Subject(s)
Early Detection of Cancer , Idiopathic Interstitial Pneumonias , Lung Neoplasms , Practice Guidelines as Topic , Humans , Early Detection of Cancer/methods , Idiopathic Interstitial Pneumonias/diagnostic imaging , Idiopathic Interstitial Pneumonias/diagnosis , Lung Neoplasms/diagnostic imaging , Lung Neoplasms/diagnosis , Male , Female , Middle Aged , Aged , Tomography, X-Ray ComputedABSTRACT
BACKGROUND: Respirable crystalline silica is a well-known cause of silicosis but may also be associated with other types of interstitial lung disease. We examined the associations between occupational exposure to respirable crystalline silica and the risk of idiopathic interstitial pneumonias, pulmonary sarcoidosis and silicosis. METHODS: The total Danish working population was followed 1977-2015. Annual individual exposure to respirable crystalline silica was estimated using a quantitative job exposure matrix. Cases were identified in the Danish National Patient Register. We conducted adjusted analyses of exposure-response relations between cumulative silica exposure and other exposure metrics and idiopathic interstitial pneumonias, pulmonary sarcoidosis and silicosis. RESULTS: Mean cumulative exposure was 125 µg/m3-years among exposed workers. We observed increasing incidence rate ratios with increasing cumulative silica exposure for idiopathic interstitial pneumonias, pulmonary sarcoidosis and silicosis. For idiopathic interstitial pneumonias and pulmonary sarcoidosis, trends per 50 µg/m3-years were 1.03 (95% CI 1.02 to 1.03) and 1.06 (95% CI 1.04 to 1.07), respectively. For silicosis, we observed the well-known exposure-response relation with a trend per 50 µg/m3-years of 1.20 (95% CI 1.17 to 1.23). CONCLUSION: This study suggests that silica inhalation may be related to pulmonary sarcoidosis and idiopathic interstitial pneumonias, though these findings may to some extent be explained by diagnostic misclassification. The observed exposure-response relations for silicosis at lower cumulative exposure levels than previously reported need to be corroborated in analyses that address the limitations of this study.
Subject(s)
Idiopathic Interstitial Pneumonias , Occupational Diseases , Occupational Exposure , Sarcoidosis, Pulmonary , Silicon Dioxide , Silicosis , Humans , Occupational Exposure/adverse effects , Occupational Exposure/statistics & numerical data , Sarcoidosis, Pulmonary/epidemiology , Sarcoidosis, Pulmonary/etiology , Silicon Dioxide/adverse effects , Denmark/epidemiology , Male , Middle Aged , Silicosis/epidemiology , Silicosis/etiology , Adult , Prospective Studies , Idiopathic Interstitial Pneumonias/epidemiology , Idiopathic Interstitial Pneumonias/etiology , Female , Follow-Up Studies , Occupational Diseases/epidemiology , Occupational Diseases/etiology , Incidence , AgedABSTRACT
Effective treatment for advanced lung cancer and idiopathic interstitial pneumonia (IIP) remains an unmet medical need. The relationship between chemotherapy's effectiveness in advanced lung cancer and the risk of acute exacerbation of IIP is poorly investigated. There is limited evidence that patients who experience an acute exacerbation of IIPs during cytotoxic chemotherapy have poorer outcomes than those who do not. Among 1004 patients with advanced lung cancer and IIPs enrolled in our published multi-centre retrospective study from 110 Japanese institutions, 708 patients (male: female, 645:63; mean age, 70.4) received first-line chemotherapy. The occurrence of chemotherapy-triggered acute exacerbations of IIPs and overall survival (OS) were analysed. The OS between groups of patients with and without the occurrence of acute exacerbation was compared at four landmark time points (30, 60, 90, and 120 days), starting from the first-line chemotherapy, using the landmark method. The incidence of acute exacerbation in patients who received first-line chemotherapy with small cell lung cancer (SCLC) and non-small cell lung cancer (NSCLC) was more frequent in NSCLC patients than in SCLC (4.2% vs 12.6%; odds ratio [OR]: 3.316; 95% confidence interval [CI] 1.25-8.8). Median survival time was 9.9 months (95% CI 9.2-10.7). Patients who experienced acute exacerbation had significant worse survival outcomes than those who did not at various time points (30 days, hazard ratio [HR]: 5.191, 95% CI 2.889-9.328; 60 days, HR: 2.351, 95% CI 1.104-5.009; 90 days, HR: 2.416, 95% CI 1.232-4.739; and 120 days, HR: 2.521, 95% CI 1.357-4.681). Acute exacerbation during first-line chemotherapy can predict poor survival.Trial Registration number: UMIN000018227.
Subject(s)
Idiopathic Interstitial Pneumonias , Lung Neoplasms , Humans , Male , Female , Aged , Lung Neoplasms/drug therapy , Lung Neoplasms/mortality , Lung Neoplasms/pathology , Idiopathic Interstitial Pneumonias/drug therapy , Idiopathic Interstitial Pneumonias/mortality , Retrospective Studies , Middle Aged , Carcinoma, Non-Small-Cell Lung/drug therapy , Carcinoma, Non-Small-Cell Lung/mortality , Prognosis , Disease Progression , Antineoplastic Agents/therapeutic use , Antineoplastic Agents/adverse effects , Treatment Outcome , Small Cell Lung Carcinoma/drug therapy , Small Cell Lung Carcinoma/mortality , Aged, 80 and overABSTRACT
While idiopathic interstitial pneumonia (IIP) centering on idiopathic pulmonary fibrosis (IPF) is the most prevalent interstitial lung disease (ILD), especially in the older adult population, connective tissue disease (CTD)-related ILD is the second most prevalent ILD. The pathogenesis of IPF is primarily fibrosis, whereas that of other ILDs, particularly CTD-ILD, is mainly inflammation. Therefore, a precise diagnosis is crucial for selecting appropriate treatments, such as antifibrotic or immunosuppressive agents. In addition, some patients with IIP have CTD-related features, such as arthritis and skin eruption, but do not meet the criteria for any CTD, this is referred to as interstitial pneumonia with autoimmune features (IPAF). IPAF is closely associated with idiopathic nonspecific interstitial pneumonia (iNSIP) and cryptogenic organizing pneumonia (COP). Furthermore, patients with iNSIP or those with NSIP with OP overlap frequently develop polymyositis/dermatomyositis after the diagnosis of IIP. Acute exacerbation of ILD, the most common cause of death, occurs more frequently in patients with IPF than in those with other ILDs. Although acute exacerbation of CTD-ILD occurs at a low rate of incidence, patients with rheumatoid arthritis, microscopic polyangiitis, or systemic sclerosis experience more acute exacerbation of CTD-ILD than those with other CTD. In this review, the features of each IIP, focusing on CTD-related signatures, are summarized, and the pathogenesis and appropriate treatments to improve the prognoses of patients with various ILDs are discussed.
Subject(s)
Connective Tissue Diseases , Idiopathic Interstitial Pneumonias , Idiopathic Pulmonary Fibrosis , Lung Diseases, Interstitial , Humans , Aged , Lung Diseases, Interstitial/diagnosis , Idiopathic Interstitial Pneumonias/complications , Idiopathic Interstitial Pneumonias/diagnosis , Idiopathic Pulmonary Fibrosis/complications , Connective Tissue Diseases/complications , Connective Tissue Diseases/diagnosis , PrognosisABSTRACT
BACKGROUND: Acute exacerbations (AEs) of fibrotic idiopathic interstitial pneumonia (fIIP) that require hospitalization occur in some patients. During hospitalization, these patients can develop hospital-acquired pneumonia (HAP), a common hospital-acquired infection with a high mortality rate. However, the characteristics of HAP in AE-fIIP remain unknown. The purpose of this study was to determine the incidence, causative pathogens, and outcomes of HAP in patients with AE-fIIP. METHODS: The medical records of consecutive patients who were hospitalized with AE-fIIP from January 2008 to December 2019 were analyzed for the incidence, causative pathogen, and survival of HAP. The records of patients with an obvious infection-triggered AE were excluded from analysis. RESULTS: There were 128 patients with AE-fIIP (89 with idiopathic pulmonary fibrosis [IPF] and 39 with non-IPF fIIP) who were hospitalized a total of 155 times (111 with IPF and 44 with non-IPF fIIP). HAP occurred in 49 patients (40 with IPF and 9 with non-IPF fIIP). The incidence and the in-hospital mortality rates of HAP in patients with AE-fIIP were high, at 32.2% and 48.9%, respectively. Corynebacterium spp. was the most common causative pathogen, which was followed by human cytomegalovirus (HCMV). CONCLUSIONS: The incidence and the in-hospital mortality rates of HAP in patients with AE-fIIP are high. To improve their survival, patients with fIIP who had AEs and HAP should receive prompt empirical treatment for possible infections with Corynebacterium spp. and testing for HCMV.
Subject(s)
Hamman-Rich Syndrome , Idiopathic Interstitial Pneumonias , Idiopathic Pulmonary Fibrosis , Lung Diseases, Interstitial , Pneumonia , Humans , Incidence , Idiopathic Interstitial Pneumonias/therapy , Lung Diseases, Interstitial/epidemiology , Lung Diseases, Interstitial/etiology , Hospitals , Disease Progression , Retrospective StudiesABSTRACT
BACKGROUND: Autoantibodies are a hallmark feature of Connective Tissue Diseases (CTD). Their presence in patients with idiopathic interstitial lung disease (ILD) may suggest covert CTD. We aimed to determine the prevalence of CTD autoantibodies in patients diagnosed with idiopathic ILD. METHODS: 499 patient sera were analysed: 251 idiopathic pulmonary fibrosis (IPF), 206 idiopathic non-specific interstitial pneumonia (iNSIP) and 42 cryptogenic organising pneumonia (COP). Autoantibody status was determined by immunoprecipitation. RESULTS: 2.4% of IPF sera had a CTD-autoantibody compared to 10.2% of iNSIP and 7.3% of COP. 45% of autoantibodies were anti-synthetases. A novel autoantibody targeting an unknown 56 kDa protein was found in seven IPF patients (2.8%) and two NSIP (1%) patients. This was characterised as anti-annexin A11. CONCLUSION: Specific guidance on autoantibody testing and interpretation in patients with ILD could improve diagnostic accuracy. Further work is required to determine the clinical significance of anti-annexin A11.
Subject(s)
Autoantibodies , Connective Tissue Diseases , Lung Diseases, Interstitial , Humans , Connective Tissue Diseases/diagnosis , Idiopathic Interstitial Pneumonias/diagnosis , Idiopathic Pulmonary Fibrosis , Lung Diseases, Interstitial/diagnosisABSTRACT
Idiopathic Interstitial Pneumonias (IIPs) are a heterogeneous group of the broader category of Interstitial Lung Diseases (ILDs), pathologically characterized by the distortion of lung parenchyma by interstitial inflammation and/or fibrosis. The American Thoracic Society (ATS)/European Respiratory Society (ERS) international multidisciplinary consensus classification of the IIPs was published in 2002 and then updated in 2013, with the authors emphasizing the need for a multidisciplinary approach to the diagnosis of IIPs. The histological evaluation of IIPs is challenging, and different types of IIPs are classically associated with specific histopathological patterns. However, morphological overlaps can be observed, and the same histopathological features can be seen in totally different clinical settings. Therefore, the pathologist's aim is to recognize the pathologic-morphologic pattern of disease in this clinical setting, and only after multi-disciplinary evaluation, if there is concordance between clinical and radiological findings, a definitive diagnosis of specific IIP can be established, allowing the optimal clinical-therapeutic management of the patient.
Subject(s)
Idiopathic Interstitial Pneumonias , Pathologists , Humans , Consensus , Interdisciplinary Studies , Respiratory Rate , Idiopathic Interstitial Pneumonias/diagnosisSubject(s)
Hypertension, Pulmonary , Idiopathic Interstitial Pneumonias , Humans , Hypertension, Pulmonary/physiopathology , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/diagnostic imaging , Hypertension, Pulmonary/complications , Male , Female , Middle Aged , Idiopathic Interstitial Pneumonias/complications , Idiopathic Interstitial Pneumonias/diagnostic imaging , Idiopathic Interstitial Pneumonias/physiopathology , Aged , Lung/diagnostic imaging , Lung/blood supply , Lung/physiopathologyABSTRACT
PURPOSE: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is the primary cause of death in patients with IPF, characterised by diffuse, bilateral ground-glass opacification on high-resolution CT (HRCT). This study proposes a three-dimensional (3D)-based deep learning algorithm for classifying AE-IPF using HRCT images. MATERIALS AND METHODS: A novel 3D-based deep learning algorithm, SlowFast, was developed by applying a database of 306 HRCT scans obtained from two centres. The scans were divided into four separate subsets (training set, n=105; internal validation set, n=26; temporal test set 1, n=79; and geographical test set 2, n=96). The final training data set consisted of 1050 samples with 33 600 images for algorithm training. Algorithm performance was evaluated using accuracy, sensitivity, specificity, positive predictive value, negative predictive value, receiver operating characteristic (ROC) curve and weighted κ coefficient. RESULTS: The accuracy of the algorithm in classifying AE-IPF on the test sets 1 and 2 was 93.9% and 86.5%, respectively. Interobserver agreements between the algorithm and the majority opinion of the radiologists were good (κw=0.90 for test set 1 and κw=0.73 for test set 2, respectively). The ROC accuracy of the algorithm for classifying AE-IPF on the test sets 1 and 2 was 0.96 and 0.92, respectively. The algorithm performance was superior to visual analysis in accurately diagnosing radiological findings. Furthermore, the algorithm's categorisation was a significant predictor of IPF progression. CONCLUSIONS: The deep learning algorithm provides high auxiliary diagnostic efficiency in patients with AE-IPF and may serve as a useful clinical aid for diagnosis.
Subject(s)
Deep Learning , Idiopathic Interstitial Pneumonias , Idiopathic Pulmonary Fibrosis , Humans , Idiopathic Pulmonary Fibrosis/diagnostic imaging , Tomography, X-Ray Computed/methods , ROC CurveABSTRACT
BACKGROUND: Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) affect a significant proportion of patients with IPF. There are limited data to inform therapeutic strategies for AE-IPF, despite its high mortality. We discuss the rationale and design of STRIVE-IPF, a randomized, multi-center, open-label Phase IIb clinical trial to determine the efficacy of combined therapeutic plasma exchange (TPE), rituximab, and intravenous immunoglobulin (IVIG), in comparison to treatment as usual (TAU), among patients with acute IPF exacerbations. METHODS: The STRIVE-IPF trial will randomize 51 patients among five sites in the United States. The inclusion criteria have been designed to select a study population with AE-IPF, as defined by American Thoracic Society criteria, while excluding patients with an alternative cause for a respiratory decompensation. The primary endpoint of this trial is six-month survival. Secondary endpoints include supplement oxygen requirement and six-minute walk distance which will be assessed immediately prior to treatment and after completion of therapy on day 19, as well as at periodic subsequent visits. DISCUSSION: The experimental AE-IPF therapy proposed in this clinical trial was adapted from treatment regimens used in other antibody-mediated diseases. The regimen is initiated with TPE, which is expected to rapidly reduce circulating autoantibodies, followed by rituximab to reduce B-cells and finally IVIG, which likely has multiple effects, including affecting feedback inhibition of residual B-cells by Fc receptor occupancy. We have reported potential benefits of this experimental therapy for AE-IPF in previous anecdotal reports. This clinical trial has the potential to profoundly affect current paradigms and treatment approaches to patients with AE-IPF. TRIAL REGISTRATION: ClinicalTrials.gov identifier: NCT03286556.
Subject(s)
Idiopathic Interstitial Pneumonias , Idiopathic Pulmonary Fibrosis , Humans , Idiopathic Interstitial Pneumonias/complications , Idiopathic Pulmonary Fibrosis/drug therapy , Immunoglobulins, Intravenous/therapeutic use , Plasma Exchange , Rituximab/therapeutic useABSTRACT
BACKGROUND: Gastrointestinal symptoms, such as diarrhea and nausea, are common adverse events associated with nintedanib. Systemic sclerosis is associated with a high prevalence of gastrointestinal symptoms that may increase with nintedanib administration. In clinical practice, we aimed to determine whether patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) experience more adverse gastrointestinal events associated with nintedanib than patients with idiopathic interstitial pneumonias (IIPs). METHODS: We retrospectively examined the clinical records of patients with SSc-ILD and IIPs newly treated with nintedanib at Kumamoto University Hospital between January 2020 and September 2022 and compared adverse events. RESULTS: In total, 27 patients with SSc-ILD and 34 with IIPs were enrolled. No significant differences were observed in the duration of nintedanib treatment. The most frequent adverse event in both groups was diarrhea, which was more frequent in the SSc-ILD group (81.5 % vs. 61.8 %, p = 0.157). Nausea was significantly more frequent in the SSc-ILD group than in the IIPs group (37.0 % vs. 11.8 %, p = 0.031). The permanent discontinuation rate of nintedanib during the study period between the two groups was not different (40.7 % vs. 32.4 %, p = 0.595). However, the most common reasons for discontinuation varied. The most frequent reason in the SSc-ILD group was nausea, due to the progression of ILD in the IIPs group. CONCLUSIONS: Patients with SSc-ILD experienced significantly more nintedanib-induced nausea than those with IIPs. Gastrointestinal adverse events are often the reason for discontinuation of nintedanib in the SSc-ILD group, which requires better management of gastrointestinal symptoms.
Subject(s)
Idiopathic Interstitial Pneumonias , Indoles , Lung Diseases, Interstitial , Scleroderma, Systemic , Humans , Lung Diseases, Interstitial/etiology , Lung Diseases, Interstitial/complications , Retrospective Studies , Idiopathic Interstitial Pneumonias/complications , Scleroderma, Systemic/complications , Scleroderma, Systemic/drug therapy , Diarrhea/chemically induced , Nausea/chemically induced , Nausea/epidemiologyABSTRACT
BACKGROUND: Patients with idiopathic interstitial pneumonia (IIP) have a favourable prognosis when they have interstitial pneumonia with autoimmune features (IPAF). However, precise IPAF-related findings from high-resolution computed tomography (HRCT) and lung histopathological specimens and the treatment response have not been fully determined. Therefore, this study was conducted to evaluate the relationship between findings on HRCT or lung histopathological specimens and the progression of interstitial pneumonia in patients with IPAF. METHODS: This multicentre cohort study prospectively enrolled consecutive patients with IIP. At the diagnosis of IIP, we systematically evaluated 74 features suggestive of connective tissue diseases and followed them up. HRCT, lung specimens, serum antibodies, and the clinical course were also evaluated. RESULTS: Among 222 patients with IIP, 26 (11.7%) fulfilled the IPAF criteria. During a median observation period of 36 months, patients with IPAF showed better survival than those without IPAF (p = 0.034). While histopathological findings were not related to IPAF, nonspecific interstitial pneumonia (NSIP) with organizing pneumonia (OP) overlap was the most prevalent HRCT pattern (p < 0.001) and the consolidation opacity was the most common radiological finding in IPAF (p = 0.017). Furthermore, in patients with IPAF, the diagnosis of COP or NSIP with OP overlap was associated with a higher increase in %FVC in 1 year than in those with idiopathic pulmonary fibrosis, NSIP, or unclassifiable IIP (p = 0.002). CONCLUSIONS: This study shows the presence of consolidation opacity on HRCT and the diagnosis of COP or NSIP with OP overlap are associated with IPAF and its favourable treatment response in patients with IPAF.
Subject(s)
Autoimmune Diseases , Connective Tissue Diseases , Idiopathic Interstitial Pneumonias , Lung Diseases, Interstitial , Humans , Cohort Studies , Prospective Studies , Autoimmune Diseases/complications , Autoimmune Diseases/diagnostic imaging , Retrospective Studies , Lung Diseases, Interstitial/diagnosis , Idiopathic Interstitial Pneumonias/diagnosis , Connective Tissue Diseases/complications , Connective Tissue Diseases/diagnostic imagingABSTRACT
Patients with idiopathic fibrosing interstitial pneumonias (f-IIPs) mainly suffer from dyspnea. Refractory dyspnea, defined as persistent dyspnea despite optimal treatment, could be the signal to prescribe dyspnea relievers. We aimed to examine the prevalence and characteristics of refractory dyspnea in consecutive patients with f-IIPs. Refractory dyspnea was defined by an mMRC≥3 and also by a VAS dyspnea score≥2 at rest. The sensory and affective characteristics of refractory dyspnea (mMRC≥3) and associated quality of life (QoL) anxiety and depression were compared with non-refractory dyspnea (mMRC1-2) using the Multidimensional Dyspnea Profile (MDP), King's Brief Interstitial Lung Disease (KBILD) and Hospital Anxiety and Depression scale (HADs). We included 40 patients (24 men), aged 72 [68-79], FVC of 71 % [59-86] and DLCO 47 % [40-49]. Refractory dyspnea, was found in 38 % (95%CI:23-54) when defined by mMRC≥3 and in 67 % (95%CI:50-81) using a resting VAS dyspnea score ≥2. The agreement between the two definitions was low. Patients with refractory dyspnea (mMRC≥3) were more often women (60 % vs.28 %, p = 0.046), had a lower DLCO (24 % [22-43] vs.47 % [43-51], p = 0.014) and more frequently used oxygen (60 % vs.12 %, p = 0.003); they experience more intense air hunger (5/10 [3-6] vs.2/10 [0-5], p = 0.018)). No significant differences were observed in VAS, MDP, KBILD, or HADs scores between refractory and non-refractory dyspnea patients. Our results indicate a significant frequency of refractory dyspnea in patients with f-IIPs and an association with air hunger but no impact on the affective dimension of dyspnea, anxiety, depression and QoL, suggesting that the mMRC score might not accurately identify patients distressed by their breathlessness.
Subject(s)
Idiopathic Interstitial Pneumonias , Lung Diseases, Interstitial , Male , Humans , Female , Quality of Life , Idiopathic Interstitial Pneumonias/complications , Idiopathic Interstitial Pneumonias/epidemiology , Lung Diseases, Interstitial/complications , Lung Diseases, Interstitial/epidemiology , Dyspnea/diagnosis , Dyspnea/epidemiology , Dyspnea/etiologyABSTRACT
BACKGROUND AND OBJECTIVE: The lung immune prognostic index (LIPI), a simple index calculated from the blood lactate dehydrogenase level and derived neutrophil-to-lymphocyte ratio, is thought to be associated with host immune status. However, the utility of LIPI in patients with idiopathic interstitial pneumonias (IIPs) is unknown. METHODS: In this multicentre, retrospective, observational study, an association between LIPI and the survival of patients with IIPs was evaluated. RESULTS: Exploratory and validation cohorts consisting of 460 and 414 patients with IIPs, respectively, were included (159 and 159 patients had idiopathic pulmonary fibrosis [IPF], and 301 and 255 had non-IPF, respectively). In the exploratory cohort, patients with IPF and a low LIPI had significantly better survival than those with a high LIPI (median of 5.6 years vs. 3.9 years, p = 0.016). The predictive ability of LIPI for the survival of patients with IPF was validated in the validation cohort (median of 8.5 years vs. 4.4 years, p = 0.003). In a multivariate Cox proportional hazard analysis, LIPI was selected as an independent predictive factor for the survival of IPF patients. There was no significant association between LIPI and survival of non-IPF patients in the exploratory and validation cohorts. CONCLUSION: The LIPI was a predictive factor for the survival of patients with IPF and could aid the management of IPF.
Subject(s)
Idiopathic Interstitial Pneumonias , Idiopathic Pulmonary Fibrosis , Humans , Prognosis , Retrospective Studies , LungABSTRACT
BACKGROUND: According to current guidelines, a surgical biopsy is rarely required when a high-confidence radiologic interstitial lung disease (ILD) diagnosis is made on thin-section high-resolution computed tomography (HRCT). Nevertheless, disowning HRCT scans diagnosed by biopsy are more common than presumed. Our study aimed to describe the concordance rate between HRCT scans and pathological diagnoses of ILDs obtained by surgical biopsy. The current guideline suggests the use of surgical lung biopsy (SLB) in patients with newly detected ILD of unknown cause. METHODS: Patients who underwent mini-invasive surgical biopsies for interstitial lung diseases from January 2018 to August 2022 were analyzed. The HRCT scans were reviewed by an observer blinded to the patient's clinical information. The concordance between histological and HRCT-scan were assessed. RESULTS: Data from 104 patients with uncertain low confidence diagnosis of interstitial lung diseases at HRCT were analyzed. Most of the patients are male (65; 62.5%). The more frequent HRCT pattern were: alternative diagnoses (46; 44.23%), UIP probable (42; 40.38%), UIP indeterminate (7; 6.73%), and non-specific interstitial pneumonia (NSIP) (9, 8.65%). The more common histological diagnosis was UIP definite (30; 28.84%), hypersensitivity pneumonia [HP](19; 18.44%), NSIP (15; 14.42%), sarcoidosis (10; 9.60%). In 7 (20%) cases, the final pathological finding denies HRCT-scans diagnoses; indeed, a moderate agreement was observed between HRCT-scan findings and the definitive histological diagnosis (kappa index: 0.428). CONCLUSIONS: HRCT-scan has limitations if the objective is to define interstitial lung diseases accurately. Consequently, pathological assessment should be taken into account in order to provide more accurate tailored treatment strategies because the risk is to wait from 12 to 24 months to ascertain if the ILD will be treatable as progressive pulmonary fibrosis (PPF). Undeniably true, video-assisted surgical lung biopsy (VASLB) with endotracheal intubation and mechanical ventilation is associated with a risk of mortality and morbidity that is far from nil. Nevertheless, in recent years a VASLB approach performed in awake subjects under loco-regional anesthesia (awake-VASLB) has been suggested as an effective method to obtain a highly confident diagnosis in patients with diffuse pathologies of the lung parenchyma.
Subject(s)
Idiopathic Interstitial Pneumonias , Lung Diseases, Interstitial , Pulmonary Fibrosis , Humans , Male , Female , Lung Diseases, Interstitial/diagnostic imaging , Lung/diagnostic imaging , Lung/pathology , Idiopathic Interstitial Pneumonias/pathology , Pulmonary Fibrosis/pathology , TomographyABSTRACT
PURPOSE: To establish accurate diagnostic criteria and predictors of treatment response for postoperative acute exacerbation (AE) in patients with lung cancer and idiopathic interstitial pneumonia (IIP). METHODS: Among 93 patients with IIP who underwent surgery for lung cancer, suspected postoperative AE developed in 20 (21.5%). Patients were divided into a progressive AE group, comprising patients with bilateral alveolar opacities and decreasing PaO2 ≥ 10 mmHg (n = 5); an incipient AE group, comprising patients with unilateral alveolar opacities and decreasing PaO2 ≥ 10 mmHg (n = 10); and an indeterminate AE group, comprising patients with alveolar opacities but decreasing PaO2 < 10 mmHg (n = 5). RESULTS: The progressive AE group had significantly higher 90-day mortality (80%) than the incipient AE group (10%, P = 0.017) or the indeterminate AE group (0%, P = 0.048). Bilateral opacities may indicate advanced AE and poor prognosis, whereas unilateral opacities may indicate an early stage of AE and a good prognosis. PaO2 < 10 mmHg may indicate conditions other than AE. CONCLUSIONS: In patients with lung cancer and IIP, decreasing PaO2 and HRCT findings may allow for the initiation of rapid and accurate treatment strategies for postoperative AE.
Subject(s)
Idiopathic Interstitial Pneumonias , Lung Neoplasms , Humans , Lung Neoplasms/complications , Lung Neoplasms/surgery , Oxygen , Partial Pressure , Retrospective Studies , Idiopathic Interstitial Pneumonias/diagnostic imaging , Prognosis , Disease ProgressionABSTRACT
BACKGROUND AND OBJECTIVE: Corticosteroids are commonly used for the treatment of acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF); however, the optimal initial dose of corticosteroids remains uncertain due to a lack of sufficient evidence. We evaluated whether the administration of a pulse dose of corticosteroids resulted in improved survival outcomes compared with conventional non-pulse dose of corticosteroids. METHODS: We retrospectively analysed 238 patients with AE-IPF treated with corticosteroids at a tertiary referral hospital between January 2013 and December 2021. Based on whether a pulse dose of corticosteroids (methylprednisolone of ≥250 mg/day or equivalent) was administered within 7 days of hospitalization for AE-IPF, the patients were divided into the pulse and non-pulse regimen groups. The survival outcomes were compared between the two groups using multivariable regression and propensity score-matched analyses. RESULTS: Among the 238 patients, 59 patients received pulse dose of corticosteroids, whereas 179 patients received conventional non-pulse dose of corticosteroids. After adjusting for the confounding factors related to the baseline clinical and radiographic severity, compared with the conventional non-pulse regimen, the pulse regimen of corticosteroids did not reduce the risk of mortality at the 3-month (aHR 0.84, 95% CI 0.45-1.38) or 12-month (aHR 0.96, 95% CI 0.60-1.25) follow-ups. Propensity score-matched analysis revealed similar results. CONCLUSION: The survival outcomes of patients with AE-IPF who received a pulse dose of corticosteroids did not differ from those of patients who received conventional non-pulse dose of corticosteroids. Further prospective studies are required to establish the optimal initial dose of corticosteroids for the treatment of AE-IPF.
Subject(s)
Idiopathic Interstitial Pneumonias , Idiopathic Pulmonary Fibrosis , Humans , Disease Progression , Retrospective Studies , Treatment Outcome , Idiopathic Interstitial Pneumonias/drug therapy , Idiopathic Pulmonary Fibrosis/drug therapy , Adrenal Cortex Hormones/therapeutic useABSTRACT
OBJECTIVES: To explore clinical and laboratory characteristics of primary Sjögren's syndrome (pSS) complicated with interstitial lung disease (ILD) and investigate the risk factors for respiratory infections in pSS-ILD. METHODS: A cohort of 162 pSS-ILD patients in Peking University People's Hospital from 2015 to 2020 were included, and all medical records were completely collected. We screened 53 patients suffering from respiratory infections as study cases, compared with 109 age- and sex-matched controls. Differences between infection group and control group were compared. Univariate and multivariate binary logistic regression tests were conducted to identify potential risk factors for respiratory infections in pSS-ILD patients. RESULTS: Among 162 pSS-ILD patients, 32.72% (53/162) suffered from respiratory infections. The most frequent type of ILD was nonspecific interstitial pneumonia (32.08%, 51/159), and the most common type of pathogen was bacteria (64.25%, 34/53). Infection group showed higher levels of ESSDAI (P < 0.001), CRP (P < 0.001), ESR (P = 0.003), and C3 (P = 0.020) but lower level of DLCO-SB (P = 0.015). Univariate logistic model revealed that PAH and the use of glucocorticoid increased infection risk in pSS-ILD patients. On multivariate logistic regression analysis, PAH (OR = 3.993, 95% CI = 1.192-13.373, P = 0.025) and severe reduction of DLCO (DLCO-SB < 40%, OR = 4.625, 95% CI = 1.281-16.702, P = 0.019) were significantly associated with increased risk of respiratory infections in pSS-ILD patients. CONCLUSION: Among pSS-ILD patients, the most frequent type of ILD was nonspecific interstitial pneumonia. In patients with infection, bacteria were the most common pathogen. Higher levels of ESSDAI, CRP, ESR, and C3 may be correlated with increased infection risk. PAH and reduction of DLCO were identified as independent risk factors. Key Points ⢠ILD and infectious diseases severely affect pSS patient conditions. ⢠Higher levels of ESSDAI, CRP, ESR, and C3 may be correlated with increased infection risks in pSS-ILD. ⢠PAH and reduction of DLCO were identified as independent risk factors for lower respiratory infection.
Subject(s)
Idiopathic Interstitial Pneumonias , Lung Diseases, Interstitial , Respiratory Tract Infections , Sjogren's Syndrome , Humans , Retrospective Studies , Sjogren's Syndrome/complications , Lung Diseases, Interstitial/complications , Idiopathic Interstitial Pneumonias/complications , Respiratory Tract Infections/complicationsABSTRACT
BACKGROUND: Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) increases mortality risk, but which factors increase mortality is unknown. We aimed to perform a prognostic review of factors associated with mortality in patients with IPF. STUDY DESIGN: and methods: We searched MEDLINE, EMBASE, and CINAHL for studies that reported on the association between any prognostic factor and AE-IPF. We assessed risk of bias using the QUIPS tool. We conduced pairwise meta-analyses using REML heterogeneity estimator, and GRADE approach to assess the certainty of the evidence. RESULTS: We included 35 studies in our analysis. We found that long-term supplemental oxygen at baseline (aHR 2.52 [95 % CI 1.68 to 3.80]; moderate certainty) and a diagnosis of IPF compared to non-IPF ILD (aHR 2.19 [95 % CI 1.22 to 3.92]; moderate certainty) is associated with a higher risk of death in patients with AE-IPF. A diffuse pattern on high resolution computed tomography (HRCT) compared to a non-diffuse pattern (aHR 2.61 [95 % CI 1.32 to 2.90]; moderate certainty) is associated with a higher risk of death in patients with AE-IPF. We found that using corticosteroids prior to hospital admission (aHR 2.19 [95 % CI 1.26 to 3.82]; moderate certainty) and those with increased neutrophils (by % increase) in bronchoalveolar lavage (BAL) during the exacerbation is associated with a higher risk of death (aHR 1.02 [1.01 to 1.04]; moderate certainty). INTERPRETATION: Our results have implications for healthcare providers in making treatment decisions and prognosticating the clinical trajectory of patients, for researchers to design future interventions to improve patient trajectory, and for guideline developers in making decisions about resource allocation.
Subject(s)
Idiopathic Interstitial Pneumonias , Idiopathic Pulmonary Fibrosis , Humans , Prognosis , Disease Progression , Bronchoalveolar LavageABSTRACT
BACKGROUND: The information needs of patients and their families regarding interstitial lung disease (ILD) have yet to be studied in detail, and few reports have examined the differences in information needs according to patient status. This study aimed to determine whether there are differences in information needs between outpatients with ILD and their family caregivers and whether these differences depend on long-term oxygen therapy use. METHODS: Patients with fibrotic ILDs and their families who visited Kyoto University Hospital between February 2020 and March 2022 were recruited for this descriptive study. Fibrotic ILDs included idiopathic pulmonary fibrosis (IPF), other idiopathic interstitial pneumonias (IIPs) than IPF, connective tissue disease-associated ILD (CTD-ILD), and fibrotic hypersensitivity pneumonia. Data were obtained from electronic patient records and questionnaires. Descriptive data analyses were performed. RESULTS: Sixty-five patients and their family caregivers were analyzed. Twenty-seven (41.5%) patients had IIPs (IPF 9 and other IIPs 18), 34 (52.3%) had CTD-ILD, and 4 (6.2%) had fibrotic hypersensitivity pneumonia. The most common relationship between the patient and their family was a spouse (67.7%), with 80% living together. The primary information needs among patients and their family caregivers were common up to the third rank but differed from the rest. Patients were interested in "when and where to contact health care providers" and "end-of-life care and advanced directives," while family caregivers were interested in "diet and nutrition" and "care and support at home." Patients with long-term oxygen therapy had higher needs for "end-of-life care and advanced directives" and "how to manage breathlessness, cough, and fatigue," while the needs for "drugs for ILD" and "acute exacerbation of ILD" were relatively low. Family caregivers were interested in "diet and nutrition" in the long-term oxygen therapy group and "acute exacerbation of ILD" in the no long-term oxygen therapy group. CONCLUSIONS: This study found that the information needs of patients and their family caregivers were not the same and that the aspect of information needs differed by long-term oxygen therapy status. Healthcare providers should consider the position of the recipient of information, the appropriate time based on the patient's condition, and the necessary information.