Relative environmental and social disadvantage in patients with idiopathic pulmonary fibrosis

Thorax ◽  
2021 ◽  
pp. thoraxjnl-2021-217652
Author(s):  
Na'ama Avitzur ◽  
Elizabeth M Noth ◽  
Mubasiru Lamidi ◽  
Steven D Nathan ◽  
Harold R Collard ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
San Francisco ◽  
Proportional Hazards ◽  
Social Disadvantage ◽  
Sensitivity Analyses ◽  
Study Cohort ◽  
Antifibrotic Therapy ◽  
Lower Baseline ◽  
Composite Scores

BackgroundAir pollution exposure is associated with disease severity, progression and mortality in patients with idiopathic pulmonary fibrosis (IPF). Combined impacts of environmental and socioeconomic factors on outcomes in patients with IPF are unknown. The objectives of this study were to characterise the relationships between relative environmental and social disadvantage with clinical outcomes in patients with IPF.MethodsPatients with IPF were identified from a longitudinal database at University of California, San Francisco. Residential addresses were geocoded and linked to the CalEnviroScreen 3.0 (CES), a tool that quantifies environmental burden in California communities, combining population, environmental and pollution vulnerability into individual and composite scores (higher scores indicating greater disadvantage). Unadjusted and adjusted linear and logistic regression and Fine and Gray proportional hazards models were used.Results603 patients were included. Higher CES was associated with lower baseline forced vital capacity (β=−0.073, 95% CI −0.13 to −0.02; p=0.006) and diffusion capacity of the lung for carbon monoxide (β=−0.11, 95% CI −0.16 to −0.06; p<0.001). Patients in the highest population vulnerability quartile were less likely to be on antifibrotic therapy (OR=0.33; 95% CI 0.18 to 0.60; p=0.001) at time of enrolment, compared with those in the lowest quartile. An association between CES and mortality was suggested, but sensitivity analyses demonstrated inconsistent results. Relative disadvantage of the study cohort appeared lower compared with the general population.ConclusionsHigher environmental exposures and vulnerability were associated with lower baseline lung function and lower antifibrotic use, suggesting that relative socioenvironmental disadvantage has meaningful impacts on patients with IPF.

scholarly journals Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD

2021 ◽  
Vol 10 (11) ◽  
pp. 2285
Author(s):  
John N. Shumar ◽  
Abhimanyu Chandel ◽  
Christopher S. King
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Treatment Pathway ◽  
Antifibrotic Therapy ◽  
New Treatment ◽  
Fibrotic Lung Diseases

Progressive fibrosing interstitial lung disease (PF-ILD) describes a phenotypic subset of interstitial lung diseases characterized by progressive, intractable lung fibrosis. PF-ILD is separate from, but has radiographic, histopathologic, and clinical similarities to idiopathic pulmonary fibrosis. Two antifibrotic medications, nintedanib and pirfenidone, have been approved for use in patients with idiopathic pulmonary fibrosis. Recently completed randomized controlled trials have demonstrated the clinical efficacy of antifibrotic therapy in patients with PF-ILD. The validation of efficacy of antifibrotic therapy in PF-ILD has changed the treatment landscape for all of the fibrotic lung diseases, providing a new treatment pathway and opening the door for combined antifibrotic and immunosuppressant drug therapy to address both the fibrotic and inflammatory components of ILD characterized by mixed pathophysiologic pathways.

scholarly journals Switching antifibrotics in patients with idiopathic pulmonary fibrosis: a multi-center retrospective cohort study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Yuzo Suzuki ◽  
Kazutaka Mori ◽  
Yuya Aono ◽  
Masato Kono ◽  
Hirotsugu Hasegawa ◽  
...  
Keyword(s):  
Cohort Study ◽  
Propensity Score ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Progression ◽  
Retrospective Cohort Study ◽  
Retrospective Cohort ◽  
Survival Times ◽  
Antifibrotic Therapy ◽  
Median Period

Abstract Background Currently, there are two antifibrotics used to treat idiopathic pulmonary fibrosis (IPF): pirfenidone and nintedanib. Antifibrotics slow disease progression by reducing the annual decline of forced vital capacity (FVC), which possibly improves outcomes in IPF patients. During treatment, patients occasionally switch antifibrotic treatments. However, prognostic implication of changing antifibrotics has not yet been evaluated. Methods This multi-center retrospective cohort study examined 262 consecutive IPF patients who received antifibrotic therapy. Antifibrotic agents were switched in 37 patients (14.1%). The prognoses were compared between the patient cohort that switched antifibrotics (Switch-IPF) and those without (Non-Switch-IPF) using propensity-score matched analyses. Results The median period between the initiation of antifibrotic therapy and the drug switch was 25.8 (12.7–35.3) months. The most common reasons for the switch were disease progression (n = 17) followed by gastrointestinal disorders (n = 12). Of the 37 patients that switched antifibrotics, only eight patients disrupted switched antifibrotics by their adverse reactions. The overall prognosis of the Switch-IPF cohort was significantly better than the Non-Switch-IPF cohort (median periods: 67.2 vs. 27.1 months, p < 0.0001). In propensity-score matched analyses that were adjusted to age, sex, FVC (%), history of acute exacerbation, and usage of long-term oxygen therapy, the Switch-IPF cohort had significantly longer survival times than the Non-Switch-IPF group (median 67.2 vs. 41.3 months, p = 0.0219). The second-line antifibrotic therapy showed similar survival probabilities than those in first-line antifibrotic therapy in multistate model analyses. Conclusion Switching antifibrotics is feasible and may improve prognosis in patients with IPF. A further prospective study will be required to confirm clinical implication of switching the antifibrotics.

scholarly journals Clinical outcomes of pediatric macular edema associated with non-infectious uveitis

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Anh Hong Nguyen ◽  
Bethlehem Mekonnen ◽  
Eric Kim ◽  
Nisha R. Acharya
Keyword(s):  
Visual Acuity ◽  
Macular Edema ◽  
San Francisco ◽  
Pediatric Patients ◽  
Proportional Hazards ◽  
Cox Proportional Hazards ◽  
Study Cohort ◽  
Common Diagnosis ◽  
Corticosteroid Injections

Abstract Background Macular edema (ME) is the most frequent cause of irreversible visual impairment in patients with uveitis. To date, little data exists about the clinical course of ME in pediatric patients. A retrospective, observational study was performed to examine the visual and macular thickness outcomes of ME associated with chronic, noninfectious uveitis in pediatric patients. Methods Pediatric patients with noninfectious uveitis complicated by ME seen in the University of California San Francisco Health System from 2012 to 2018 were identified using ICD-9 and ICD-10 codes. Data were collected from medical records including demographics, diagnoses, ocular history, OCT imaging findings, complications, and treatments at first encounter and at 3, 6, 9, and 12-month follow-up visits. Cox proportional hazards regression was used to investigate the association between different classes of treatment (steroid drops, steroid injections, oral steroids and other immunosuppressive therapies) and resolution of macular edema. Results The cohort comprised of 21 children (26 eyes) with a mean age of 10.5 years (SD 3.3). Undifferentiated uveitis was the most common diagnosis, affecting 19 eyes (73.1%). The majority of observed macular edema was unilateral (16 patients, 76.2%) and 5 patients had bilateral macular edema. The mean duration of follow-up at UCSF was 35.3 months (SD 25.7). By 12 months, 18 eyes (69.2%) had achieved resolution of ME. The median time to resolution was 3 months (IQR 3–6 months). Median best-corrected visual acuity (BCVA) at baseline was 0.54 logMAR (Snellen 20/69, IQR 20/40 to 20/200). Median BCVA at 12 months was 0.1 logMAR (Snellen 20/25, IQR 20/20 to 20/50) Corticosteroid injections were associated with a 4.0-fold higher rate of macular edema resolution (95% CI 1.3–12.2, P = 0.01). Conclusions Although only 15% of the pediatric patients with uveitis in the study cohort had ME, it is clinically important to conduct OCTs to detect ME in this population. Treatment resulted in 69% of eyes achieving resolution of ME by 12 months, accompanied with improvement in visual acuity. Corticosteroid injections were significantly associated with resolution of macular edema.

FRI0485 RISK OF PROGRESSION OF IDIOPATHIC PULMONARY FIBROSIS TO CONNECTIVE TISSUE DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 840-841
Author(s):  
B. Ghang ◽  
S. H. Nam ◽  
Y. G. Kim ◽  
B. Yoo ◽  
C. K. Lee
Keyword(s):  
Risk Factors ◽  
Idiopathic Pulmonary Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Rheumatoid Factor ◽  
Connective Tissue Disease ◽  
Proportional Hazards ◽  
Cox Proportional Hazards ◽  
Survival Duration ◽  
Citrullinated Protein

Background:Connective tissue disease (CTD) may be observed during the course of idiopathic pulmonary fibrosis (IPF). However, clinical factors associated with the development of CTD in patients with IPF have not yet been identified. These factors might be valuable clues for determining the pathogenesis of pulmonary fibrosis in patients with CTD. We hypothesize that some IPF patients have a clinically significant association with autoimmunity, and that autoantibodies are important biomarkers for identifying these patients.Objectives:Based on this hypothesis, we investigated whether the serology criteria (anti-neutrophil cytoplasmic antibody (ANCA) or autoantibodies that met the serology criteria for interstitial pneumonitis with autoimmune features (IPAF)) were associated with the development of CTD during the clinical course of IPF in the patients from our previous study(1), with a particular focus on which antibodies have a significant association with the development of CTD.Methods:We retrospectively reviewed the records of 527 patients with a first diagnosis of IPF between January 2007 and March 2014, and investigated the length of time from first visit to the clinic for IPF diagnosis (baseline) to CTD diagnosis by an expert rheumatologist in patients with IPF. Multivariable Cox proportional-hazards models with backward elimination were used to investigate the risk factors for the development of CTD.Results:CTD developed in 15 patients at a median of 2.1 years (range 1.2 to 4.8) after IPF diagnosis. All these patients had ANCA or autoantibodies that met the serology criteria for IPAF. A significant number of IPF patients with high titers of RF, ACPA or MPO-ANCA tested at first visit to the clinic progressed to CTD(Figure 1). Survival duration for IPF patients with progression to CTD was 5.3 [3.8; 6.7] years, which was significantly longer than for the IPF patients without progression to CTD (2.9 [1.7; 4.8], p = 0.001). Independent risk factors for development of CTD in IPF patients included female gender (adjusted hazard ratio (HR) 5.319, p = 0.0082), titer of rheumatoid factor (RF) (adjusted HR 1.006, p = 0.022), titer of anti-citrullinated protein antibody (ACPA) (adjusted HR 1.009, p = 0.0011), and titer of myeloperoxidase (MPO) ANCA (adjusted HR 1.02, p < 0.0001).Figure 1.Connective tissue disease development in each autoantibody positive IPF patient. ACPA = anti–citrullinated protein antibody; ANA = antinuclear antibody; CTD = connective tissue disease; MPA = microscopic polyangiitis; PAN = polyarteritis nodosa; RA = rheumatoid arthritis; RF = rheumatoid factor; UCTD = Undifferentiated connective tissue disease; SjS = Sjögren’s syndrome.Conclusion:We observed development of CTD in IPF patients with ANCA or autoantibodies that met the IPAF serology criteria. Among these autoantibodies, RF, ACPA, and MPO-ANCA were significantly associated with the development of CTD in IPF patients. Progression to CTD is uncommon in IPF patients, but a significant number of IPF patients with high titers of RF, ACPA or MPO-ANCA progressed to connective tissue disease. IPF with high titers of RF, ACPA or MPO-ANCA might be the initial clinical manifestation of connective tissue disease. Further studies are needed to investigate the role of RF, ACPA, and MPO-ANCA in development of pulmonary fibrosis.References:[1]Ghang B, Lee J, Chan Kwon O, Ahn SM, Oh JS, Hong S, et al. Clinical significance of autoantibody positivity in idiopathic pulmonary fibrosis. Respir Med. 2019;155:43-8.Disclosure of Interests:None declared

scholarly journals Physician characteristics associated with treatment initiation patterns in idiopathic pulmonary fibrosis

2019 ◽  
Vol 16 ◽  
pp. 147997311987967 ◽  
Author(s):  
Peter P LaCamera ◽  
Susan L Limb ◽  
Tmirah Haselkorn ◽  
Elizabeth A Morgenthien ◽  
John L Stauffer ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Treatment Initiation ◽  
Group Versus ◽  
Direct Patient Care ◽  
Real World Data ◽  
Antifibrotic Therapy ◽  
Therapy Initiation ◽  
Physician Characteristics ◽  
Delayed Group

Pirfenidone and nintedanib are oral antifibrotic agents approved for the treatment of idiopathic pulmonary fibrosis (IPF). Real-world data on factors that influence IPF treatment decisions are limited. Physician characteristics associated with antifibrotic therapy initiation following an IPF diagnosis were examined in a sample of US pulmonologists. An online, self-administered survey was fielded to pulmonologists between April 10, 2017, and May 17, 2017. Pulmonologists were included if they spent >20% of their time in direct patient care and had ≥5 patients with IPF receiving antifibrotics. Participants answered questions regarding timing and reasons for considering the initiation of antifibrotic therapy after an IPF diagnosis. A total of 169 pulmonologists participated. The majority (81.7%) considered initiating antifibrotic therapy immediately after IPF diagnosis all or most of the time (immediate group), while 18.3% considered it only some of the time or not at all (delayed group). Pulmonologists in the immediate group were more likely to work in private practice (26.1%), have a greater mean percentage of patients receiving antifibrotic therapy (60.8%), and decide to initiate treatment themselves (31.2%) versus those in the delayed group (16.1%, 30.5%, and 16.1%, respectively). Most pulmonologists consider initiating antifibrotic treatment immediately after establishing an IPF diagnosis all or most of the time versus using a “watch-and-wait” approach. Distinguishing characteristics between pulmonologists in the immediate group versus the delayed group included practice setting, percentage of patients receiving antifibrotic therapy, and the decision-making dynamics between the patient and the pulmonologist.

Cause of mortality and sarcopenia in patients with idiopathic pulmonary fibrosis receiving antifibrotic therapy

Respirology ◽  
2020 ◽  
Author(s):  
Yuzo Suzuki ◽  
Yuya Aono ◽  
Masato Kono ◽  
Hirotsugu Hasegawa ◽  
Koushi Yokomura ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Antifibrotic Therapy

scholarly journals Risk of Appendicitis among Children with Different Piped Water Supply: A Nationwide Population-Based Study

2018 ◽  
Vol 15 (8) ◽  
pp. 1601
Author(s):  
Hao-Ming Li ◽  
Shi-Zuo Liu ◽  
Ying-Kai Huang ◽  
Yuan-Chih Su ◽  
Chia-Hung Kao
Keyword(s):  
Water Supply ◽  
Proportional Hazards ◽  
Population Based ◽  
Incidence Rates ◽  
Cox Proportional Hazards ◽  
Sensitivity Analyses ◽  
Study Cohort ◽  
Research Database ◽  
Piped Water ◽  
Increased Risk

Appendicitis is a common surgical condition for children. However, environmental effects, such as piped water supply, on pediatric appendicitis risk remain unclear. This longitudinal, nationwide, cohort study aimed to compare the risk of appendicitis among children with different levels of piped water supply. Using data from Taiwan Water Resource Agency and National Health Insurance Research Database, we identified 119,128 children born in 1996–2010 from areas of the lowest piped water supply (prevalence 51.21% to 63.06%) as the study cohort; additional 119,128 children of the same period in areas of the highest piped water supply (prevalence 98.97% to 99.63%) were selected as the controls. Both cohorts were propensity-score matched by baseline variables. We calculated the hazard ratios (HRs) and 95% confidence intervals (CIs) of appendicitis in the study cohort compared to the controls by Cox proportional hazards regression. The study cohort had a raised overall incidence rates of appendicitis compared to the control cohort (12.8 vs. 8.7 per 10,000 person-years). After covariate adjustment, the risk of appendicitis was significantly increased in the study cohort (adjusted HR = 1.46, 95% CI: 1.35, 1.58, p < 0.001). Subgroup and sensitivity analyses showed consistent results that children with low piped water supply had a higher risk of appendicitis than those with high piped water supply. This study demonstrated that children with low piped water supply were at an increased risk of appendicitis. Enhancement of piped water availability in areas lacking adequate, secure, and sanitized water supply may protect children against appendicitis.

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