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.

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.

A score without diffusion capacity of the lung for carbon monoxide for estimating survival in idiopathic pulmonary fibrosis.

Prediction models for survival at baseline evaluation have been proposed in idiopathic pulmonary fibrosis (IPF) but include diffusion capacity of the lung for carbon monoxide, a test not available in many places. The aim of the present study was to develop a simple new mortality risk scoring system for patients with IPF at initial evaluation without diffusion capacity of the lung for carbon monoxide measurement.A total of 173 patients, 72% males, mean age 70 years, 64% smokers/ex-smokers, were included in a retrospective study. The diagnosis was made by surgical lung biopsy in 40 (23%); in the remaining patients, a usual interstitial pneumonia pattern was present in high-resolution computed tomography. Patients with forced expiratory volume in 1 second/forced vital capacity ratio (FEV1/FVC) <0.70 were excluded. Dyspnea was evaluated by magnitude of task on the Mahler scale (Chest 1984). Peripheral oxygen saturation was measured by oximetry at rest and at the end of a 4 minutes step test or a 6-minute walk test.At the end of the follow-up period, 154 (89%) of the patients had died. Based on the univariate Cox proportional-hazards model, survival (P ≤ .10) was related directly to the dyspnea score, presence of cough, lower values of FVC% and FEV1%, lower rest and oxygen desaturation during exercise, and greater FEV1/FVC. By Cox multivariate analysis, the results remained correlated to the survival dyspnea score, FVC%, and exercise peripheral oxygen saturation. A score, using these variables, was developed and was able to discriminate among 3 groups, with high, low, and intermediate survival curves.A prognostic score, taking into account dyspnea, FVC%, and oxygen desaturation during exercise, can estimate survival in IPF.

Squawks in interstitial lung disease prevalence and causes in a cohort of one thousand patients.

Squawks are lung adventitious sounds with a mix of both musical and nonmusical components heard during the inspiratory phase. Small series have described squawks in interstitial lung diseases. Hypersensitivity pneumonitis and other diseases involving small airways can result in squawks, but new interstitial lung diseases (ILDs) involving peripheral airways are being described. A retrospective analysis was performed on 1000 consecutive patients from a database of ILD of a tertiary referral center. Squawks were recorded in 49 cases (4.9%), hypersensitivity pneumonitis (23 cases), connective tissue disease (7), microaspiration (4), pleuroparenchymal fibroelastosis (4), fibrosing cryptogenic organizing pneumonia (, 3), familial ILD (2), sarcoidosis (2), idiopathic pulmonary fibrosis (IPF; 1), bronchiolitis (2), and nonspecific interstitial pneumonia (1). One patient had a final diagnosis of IPF. There was a significant association between mosaic pattern and squawks: 20 cases with squawks (40.8%) had mosaic pattern compared with 140 (14.7%) cases without squawks (x = 23.6, P < .001).Findings indicative of fibrosis were described on high-resolution chest tomography (HRCT) in 715 cases (71.5%). Squawks were more common in patients with findings indicative of fibrosis on HRCT: 45 of 715 (6.3%) compared with 4 of 285 (1.4%) of those without findings indicative of fibrosis (x = 10.46, P = .001).In conclusion, squawks are an uncommon finding on physical examination in patients with ILD, but when present suggest fibrosing ILD associated with bronchiolar involvement. However, squawks are rare in IPF.

Diagnostic accuracy of home-based monitoring system in morbidly obese patients with high risk for sleep apnea.

BACKGROUND: No previous studies have validated the use of portable monitoring (PM) for the diagnosis of obstructive sleep apnea (OSA) in morbidly obese individuals. Our aim was to investigate the accuracy of PM for detecting respiratory events in morbidly obese patients that will be undergoing bariatric surgery. METHODS: This was a prospective study involving patients with body mass index (BMI) ≥35 kg/m(2) who were recruited from the Sleep Clinic of Universidade Federal de São Paulo. Sleep-disordered breathing (SDB) was evaluated during full-night polysomnography (PSG). PM use was randomized and used on two consecutive nights: (1) at home (STDHome) and (2) at the sleep laboratory with PSG (PSG_STDLab). RESULTS: Although 58 participants initially underwent the recordings, 26 (45%) were excluded because of technical problems. The patients' mean age was 42.9 ± 10.9 (SD) years, and 56% were female. The mean BMI was 40.8 ± 5.2 kg/m(2). All patients had high risk for OSA, as defined by the Stop-Bang questionnaire, and the mean apnea-hypopnea index (AHI) was 46.9 ± 30.4/h. The intraclass coefficient of the correlation between AHI_PSG and AHI_STDLab was r = 0.92 (p = 0.0001); the intraclass coefficient for AHI_PSG and AHI_STDHome was r = 0.84 (p = 0.0001). The Kappa index was 0.87 (p > 0.0001) for severe cases. The sensitivity and the positive predictive value increased with the disease severity. A Bland-Altman analysis showed good agreement between the investigated methods. CONCLUSIONS: PM is an efficacious method for diagnosing OSA in obese patients who have a high clinical probability of the disease. The method displays good sensitivity and specificity in severe cases; nevertheless, the high rate of data loss must be taken into account.