Recognition of Connective Tissue Disease-Related Interstitial Pneumonia Based on Histological Score-A Validation Study of an Online Diagnostic Decision Support Tool.

Mutsumi Ozasa,Hiroshi Mukae,Kensuke Kataoka,Noriho Sakamoto,Yasuhiro Kondoh,Kazuhiro Tabata,Shuntaro Sato,Yoshiaki Zaizen,Andrey Bychkov,Junya Fukuoka

doi:10.3390/diagnostics11081359

Abstract

Objectives: to evaluate the number of cases of idiopathic pulmonary fibrosis (IPF) that included histological features of connective tissue disease (CTD) and to check whether they demonstrated the clinical features of CTD, using a previously reported CTD-interstitial pneumonia (IP) index that histologically differentiates CTD-associated and idiopathic IP. Methods: patients diagnosed with IPF following video-assisted thoracoscopic biopsy through multidisciplinary team diagnosis between 2014 and 2017 were selected. Pathological observation was made by four pathologists who scored eight observational items needed for the CTD-IP index. Cases determined as CTD, by the CTD-IP index, were extracted, and their clinical features were compared. Results: a total of 94 cases of IPF were identified, of which 20 were classified into the CTD group using the CTD-IP index with reasonable interobserver agreement (k = 0.76). Cases pathologically classified into the CTD group were significantly associated with female sex, non-smoking history, autoantibody positivity, and CTD symptoms (p = 0.01, 0.03, 0.01, and 0.04, respectively). Conclusions: patients with IPF with pathological findings of CTD showed clinical characteristics similar to those of patients with CTD.

Highlights

Idiopathic interstitial pneumonias (IIPs) are interstitial pneumonias (IPs) that have no clear systemic disease or cause; they are classified into various types based on the clinical, radiologic, and pathological findings [1,2,3]
There are a number of idiopathic pulmonary fibrosis (IPF) cases where clinical and pathological findings are suggestive of connective tissue disease (CTD) [7,8,9]
94 cases were included in the study, which had a usual IP (UIP) pattern and were diagnosed as IPF through multidisciplinary discussions (MDD)

Summary

Introduction

Idiopathic interstitial pneumonias (IIPs) are interstitial pneumonias (IPs) that have no clear systemic disease or cause; they are classified into various types based on the clinical, radiologic, and pathological findings [1,2,3]. Among the different types of IIPs, idiopathic pulmonary fibrosis (IPF) has the worst prognosis. There are a number of IPF cases where clinical and pathological findings are suggestive of connective tissue disease (CTD) [7,8,9]. Such cases are constantly debated in multidisciplinary discussions (MDD) for inconsistency in the interpretation of histological diagnosis among pathologists. Some MDD teams may diagnose the usual IP (UIP) cases with CTD features as unclassifiable IIP [10,11]

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