Differentiating Usual Interstitial Pneumonia From Nonspecific Interstitial Pneumonia Using High-resolution Computed Tomography: The "Straight-edge Sign".

PURPOSE: The purpose of this article was to determine whether a novel finding on coronal computed tomography (CT) can help differentiate usual interstitial pneumonia (UIP) from nonspecific interstitial pneumonia (NSIP) in order to obviate lung biopsy. MATERIALS AND METHODS: Two chest radiologists, blinded to clinical data, reviewed 3 preselected coronal images from CT scans, performed within 1 year of surgical lung biopsy (SLB), from 51 patients with biopsy-proven UIP and 15 with biopsy-proven NSIP. The 198 (66×3) images were anonymized and randomized. The radiologists assessed each coronal image for the presence or absence of the straight-edge sign (SES) on both the right and left sides, anecdotally thought to be more common in NSIP than in UIP. The SES was defined as reticulation isolated to the lung bases with sharp demarcation in the craniocaudal plane and without substantial extension along the lateral margins of the lungs. A validation cohort from a second medical center was also evaluated to reassess our findings. RESULTS: The absence of a bilateral SES yielded a sensitivity, specificity, positive predictive value (PPV), and negative predictive value of 56.9%, 93.3%, 96.7%, and 38.9%, respectively, for UIP on SLB. The unilateral or bilateral absence of the SES yielded a sensitivity, specificity, PPV, and negative predictive value of 76.5%, 66.7%, 88.6%, and 45.5%, respectively, for UIP on SLB. For the 11 subjects with an overall CT pattern consistent with NSIP but a pathologic diagnosis of UIP, the SES was absent in 6 (54.5%) subjects. In the validation cohort, the SES was much more common in NSIP than in UIP (46.6% compared with 3.3%, respectively; P<0.001). CONCLUSION: The absence of the SES has a high PPV for biopsy-proven UIP. Bilateral absence of the SES has high specificity (93.3%) for biopsy-proven UIP. The SES may be useful for differentiating UIP from NSIP.

Physical activity and activity space in patients with pulmonary fibrosis not prescribed supplemental oxygen.

BACKGROUND: Patients with pulmonary fibrosis (PF) have impaired quality of life, and research suggests that dyspnea and physical activity are primary drivers. As PF progresses, some patients notice the disease "shrinks their worlds". The objective of this study is to describe movement (both physical activity and activity space) in a cohort of patients with PF of various etiologies who have not been prescribed supplemental oxygen (O2). METHODS: Subjects with PF not on supplemental O2 during the day were enrolled from across the U.S. from August 2013 to October 2015. At enrollment, each subject completed questionnaires and, for seven consecutive days, wore an accelerometer and GPS tracker. RESULTS: One hundred ninety-four subjects had a confirmed diagnosis of PF and complete, analyzable GPS data. The cohort was predominantly male (56%), Caucasian (95%) and had idiopathic pulmonary fibrosis (30%) or connective tissue disease related-PF (31%). Subjects walked a median 7497 (interquartile range [IQR] 5766-9261) steps per day. Steps per day were correlated with symptoms and several quality of life domains. In a model controlling for age, body mass index, wrist- (vs. waist) worn accelerometer and percent predicted diffusing capacity (DLCO%), fatigue (beta coefficient = -51.5 ± 11.7, p < 0.0001) was an independent predictor of steps per day (model R2=0.34). CONCLUSIONS: Patients with PF, who have not been prescribed O2 for use during the day, have wide variability in their mobility. Day-to-day physical activity is related to several domains that impact quality of life, but GPS-derived activity space is not. Wearable data collection devices may be used to determine whether and how therapeutic interventions impact movement in PF patients. TRIAL REGISTRATION: NCT01961362 . Registered 9 October, 2013.

Plasma Leptin Is Elevated in Acute Exacerbation of Idiopathic Pulmonary Fibrosis.

Background. The natural history of idiopathic pulmonary fibrosis (IPF) is very complex and unpredictable. Some patients will experience acute exacerbation (AE) and fatal outcomes. Methods. The study included 30 AE-IPF patients, 32 stable IPF (S-IPF) patients, and 12 healthy controls. We measured the plasma concentrations of leptin and KL-6. Simple correlation was used to assess associations between leptin and other variables. Plasma leptin levels were compared between AE-IPF and S-IPF subjects, decedents, and survivors. Kaplan-Meier curves were used to display survival and Cox proportional hazards regression was used to examine risk factors for survival. Results. In subjects with AE-IPF, plasma leptin was significantly greater than in subjects with S-IPF (p = 0.0003) or healthy controls (p < 0.0001). Plasma leptin was correlated with BMI, KL-6, LDH, CRP, and PaO2/FiO2 (p = 0.007; p = 0.005; p = 0.003; p = 0.033; and p = 0.032, resp.). Plasma leptin was significantly greater in 33 decedents than in the 23 survivors (p = 0.007). Multivariate Cox regression analysis showed leptin (>13.79 ng/mL) was an independent predictor of survival (p = 0.004). Conclusions. Leptin could be a promising plasma biomarker of AE-IPF occurrence and predictor of survival in IPF patients.

Patient Perspectives in OMERACT Provide an Anchor for Future Metric Development and Improved Approaches to Healthcare Delivery in Connective Tissue Disease Related Interstitial Lung Disease (CTD-ILD).

OBJECTIVE: The impact and natural history of connective tissue disease related interstitial lung disease (CTD-ILD) are poorly understood; and have not been previously described from the patient's perspective. This investigation sought insight into CTD-ILD from the patients' perspective to add to our knowledge of CTD-ILD, identify disease-specific areas of unmet need and gather potentially meaningful information towards development of disease-specific patient-reported outcome measures (PROMs). METHODS: A mixed methods design incorporating patient focus groups (FGs) querying disease progression and life impact followed by questionnaires with items of importance generated by >250 ILD specialists were implemented among CTD-ILD patients with rheumatoid arthritis, idiopathic inflammatory myopathies, systemic sclerosis, and other CTD subtypes. FG data were analyzed through inductive analysis with five independent analysts, including a patient research partner. Questionnaires were analyzed through Fisher's Exact tests and hierarchal cluster analysis. RESULTS: Six multicenter FGs included 45 patients. Biophysiologic themes were cough and dyspnea, both pervasively impacting health related quality of life (HRQoL). Language indicating dyspnea was unexpected, unique and contextual. Psycho-social themes were Living with Uncertainty, Struggle over Self-Identity, and Self-Efficacy - with education and clinician communication strongly emphasised. All questionnaire items were rated 'moderately' to 'extremely' important with 10 items of highest importance identified by cluster analysis. CONCLUSION: Patients with CTD-ILD informed our understanding of symptoms and impact on HRQoL. Cough and dyspnea are central to the CTD-ILD experience. Initial FGs have provided disease-specific content, context and language essential for reliable PROM development with questionnaires adding value in recognition of patients' concerns.