Usual interstitial pneumonia

OBJECTIVES: The purpose of this study was to retrospectively assess cystic changes in findings on follow-up CT scans of patients with fibrotic nonspecific interstitial pneumonia (NSIP). METHODS: The initial and last high-resolution CT scans of 58 patients with pathologically proven fibrotic NSIP were evaluated retrospectively. The median follow-up periods were 48 months (range, 12-183 months). The pattern, extent, and distribution of abnormal CT findings were compared with findings in the same region on previous and subsequent CT scans with a focus on cystic lesions...

BACKGROUND: Interstitial lung disease (ILD) is a common pulmonary manifestation of Sjögren's syndrome (SjS) and associated with an increased risk of death. Early detection and treatment of ILDs and knowing the risk factors are very important for prognosis in rheumatic diseases. AIMS: This study was performed to determine ILD and associated factors in patients with SjS. METHODS: Four hundred three SjS patients were evaluated in this cross-sectional cohort study...

While idiopathic pulmonary fibrosis (IPF) is the most common type of fibrotic lung disease, there are numerous other causes of pulmonary fibrosis that are often characterized by lung injury and inflammation. Although often gradually progressive and responsive to immune modulation, some cases may progress rapidly with reduced survival rates (similar to IPF) and with imaging features that overlap with IPF, including usual interstitial pneumonia (UIP)-pattern disease characterized by peripheral and basilar predominant reticulation, honeycombing, and traction bronchiectasis or bronchiolectasis...

Cerebral arterial air embolism (CAE), a rare subtype of air embolism, carries a 21% mortality rate. We present a unique case involving a 69-year-old female with a history of usual interstitial pneumonia (UIP) who suffered a transient ischemic attack (TIA) due to CAE. Unlike typical cases, CAE in this instance resulted from spontaneous pneumothorax, not the more common iatrogenic causes. Adding complexity, an unexpected discovery emerged during evaluation: a patent foramen ovale, contributing to paradoxical embolism...

BACKGROUND AND OBJECTIVE: Variants in surfactant genes SFTPC or ABCA3 are responsible for interstitial lung disease (ILD) in children and adults, with few studies in adults. METHODS: We conducted a multicentre retrospective study of all consecutive adult patients diagnosed with ILD associated with variants in SFTPC or ABCA3 in the French rare pulmonary diseases network, OrphaLung. Variants and chest computed tomography (CT) features were centrally reviewed. RESULTS: We included 36 patients (median age: 34 years, 20 males), 22 in the SFTPC group and 14 in the ABCA3 group...

We previously validated Fibresolve, a machine learning classifier system that non-invasively predicts idiopathic pulmonary fibrosis (IPF) diagnosis. The system incorporates an automated deep learning algorithm that analyzes chest computed tomography (CT) imaging to assess for features associated with idiopathic pulmonary fibrosis. Here, we assess performance in assessment of patterns beyond those that are characteristic features of usual interstitial pneumonia (UIP) pattern. The machine learning classifier was previously developed and validated using standard training, validation, and test sets, with clinical plus pathologically determined ground truth...

Patients with lung cancer complicated by interstitial pneumonia (IP) often lose treatment options early owing to acute exacerbation of IP concerns. Carbon-ion radiotherapy (CIRT) can provide superior tumor control and low toxicity at high dose concentrations. We conducted a retrospective analysis of the efficacy and tolerability of a single-fraction CIRT using 50 Gy for IP-complicated lung cancer. The study included 50 consecutive patients treated between April 2013 and September 2022, whose clinical stage of lung cancer (UICC 7th edition) was 1A:1B:2A:2B = 32:13:4:1...

OBJECTIVES: Interstitial lung disease (ILD) in connective tissue diseases (CTD) have highly variable morphology. We aimed to identify imaging features and their impact on ILD progression, mortality and immunosuppression response. METHODS: Patients with CTD-ILD had high-resolution chest computed tomography (HRCT) reviewed by expert radiologists blinded to clinical data for overall imaging pattern (usual interstitial pneumonia [UIP]; non-specific interstitial pneumonia [NSIP]; organizing pneumonia [OP]; fibrotic hypersensitivity pneumonitis [fHP]; and other)...

OBJECTIVES: Myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) are associated with distinctive dermatomyositis (DM) clinical phenotypes. The aim of this study is to explicate the clinical and immunological features of MSAs-negative DM patients. METHODS: A total of 515 individuals diagnosed with DM was screened from 2013 to 2022 and 220 DM patients were enrolled in this retrospective cohort. Clinical and laboratory data of these patients were analyzed...

The objective of this article is to describe and classify usual interstitial pneumonia (UIP) changes according to their relevance in the pathology of the idiopathic pulmonary fibrosis (IPF) process. In a cohort of 50 patients (25♀, 25♂) with UIP findings, the percentage ratio between fibrotic and preserved parts of the lungs was quantified. Three quantitative stages of fibrotic involvement of the lung parenchyma and concomitant changes were defined. These are initial (≤20%), advanced (21-40%), and diffuse (≥41%) fibrosis of the lungs...

OBJECTIVES: Real-world data regarding rheumatoid arthritis (RA) and its association with interstitial lung disease (ILD) is still scarce. This study aimed to estimate the prevalence of RA and ILD in patients with RA (RAILD) in Spain, and to compare clinical characteristics of patients with RA with and without ILD using natural language processing (NLP) on electronic health records (EHR). METHODS: Observational case-control, retrospective and multicentre study based on the secondary use of unstructured clinical data from patients with adult RA and RAILD from nine hospitals between 2014 and 2019...

BACKGROUND: The diagnostic process for fibrotic interstitial lung disease (F-ILD) is notably intricate, necessitating a multidisciplinary discussion to achieve consensus based on both clinical and radiological features. This study investigated the shared and distinctive long-term mortality predictors among the two primary phenotypes of F-ILD, namely idiopathic pulmonary fibrosis (IPF) and connective tissue disease-associated interstitial lung disease (CTD-ILD). METHODS: We included patients with F-ILD diagnosed from December 2018 to December 2019 and conducted follow-up assessments until February 2023...

BACKGROUND: Interstitial lung disease (ILD) related to rheumatoid arthritis (RA) is among the leading causes of death and an essential prognostic factor. There is only limited evidence for the safety of anti-rheumatic drugs for patients with RA-ILD. The aim of this study is to investigate the safety and efficacy of Janus kinase inhibitors (JAKis) by comparing it with abatacept (ABT) in patients with RA-ILD. METHODS: This single centre, retrospective nested case-control study enrolled patients with RA-ILD treated with JAKi or ABT...

BACKGROUND: We proposed the term "UIPAF" to define patients with Usual Interstitial Pneumonia (UIP) associated with only one domain of the classification called "Interstitial Pneumonia with Autoimmune Features" (IPAF). The objective of this study was to evaluate the clinical presentation and prognosis of UIPAF patients, compared with two cohorts, composed of IPAF and idiopathic pulmonary fibrosis (IPF) patients, respectively. METHODS: The patients were enrolled as IPAF, UIPAF, or IPF based on clinical, serological, and radiological data and evaluated by a multidisciplinary team...

Histopathologic criteria of usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF) were defined over the years and endorsed by leading organizations decades after Dr. Averill A. Liebow first coined the term UIP in the 1960s as a distinct pathologic pattern of fibrotic interstitial lung disease. Novel technology and recent research on interstitial lung diseases with genetic component shed light on molecular pathogenesis of UIP/IPF. Two antifibrotic agents introduced in the mid-2010s opened a new era of therapeutic approaches to UIP/IPF, albeit contentious issues regarding their efficacy, side effects, and costs...

One of the most frequent diffuse interstitial lung diseases is hypersensitivity pneumonitis. It is related to exposure to diverse antigens, causing fibrosis in advanced stages, making the differential diagnosis with interstitial pulmonary fibrosis difficult as it overlaps with the usual interstitial pneumonia pattern. On the other hand, there are interstitial lung diseases associated with ANCA, such as microscopic polyangiitis, which is also related to the usual interstitial pneumonia pattern. We present the case of a 74-year-old male patient with chronic dyspnea, history of smoking and exposure to organic particles, in addition to a pattern suggestive of moderately severe restriction...

Computed tomography (CT) imaging findings of pulmonary fibrosis are well established for adults and have been shown to correlate with prognosis and outcome. Recognition of fibrotic CT findings in children is more limited. With approved treatments for adult pulmonary fibrosis, it has become critical to define CT criteria for fibrosis in children, to identify patients in need of treatment and those eligible for clinical trials. Understanding how pediatric fibrosis compares with idiopathic pulmonary fibrosis and other causes of fibrosis in adults is increasingly important as these patients transition to adult care teams...

RATIONALE: Computed tomography (CT) enables noninvasive diagnosis of usual interstitial pneumonia (UIP), but enhanced image analyses are needed to overcome the limitations of visual assessment. OBJECTIVES: Apply multiple instance learning (MIL) to develop an explainable deep learning algorithm for prediction of UIP from CT and validate its performance in independent cohorts. METHODS: We trained a MIL algorithm using a pooled dataset (n=2,143) and tested it in three independent populations: data from a prior publication (n=127), a single-institution clinical cohort (n=239), and a national registry of patients with pulmonary fibrosis (n=979)...

OBJECTIVES: An association between physical inactivity and worse outcome during infectious disease has been reported. The effect of moderate exercise preconditioning on the immune response during an acute pneumonia in a murine model was evaluated. SETTING: Laboratory experiments. SUBJECTS: C57BL6/j male mice. INTERVENTIONS: Six-week-old C57BL/6J mice were divided in two groups: an exercise group and a control group. In the exercise group, a moderate, progressive, and standardized physical exercise was applied for 8 weeks...

We diagnosed and treated a case of Primary SjoGren's Syndrome with Idiopathic Pulmonary Fibrosis (IPF) in a 65 years old woman who presented with dyspnoea and multiple joint pains for 5 years and remained undiagnosed. She had variable presentation and was initially established as a case of mixed connective tissue disease which consists of Systemic Lupus Erythematosus (SLE), Systemic Sclerosis and Dermatomyositis. She complained of xerostomia, xerophthalmia, difficulty in opening mouth, progressive dysphagia with solid foods and raynaud's phenomenon...