Development and Validation of a Clinical Diagnostic Scoring System for the Diagnosis of Idiopathic Pulmonary Fibrosis.

Rationale: Interpreting the radiologic data in conjunction with an objective clinical score could help to harmonize idiopathic pulmonary fibrosis (IPF) diagnosis and improve accuracy. Objectives: We sought to establish and validate a multivariable objective scoring model based on clinical parameters by stratifying the risk of patients having IPF diagnosed versus having other forms of interstitial lung disease (ILD) diagnosis. Methods: A clinical score was derived from review of patients evaluated at the Inova Fairfax ILD Program and validated in three distinct cohorts. On the basis of known IPF clinical characteristics, a multivariable model was created and assessed by using receiver operating characteristic curves. Results: There were 844 patients with ILD with either IPF (n = 347, 41%) or non-IPF ILD (n = 497, 59%) diagnosis. On the basis of calculated odds ratios, a score was assigned to each of the following clinical parameters: age, sex, smoking history, race or ethnicity, ILD family history, exposures, presence of connective tissue disease 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 (0.88) in the derivation cohort, with similar areas under the curve of 0.91, 0.81, and 0.71 being demonstrated in the respective validation cohorts. Conclusions: 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 high-resolution computed tomographic patterns remains to be determined. This model could ultimately be useful for increasing the degree of confidence in the final diagnosis and could help to obviate the need for lung biopsy in cases with non-usual interstitial pneumonia patterns on high-resolution computed tomographic images.

Idiopathic pulmonary fibrosis patients with severe physiologic impairment: characteristics and outcomes.

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.

Surgical Lung Biopsy for Interstitial Lung Disease. Safety and Feasibility at a Tertiary Referral Center.

Rationale: Video-assisted thoracoscopic surgery (VATS) remains the gold standard for interstitial lung disease (ILD) characterization when histology is deemed necessary. There is diminishing use 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.Objectives: The goal of this study was to evaluate the safety and tolerability of VATS among a broad group of patients with ILD 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 from December 2012 to September 2019 were collected. Clinical, physiologic, and functional parameters as well as postoperative 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 idiopathic pulmonary fibrosis. Two hundred twenty-nine patients were scheduled (85%, Elective VATS group) whereas 39 were inpatients (15%). In the elective group, the 1-month complication rate was 8%, whereas 4% had a severe complication, and there were no deaths. The only mortalities were in the group who were hospitalized before 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.Conclusions: 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.

HRCT evaluation of patients with interstitial lung disease: comparison of the 2018 and 2011 diagnostic guidelines.

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.