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Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with a median survival of 3–4 years from time of initial diagnosis, similar to the time course of many malignancies. A hallmark of IPF is its unpredictable disease course, ranging from long periods of clinical stability to acute exacerbations with rapid decompensation. As the disease progresses, patients with chronic cough and progressive exertional dyspnea become oxygen dependent. They may experience significant distress due to concurrent depression, anxiety, and fatigue, which often lead to increased symptom burden and decreased quality of life. Despite these complications, palliative care is an underutilized, and often underappreciated, resource before end-of-life care in this population. While there is growing recognition about early palliative care in IPF, current data suggest referral patterns vary widely based on institutional practices. In addition to focusing on symptom management, there is emphasis on supplemental oxygen use, pulmonary rehabilitation, quality of life, and end-of-life care. Importantly, increased use of support groups and national foundation forums have served as venues for further disease education, communication, and advanced care planning outside of the hospital settings. The purpose of this review article is to discuss the clinical features of IPF, the role of palliative care in chronic disease management, current data supporting benefits of palliative care in IPF, its role in symptom management, and practices to help patients and their caregivers achieve their best quality of life.

Exercise pulmonary hypertension is an underappreciated form of physical limitation related to early pulmonary vascular disease. A low diffusing capacity of lungs for carbon monoxide (DLco) can be seen in patients with resting pulmonary hypertension as well as parenchymal lung disease. It remains unclear whether low DLco% identifies early pulmonary vascular disease. We hypothesize that a reduced DLco% differentiates the presence of exercise pulmonary hypertension in patients with parenchymal lung disease. Fifty-six patients referred for unexplained exertional dyspnea with pulmonary function tests within six months of hemodynamic testing underwent exercise right heart catheterization. Exclusion criteria included resting pulmonary arterial or venous hypertension. Receiver operator characteristic curve determined the optimal DLco% cutoffs based on the presence or absence of parenchymal lung disease. Twenty-one (37%) patients had parenchymal lung disease, most common manifesting as chronic obstructive lung disease or interstitial lung disease. In patients with parenchymal lung disease, a DLco of 46% demonstrated 100% sensitivity and 73% specificity for detecting exercise pulmonary hypertension. In patients without parenchymal lung disease, a DLco of 73% demonstrated 58% sensitivity and 94% specificity for detecting exercise pulmonary hypertension. In both cohorts, DLco% below the optimum cutoffs were associated with higher peak mean pulmonary arterial pressure and peak total pulmonary resistance consistent with the hemodynamic definition of exercise pulmonary hypertension. Patients with a DLco

IntroductionMuscle loss is an important extrapulmonary manifestation of COPD. Dual energy X-ray absorptiometry (DXA) is the method of choice for body composition measurement but is not widely used for muscle mass evaluation. The pectoralis muscle area (PMA) is quantifiable by CT and predicts cross-sectional COPD-related morbidity. There are no studies that compare PMA with DXA measures or that evaluate longitudinal relationships between PMA and lung disease progression.MethodsParticipants from our longitudinal tobacco-exposed cohort had baseline and 6-year chest CT (n=259) and DXA (n=164) data. Emphysema was quantified by CT density histogram parenchymal scoring using the 15th percentile technique. Fat-free mass index (FFMI) and appendicular skeletal mass index (ASMI) were calculated from DXA measurements. Linear regression model relationships were reported using standardised coefficient (β) with 95% CI.ResultsPMA was more strongly associated with DXA measures than with body mass index (BMI) in both cross-sectional (FFMI: β=0.76 (95% CI 0.65 to 0.86), pConclusionsPMA is an accessible measure of muscle mass and may serve as a useful clinical surrogate for assessing skeletal muscle loss in smokers. Decreased PMA correlated with emphysema progression but not lung function decline, suggesting a difference in the pathophysiology driving emphysema, airflow obstruction and comorbidity risk.

Pulmonary arterial hypertension (PAH) is a deadly vascular disease, characterized by increased pulmonary arterial pressures and right heart failure. Considering prior non-US studies of atrial arrhythmias in PAH, this retrospective, regional multi-center US study sought to define more completely the risk factors and impact of paroxysmal and non-paroxysmal forms of atrial fibrillation and flutter (AF/AFL) on mortality in this disease. We identified patients seen between 2010 and 2014 at UPMC (Pittsburgh) hospitals with hemodynamic and clinical criteria for PAH or chronic thromboembolic pulmonary hypertension (CTEPH) and determined those meeting electrocardiographic criteria for AF/AFL. We used Cox proportional hazards regression with time-varying covariates to analyze the association between AF/AFL occurrence and survival with adjustments for potential cofounders and hemodynamic severity. Of 297 patients with PAH/CTEPH, 79 (26.5%) suffered from AF/AFL at some point. AF/AFL was first identified after PAH diagnosis in 42 (53.2%), identified prior to PAH diagnosis in 27 (34.2%), and had unclear timing in the remainder. AF/AFL patients were older, more often male, had lower left ventricular ejection fractions, and greater left atrial volume indices and right atrial areas than patients without AF/AFL. AF/AFL (whether diagnosed before or after PAH) was associated with a 3.81-fold increase in the hazard of death (95% CI 2.64–5.52, p

Background Our hospital system created system guidelines to standardize care across 24 hospitals for COVID-19 treatment during the pandemic. Guidelines changed over time. Hydroxychloroquine (HCQ) was unrestricted during phase 1, then restricted by pharmacy outside of a randomized clinical trial (RCT) during phase 2 (excepting those ineligible for RCTs). Methods This was a prospective study to assess system-wide adherence to COVID-19 treatment guidelines, and to evaluate patient outcomes. Results Of 261 patients, median age was 67 years (IQR 56–76); 49% (129/261) were male, and 45% (118/261) required ICU care. Overall, 47% (122/261) were in phase 1; HCQ was offered to 57% (69/122) during this phase. The rate of HCQ prescription in phase 2 decreased significantly to 10% (14/136), (p < 0.001). Adherence to COVID-19 treatment protocol was 97% (135/139) during phase 2. Mortality was similar in both phases (22% vs 28%, p=0.32), as was median length of stay (8 vs 7 days, p=0.3). Overall 66 patients (25%) died in the hospital; neither non-adherence (p=1) to system guidelines nor receipt of HCQ (p=0.17) were risk factors for death. Independent predictors of mortality included: new renal replacement therapy (OR 61, 95%CI 6.7–560, p < 0.001), mechanical ventilation (OR 4.9, 95%CI 2.0–11, p < 0.001), abnormal chest X-ray (OR 4.3, 95%CI 1.4–12.6, p =0.009), history of heart failure (OR 3.9, 95%CI 1.5–11, p=0.006), lack of fever on admission (OR 3.5, 95%CI 1.7 -7.6, p =0.001), receipt of corticosteroids (OR 2.7 95%CI 1.1–6.6, p=0.026) and increased age (OR 1.07 per year, 95%CI 1.04–1.1, p < 0.001). Bacterial pneumonia occurred in 8% (21/261), more commonly in those who died (p=0.02). Black patients had a higher race-specific death rate (308 vs 197) per 1000 than white patients (p< 0.001). Conclusion During the COVID-19 pandemic, our health system guidelines and pharmacy restrictions were successful in delivering consistent care across hospitals. Restriction of HCQ for COVID-19 treatment to RCTs reduced its use in phase two. Non-adherence to systemic guidelines was infrequent, and not associated with adverse outcomes. A COVID-19 treatment team of physicians and pharmacists can effectively coordinate therapy across hospitals in the setting of rapidly changing guidelines. Disclosures J. Ryan Bariola, MD, Infectious Disease Connect (Employee)Mayne Pharma (Advisor or Review Panel member)Merck (Research Grant or Support)

Background Lung Cancer is occasionally observed in patients with Idiopathic Pulmonary Fibrosis (IPF). We sought to describe the epidemiologic and clinical characteristics of lung cancer for patients with IPF and other interstitial lung disease (ILD) using institutional and statewide data registries. Methods We conducted a retrospective analysis of IPF and non-IPF ILD patients from the ILD center registry, to compare with lung cancer registries at the University of Pittsburgh as well as with population data of lung cancer obtained from Pennsylvania Department of Health between 2000 and 2015. Results Among 1108 IPF patients, 31 patients were identified with IPF and lung cancer. The age-adjusted standard incidence ratio of lung cancer was 3.34 (with IPF) and 2.3 (with non-IPF ILD) (between-group Hazard ratio = 1.4, p = 0.3). Lung cancer worsened the mortality of IPF (p

Foot and ankle problems are highly prevalent in patients with diabetes mellitus (DM). Increased rates of surgical site infections and noninfectious complications, such as malunion, delayed union, nonunion, and hardware failure, have also been more commonly observed in diabetic patients who undergo foot and ankle surgery. DM is a substantial contributor of perioperative morbidity in patients with solid organ transplantation. To the best of our knowledge, postoperative foot and ankle complications have not been studied in a cohort of diabetic patients who previously underwent solid organ transplantation. The aim of the present study was to evaluate the outcomes of foot and ankle surgery in a cohort of diabetic transplant patients and to compare these outcomes with those of diabetic patients without a history of transplantation. We compared the rates of infectious and noninfectious complications after foot and ankle surgery in 28 diabetic transplant patients and 56 diabetic patients without previous transplantation and calculated the odds ratios (OR) for significant findings. The diabetic transplant patients who underwent foot and ankle surgery in the present cohort were not at an increased risk of overall complications (OR 0.83, 95% confidence interval [CI] 0.33 to 2.08, p = .67), infectious complications (OR 0.54, 95% CI 0.09 to 3.09, p = .49), or noninfectious complications (OR 1.14, 95% CI 0.41 to 3.15, p = .81). Four transplant patients (14.3%) died of non-orthopedic surgery-related events during the follow-up period; however, no deaths occurred in the control group. Diabetic patients with previous solid organ transplantation were not at an increased risk of developing postoperative complications after foot and ankle surgery, despite being immunocompromised. The transplant patients had a greater mortality rate, but their premature death was unrelated to their foot and ankle surgery. Surgeons treating transplant patients can recommend foot and ankle surgery when indicated. However, owing to the increased mortality rate and comorbidities associated with this high-risk group, we recommend preoperative clearance from the transplant team and medical consultations before performing surgery.

Abdominal contour deformities after massive weight loss are highly variable, ranging from a mild upper protuberance to multiple rolls. Correction of these deformities is challenging and may require advanced surgical techniques. Evaluating the incidence of patients presenting with various abdominal deformities and the factors influencing these deformities could aid patients undergoing weight loss.All massive weight loss patients presenting for abdominal contouring from 2002 to 2012 were reviewed, and abdomens were graded using a modified Pittsburgh Rating Scale. Data collected for each patient included body mass indices, history of bariatric surgery, and type of abdominal contouring surgery.One thousand six patients were evaluated, with a mean age of 44.2 ± 10.5 years. The mean maximum body mass index was 51.7 ± 9.6 kg/m2, mean current body mass index was 30.0 ± 6.4 kg/m2, and mean change in body mass index was 21.7 ± 7.0 kg/m2. Sixty-four percent had a high-grade deformity. Patients with a larger change in body mass index had higher deformity grades (p0.001). Patients with higher deformity grades were more likely to undergo a fleur-de-lis abdominoplasty (p0.001).Most patients presenting for body contouring after massive weight loss have high-grade abdominal deformities with multiple rolls. Change in body mass index is positively correlated with deformity grade and more aggressive contouring procedures. Patients interested in massive weight loss should be counseled that, depending on desire for eventual outcomes, more complex procedures may be required to correct the resultant abdominal deformity.