Nonspecific interstitial pneumonia

Approximately 20% of rheumatoid arthritis (RA) patients have RA-related interstitial lung disease (RA-ILD). Stattic, an STAT3 inhibitor, has been confirmed to be relevant to both RA and ILD. Therefore, this study explored the effect of Stattic on the progression of joint disease and pulmonary fibrosis in zymosan-treated female SKG mice, an established model for autoimmune arthritis. The experimental mice developed pulmonary interstitial pneumonia, which is similar to human cellular and fibrotic nonspecific interstitial pneumonia...

OBJECTIVES: Interstitial lung disease (ILD) is common in anti-synthetase syndrome (ASS). Progressive fibrosing ILD (PF-ILD) may develop in ILD with autoimmune features. Data on PF-ILDs in ASS as a group are scarce. This study aimed to explore the characteristics and predictors of PF-ILD in ASS patients. METHODS: This retrospective study enrolled 96 ASS-ILD patients. Baseline clinical data were collected. PF-ILD assessments were conducted at every hospital visit during windows of 24 months after initial diagnosis...

BACKGROUND: Acute onset of interstitial lung disease (ILD) has been described in patients with idiopathic inflammatory myositis (IIM), but controlled studies about this issue are sparse. The aim of this study was to compare disease onset, demographics, and high-resolution computed tomography (HRCT) patterns in IIM-ILD and other connective tissue disease (CTD)-ILDs. METHODS: Clinical and radiological data of 22 IIM-ILD and 132 other CTD-ILD patients was retrospectively gathered from hospital registries between January 2000 and November 2019...

INTRODUCTION: Interstitial lung disease (ILD) is a frequent manifestation of connective tissue disease (CTD) with substantial variability in prevalence and outcomes reported across CTD subtypes. This systematic review summarises the prevalence, risk factors and ILD patterns on chest computed tomography of CTD-ILD. METHODS: A comprehensive search was performed in Medline and Embase to identify eligible studies. Meta-analyses were completed using a random effects model to determine the pooled prevalence of CTD-ILD and ILD patterns...

BACKGROUND: With the application of immune checkpoint inhibitors (ICIs) in cancer treatment, more and more attention has been paid to checkpoint inhibitor-related pneumonitis (CIP), which requires a better understanding of its clinical characteristics and therapeutic effects. METHODS: The clinical and imaging data of 704 patients with non-small cell lung cancer (NSCLC) who received immunotherapy were analyzed retrospectively; the clinical characteristics of CIP were summarized, and the therapeutic regimens and effects of the patients were summarized...

The following case report demonstrates a surgical biopsy performed through a subxiphoid uniportal video-assisted thoracoscopic surgery approach. The patient was a 35-year-old male, non-smoker, obese, with a history of arterial hypertension. He was referred for a thoracic surgery consultation because nonspecific interstitial pneumonia was suspected. The histological analysis confirmed the nonspecific interstitial pneumonia. We describe the procedure step by step. The postoperative course was uneventful. The subxiphoid approach is associated with less postoperative pain than the transthoracic approaches and could be an alternative, even for patients who have to undergo major lung resection surgery...

Interstitial pneumonia with autoimmune features (IPAF) is a new disease entity proposed in 2015. Numerous questions regarding IPAF require clarification, including diagnostic criteria, standard managements for stable disease and exacerbation, and prognosis. We report a case of a 67-year-old Asian woman who presented with progressive dyspnea. Chest computed tomography (CT) scans revealed nonspecific interstitial pneumonia. Serologic testing indicated positive anti-Jo-1 without presence of extrathoracic manifestations...

To investigate the characteristics of elderly-onset primary Sjögren syndrome (pSS) using chest high-resolution computed tomography and pulmonary function tests (PFTs). The data of 102 patients with pSS with interstitial lung disease were retrospectively analyzed. The chest high-resolution computed tomography, PFTs, and clinical and laboratory data were evaluated based on the age of onset: elderly-onset pSS (EopSS) (≥65 years) versus adult-onset pSS (AopSS) (<65 years). Among the 102 patients with pSS-interstitial lung disease, there were 34 of EopSS and 68 of AopSS...

BACKGROUND: The ILD-GAP scoring system has been widely used to predict the prognosis of patients with interstitial lung disease (ILD). The ability of the ILD-GAP scoring system combined with the Charlson Comorbidity Index score (CCIS) (ILD-GAPC) to predict ILD prognosis was investigated. METHODS: In ILD patients, including idiopathic pulmonary fibrosis (IPF), idiopathic nonspecific interstitial pneumonia (iNSIP), collagen vascular disease-related interstitial pneumonia (CVD-IP), chronic hypersensitivity pneumonitis (CHP), and unclassifiable ILD (UC-ILD), treated between April 2013 and April 2017, the relationships between baseline clinical parameters, including age, sex, CCIS, ILD diagnosis, pulmonary function test results, and disease outcomes, were retrospectively assessed, and the ability to predict prognosis was compared between the ILD-GAP and ILD-GAPC models, respectively...

Higher blood monocyte counts are related to worse survival in idiopathic pulmonary fibrosis. However, studies evaluating the association between blood monocyte counts and clinical outcomes of idiopathic nonspecific interstitial pneumonia (iNSIP) are lacking. We evaluated the impact of monocyte counts on iNSIP prognosis. iNSIP patients (n = 126; median age, 60 years; female, n = 64 [50.8%]) diagnosed by surgical lung biopsy were enrolled and categorized into low (monocyte < 600/µL) and high (monocyte ≥ 600/µL) monocyte groups...

The development of a progressive phenotype of interstitial lung disease (ILD) is still unpredictable. Whereas tools to predict mortality in ILD exist, scores to predict disease progression are missing. The aim of this study was to investigate whether baseline serum KL-6 as an established marker to assess disease activity in ILD, alone or in combination with clinical variables, could improve stratification of ILD patients according to progression risk at any time. Consecutive patients with fibrotic ILD, followed at our institution between 2008 and 2015, were investigated...

Exposure to smoke is associated with the development of diseases of the airways and lung parenchyma. Apart from chronic obstructive pulmonary disease (COPD), in some individuals, tobacco smoke can also trigger mechanisms of interstitial damage that result in various pathological changes and pulmonary fibrosis. A causal relation has been established between tobacco smoke and a group of entities that includes respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), Langerhans cell histiocytosis (LCH), and acute eosinophilic pneumonia (AEP)...

Systemic autoimmune diseases comprise a complex, heterogeneous group of entities. Noteworthy among the pulmonary complications of these entities is interstitial involvement, which manifests with the same radiopathologic patterns as in idiopathic interstitial pneumonia. High-resolution computed tomography is the gold-standard imaging technique; it enables us to identify and classify the disease and to determine its extent, providing useful information about the prognosis. In this group of processes, the most common pattern of presentation is nonspecific interstitial pneumonia...

Organizing pneumonia is a nonspecific pathologic pattern of response to lung damage. It can be idiopathic, or it can occur secondary to various medical processes, most commonly infections, connective tissue disease, and pharmacological toxicity. Although there is no strict definition of the pattern of organising pneumonia as in other idiopathic interstitial pneumonias, the characteristic pattern of this disease could be considered to include patchy consolidations and ground-glass opacities in the peribronchial and subpleural areas of both lungs...

We present a 61-year-old woman with a history of scleroderma and suspicion of osteomyelitis in her left wrist. She underwent a 3-phase bone scan for evaluation of osteomyelitis. Incidentally, the scan showed bilateral pulmonary MDP uptake, especially in lower lobes, which was proven to be due to the nonfibrotic form of nonspecific interstitial pneumonia.

BACKGROUND: Interstitial lung disease (ILD) is a common pulmonary manifestation of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). OBJECTIVES: We aimed to clarify the clinical predictors of mortality in a cohort of patients with AAV-related ILD (AAV-ILD). METHOD: We retrospectively identified AAV-ILD patients seen at Peking University First Hospital from January 2010 to June 2020 and manually screened for study inclusion. Baseline computed tomography (CT) images were further classified as nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), and unclassified ILD...

BACKGROUND: VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome is a newly described auto-inflammatory disease. Many cases feature pulmonary infiltrates or respiratory failure. This systematic review aimed to summarize respiratory manifestations in VEXAS syndrome described to date. METHODS: Databases were searched for articles discussing VEXAS syndrome until May 2022. The research question was: What are the pulmonary manifestations in patients with VEXAS syndrome? The search was restricted to English language and those discussing clinical presentation of disease...

Interstitial lung disease comprises numerous clinical entities posing significant challenges towards a prompt and accurate diagnosis. Amongst the contributing factors are intricate pathophysiological mechanisms, an overlap between conditions, and interobserver disagreement. We developed a model for patient clustering offering an additional approach to such complex clinical cases. The model is based on surface phenotyping of over 40 markers on immune cells isolated from bronchoalveolar lavage in combination with clinical data...

BACKGROUND: Nonspecific interstitial pneumonia is a rare clinical condition and usually precedes the full manifestation of connective tissue disorders, drug-induced interstitial lung disease or chronic hypersensitivity pneumonitis. OBJECTIVE: This article reports a twenty-eight (28)-year-old Nigerian female with nonspecific interstitial pneumonia (NSIP) complicated by Cor pulmonale. The unusual characteristics of our index case favors NSIP rather than idiopathic pulmonary fibrosis (age of 28 years, female, chest high-resolution computerized tomographic features and response to glucocorticoids)...

A man with non-small-cell lung cancer who was negative for anti-nuclear antibodies was admitted for dyspnea after immune checkpoint inhibitor (ICI) administration. Computed tomography (CT) showed complexed radiologic features, including subpleural and basal predominant reticular shadow with cystic structures and peribronchovascular consolidation. Although we treated him with high-dose steroid under a diagnosis of ICI-related pneumonitis, he developed acute exacerbation of pneumonitis with progressive fibrosis and volume loss...