the rate of decline of FVC in IPF, suggesting a potential mechanistic role for air pollution in the progression of disease.