Evolution of diagnostic UIP computed tomography patterns in idiopathic pulmonary fibrosis: Disease spectrum and implications for survival

Giacomi, Federica De; White, Darin; Cox, Christian W.; Moua, Teng

doi:10.1016/j.rmed.2018.07.014

Cited by 9 publications

(5 citation statements)

References 15 publications

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“…Yamauchi et al reported that in IPF, 53.3% of the patients developed honeycombing over a mean follow-up period of 5.9 years [15]. Giacomi et al also reported the development of honeycombing in 32% of their patients over a median follow-up period of 4.8 years [16]. The present study is, to our knowledge, the first to focus on the development of honeycombing during follow-up for RA-ILD.…”

Section: Discussionsupporting

confidence: 51%

Impact of radiological honeycombing in rheumatoid arthritis-associated interstitial lung disease

et al. 2020

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Background Interstitial lung disease (ILD) is the most common and important pulmonary manifestation of rheumatoid arthritis (RA). A radiological honeycomb pattern has been described in diverse forms of ILD that can impact survival. However, the clinical course and sequential radiological changes in the formation of the honeycomb pattern in patients with RA-ILD is not fully understood. Methods We evaluated the sequential changes in computed tomography findings in 40 patients with chronic forms of RA-ILD without the honeycomb pattern at initial diagnosis. We classified the patients into the Non-honeycomb group and Honeycomb group, and then analyzed the characteristics and prognosis of the two groups. The term “honeycomb formation” indicated a positive finding of honeycombing on any available follow-up CT. Results Our RA-ILD cohort included patients with probable usual interstitial pneumonia (UIP) (35%), nonspecific interstitial pneumonia (NSIP) (20%), and mixed NSIP/UIP (45%). Among all RA-ILD patients, 16 (40%) showed honeycomb formation on follow-up CT (median time between initial and last follow-up CT was 4.7 years). Patient characteristics and prognosis were not significantly different between the Non-honeycomb and Honeycomb groups. However, Kaplan-Meier survival curve for the time from the date of honeycomb formation to death showed a poor median survival time of 3.2 years. Conclusions A certain number of patients with RA-ILD developed a honeycomb pattern during long-term follow-up, regardless of whether they had UIP or NSIP. Prognosis in the patients with characteristics of both progressive ILD and honeycomb formation could be poor. Although radiological findings over the disease course and clinical disease behavior in RA-ILD are heterogenous, clinicians should be alert to the possibility of progressive disease and poor prognosis in patients with RA-ILD who form a honeycomb pattern during follow-up observation.

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Section: Discussionsupporting

confidence: 51%

Impact of radiological honeycombing in rheumatoid arthritis-associated interstitial lung disease

et al. 2020

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“…Looking at the features of fibrosis, the presence of honeycombing and traction bronchiectasis may indicate advanced features of IPF, as some patients with an inconsistent or possible UIP pattern on their HRCT eventually develop a definite UIP pattern over months or years [ 30 , 31 ]. Several studies have identified a poor prognosis for patients with fibrotic lung disease that shows features of honeycombing on HRCT [ 27 , 32 , 33 ].…”

Section: Early-stage Diagnosis In Ipfmentioning

confidence: 99%

Early Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis: A Narrative Review

et al. 2023

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Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease of unknown aetiology. Patients typically present with symptoms of chronic dyspnoea and cough over a period of months to years. IPF has a poor prognosis, with an average life expectancy of 3–5 years from diagnosis if left untreated. Two anti-fibrotic medications (nintedanib and pirfenidone) have been approved for the treatment of IPF. These drugs slow disease progression by reducing decline in lung function. Early diagnosis is crucial to ensure timely treatment selection and improve outcomes. High-resolution computed tomography (HRCT) plays a major role in the diagnosis of IPF. In this narrative review, we discuss the importance of early diagnosis, awareness among primary care physicians, lung cancer screening programmes and early IPF detection, and barriers to accessing anti-fibrotic medications.

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“…While anecdotally, patients may proceed from so-called earlier or atypical (nondefinitive) UIP to more consistent or definite UIP CT patterns, systematic analysis of this progression is incomplete and may not reflect the extent to which radiologic severity affects survival or morbidity. Prior studies have suggested patients with “possible” UIP CT pattern at diagnosis have improved survival (suggesting perhaps less severe or “earlier” disease),24,25 while a recent study suggested no difference in long-term survival among various UIP CT pattern types 26. Two studies have confirmed radiologic progression from “possible” or “inconsistent” UIP CT pattern to more “consistent UIP” in about third to half of patients 26,27.…”

Section: Introductionmentioning

confidence: 98%

“…Prior studies have suggested patients with “possible” UIP CT pattern at diagnosis have improved survival (suggesting perhaps less severe or “earlier” disease),24,25 while a recent study suggested no difference in long-term survival among various UIP CT pattern types 26. Two studies have confirmed radiologic progression from “possible” or “inconsistent” UIP CT pattern to more “consistent UIP” in about third to half of patients 26,27. What is more interesting is the observation that relative pattern stability may be seen in some over many years of follow-up, making the supposition of so-called earlier radiologic disease based on the absence of honeycombing or more extensive fibrotic changes less reliable for delineating how long disease has been present.…”

Section: Introductionmentioning

confidence: 99%

Obstacles to early treatment of idiopathic pulmonary fibrosis: current perspectives

Moua

Ryu

2019

TCRM

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Idiopathic pulmonary fibrosis is a progressive and fatal fibrotic lung disease which has seen new opportunity for drug treatment in the last several years with the approval of nintedanib and pirfenidone, two antifibrotic agents aimed at slowing decline in lung function as defined by FVC on pulmonary function testing. Despite these promising effects, delays in drug initiation have been reported undermining the premise that earlier drug initiation may sustain lung function and prolong survival. This review explores obstacles to earlier treatment, inclusive of defining so-called early idiopathic pulmonary fibrosis, difficulties in achieving a confident diagnosis in that setting, and uncertainties regarding drug-related benefits among specific patient subgroups such as those with no symptoms or advanced disease at presentation. Goals of therapy balanced with the burdens associated with antifibrotic drug therapy are negotiated on an individual basis. We review the evidence for and against earlier initiation of antifibrotic drug therapy along with its role in patient-centered outcomes.

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Evolution of diagnostic UIP computed tomography patterns in idiopathic pulmonary fibrosis: Disease spectrum and implications for survival

Cited by 9 publications

References 15 publications

Impact of radiological honeycombing in rheumatoid arthritis-associated interstitial lung disease

Impact of radiological honeycombing in rheumatoid arthritis-associated interstitial lung disease

Early Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis: A Narrative Review

Obstacles to early treatment of idiopathic pulmonary fibrosis: current perspectives

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