Diagnosis of idiopathic pulmonary fibrosis by virtual means using “IPFdatabase”- a new software

Abstract

Background: The diagnostic algorithm for idiopathic pulmonary fibrosis (IPF) guidelines has some shortcomings. The aim of the present study was to develop a novel software, "IPFdatabase", that could readily apply the diagnostic criteria per IPF guidelines and make a 'virtual' diagnosis of IPF. Methods: Software was developed as a step-by-step compilation of necessary information according to guidelines to enable a diagnosis of IPF. Software accuracy was validated primarily by comparing software diagnoses to those previously made at a Center for Interstitial Lung Diseases. Results: Clinical validation on 98 patients (68 male, age 61.0 ± 8.5 years), revealed high software accuracy for IPF diagnosis when compared to historical diagnoses (sensitivity 95.5%, specificity 96.2%; positive predictive value 95.5%, negative predictive value 96.2%). A general radiologist and a general pathologist reviewed relevant data with and without the new software: interobserver agreement increased when they used the IPFdatabase (kappa 0.18 to 0.64 for radiology, 0.13 to 0.59 for pathology). Conclusion: IPFdatabase is a useful diagnostic tool for typical cases of IPF, and potentially restricts the need for MDDs to atypical and complex cases. We propose this web-designed software for instant accurate diagnosis of IPF by virtual means and for educational purposes; the software is readily accessed with mobile apps, allows incorporation of updated version of guidelines, can be utilized for gathering data useful for future studies and give physicians rapid feedback in daily practice.

Computed tomography patterns predict clinical course of idiopathic pulmonary fibrosis

“…However, interobserver agreement in our study is comparable to that of previous study (kappa value 0.66-0.69) [29]. A recent study of 98 patients with ILD showed that the new software, IPFdatabase, can improve interobserver agreement among radiologists (kappa value of 0.18 to 0.64) [30]. Further studies on enhancing the interobserver agreement are warranted.…”

Combined sarcoidosis and idiopathic pulmonary fibrosis (CSIPF): A novel disease phenotype?

“…The physical examination identified bilateral basal crackles, lung function tests revealed normal lung volumes and reduced DLCO (40%). HRCT with spiral technique [5,6] demonstrated a definite UIP-pattern associated with the presence of parenchymal consolidations, lymphnodes enlargement and upper lobe emphysema. Cellular analysis of bronchoalveolar lavage (BAL) revealed: macrophages 68%, eosinophils 14%, neutrophils 9% and 9% lymphocytes with a CD4+/ CD8+ ratio 2.93.…”

“…He has worked as a bricklayer, in an oil refinery and finally as truck driver. In 2014 due to chest pain the patient performed high resolution CT (HRCT) with spiral technique [5,6] showing emphysema, fine mantellar and peripheral reticulation, widespread nodules predominantly in the upper lobes and concomitant lymphoadenopathies indicative of sarcoidosis. The patient stayed stable since 2017 when, due to clinical deterioration with dyspnea and dry cough, he performed pulmonary function tests (PFT) revealing mild restrictive deficit and DLCO 52%; the 6 minute walking test and autoantibodies were negative.…”

“…The patient stayed stable since 2017 when, due to clinical deterioration with dyspnea and dry cough, he performed pulmonary function tests (PFT) revealing mild restrictive deficit and DLCO 52%; the 6 minute walking test and autoantibodies were negative. HRCT made with spiral technique [5,6] was repeated revealing an increase in diffuse and widespread thickening of the subpleural interstium. The absence of honeycombing and the definite diagnosis of IPF prompted a videothoracoscopic biopsy after multidisciplinary discussion (MDD).…”

“…He underwent pneumologic evaluation for a persistent dry cough and dyspnea. HRCT with spiral technique [5,6] revealed mediastinal lymphadenopathies, reticular thickening of the interstitium in the lower and middle lobes and in the subpleural areas of the upper lobes with honeycombing areas and traction bronchiectasis. Functional lung test showed a restrictive deficit with severe reduction of DLCO (31% of theoretical value).…”