No increased prevalence of autoantibodies neutralizing type I IFNs in idiopathic pulmonary fibrosis patients.

SARS-CoV2 infection has a poor prognosis in patients affected of idiopathic pulmonary fibrosis (IPF). Autoantibodies (auto-Abs) neutralizing type I interferons (IFNs) are found in the blood of at least 15% of patients with life-threatening COVID-19 pneumonia. Because of the elevated prevalence of some auto-Abs in IPF patients, we hypothesize that the prevalence of auto-Abs neutralizing type I IFNs might be increased in the IPF population and then explained specific poor outcome after COVID-19. We screened the plasma of 247 consecutive IPF patients for the presence of auto-Abs neutralizing type I IFNs. Three patients displayed auto-Abs neutralizing type I IFNs. Among them, the only patient with documented SARS-CoV-2 infection experienced life threatening COVID-19 pneumonia. The prevalence of auto-Abs neutralizing type I IFNs in this cohort of IPF patients was not significantly different from the one of the general population. Overall, this study did not suggest any association between auto-Abs neutralizing type I IFNs and IPF.

What Do We Need to Know About Rising Rates of Idiopathic Pulmonary Fibrosis? A Narrative Review and Update.

The most common type of idiopathic interstitial pneumonia is idiopathic pulmonary fibrosis (IPF), an irreversible, progressive disorder that has lately come into question for possible associations with COVID-19. With few geographical exceptions, IPF is a rare disease but its prevalence has been increasing markedly since before the pandemic. Environmental exposures are frequently implicated in IPF although genetic factors play a role as well. In IPF, healthy lung tissue is progressively replaced with an abnormal extracellular matrix that impedes normal alveolar function while, at the same time, natural repair mechanisms become dysregulated. While chronic viral infections are known risk factors for IPF, acute infections are not and the link to COVID-19 has not been established. Macrophagy may be a frontline defense against any number of inflammatory pulmonary diseases, and the inflammatory cascade that may occur in patients with COVID-19 may disrupt the activity of monocytes and macrophages in clearing up fibrosis and remodeling lung tissue. It is unclear if COVID-19 infection is a risk factor for IPF, but the two can occur in the same patient with complicating effects. In light of its increasing prevalence, further study of IPF and its diagnosis and treatment is warranted.

Incidental discovery of interstitial lung disease: diagnostic approach, surveillance and perspectives.

The incidental discovery of pre-clinical interstitial lung disease (ILD) has led to the designation of interstitial lung abnormalities (ILA), a radiological entity defined as the incidental finding of computed tomography (CT) abnormalities affecting more than 5% of any lung zone. Two recent documents have redefined the borders of this entity and made the recommendation to monitor patients with ILA at risk of progression. In this narrative review, we will focus on some of the limits of the current approach, underlying the potential for progression to full-blown ILD of some patients with ILA and the numerous links between subpleural fibrotic ILA and idiopathic pulmonary fibrosis (IPF). Considering the large prevalence of ILA in the general population (7%), restricting monitoring only to cases considered at risk of progression appears a reasonable approach. However, this suggestion should not prevent pulmonary physicians from pursuing an early diagnosis of ILD and timely treatment where appropriate. In cases of suspected ILD, whether found incidentally or not, the pulmonary physician is still required to make a correct ILD diagnosis according to current guidelines, and eventually treat the patient accordingly.

Nutrition implications of intrinsic restrictive lung disease.

Restrictive lung disease is defined as a reduction in lung volume that may be due to intraparenchymal or extraparenchymal causes. Intraparenchymal causes falls under the umbrella term of interstitial lung disease (ILD) and includes idiopathic pulmonary fibrosis. This manuscript provides an overview of ILD and can be beneficial for all clinicians working with patients with ILD. Although not well documented, the prevalence of malnutrition in patients with ILD has been reported to be between ~9% and 55%. Body mass index has been shown to predict survival; but more recently, research has suggested that fat-free mass has a larger influence on survival. There is insufficient evidence to support the use of antioxidant or vitamin supplementation to help diminish the chronic inflammatory process that is seen in this patient population. There are data from studies examining the vitamin D status in this patient population, but research on vitamin D supplementation appears to be lacking. Registered dietitian nutritionists should continue to advocate and play a more prominent role in the nutrition management of patients with ILD as part of standard of care.

Characteristics and outcomes of COVID-19 patients with IPF: A multi-center retrospective study.

BACKGROUND: There are few data on severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) (COVID-19) infection in patients with idiopathic pulmonary fibrosis (IPF). The objective of this study is to describe the characteristics and outcomes of IPF patients confirmed COVID-19 infection. METHODS: In this retrospective, multi-center, cohort study, patients from 4 hospital medical records with known IPF and a COVID-19 diagnosis were identified. Demographic and clinical outcome data were abstracted through a review of electronic medical records. RESULTS: Records for 46 patients with IPF and COVID-19 were abstracted. The mean age was 65±10 years. The most common symptom was dyspnea, followed by fever and cough. Ground-glass opacities (n = 35, 83.3%) and consolidations (n = 11, 26.1%) were the main imaging features of the disease in thorax computed tomography (CT). Twenty-four patients (52.1%) required hospitalization. Among the hospitalized patients, 16 (66.6%) were admitted to the intensive care unit (ICU), and 10 (41.6%) underwent invasive mechanical ventilation. Thirteen patients (28.2%) died of COVID-19 complications. Mortality rate was significantly associated with lower DLCO/VA, long term oxygen therapy and consolidation finding on CT of thorax (p<0.05). On multivariable analysis, neither factor was associated with hospitalization or mortality. CONCLUSIONS: IPF patients represent a vulnerable population for COVID-19, according to the high rate of hospitalization, ICU requirement, and mortality rate. Measures to minimize the risk of COVID-19 infection remain key to protect IPF patients.

The impact of SARS-COV2 pandemic on the management oF IPF patients: Our narrative experience.

BACKGROUND: The SARS-CoV-2 pandemic has changed the health-care systems around the world in a remarkable way. We describe the strategies adopted to cope with the limitations imposed by the pandemic to the access to health care by patients diagnosed with idiopathic Pulmonary Fibrosis (IPF). MATERIAL AND METHODS: We conducted a retrospective observational analysis including IPF patients under antifibrotic drugs (nintedanib and pirfenidone) that accessed to the Outpatient clinic of the University of Palermo, Italy. Patients received a phone number and an email address in case of any urgency and a virtual meeting was settled up monthly. RESULTS: 40 patients (M/F: 30/10) were followed up, 33 under nintedanib treatment, 7 under pirfenidone. Among patients under nintedanib, 1 patient reported high fever (T max 39 °C) and purulent sputum with no sign of infections, 1 had hemoptysis that was spontaneously resolved. 2 patients accessed to the emergency department for the worsening of dyspnea; 5 patients had diarrhea that resolved with symptomatic drugs in few days. 3 patients had an increase of alkaline phosphatase levels, leading to the withdrawal of the antifibrotic drug for 15 days, and subsequent normalization of the plasmatic levels. Among patients under pirfenidone, one subject had an increase of ferritin serum levels with no symptoms. The remaining subjects were in stable clinical conditions. None of the patients reported hospitalization or exacerbations, and did not experience antifibrotic withdrawal. CONCLUSIONS: We were able to demonstrate that by implementing alternative ways to monitor the disease, patients did not incur in increased rates of acute exacerbations or higher frequency of side effects and antifibrotic treatment withdrawal.

Effects of the COVID-19 pandemic on the follow-up and treatment of patients with idiopathic pulmonary fibrosis: a cross-sectional, multicentre phone call survey.

OBJECTIVE: To learn about the attitudes and behaviours of patients with idiopathic pulmonary fibrosis (IPF) in relation to the difficulties experienced during the COVID-19 pandemic. DESIGN: A cross-sectional, multicentre phone call survey. SETTING: Four university hospitals in Turkey. PARTICIPANTS: The study included patients with IPF receiving antifibrotics for at least 3 months and with doctor appointment and/or scheduled routine blood analysis between March and May 2020 (the first 3 months after the official announcement of the COVID-19 pandemic in Turkey). INTERVENTIONS: Phone calls (a 5 min interview) were performed in June 2020. A questionnaire and the Hospital Anxiety-Depression Scale were applied. MAIN OUTCOME MEASURES: Patients' preferences for disease monitoring, patients' attitudes and behaviours towards IPF, drug continuation, COVID-19 diagnosis and anxiety/depression status. RESULTS: The study included 115 patients with IPF (82 male; mean age, 68.43±7.44 years). Of the patients, 73.9% had doctor appointment and 52.2% had scheduled routine blood testing; 54.5% of patients with doctor appointment self-cancelled their appointments and 53.3% of patients with scheduled routine blood testing did not undergo testing. Of the patients, 32.2% were on nintedanib and 67.8% were on pirfenidone; self-initiated drug discontinuation rate was 22.6%. The percentage of patients communicating with their physicians was 35.7%. The route of communication was by phone (34.8%). The frequency of depression and anxiety was 27.0% and 38.3%, respectively. The rates of drug discontinuation (35.1% vs 16.7%, p<0.05) and depression (37.8% vs 21.8%, p=0.07) were higher in nintedanib users than in pirfenidone users. Only two (1.7%) patients had COVID-19 diagnosis. CONCLUSIONS: During the COVID-19 pandemic, a significant proportion (>50%) of patients self-cancelled their appointments and nearly a quarter of patients discontinued their medications. Providing a documentation of the problems experienced by patients with IPF about management of the necessary requirements during the COVID-19 pandemic, this study may be a model for patients with chronic diseases.

Patients with idiopathic pulmonary fibrosis have poor clinical outcomes with COVID-19 disease: a propensity matched multicentre research network analysis.

INTRODUCTION: Outcomes of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in patients with pre-existing idiopathic pulmonary fibrosis (IPF) remain understudied, and it is unknown if IPF is an independent predictor of worse disease course. Herein, we report the clinical outcomes in a large cohort of 251 patients with COVID-19 in the setting of known IPF. Outcomes were compared with a propensity matched cohort of patients with COVID-19 without IPF. METHODS: Analysis of a federated multicentre research network TriNetX was performed including patients more than 16 years of age diagnosed with SARS-CoV-2 infection. Outcomes in patients diagnosed as positive for SARS-CoV-2 infection with concurrent IPF were compared with a propensity matched cohort of patients without IPF. RESULTS: A total of 311 060 patients with SARS-CoV-2 infection on the research network were identified, 251 patients (0.08%) carried a diagnosis of IPF. Mean age of patients with IPF was 68.30±12.20 years, with male predominance (n=143, 56.97%). Comorbidities including chronic lower respiratory diseases, diabetes mellitus, ischaemic heart disease and chronic kidney disease were more common in patients with IPF when compared with the non-IPF cohort. After propensity matching, higher rates of composite primary outcome (death or mechanical ventilation) at 30 and 60 days, as well as need for hospitalisation, critical care, and acute kidney injury were observed in the IPF cohort. CONCLUSION: Poor outcomes of COVID-19 disease were observed in patients with IPF after robust matching of confounders. Our data confirm that patients with IPF constitute a high-risk cohort for poor outcomes related to COVID-19 disease.