Objective Patients with idiopathic interstitial pneumonia (IIP) often test positive for systemic scleroderma-specific autoantibodies (SSc-Ab), even if they do not meet the diagnostic criteria for systemic scleroderma (SSc). However, the significance of SSc-Ab in IIP is unknown. Methods We retrospectively studied the medical records of all patients suspected of interstitial lung disease (ILD) who visited our center between January 2016 and December 2021. We evaluated the association between SSc-Ab subtypes and clinical characteristics, prognosis, and incidence of acute exacerbation (AE) of IIP. Among 571 patients suspected of having IIP and SSc-Ab measured, we excluded cases with clear causes of ILD or those diagnosed with other diseases and analyzed 386 cases diagnosed as IIP. Results Among 386 IIP patients, 48 were SSc-Ab positive (platelet-derived growth factor receptor (PDGFR) in 0, Th/To in 10, anti-nucleolar organizer region 90 antibodies (NOR90) in 12, fibrillarin in five, RP155 in 14, RP11 in three, CENP A in seven, CENP B in 10, and Scl-70 in six). There was no significant difference in survival rate or incidence of AE between patients with or without SSc-Ab. Multivariate logistic regression analysis showed that age and malignancy were significant risk factors for death, whereas age, male sex, and anti-fibrillarin antibodies were significant risk factors for AE of IIP. Conclusion None of the SSc-Abs were associated with the risk of mortality, and anti-fibrillarin antibodies, along with age and male sex may contribute to the risk of AE of IIP, predicting severe lung involvement and warranting multidisciplinary treatment and careful follow-up.

Background: There are only a few reports on the nutritional status and mortality of patients with idiopathic pulmonary fibrosis (IPF). As such, this study aims to investigate the relationship between controlling nutritional status (CONUT) and the mortality of elderly patients with IPF. Methods: A total of 170 IPF patients aged ≥65 years old who visited the rehabilitation department of our hospital between July 2014 and July 2021 (mean age: 75.7 ± 6.3 years, sex (male/female): 138/32, %FVC: 78.3 ± 18.3%) were retrospectively analyzed. The Kaplan-Meier method and log-rank test were applied. Furthermore, using a Cox proportional hazards model with multivariate analysis, we analyzed the relationship between all-cause mortality and baseline characteristics including CONUT. Results: Based on the CONUT score, the normal group included 101 cases, the mild group included 58 cases, the moderate group included 11 cases, and the severe group had 0 cases. There were 49 cases of all-cause mortality events, suggesting that the mortality of the moderate group was significantly poorer than that of the normal and mild groups (p < 0.05). Furthermore, multivariate analysis identified GAP stage (HR: 5.972, 95%CI: 2.901~12.291, p < 0.0001), mMRC scale (HR: 0.615, 95%CI: 0.389~0.971, p = 0.009), and CONUT (HR: 2.012, 95%CI: 1.192~3.395, p = 0.037) as factors significantly influencing mortality. Conclusions: Severe malnutrition was not observed in elderly patients with IPF. Moderate malnutrition was associated with a significantly higher risk of all-cause mortality, suggesting that CONUT is an important indicator for predicting mortality.

BACKGROUND: Acute exacerbation of idiopathic interstitial pneumonias (AE-IIPs) has a high mortality. However, there is no established treatment for AE-IIPs. Therefore, we aimed to compare the efficacy of high- and low-dose corticosteroid therapies in AE-IIPs patients.
METHODS: Data were retrospectively collected from the Japanese Diagnosis Procedure Combination database from July 2010 to March 2018. Adult patients with AE-IIPs who received high-dose (methylprednisolone at a dose of 500-1000 mg/day for 3 days starting within 4 days after admission) or low-dose (methylprednisolone at a dose of 100-200 mg/day for at least 5 days starting within 4 days after admission) corticosteroid therapy were identified. Eligible patients (n = 17,317) were divided into the high-dose (n = 16,998) and low-dose (n = 319) groups. A stabilized inverse probability of treatment weighting using propensity scores was performed to compare outcomes between the groups.
RESULTS: The primary outcome was in-hospital mortality, and the secondary outcomes were 28-day mortality, infections during hospitalization, length of hospitalization, duration of steroid use, and discharge to home. The in-hospital mortality rates of the high- and low-dose corticosteroid groups were 50.6% and 47.0%, respectively. In-hospital mortality did not significantly differ between the two groups after stabilized inverse probability of treatment weighting, and the odds ratio in the low-dose corticosteroid group was 0.86 (95% confidence interval: 0.64-1.16; p = 0.33). The secondary outcomes also did not significantly differ between the groups.
CONCLUSIONS: There was no significant difference in outcomes between patients with AE-IIPs who received high- and low-dose corticosteroid therapies.

暂无摘要。

UNASSIGNED: Published data on the epidemiology of interstitial lung disease (ILD) in Asia is scarce. Understanding the epidemiology is important for authorities in the health management planning. This study aimed to estimate the prevalence, incidence, and survival of ILD in Hong Kong from 2005 to 2020 and evaluate the change of trend over time.
UNASSIGNED: In this retrospective cohort study, we identified ILD patients between 2005 and 2020 using a territory-wide electronic health record database. Prevalence, incidence rates, and age- and sex-standardised incidence rates with United Nations population in 2020 as a reference were estimated. Trends in prevalence and incidence were analysed using joinpoint regression and the average annual percent change (AAPC) was estimated. Median survival, and risk factors of mortality were evaluated using Cox proportional hazard regression.
UNASSIGNED: We identified 5924 patients and included 5884 of them for analysis. The prevalence of ILD increased from 24.7 to 33.6 per 100,000 population from 2005 to 2020 with an AAPC of 1.94 (95% confidence interval, CI: 1.69-2.34). The standardized incidence rate decreased from 5.36 to 2.57 per 100,000 person from 2005 to 2020 (AAPC -3.56, 95% CI, -4.95 to -1.78). The median survival of ILD was 2.50 (95% CI, 2.32-2.69) years. Male, older age, higher Charlson comorbidity index, and IIP subtype were associated with increased mortality with statistical significance.
UNASSIGNED: This study provided the first epidemiological evaluation of ILD in Hong Kong. Further studies on ILD in multiple Asian cities and countries are warranted.
UNASSIGNED: None.

Hypersensitivity pneumonitis (HP) is a diffuse parenchymal lung disease (DLPD) characterized by complex interstitial lung damage with polymorphic and protean inflammatory aspects affecting lung tissue targets including small airways, the interstitium, alveolar compartments and vascular structures. HP shares clinical and often radiological features with other lung diseases in acute or chronic forms. In its natural temporal evolution, if specific therapy is not initiated promptly, HP leads to progressive fibrotic damage with reduced lung volumes and impaired gas exchange. The prevalence of HP varies considerably worldwide, influenced by factors like imprecise disease classification, diagnostic method limitations for obtaining a confident diagnosis, diagnostic limitations in the correct processing of high-resolution computed tomography (HRCT) radiological parameters, unreliable medical history, diverse geographical conditions, heterogeneous agricultural and industrial practices and occasionally ineffective individual protections regarding occupational exposures and host risk factors. The aim of this review is to present an accurate and detailed 360-degree analysis of HP considering HRCT patterns and the role of the broncho-alveolar lavage (BAL), without neglecting biopsy and anatomopathological aspects and future technological developments that could make the diagnosis of this disease less challenging.

Cryptogenic organizing pneumonia (COP) is a form of idiopathic interstitial pneumonia that commonly presents with exertional dyspnea. The mainstay diagnostic criterion is with histopathological confirmation alongside excluding secondary causes of interstitial lung disease. The COVID-19 pandemic left many mysteries regarding the long-term sequelae of this disease. We explore a case of post-COVID-19 syndrome organizing pneumonia (PCOP) in a patient presenting with new-onset respiratory symptoms seven weeks after recovery from COVID-19 infection. Upon further review of the literature, there were no published case reports on PCOP in Trinidad and Tobago. We describe a case of PCOP presented at Apley Medical Clinic, Trinidad, and Tobago, West Indies, with the aim of increasing awareness of this condition to allow for early identification and effective management.

An 82-year-old man diagnosed with interstitial lung disease through computed tomography (CT) 1 year prior received a bivalent (tozinameran and famtozinameran) mRNA COVID-19 vaccine. He developed respiratory symptoms 1.5 months later, and chest high-resolution CT revealed new ground-glass opacities showing traction bronchiectasis. Transbronchial lung cryobiopsy revealed organizing acute lung injury and fibrosis with architectural destruction. The patient was diagnosed with an acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF). The bivalent mRNA COVID-19 vaccination was determined as the cause of the AE-IPF based on detailed medical history and examination findings. High-dose corticosteroid therapy improved the patient\'s symptoms and radiological findings.

BACKGROUND: \"Interstitial lung disease\" (ILD) is a broad term encompassing diseases of different backgrounds. \"Interstitial pneumonia with autoimmune features\" (IPAF) is a recent term that implies the presence of autoimmunity.
OBJECTIVE: This study aims to determine the characteristics of Polish patients with IPAF, compare them with patients with other interstitial pneumonias, and search for the prognostic and diagnostic biomarkers of IPAF in serum and bronchoalveolar lavage fluid (BALF).
METHODS: This multicenter prospective study plans to recruit 240 participants divided into 1 study group and 2 control groups. Biological fluid samples will be collected according to Polish Respiratory Society management guidelines and stored at -80°C for further tests. Prospective 5-year observations of 60 newly diagnosed individuals are planned. The study will be divided into subsections. First, we plan to characterize Polish patients with IPAF (study group) against their peers with other ILDs (2 control groups). Control group 1 will comprise patients with idiopathic ILDs, including mainly idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Control group 2 will comprise patients with connective tissue disease-associated interstitial lung diseases, such as rheumatoid arthritis, systemic sclerosis, polymyositis, dermatomyositis, Sjögren\'s syndrome, mixed connective tissue disease, and systemic lupus erythematosus. Radiological and functional parameters will be analyzed. Patients will be compared in terms of high-resolution computed tomography results, the 6-minute walking test performance, and pulmonary function test parameters. The diagnosis of IPAF will be reassessed on a regular basis through multidisciplinary discussion in order to determine its clinical stability. In the laboratory arm, inflammation and fibrosis pathways will be assessed. Cytokine levels (interleukin 8, transforming growth factor beta 1, chemokine C-C motif ligand [CXCL]18, CXCL1, surfactant protein [SP]-A, SP-D, Krebs von den Lungen-6 protein, and chitinase 1) will be measured in serum and BALF. A comparative analysis of serum and BALF cytokine levels will be performed in order to establish potential differences between systemic and local inflammatory pathways. In the quality of life (QoL) arm of the study, dyspnea and cough and their impact on various aspects of the QoL will be assessed. Depression and anxiety will be measured with the Hospital Anxiety and Depression Modified Scale and the 9-item Patient Health Questionnaire, and potential correlations with symptom prevalence will be assessed.
RESULTS: This study will start recruiting patients to phase 1 in October 2023. The final results will be available in 2028. We plan to publish preliminary results after 2-3 years from the start of phase 1.
CONCLUSIONS: This study will be a step toward a better understanding of IPAF etiopathogenesis and outcomes.
UNASSIGNED: PRR1-10.2196/44802.

Acute exacerbation (AE) of interstitial pneumonia (IP) shows poor prognosis, due to the typical histological pattern of diffuse alveolar damage superimposed upon lung fibrosis. The previous reports comparing clinical features between AE of idiopathic interstitial pneumonias (IIPs) and those of IPs with known etiology are limited. We retrospectively compared clinical parameters including age, sex, Charlson Comorbidity Index score (CCIS), blood biomarkers at diagnosis of AE, treatment, and 3-month mortality between patients with AE of IIPs and collagen vascular disease-associated interstitial pneumonia (CVD-IP). We assessed 85 patients, comprising 66 patients with AE of IIPs (78%) and 19 patients with AE of CVD-IP (22%). The least absolute shrinkage and selection operator regression selected CCIS (hazard ratio, 1.281; 95% confidence interval, 1.055-1.556; P = 0.012) and log serum lactate dehydrogenase (LDH) (hazard ratio, 6.267; 95% confidence interval, 2.172-18.085; P < 0.001) as significant predictors of 3-month mortality among these patients. Also, the adjusted survival curves using sex, CCIS, and serum LDH showed no significant differences between these two groups. In conclusion, among AE patients, CCIS and serum LDH level may be more important prognostic factors for 3-month mortality rather than two classification of IP subtypes: IIPs and CVD-IP.