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1.
Ann Am Thorac Soc ; 2021 Apr 12.
Artigo em Inglês | MEDLINE | ID: mdl-33844935

RESUMO

RATIONALE: Interpreting the radiological data in conjunction with an objective clinical score could help to harmonize IPF diagnostic and improve accuracy. OBJECTIVES: We sought to establish and validate a multivariable objective scoring model based on clinical parameters, by stratifying the risk for patients to be diagnosed as IPF versus other forms of interstitial lung disease (ILD). METHODS: A clinical score was derived from review of patients evaluated at the Inova Fairfax ILD program and validated in three distinct cohorts. Based on known IPF clinical characteristics, a multivariable model was created and assessed by receiver operator curve (ROC) characteristics. RESULTS: There were 844 ILD patients diagnosed as either IPF (n=347, 41%) or non-IPF ILD (n=497, 59%). Based on calculated odds ratios, a score was assigned to each of the following clinical parameters: age, sex, smoking history, ethnicity, ILD family history, exposures, presence of CTD signs or symptoms, and Velcro crackles. The final Fairfax IPF clinical score (FICS) ranged from 1 to 25. The clinical diagnostic score system was accurate in predicting IPF, as measured by the area under the curve (AUC 0.88) in the derivation cohort with similar AUCs of 0.91, 0.81 and 0.71 in the respective validation cohorts. CONCLUSION: The FICS appears to be an accurate tool for estimating the pretest probability of IPF in patients with ILD. How the FICS performs in conjunction with the various HRCT patterns remains to be determined. This model could ultimately be useful for increasing the degree of confidence in the final diagnosis and help to obviate the need for lung biopsy in cases of non-UIP patterns on HRCT.

2.
Artigo em Inglês | MEDLINE | ID: mdl-33814284

RESUMO

BACKGROUND: Primary graft dysfunction (PGD) is a risk factor for chronic lung allograft dysfunction (CLAD). However, the association between PGD and degree of allograft injury remains poorly defined. In this study, we leverage a novel biomarker for allograft injury, percentage donor-derived cell-free DNA (%ddcfDNA), to study the association between PGD, degree of allograft injury, and the development of CLAD. METHODS: This prospective cohort study recruited 99 lung transplant recipients and collected plasma samples on days 1, 3, and 7 for %ddcfDNA measurements. Clinical data on day 3 was used to adjudicate for PGD. %ddcfDNA levels were compared between PGD grades. In PGD patients, %ddcfDNA was compared between those who developed CLAD and those who did not. RESULTS: On posttransplant day 3, %ddcfDNA was higher in PGD than in non-PGD patients (median [IQR]: 12.2% [8.2, 22.0] vs 8.5% [5.6, 13.2] p = 0.01). %ddcfDNA correlated with the severity grade of PGD (r = 0.24, p = 0.02). Within the PGD group, higher levels of %ddcfDNA correlated with increased risk of developing CLAD (log OR(SE) 1.38 (0.53), p = 0.009). PGD patients who developed CLAD showed ∼2-times higher %ddcfDNA levels than patients who did not develop CLAD (median [IQR]: 22.4% [11.8, 27.6] vs 9.9% [6.7, 14.9], p = 0.007). CONCLUSION: PGD patients demonstrated increased early posttransplant allograft injury, as measured by %ddcfDNA, in comparison to non-PGD patients, and these high %ddcfDNA levels were associated with subsequent development of CLAD. This study suggests that %ddcfDNA identifies PGD patients at greater risk of CLAD than PGD alone.

3.
Artigo em Inglês | MEDLINE | ID: mdl-33744088

RESUMO

BACKGROUND: Riociguat in Patients with Symptomatic Pulmonary Hypertension associated with Idiopathic Interstitial Pneumonias (RISE-IIP), a randomized, controlled, phase 2b trial of riociguat for pulmonary hypertension associated with idiopathic interstitial pneumonia, was terminated early due to increased mortality in riociguat-treated patients. Baseline characteristics of enrolled patients demonstrated a low diffusing capacity of the lung for carbon monoxide (DLCO) with preserved lung volumes at baseline, suggesting the presence of combined pulmonary fibrosis and emphysema (CPFE) in some patients. This post hoc analysis of RISE-IIP was undertaken to explore lung morphology, assessed by high-resolution computed tomography, and associated clinical outcomes. METHODS: Available baseline/pre-baseline high-resolution computed tomography scans were reviewed centrally by 2 radiologists. The extent of emphysema and fibrosis was retrospectively scored and combined to provide the total CPFE score. RESULTS: Data were available for 65/147 patients (44%), including 15/27 fatal cases (56%). Of these, 41/65 patients (63%) had CPFE. Mortality was higher in patients with CPFE (12/41; 29%) than those without (3/24; 13%). Fourteen patients with CPFE had emphysema > fibrosis (4 died). No relationship was observed between CPFE score, survival status, and treatment assignment. A low DLCO, short 6-min walking distance, and high forced vital capacity:DLCO ratio at baseline also appeared to be risk factors for mortality. CONCLUSIONS: High parenchymal lung disease burden and the presence of more emphysema than fibrosis might have predisposed patients with pulmonary hypertension associated with idiopathic interstitial pneumonia to poor outcomes in RISE-IIP. Future studies of therapy for group 3 pulmonary hypertension should include centrally adjudicated imaging for morphologic phenotyping and disease burden evaluation during screening.

4.
N Engl J Med ; 384(4): 325-334, 2021 01 28.
Artigo em Inglês | MEDLINE | ID: mdl-33440084

RESUMO

BACKGROUND: No therapies are currently approved for the treatment of pulmonary hypertension in patients with interstitial lung disease. The safety and efficacy of inhaled treprostinil for patients with this condition are unclear. METHODS: We enrolled patients with interstitial lung disease and pulmonary hypertension (documented by right heart catheterization) in a multicenter, randomized, double-blind, placebo-controlled, 16-week trial. Patients were assigned in a 1:1 ratio to receive inhaled treprostinil, administered by means of an ultrasonic, pulsed-delivery nebulizer in up to 12 breaths (total, 72 µg) four times daily, or placebo. The primary efficacy end point was the difference between the two groups in the change in peak 6-minute walk distance from baseline to week 16. Secondary end points included the change in N-terminal pro-B-type natriuretic peptide (NT-proBNP) level at week 16 and the time to clinical worsening. RESULTS: A total of 326 patients underwent randomization, with 163 assigned to inhaled treprostinil and 163 to placebo. Baseline characteristics were similar in the two groups. At week 16, the least-squares mean difference between the treprostinil group and the placebo group in the change from baseline in the 6-minute walk distance was 31.12 m (95% confidence interval [CI], 16.85 to 45.39; P<0.001). There was a reduction of 15% in NT-proBNP levels from baseline with inhaled treprostinil as compared with an increase of 46% with placebo (treatment ratio, 0.58; 95% CI, 0.47 to 0.72; P<0.001). Clinical worsening occurred in 37 patients (22.7%) in the treprostinil group as compared with 54 patients (33.1%) in the placebo group (hazard ratio, 0.61; 95% CI, 0.40 to 0.92; P = 0.04 by the log-rank test). The most frequently reported adverse events were cough, headache, dyspnea, dizziness, nausea, fatigue, and diarrhea. CONCLUSIONS: In patients with pulmonary hypertension due to interstitial lung disease, inhaled treprostinil improved exercise capacity from baseline, assessed with the use of a 6-minute walk test, as compared with placebo. (Funded by United Therapeutics; INCREASE ClinicalTrials.gov number, NCT02630316.).


Assuntos
Anti-Hipertensivos/uso terapêutico , Epoprostenol/análogos & derivados , Hipertensão Pulmonar/tratamento farmacológico , Doenças Pulmonares Intersticiais/complicações , Teste de Caminhada , Administração por Inalação , Adulto , Idoso , Idoso de 80 Anos ou mais , Anti-Hipertensivos/efeitos adversos , Método Duplo-Cego , Epoprostenol/efeitos adversos , Epoprostenol/uso terapêutico , Tolerância ao Exercício/efeitos dos fármacos , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Análise dos Mínimos Quadrados , Masculino , Pessoa de Meia-Idade , Qualidade de Vida
5.
Respir Res ; 22(1): 5, 2021 Jan 06.
Artigo em Inglês | MEDLINE | ID: mdl-33407450

RESUMO

RESEARCH QUESTION: There is no widely accepted grading system for IPF disease severity, although physiologic impairment based on pulmonary function testing is frequently employed. We sought to describe clinical and functional characteristics as well as outcomes of patients with severe physiologic impairment. PATIENTS AND METHODS: IPF patients with severe physiologic impairment defined by FVC ≤ 50% and/or DLco ≤ 30% predicted evaluated in the Inova Advanced Lung Disease Program between 2011 and 2019 were included. Demographic, physiologic, functional treatment and outcome data were collated. RESULTS: There were 531 patients with IPF evaluated of whom 242 (46%) had severe physiologic impairment. Mean age was 72 ± 8 years; baseline FVC was 53 ± 17% and DLCO 28 ± 9% of predicted. The mean 6 min walks test (6MWT) distance was 304 ± 121 m with 59% of the patients requiring supplemental oxygen ([Formula: see text] group). There was a poor correlation between the 6MWT distance and both FVC% and DLco%. Patients in the 6MWTRA group had a better transplant-free survival than the [Formula: see text] group (p = 0.002). Patients managed before October 2014 and not receiving antifibrotic therapy had worse outcomes with reduced transplant-free survival compared with patients presenting after this date who did receive antifibrotic therapy (n = 113) (log rank p < 0.0001). CONCLUSION: IPF patients often present with severe physiologic impairment which may be poorly correlated with their functional status. Assessment of IPF disease severity should not be based on physiologic impairment alone, but should also encompass functional status as well as need for supplemental oxygen. Antifibrotic therapy in patients with severe physiologic impairment is associated with improved outcomes.

6.
Respir Care ; 2020 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-33328179

RESUMO

BACKGROUND: Optimal timing of mechanical ventilation in COVID-19 is uncertain. We sought to evaluate outcomes of delayed intubation and examine the ROX index (ie, [SpO2 /FIO2 ]/breathing frequency) to predict weaning from high-flow nasal cannula (HFNC) in patients with COVID-19. METHODS: We performed a multicenter, retrospective, observational cohort study of subjects with respiratory failure due to COVID-19 and managed with HFNC. The ROX index was applied to predict HFNC success. Subjects that failed HFNC were divided into early HFNC failure (≤ 48 h of HFNC therapy prior to mechanical ventilation) and late failure (> 48 h). Standard statistical comparisons and regression analyses were used to compare overall hospital mortality and secondary end points, including time-specific mortality, need for extracorporeal membrane oxygenation, and ICU length of stay between early and late failure groups. RESULTS: 272 subjects with COVID-19 were managed with HFNC. One hundred sixty-four (60.3%) were successfully weaned from HFNC, and 111 (67.7%) of those weaned were managed solely in non-ICU settings. ROX index >3.0 at 2, 6, and 12 hours after initiation of HFNC was 85.3% sensitive for identifying subsequent HFNC success. One hundred eight subjects were intubated for failure of HFNC (61 early failures and 47 late failures). Mortality after HFNC failure was high (45.4%). There was no statistical difference in hospital mortality (39.3% vs 53.2%, P = .18) or any of the secondary end points between early and late HFNC failure groups. This remained true even when adjusted for covariates. CONCLUSIONS: In this retrospective review, HFNC was a viable strategy and mechanical ventilation was unecessary in the majority of subjects. In the minority that progressed to mechanical ventilation, duration of HFNC did not differentiate subjects with worse clinical outcomes. The ROX index was sensitive for the identification of subjects successfully weaned from HFNC. Prospective studies in COVID-19 are warranted to confirm these findings and to optimize patient selection for use of HFNC in this disease.

7.
Contemp Clin Trials ; 100: 106227, 2020 Nov 25.
Artigo em Inglês | MEDLINE | ID: mdl-33246099

RESUMO

The 6-min walk test (6MWT) is an important measure of functional capacity in idiopathic pulmonary fibrosis (IPF) and has been an endpoint of several IPF clinical trials. However, current guidance for the 6MWT offers insufficient advice on standardization, particularly oxygen supplementation, for clinical trials. Three physicians experienced with the 6MWT and IPF developed a standardized protocol for the 6MWT based on existing clinical guidelines and published literature. The protocol comprises guidance on test conditions, pre-defined parameters to measure at specified timepoints, and step-by-step instructions on conducting the test. The standardized test will be evaluated in the large-scale phase 3 ISABELA trials (NCT03711162; NCT03733444). The test is conducted indoors, using standardized equipment, along a flat, straight, 30-m unobstructed corridor; tests for each individual are performed by the same administrators at the same time of day; warm-up prior to testing is prohibited; supplemental oxygen tanks are permitted and moved by the patient in the same manner for each test; precise wording is used to instruct and encourage patients. Contraindications and stopping criteria are specified. Key assessments include: 6-min walk distance, distance walked at 1 and 3 min, the Borg CR10 scale, heart rate, blood pressure, and oxygen desaturation levels. A standardized 6MWT for IPF will enable more reliable comparisons between clinical trials and limit variability, optimizing use as an endpoint. Application of the standardized 6MWT in the ISABELA program will allow its correlation with other clinically important endpoints and may lead to novel composite endpoints for use in future trials. Submission category: Study Design, Statistical Design, Study Protocols. Submission classifications: Clinical study methodology; Clinical trial design; Clinical trials; Pulmonary disease; Pulmonary disease clinical trial; Respiratory medicine.

8.
Chest ; 2020 Oct 16.
Artigo em Inglês | MEDLINE | ID: mdl-33075376

RESUMO

BACKGROUND: Aberrations in the coagulation system have been implicated in the pathogenesis of interstitial lung disease (ILD). Anticoagulants have been proposed as a potential therapy in ILD; however, a randomized control trial examining warfarin as a treatment for IPF was terminated early for harm. This has led some to speculate that warfarin specifically may be harmful in ILD, and use of direct oral anticoagulants (DOACs) may result in superior outcomes. RESEARCH QUESTION: We sought to delineate the relationship between anticoagulation and outcomes in patients with ILD through an analysis of the Pulmonary Fibrosis Foundation (PFF) registry. STUDY DESIGN: and Methods: An analysis of all patients in the PFF registry was performed. Patients were stratified into three groups: no anticoagulation (AC), DOAC use, or warfarin use. Survival was analyzed using both Kaplan Meier curves and Cox proportional hazards models. RESULTS: Of 1,911 patients included in the analysis, 174 (9.1%) were anticoagulated, 93 (4.9%) with DOACs, and 81 (4.2%) with warfarin. There was a two-fold increased risk of death or transplant for patients receiving DOACS, while for warfarin this was over a two and half times greater risk. DOACs were not associated with an increased risk of mortality after adjustment for confounding variables. However, even after adjustment, patients anticoagulated with warfarin remained at increased risk of mortality. In patients with IPF, warfarin was associated with reduced transplant free survival, but DOACs were not. There was no statistically significant difference in survival between those receiving warfarin and those receiving a DOAC. INTERPRETATION: The need for anticoagulation is associated with an increased risk for death or transplant in patients with ILD, in both the IPF and non-IPF population. Further research is required to determine if warfarin and DOACs present varying safety profiles in ILD patients. (292 words).

9.
Ther Adv Respir Dis ; 14: 1753466620968496, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33121391

RESUMO

BACKGROUND AND AIMS: Chest high-resolution computed tomography (HRCT) is the central diagnostic tool in discerning idiopathic pulmonary fibrosis (IPF) from other interstitial lung disease (ILDs). In 2018, new guidelines were published and the nomenclature for HRCT interpretation was changed. We sought to evaluate how clinicians' interpretation would change based on reading HRCTs under the framework of the old versus new categorization. MATERIALS AND METHODS: We collated HRCTs from 50 random cases evaluated in the Inova Fairfax ILD clinic. Six ILD experts were provided the deidentified HRCTs. They were all instructed to independently provide two reads of each HRCT, based on the old and the new guidelines. RESULTS: The kappa statistic for concordance for HRCT reads under old guidelines was 0.5, while for the new guidelines it was 0.38. Under the framework of the old guidelines, there were 22 HRCTs with unanimous consensus reads, while only 15 with the new guidelines. There were 12 HRCTs read unanimously as usual interstitial pneumonia (UIP) pattern based on both the old and the new guidelines. Ten HRCTs were read as a possible UIP pattern based on the old guidelines and were classified in nine cases as probable UIP and one indeterminate based on the new guidelines. Of the 28 inconsistent UIP HRCTs (old guidelines), 25 were read as alternative diagnosis suggested, two were read as indeterminate and one as probable UIP. CONCLUSION: Implementation of the new guidelines to categorize HRCTs in ILD patients appears to be associated with greater inter-interpreter variability. How or whether new guidelines improve the care and management of ILD patients remains unclear.The reviews of this paper are available via the supplemental material section.

10.
Ann Am Thorac Soc ; 2020 Sep 24.
Artigo em Inglês | MEDLINE | ID: mdl-32970474

RESUMO

RATIONALE: Video-assisted thoracoscopic surgery (VATS) remains the gold standard for interstitial lung disease (ILD) characterization when histology is deemed necessary. There is diminishing utilization of VATS owing to increased reliance on high resolution computed tomographic patterns, as well as concerns regarding the potential morbidity and mortality of the procedure. OBJECTIVE: The goal of this study was to evaluate the safety and tolerability of VATS among a broad group of ILD patients referred to a tertiary care center. METHODS: Data for all patients with ILD who underwent VATS lung biopsies at Inova Fairfax hospital for the period December 2012 to September 2019, was collected. Clinical, physiologic and functional parameters as well as post-operative outcomes including any complications, hospital length of stay and mortality were collated. RESULTS: There were 268 diagnostic VATS biopsies performed during the period. The mean age of the cohort was 63 ± 13 years, 54% were male and 25% were ultimately diagnosed with IPF. 229 patients were scheduled (85%, Elective VATS group) whereas 39 were inpatients (15%). In the elective group, the 1-month complication rate was 8%, while 4% had a severe complication, and there were no deaths. The only mortalities were in the group who were hospitalized prior to the VATS (4/39=10%). Complications were less frequent when VATS was requested by the tertiary referral ILD team. Of the elective group, 87% patients were discharged the same day. CONCLUSION: This report demonstrates the safety, tolerability and feasibility of VATS lung biopsy as a same day procedure in the modern era, especially if patients are first vetted by a team with expertise in the field of ILD. These results support a lower threshold to pursue a VATS biopsy when histology is required for an ILD diagnosis.

11.
Expert Rev Respir Med ; : 1-7, 2020 Sep 28.
Artigo em Inglês | MEDLINE | ID: mdl-32985286

RESUMO

INTRODUCTION: Forced vital capacity (FVC) decline is predictive of mortality in patients with idiopathic pulmonary fibrosis (IPF) and has been used as a clinical trial endpoint to define disease progression. How to interpret FVC findings in an individual patient with IPF in the real-world setting amid uncertainty about the measurement accuracy and variability has not been well established. AREAS COVERED: This review highlights the challenges and limitations of using FVC in the clinic to monitor disease progression in patients with IPF. Spirometry is noninvasive, relatively simple, and inexpensive. FVC measurements provide evidence for trends over time in patients with IPF. When using FVC in the clinic, several important challenges and limitations, including visit-to-visit variability, dependence on patient effort, inconsistent quality control, limitations on accuracy, and the influence of comorbidities and pretest factors, must be considered. Recent studies suggest the potential for home spirometry devices to facilitate more frequent collection of data and perhaps demonstrate more accurate trends. EXPERT OPINION: Measuring FVC decline in the clinic has an important role in monitoring disease progression in patients with IPF, but additional measures of disease progression should be considered along with FVC to facilitate decision-making about disease management.

12.
Lancet Respir Med ; 2020 Aug 18.
Artigo em Inglês | MEDLINE | ID: mdl-32822614

RESUMO

BACKGROUND: The benefit of sildenafil in patients with advanced idiopathic pulmonary fibrosis (IPF) at risk of poor outcomes from pulmonary hypertension, whether already present or likely to develop, is uncertain. We aimed to assess the efficacy and safety of sildenafil added to pirfenidone versus placebo added to pirfenidone for 52 weeks in patients with advanced IPF and at risk of group 3 pulmonary hypertension. METHODS: We did a multicentre, international, double-blind, randomised, placebo-controlled, phase 2b study at 56 university clinics, research hospitals, and tertiary sites in Canada, Europe (Belgium, Czech Republic, Germany, Greece, Hungary, Italy, the Netherlands, Spain, and Turkey), Israel, and Africa (Egypt and South Africa). Eligible patients (aged 40-80 years) had advanced IPF (carbon monoxide diffusing capacity ≤40% predicted at screening), and were at risk of group 3 pulmonary hypertension (mean pulmonary artery pressure of ≥20 mm Hg with pulmonary artery wedge pressure of ≤15 mm Hg on previous right-heart catheterisation, or intermediate or high probability of group 3 pulmonary hypertension on echocardiography as defined by the 2015 European Society of Cardiology and European Respiratory Society guidelines). Patients were randomly assigned 1:1 to oral sildenafil tablets (20 mg three times daily) or placebo, both in addition to oral pirfenidone capsules (801 mg three times daily), using a validated interactive voice-based or web-based response system with permuted block randomisation, stratified by previous right-heart catheterisation (yes or no) and forced expiratory volume in 1 s to forced vital capacity ratio (<0·8 or ≥0·8). The composite primary endpoint was disease progression, defined as either a relevant decline in 6-min walk distance, respiratory-related admission to hospital, or all-cause mortality, after 52 weeks and was assessed in the intention-to-treat population; safety was assessed in all patients who received at least one dose of the study drug. This trial is registered with ClinicalTrials.gov, NCT02951429, and is no longer recruiting. The 11-month safety follow-up is ongoing. FINDINGS: Between Jan 13, 2017, and Aug 30, 2018, 247 patients were screened for eligibility, 177 of whom were randomly assigned to a treatment group (n=88 sildenafil; n=89 placebo) and were assessed for the primary outcome. There was no difference in the proportion of patients with disease progression over 52 weeks between the sildenafil (64 [73%] of 88 patients) and placebo groups (62 [70%] of 89 patients; between-group difference 3·06% [95% CI -11·30 to 17·97]; p=0·65). Serious treatment-emergent adverse events were reported in 54 (61%) patients in the sildenafil group and 55 (62%) patients in the placebo group. Treatment-emergent adverse events leading to mortality occurred in 22 (25%) patients in the sildenafil group and 26 (29%) in the placebo group. INTERPRETATION: Addition of sildenafil to pirfenidone did not provide a treatment benefit versus pirfenidone plus placebo up to 52 weeks in patients with advanced IPF and risk of pulmonary hypertension. No new safety signals were identified with either treatment. Although the absence of a beneficial treatment effect suggests that sildenafil is not an appropriate treatment in the overall population, further research is required to establish if specific subgroups of patients with IPF might benefit from sildenafil. FUNDING: F Hoffmann-La Roche.

13.
Ann Am Thorac Soc ; 17(12): 1620-1628, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32776789

RESUMO

Detailed understanding of longitudinal behavior, response to therapy, and applicable biomarkers for interstitial lung diseases (ILDs) is lacking. There is a need for a large multicenter registry that provides researchers and clinicians access to well-characterized data not limited to patients with idiopathic pulmonary fibrosis. The Pulmonary Fibrosis Foundation Patient Registry (PFF-PR) is a database that collects baseline and longitudinal demographic and clinical information about patients with ILDs in the United States. The objective of this study is to describe the patient population, data collection process, and opportunities for retrospective and prospective research with the PFF-PR. Individuals 18 years or older who had ILD diagnosed and who were seen at PFF-PR centers who provided informed consent were eligible to participate. Baseline and longitudinal demographic, spirometric, radiographic, morbidity, and mortality data are recorded into a secure electronic data capture system. Starting in 2016, the PFF-PR has collected data on 2,003 patients at 42 clinical sites in the United States. At the time of enrollment, the mean age of participants was 68 years old. Most (62%) of participants were male, and 58% had a positive smoking history. The mean forced vital capacity was 69% predicted, and the mean diffusing capacity of the lung for carbon monoxide was 43% predicted. Forty-one percent of patients were using supplemental oxygen, and 39% were on antifibrotic therapy. Reasons for attrition were mostly death or transplant, with low rates of loss to follow-up or withdrawal. The PFF-PR is a large multicenter United States-based registry that provides researchers and clinicians access to well-characterized ILD patient data.

14.
Respir Med ; 170: 106068, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32843183

RESUMO

BACKGROUND: The course of idiopathic pulmonary fibrosis (IPF) is uncertain with variable patterns of disease progression. We sought to evaluate the prognostic utility of the WBC, a routinely performed lab test, in a well-defined cohort of outpatient IPF subjects. METHODS: We reviewed IPF patient records from two independent ILD centers (Inova Fairfax in Falls Church, VA, USA and Ege University Hospital in Izmir, Turkey) between 2007 and 2018. Demographics, CBC data, and patient outcomes were obtained. Survival differences were analyzed. RESULTS: There were 436 IPF outpatients in the cohort with a median WBC of 8.9 × 109 cells per liter. For pragmatic purposes, patients were categorized into two groups, WBC ≥9 or WBC <9. Patients with WBC <9 had a median transplant-free survival of 50.5 months from the time of the CBC, compared to 32.4 months for those with WBC ≥9 (p < 0.0001). The association between WBC and attenuated survival remained significant after adjusting for GAP stage, steroid use, and antifibrotic use when WBC was analyzed both as a continuous (HR: 1.11; 95% CI: 1.05-1.17) and a dichotomized variable (high (WBC ≥9) vs. low (WBC <9), (HR: 1.53; 95% CI:1.09-2.15). WBC and absolute neutrophil count (ANC) were highly correlated suggesting that PMNs account for most of this association (r = 0.92). CONCLUSIONS: Baseline WBC may impart important and readily available prognostic information in outpatients with IPF. Further studies are warranted to validate this as a potential biomarker for IPF, as well as to define the biologic basis for the association.

15.
Artigo em Inglês | MEDLINE | ID: mdl-32721166

RESUMO

Rationale Usual interstitial pneumonia (UIP) is the defining morphology of idiopathic pulmonary fibrosis (IPF). Guidelines for IPF diagnosis conditionally recommend surgical lung biopsy (SLB) for histopathology diagnosis of UIP when radiology and clinical context are not definitive. A 'molecular diagnosis of UIP' in transbronchial lung biopsy (TBBx), the Envisia Genomic Classifier, accurately predicted histopathologic UIP. Objectives We evaluated the combined accuracy of the Envisia Genomic Classifier and local radiology in the detection of UIP pattern. Methods Ninety-six patients who had diagnostic lung pathology, as well as a TBBx for molecular testing with Envisia Genomic Classier, were included in this analysis. The classifier results were scored against reference pathology. UIP identified on HRCT as documented by features in local radiologists' reports was compared to histopathology. Measurements and Main Results In 96 patients, the Envisia classifier achieved a specificity of 92.1% [CI:78.6%-98.3%] and a sensitivity of 60.3% [CI:46.6%-73.0%] for histology-proven UIP pattern. Local radiologists identified UIP in 18 of 53 patients with UIP histopathology with a sensitivity of 34.0% [CI:21.5%-48.3%], and a specificity of 96.9% [CI:83.8 - 100]). In conjunction with HRCT patterns of UIP, the Envisia classifier results identified 24 additional UIP patients (sensitivity 79.2% specificity 90.6%). Conclusions In 96 patients with suspected ILD, the Envisia Genomic Classifier identified UIP regardless of HRCT pattern. These results suggest that recognition of a UIP pattern by the Envisia Genomic Classifier combined with HRCT and clinical factors in a multidisciplinary discussion may assist clinicians in making an ILD (especially IPF) diagnosis without the need for SLB.

16.
BMC Pulm Med ; 20(1): 191, 2020 Jul 14.
Artigo em Inglês | MEDLINE | ID: mdl-32664913

RESUMO

BACKGROUND: In patients with idiopathic pulmonary fibrosis (IPF) treated with pirfenidone (Esbriet®, Genentech USA, Inc. South San Francisco, CA.), effectively managing treatment-related adverse events (AEs) may improve adherence. Due to a lack of clinical evidence and expertise, managing these AEs can be challenging for patients and physicians alike. In the absence of evidence, consensus recommendations from physicians experienced in using pirfenidone to treat IPF are beneficial. METHODS: Using a modified Delphi process, expert recommendations were developed by a panel of physicians experienced with using pirfenidone for IPF. Over three iterations, panelists developed and refined a series of statements on the use of pirfenidone in IPF. Their agreement on each statement was ranked using a Likert scale. RESULTS: A panel of 12 physicians participated and developed a total of 286 statements on dosing and administration, special populations, drug-drug interactions, laboratory analysis, warnings and precautions, and AE management. Expert recommendations were achieved with regard to slower initial titrations and slower titrations for AEs, dosing with meal(s) or substantial meals, and adding other prescribed pharmacological agents for AEs. CONCLUSION: Until there is further clinical evidence, the resulting consensus recommendations are intended to provide direction on the practical management of IPF with pirfenidone, by encompassing a broad experience from the real world to complement data gleaned from clinical trials.

18.
Chest ; 157(6): 1415, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32505306
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