Management Of Interstitial Lung Disease Research Articles

The Management of Interstitial Lung Disease in the ICU: A Comprehensive Review

Interstitial lung disease (ILD) encompasses a diverse group of parenchymal lung diseases characterized by varying degrees of inflammation and/or fibrosis. Patients with ILD frequently require hospitalization, with many needing intensive care unit (ICU) admission, most often due to respiratory failure. The diagnosis and management of ILD in the ICU present unique challenges. Diagnosis primarily relies on chest CT imaging to identify fibrosis and inflammation. Acute exacerbations, whether in idiopathic pulmonary fibrosis (IPF) or non-IPF ILD, require careful evaluation of potential triggers and differential diagnoses. Bronchoalveolar lavage may provide valuable information, such as the identification of infections, but carries risks of complications. Biopsies, whether transbronchial or surgical, can also be informative but pose significant procedural risks. Corticosteroids are the cornerstone of treatment for acute exacerbations of IPF, with higher doses potentially benefiting non-IPF ILD. Additional immunosuppressive agents may be used in cases with evidence of inflammation. Oxygen supplementation, particularly with high-flow nasal cannula, is often employed to manage severe hypoxemia, while noninvasive ventilation can be useful for worsening hypoxemia and/or hypercapnia. When mechanical ventilation is used, it is recommended to target low tidal volumes to minimize lung injury; high PEEP may be less effective and even associated with increased mortality. Prone positioning can improve oxygenation in severely hypoxemic patients. In addition to ventilatory strategies, careful fluid management and addressing concomitant pulmonary hypertension are essential components of care. Extracorporeal membrane oxygenation is a high-risk intervention reserved for the most severe cases. Lung transplantation may be considered for end-stage ILD patients in the ICU, with outcomes dependent on the urgency of transplantation and the patient’s overall condition. Managing ILD in the ICU requires a multidisciplinary approach, and despite recent advances, mortality remains high, emphasizing the need for continued research and individualized treatment strategies.

P14 A case of worsening scleroderma-associated interstitial lung disease in pregnancy – treated with tocilizumab

Abstract Introduction Systemic sclerosis is a rare, multi-system connective tissue disorder, predominantly affecting women of reproductive age, with remarkable heterogeneity in organ involvement. Pulmonary involvement, such as interstitial lung disease (ILD) and pulmonary hypertension, remains the leading cause of death. There have been reports of disease progression during pregnancy and trials are ongoing to establish a safe treatment regimen for patients with exacerbation of ILD during pregnancy. We present an interesting case where interleukin-6 (IL-6) inhibitor, tocilizumab, was successfully used to control inflammation in the lung during pregnancy, resulting in a positive outcome for both the mother and the baby. Case description Our patient was diagnosed with diffuse cutaneous systemic sclerosis with overlapping facial dermatomyositis at the age of 25. She was found to have anti Scl-70 antibody, anti-Ro antibody and weakly positive anti Jo–1 antibody. On review, she was noted to have significant Raynaud’s disease, fixed flexion deformity at the proximal interphalangeal joints as a consequence of digital ulcers. At the age of 29, minor lung fibrosis was detected in the CT, with pulmonary function tests showing FEV1 at 87%, FVC at 87%, and TLCO at 73%. She was managed with mycophenolate mofetil, hydroxychloroquine, prednisolone, sildenafil, omeprazole, and vitamin D. During active disease phases with digital ulcerations, she received iloprost and bosentan. She had her first child aged 30 without complications while off mycophenolate mofetil. Planning for another pregnancy at 34, she was advised to stop mycophenolate mofetil. During her 21st week of pregnancy, she presented with palpitations, non-productive cough, and shortness of breath. A limited sequence CTPA revealed diffuse ground-glass opacity with mid and lower zone predominance, indicating progressive inflammatory changes compared to the previous imaging. Repeat lung function tests showed significant deterioration: FEV1 at 75%, FVC at 68%, and TLCO at 57%. Her echocardiogram was normal with no evidence of pulmonary hypertension. Despite the increase in her prednisolone to 10 mg and the initiation of azathioprine, she continued to experience dyspnoea at rest. After multidisciplinary discussions and review of guidelines and literature, the decision was made to use tocilizumab to stabilise her lung disease. She received two doses, one in the second and another in the third trimester. She safely delivered a healthy baby via planned C-section, and her symptoms significantly improved postpartum, allowing her to switch back to her usual mycophenolate mofetil. Discussion The incidence of ILD is highest within the first five years of a systemic sclerosis diagnosis. Patients with anti-topoisomerase antibodies are at a higher risk of developing ILD. Synthetic DMARDs, most commonly mycophenolate mofetil, are used to induce remission, but they have teratogenic side effects in pregnancy. The FDA approved tocilizumab, a humanised monoclonal antibody against the IL–6 receptor, for management of ILD associated with systemic sclerosis. Exacerbation of ILD during pregnancy is expected, with limited case reports on using non-teratogenic DMARDs and biological agents. For systemic sclerosis-ILD, the American College of Rheumatology conditionally recommends tocilizumab as a first-line treatment option. A meta-analysis suggests tocilizumab significantly prevents worsening of FVC, though it shows no significant improvement in skin disease control. The evidence regarding the teratogenicity of IL-6 inhibitors during pregnancy is limited. While it is advisable to discontinue IL-6 inhibitors at conception, exposure during pregnancy is unlikely to cause harm. If used in the third trimester, precautions such as avoiding live vaccines for the infant until six months of age are recommended. Maternal use of IL-6 inhibitors appears compatible with breastfeeding based on limited evidence. Pre-conception counselling should optimise disease control before pregnancy, including advice on timing, drug therapy, and contraception. Women planning pregnancy should avoid incompatible drugs, and the risks and benefits of treatment should be discussed. Contraindicated immunomodulatory drugs should be switched to pregnancy-compatible alternatives before conception. In severe maternal disease, controlling the disease takes priority over potential foetal outcomes, and biologic DMARDs may be continued if necessary. In this case, the patient experienced symptoms with significant deterioration in CT and lung function tests after stopping mycophenolate mofetil to conceive. After consulting with regional experts, tocilizumab was obtained through Trust funding, which helped to limit progression of disease. Key learning points • Early treatment is cruical for improving prognosis. The goal of treatment strategies is to achieve remission by halting disease progression and ideally reversing some disease-related changes. • There are studies supporting the efficacy of tocilizumab in improving lung function tests for patients with systemic sclerosis. In the above case, the off-label use of tocilizumab saved the life of this pregnant woman. This suggests that tocilizumab may help stabilise inflammatory lung disease during pregnancy, indicating its potential use in managing severe maternal disease if other pregnancy-compatible drugs are not suitable. The decision to use tocilizumab in pregnancy involves careful consideration of the risks and benefits, with input from a multidisciplinary team to ensure comprehensive patient care.

B-Lines in the Assessment of Interstitial Lung Disease Associated with Scleroderma: The Role of Handheld Devices.

Background: Timely detection and aggressive management of interstitial lung disease (ILD) in systemic sclerosis (SSc) are essential to improving outcomes and reducing risks of irreversible lung injury. Objective: to explore the usefulness of an ultraportable ultrasound device for the management of SSc-related ILD and to compare it with clinical and instrumental data. Methods: A total of 19 consecutive SSc patients underwent a comprehensive pulmonary evaluation: clinical, pulmonary function tests (PFTs) (spirometry, DLCO), lung CT (1.5 mm slice thickness reconstruction; HRCT), and lung ultrasound (LUS). A total score was calculated based on the number of color-coded B-lines recorded for each lung sliding. B-lines were analyzed against dyspnea, cough, Velcro, CT imaging (Warrick's score), and PFTs. Global and subgroup analysis were performed (diffuse versus limited cutaneous SSc, Warrick's < 7 versus >7). Results: Symptomatic lung involvement with varying degrees of dyspnea was reported in about 74% of cases (functional NYHA > 2 in more than half), chronic dry cough in one-third, Velcro rales in 42%. A total of 84.24% were classified as SSc with ILD on CT imaging. Statistically significant mild-to-moderate correlations between B-lines and clinical manifestations were demonstrated, as well as PFTs and Warrick's scores (more B-lines, lower pulmonary function, but higher extent and severity on CT) (p < 0.05); there were differences between SSc patients without and with ILD in terms of the number and distribution of B-lines (p < 0.05), as well as different B-lines patterns and numbers in diffuse versus limited SSc (p < 0.05). Conclusions: Ultraportable handheld LUS is a promising method suitable for the management (screening, early detection, and evaluation) of SSc patients.

Implications of the 2022 lung function update and GLI global reference equations among patients with interstitial lung disease

BackgroundLung function testing remains a cornerstone in the assessment and management of interstitial lung disease (ILD) patients. The clinical implications of the Global Lung function Initiative (GLI) reference equations and...

Efficacy of Rituximab Versus Cyclophosphamide and Mycophenolate for the Treatment of Interstitial Lung Disease in Systemic Sclerosis: A Systematic Review.

Interstitial lung disease (ILD) is a common complication of systemic sclerosis (SSc), contributing to significant morbidity and mortality in affected individuals. The optimal treatment approach for SSc-associated ILD remains uncertain, with rituximab, cyclophosphamide, and mycophenolate among potential therapeutic options. This systematic review aims to evaluate and synthesize the existing evidence on the efficacy of rituximab compared to cyclophosphamide and mycophenolate for the treatment of ILD in patients with systemic sclerosis. A comprehensive search of the following electronic databases, PubMed, Science Direct, Google Scholar, and Cochrane Library, has been conducted to identify relevant studies, including randomized controlled trials, systematic review and meta-analysis, prospective cohort studies, and retrospective cohort studies. Data on study characteristics, participant demographics, interventions, outcomes, and key findings have been extracted and synthesized. The risk of bias in the included studies has been assessed using appropriate tools such as the Cochrane Bias assessment tool for randomized controlled trials, the New Castle Ottawa tool for cohort studies, and the AMSTAR checklist for systematic reviews and meta-analysis. The research team ultimately selected 15 high-quality studies for review. Rituximab demonstrated similar efficacy to cyclophosphamide and mycophenolate in improving lung function (forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLCO)), with fewer severe adverse events. Cyclophosphamide, while effective, had higher toxicity, leading to more frequent adverse events such as leukopenia and infections. Mycophenolate showed comparable efficacy to cyclophosphamide but with fewer side effects, making it a well-tolerated alternative. The findings of this systematic review will provide valuable insights into the comparative efficacy of rituximab, cyclophosphamide, and mycophenolate in the management of ILD in systemic sclerosis, informing clinical decision-making and guiding future research in this area.

Interstitial Lung Disease Mortality Disparities Along the US-Mexico Border, 1999-2020

BackgroundOptimal diagnosis and management of interstitial lung diseases (ILD) needs access to specialized centers, frequent monitoring, and complex therapeutic options. In underprivileged areas, these necessities can often lead to barriers in delivering care. Research QuestionWhat are the ILD mortality disparities in the regions along the United States-Mexico (US-MX) border? Study Design and MethodsWe obtained ILD mortality information through death certificate queries from the Centers for Disease Control and Prevention repository. Death data were adjusted for age and stratified by US-MX border regions and non-border regions in the US. Log-linear regression models were utilized to analyze mortality trends in the period from 1999 to 2020 followed by calculation of annual percentage changes (APC). Age-adjusted mortality rates (AAMR) were compared across cumulative and sub-demographic populations. ResultsILD-related mortality among border regions (AAMR 5.31) was higher compared to non-border regions (AAMR 4.86). Mortality within border regions remained unchanged from 1999 to 2020 (APC +0.3, p=0.269). Non-border regions experienced a significant rise in mortality rates (APC +2.6, p=0.017) from 1999 to 2005 and remained unchanged from 2005 to 2020. Mortality was higher within both men (AAMR 6.57) and women (AAMR 4.36) populations among border regions compared to their non-border counterparts (AAMR 6.27 and 3.87, respectively). Hispanic populations among the border regions experienced higher mortality rates (AAMR 6.15) compared to Hispanic populations within non-border regions (AAMR 5.44). Non-Hispanic populations encountered similar mortality rates between the two regions. Mortality rates among Hispanic (APC +0.0, p=0.938) and non-Hispanic (APC +0.2, p=0.531) populations in the border regions remained unchanged from 1999 to 2020. InterpretationThese results revealed ILD-related mortality disparities among the US-MX border regions, emphasizing the importance of public health measures to increase access to equitable medical care and implement targeted interventions among these vulnerable populations.

Interstitial Lung Disease: Comparing Lecture and Large Group Case-Based Learning for Preclinical Medical Students.

Introduction and objective Interstitial lung disease (ILD) is a subject with which preclinical medical students often struggle. Because case-based learning (CBL) engages students in discussions centered around complex clinical scenarios, it may be effective for teaching ILD to preclinical medical students by fostering the development of critical thinking and clinical reasoning skills. Methods Lecture-based learning on ILD in the second-year Respiratory System course was replaced with a large group CBL session. Students worked in a large group team-based CBL (TB-CBL) to answer questions about the etiology, presentation, diagnostic radiology images, pathophysiology, and management of various ILDs. Performance on ILD-related final exam questions from Cohort A (Class of 2023, lecture) was compared with Cohort B (Class of 2025, TB-CBL). Student satisfaction was assessed through an anonymous end-of-course survey (5-point Likert). Results Mean performance on ILD-related final exam questions was 85.4 (SD: 16.5) for Cohort B (TB-CBL) vs. 80.0 (SD: 17.6) for Cohort A (lecture). Mean overall satisfaction was 3.31 (SD: 1.37), with 87.6%, 88.4%, and 87.6% agreeing or strongly agreeing with the statements "The session was well organized," "The session contributed to my learning," and "The session was a valuable use of my time," respectively. Conclusion Students participating in TB-CBL scored higher on ILD-related final exam questions compared with those who received lecture-based learning of the same material. Student satisfaction was acceptable but lower than expected. This TB-CBL session may be adapted at other institutions aiming to utilize active learning methods to teach the principles of diagnosis and management of ILD to preclinical medical students.

Pixel-Wise Interstitial Lung Disease Interval Change Analysis: A Quantitative Evaluation Method for Chest Radiographs Using Weakly Supervised Learning.

Interstitial lung disease (ILD) is characterized by progressive pathological changes that require timely and accurate diagnosis. The early detection and progression assessment of ILD are important for effective management. This study introduces a novel quantitative evaluation method utilizing chest radiographs to analyze pixel-wise changes in ILD. Using a weakly supervised learning framework, the approach incorporates the contrastive unpaired translation model and a newly developed ILD extent scoring algorithm for more precise and objective quantification of disease changes than conventional visual assessments. The ILD extent score calculated through this method demonstrated a classification accuracy of 92.98% between ILD and normal classes. Additionally, using an ILD follow-up dataset for interval change analysis, this method assessed disease progression with an accuracy of 85.29%. These findings validate the reliability of the ILD extent score as a tool for ILD monitoring. The results of this study suggest that the proposed quantitative method may improve the monitoring and management of ILD.

Clinical Impact of Telomere Length Testing for Interstitial Lung Disease

BackgroundShortened telomere length (TL) is a genomic risk factor for fibrotic interstitial lung disease (ILD), but its role in clinical management is unknown. Research QuestionWhat is the clinical impact of TL testing on the management of ILD? Study Design and MethodsPatients were evaluated in the Columbia University ILD clinic and underwent CLIA-certified TL testing by flow cytometry and fluorescence in-situ hybridization (FlowFISH) as part of clinical management. Short TL was defined as below the 10th age-adjusted percentile for either granulocytes or lymphocytes by FlowFISH. Patients were offered genetic counseling and testing if they had short TL or a family history of ILD. FlowFISH TL was compared against research qPCR TL measurement. ResultsA total of 108 patients underwent TL testing, including those with clinical features of short telomere syndrome such as familial pulmonary fibrosis (50%) or extrapulmonary manifestations in the patient (25%) or a relative (41%). The overall prevalence of short TL was 46% and was similar across clinical ILD diagnoses. The number of short telomere clinical features was independently associated with detecting short TL (OR 2.00, 95% CI [1.27, 3.32]). TL testing led to clinical management changes for 35 (32%) patients, most commonly resulting in reduction or avoidance of immunosuppression. Of the patients who underwent genetic testing (n=34), a positive or candidate diagnostic finding in telomere-related genes was identified in 10 (29%) patients. Inclusion of TL testing below the 1st percentile helped reclassify 8 of 9 variants of uncertain significance (VUS) into actionable findings. The qPCR test correlated with FlowFISH, but age-adjusted percentile cutoffs may not be equivalent between the two assays. InterpretationIncorporating TL testing in ILD impacted clinical management and led to the discovery of new actionable genetic variants.

Abstract PO4-12-06: Interstitial Lung Disease (ILD) in Sequential Antibody Drug Conjugate (ADC) use in patients with Metastatic Breast Cancer (MBC): A Multi-Institutional experience

Abstract Introduction: Interstitial Lung Disease (ILD) is a known adverse event associated with Antibody Drug Conjugates (ADCs), causing fibrosis of the lung. In the DESTINY-Breast 04, TROPICS-02, and ASCENT trials the rate of ILD/Pneumonitis was 12.1%, 0.4%, and 0% respectively. In these studies, the time to first cross-sectional imaging after initiation of ADC was 6 weeks. However, prior treatment with a topoisomerase-1 inhibitor ADC was not permitted in these studies, so the influence of sequential ADC treatments remains unknown. Here, we aimed to characterize the frequency and management of ILD in sequential use of ADCs in a multi-institution retrospective analysis. Methods: We identified patients (pts) with HR+/HER2-low and HR-/HER2-low MBC who had received both Trastuzumab-deruxtecan (T-DXd) and Sacituzumab govitecan (SG) monotherapy (in either order, with or without intervening therapies) who were treated at five academic centers between 2020-2023. Patients had received treatment with the ADC in either routine clinical treatment or as a prior participant on a clinical trial with ADC monotherapy. Information regarding ILD diagnosis, imaging, timing of onset, peak and resolution along with management information was collected and analyzed. Retrospective assessment of ILD toxicity was graded using CTCAE v5.0, based on available clinical and radiographic reports. Results: 10/60 pts who received ADC therapy in this multi-institutional cohort were diagnosed with ILD (all grades). No differences in age or BMI were observed in those who developed ILD versus those who did not, and 9/10 pts were White, 1 was Asian, with a median age of 59 years (47-79 years). Pertinent history in these pts included 3/10 had a prior smoking history, 8/10 had a prior cardiac or pulmonary comorbidity (including sleep apnea, lung cancer, hypertension, hyperlipidemia, thrombosis, or stroke). Prior to receiving their first ADC, 7/10 patients had metastatic disease to the lung. All 10 patients were diagnosed with ILD during treatment with T-DXd. 4 pts initially received T-DXd, then developed ILD and then upon recovery, subsequently received SG. The remaining 6 pts initially received SG (with no reports of ILD), and then received T-DXd and subsequently developed ILD. In the entire cohort, the median time from ADC initiation to first CT scan was 8 weeks (range 1-22 weeks). Within the cohort that developed ILD, the median time from initiation of T-DXd to first CT scan was also 8 weeks (range 6-13 weeks). The median time to diagnoses of ILD was 3 months after initiation of T-DXd (range 0.4-14.5 months). Dyspnea was the most common presenting symptom in 9/10 pts and at diagnosis of ILD, dyspnea was graded as III in 6/10 pts, II in 3/10 pts, and I in 1/10 pts. Diagnostic evaluation included CT chest (9/10), pulmonary consultation (8/10), pulmonary function testing (2/10), and bronchoscopy (1/10). 4/10 patients presented at their highest-grade toxicity at diagnosis. All patients stopped T-DXd treatment, 7 were hospitalized, 7 required supplemental oxygen, and 8 were prescribed steroids for treatment. Of those receiving steroids, the most common starting dose was prednisone 1 mg/kg and doses ranged from 0.5 mg/kg to 9mg/kg prednisone equivalents. No patient received a secondary immunosuppressive agent. The steroid treatment duration ranged from 1-4 months. None of the 10 pts underwent re-challenge with T-DXd and 3/10 developed grade V ILD (death). Conclusion: We observed a 16.6% incidence of all grade ILD (5% grade V ILD) with T-DXd and 0% with SG. In this small cohort study, we did not observe a clear increased risk for ILD with sequential treatment of ADCs. Practice patterns were consistent with recommended diagnostic evaluation and steroid management. Additional strategies are needed to characterize pts most at risk for developing ILD and to identify situations where re-challenge may be safe. Citation Format: Sarah Premji, Laura Huppert, Reshma Mahtani, Samantha Fisch, Naomi Dempsey, Angelina Raimonde-Taylor, Saya Jacob, Laura Quintal, Jo Chien, Michelle Melisko, Ana Sandoval-Leon, Lauren Carcas, Manmeet Ahluwalia, Natasha Harpalani, Jenna Hoppenworth, Dame Idossa, Ruta Rao, Hope Rugo, Karthik Giridhar. Interstitial Lung Disease (ILD) in Sequential Antibody Drug Conjugate (ADC) use in patients with Metastatic Breast Cancer (MBC): A Multi-Institutional experience [abstract]. In: Proceedings of the 2023 San Antonio Breast Cancer Symposium; 2023 Dec 5-9; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2024;84(9 Suppl):Abstract nr PO4-12-06.

Integration of Artificial Intelligence into Chest Computed Tomography

The integration of Artificial Intelligence (AI) in radiology, especially for chest computed tomography (CT) scan analysis, marked a significant advancement in medical diagnostics, aiming to improve patient care and streamline the workflow for radiologists. This review article examined the role of current AI technologies, including machine learning (ML), deep learning (DL), convolutional neural networks (CNN), and radiomics, in enhancing the detection and characterisation of lung diseases. These technologies are instrumental in identifying complex patterns within imaging data and constructing more informed decisions regarding disease severity, progression, and potential treatment options. Deep learning and CNN have demonstrated effectiveness in analysing the intricate details present in chest CT scans, offering a high degree of accuracy. Radiomics complements these methods by extracting quantitative features from medical images, providing deeper insights into disease characteristics that are not visible through standard imaging techniques. The application of AI has shown promise in improving the diagnosis and management of interstitial lung diseases and lung cancers, contributing to the development of personalised treatment plans. However, this review also highlights limitations, such as small sample sizes in studies, which may impact the generalisability of AI applications in this field. Despite these challenges, the ongoing incorporation of AI into radiological practices is anticipated to significantly enhance the accuracy and efficiency of lung disease diagnostics, setting a foundation for future research and improvements in clinical practice.

Therapeutic Approaches for the Treatment of Interstitial Lung Disease: An Exploratory Review on Molecular Mechanisms.

Interstitial Lung Diseases (ILDs) are characterized by shortness of breath caused by alveolar wall inflammation and/or fibrosis. Our review aims to study the depth of various variants of ILD, diagnostic procedures, pathophysiology, molecular dysfunction and regulation, subject and objective assessment techniques, pharmacological intervention, exercise training and various modes of delivery for rehabilitation. Articles are reviewed from PubMed and Scopus and search engines. ILD is a rapidly progressing disease with a high mortality rate. Each variant has its own set of causal agents and expression patterns. Patients often find it challenging to self-manage due to persistent symptoms and a rapid rate of worsening. The present review elaborated on the pathophysiology, risk factors, molecular mechanisms, diagnostics, and therapeutic approaches for ILD will guide future requirements in the quest for innovative and tailored ILD therapies at the molecular and cellular levels. The review highlights the rationale for conventional and novel therapeutic approaches for better management of ILD.

The diagnosis and management of systemic autoimmune rheumatic disease-related interstitial lung disease: British Society for Rheumatology guideline scope.

Interstitial lung disease (ILD) is a significant complication of many systemic autoimmune rheumatic diseases (SARDs), although the clinical presentation, severity and outlook may vary widely between individuals. Despite the prevalence, there are no specific guidelines addressing the issue of screening, diagnosis and management of ILD across this diverse group. Guidelines from the ACR and EULAR are expected, but there is a need for UK-specific guidelines that consider the framework of the UK National Health Service, local licensing and funding strategies. This article outlines the intended scope for the British Society for Rheumatology guideline on the diagnosis and management of SARD-ILD developed by the guideline working group. It specifically identifies the SARDs for consideration, alongside the overarching principles for which systematic review will be conducted. Expert consensus will be produced based on the most up-to-date available evidence for inclusion within the final guideline. Key issues to be addressed include recommendations for screening of ILD, identifying the methodology and frequency of monitoring and pharmacological and non-pharmacological management. The guideline will be developed according to methods and processes outlined in Creating Clinical Guidelines: British Society for Rheumatology Protocol version 5.1.

Imaging in the diagnosis and management of fibrosing interstitial lung diseases.

High-resolution computed tomography (HRCT) plays a pivotal role in the diagnosis and management of interstitial lung diseases (ILDs), particularly given the approval of antifibrotic agents for conditions like idiopathic pulmonary fibrosis and progressive pulmonary fibrosis. Diagnosing fibrotic pulmonary disorders through HRCT involves a detailed and methodical examination. The identification of specific lung tissue changes, including ground-glass opacities and reticulation, along with signs of fibrosis like honeycombing, traction bronchiectasis and lung volume loss, establishes clear HRCT patterns indicative of various ILDs. The reliability of these patterns in predicting pathological conditions depends largely on the clinical context. For instance, when a usual interstitial pneumonia pattern is present, the predictive value of this diagnosis is so high that a lung biopsy is considered to be redundant. This review intends to delineate the HRCT signs of fibrosis, elucidate the specific radiological patterns of fibrotic lung diseases, and identify the clinical circumstances under which these patterns emerge. Additionally, we introduce and discuss novel imaging techniques that hold promise for the diagnosis, screening and early detection of ILDs.

Towards the adoption of quantitative computed tomography in the management of interstitial lung disease.

The shortcomings of qualitative visual assessment have led to the development of computer-based tools to characterise and quantify disease on high-resolution computed tomography (HRCT) in patients with interstitial lung diseases (ILDs). Quantitative CT (QCT) software enables quantification of patterns on HRCT with results that are objective, reproducible, sensitive to change and predictive of disease progression. Applications developed to provide a diagnosis or pattern classification are mainly based on artificial intelligence. Deep learning, which identifies patterns in high-dimensional data and maps them to segmentations or outcomes, can be used to identify the imaging patterns that most accurately predict disease progression. Optimisation of QCT software will require the implementation of protocol standards to generate data of sufficient quality for use in computerised applications and the identification of diagnostic, imaging and physiological features that are robustly associated with mortality for use as anchors in the development of algorithms. Consortia such as the Open Source Imaging Consortium have a key role to play in the collation of imaging and clinical data that can be used to identify digital imaging biomarkers that inform diagnosis, prognosis and response to therapy.

Acneiform eruptions associated with nintedanib – A case report

Interstitial lung diseases (ILDs) these are a group of diffuse parenchymal lung disorders present with increased risk of morbidity and mortality. The patient presents with symptoms of gradual onset of dyspnea, dry cough, chest discomfort, and extreme tiredness, which causes decreased quality of life. Treatment of ILD includes the use of immunomodulatory therapies such as corticosteroids, azathioprine, cyclophosphamide, and monoclonal antibodies. Antifibrotic therapies include the use of pirfenidone and nintedanib. The most common adverse effects associated with tyrosine kinase inhibitors include hepatotoxicity, hypertension, Steven–Johnson syndrome, acne-like skin rash, and pruritus. The patient presented to the dermatology outpatient department with complaints of acneiform eruptions over the forehead and cheeks. Lesions started within 7–15 days of starting nintedanib for ILD. Post-dermatology consultation and local examination, acneiform eruptions due to the drug are the probable diagnosis made, and therefore, capsule nintedanib was withheld and prescribed Vitamin C tablets, tretinoin 0.05% with azelaic acid 10% and sunscreen. At the time of discharge tapering doses of prednisolone, tablet deriphylline 150 mg BD and tablet n-acetyl-cysteine 600 mg BD for 14 days were prescribed. Detailed history of the patient was taken and the relevant medical documents were scrutinized. This case was assessed as per the pharmacovigilance program of India. A very rare adverse reaction was seen with the use of nintedanib. After discontinuation of capsule nintedanib and acne management, patient experienced an improvement in the acne lesions. The case reported is a very rare adverse reaction seen with the use of nintedanib for the management of ILD. Our study is supported by temporal association, biological plausibility, and successful de-challenge.

Incidence of interstitial lung disease in patients with breast cancer: a nationwide database study in Japan.

Aim: This study estimated the incidence of moderate-to-severe drug-induced interstitial lung disease (ILD) among patients with breast cancer in Japan. Methods: We analyzed a large nationwide database of patients with breast cancer treated with anticancer therapies between 2009 and2022. ILD was identified using diagnostic codes and treatment records. Results: Of the 81,601 patients, 1042 developed ILD requiring corticosteroids, corresponding to an incidence rate of 1.41 per 100 person-years. The incidence varied across years and treatment regimens. Most ILD incidents occurred within the initial 90-day period post-anticancer therapy initiation. Conclusion: Increase in ILD cases and potential risk variations among treatments underlinethe importance of continued monitoring, especially during treatment onset, and ILD management in patients with breast cancer undergoing therapy.

Progressive Pulmonary Fibrosis: Where Are We Now?

Interstitial lung diseases (ILDs) are a diverse collection of lung disorders sharing similar features, such as inflammation and fibrosis. The diagnosis and management of ILD require a multidisciplinary approach using clinical, radiological, and pathological evaluation. Progressive pulmonary fibrosis (PPF) is a distinct form of progressive and fibrotic disease, occurring in ILD cases other than in idiopathic pulmonary fibrosis (IPF). It is defined based on clinical symptoms, lung function, and chest imaging, regardless of the underlying condition. The progression to PPF must be monitored through a combination of pulmonary function tests (forced vital capacity [FVC] and diffusing capacity of the lung for carbon monoxide), an assessment of symptoms, and computed tomography scans, with regular follow-up. Although the precise mechanisms of PPF remain unclear, there is evidence of shared pathogenetic mechanisms with IPF, contributing to similar disease behavior and worse prognosis compared to non-PPF ILD. Pharmacological treatment of PPF includes immunomodulatory agents to reduce inflammation and the use of antifibrotics to target progressive fibrosis. Nintedanib, a known antifibrotic agent, was found to be effective in slowing IPF progression and reducing the annual rate of decline in FVC among patients with PPF compared to placebos. Nonpharmacological treatment, including pulmonary rehabilitation, supplemental oxygen therapy, and vaccination, also play important roles in the management of PPF, leading to comprehensive care for patients with ILD. Although there is currently no cure for PPF, there are treatments that can help slow the progression of the disease and improve quality of life.

Expert consensus on the management of interstitial lung disease during the COVID-19 epidemic

Expert consensus on the management of interstitial lung disease during the COVID-19 epidemic

Chinese expert consensus on multidisciplinary discussion of interstitial lung disease

Interstitial lung disease (ILD) is a group of heterogeneous diseases characterized by interstitial lung inflammation and fibrosis. Its causes are diverse, symptoms can be non-specific, and the associated imaging and pathologic patterns can vary widely. The diagnosis and management of these conditions often require input from multiple medical disciplines. Over the past few decades, the importance of multidisciplinary discussion (MDD) in the management of ILD has grown internationally, and MDD has gradually become a part of the gold standard for ILD diagnosis and an essential reference for disease management. Recognizing this, the Chinese Medical Association-Chinese Thoracic Society and the Chinese Association of Chest Physicians have collaborated with experts to standardize the ILD-MDD process in China.Key recommendations:1. Formation of teams: ILD centers should establish multidisciplinary teams (MDT) and integrate MDD into the ILD patient care process. (strong recommendation)2. Patient preparation: Prior to ILD-MDD, patients should undergo as thorough an ILD assessment as possible, including medical history, diagnostic tests, and functional evaluations. (strong recommendation)3. MDD frequency & logistics:(1) The frequency, duration and number of cases to be discussed in ILD-MDD should be based on the clinical needs of each center, but should be held at least monthly. (2) Consistent timing and location of ILD-MDD is recommended to improve attendance. (medium recommendation)4. Team composition: The MDT should include experts from a range of disciplines with experience of ILD. These should include specialists in Pulmonary and Critical Care Medicine, Radiology, and Pathology. Where appropriate, experts in Rheumatology, Hematology, Thoracic Surgery, and Respiratory Care and Rehabilitation should also be involved. (strong recommendation)5. Case presentation & goals:(1) The physician in charge should present clinical information, preferably using standardized slides. Real-time imaging and pathology data are encouraged. (2) The primary goals of ILD-MDD discussions should be disease diagnosis and management. (strong recommendation)6. Operational leadership: Assign a dedicated professional to manage the ILD-MDD operations and maintain the information discussed. (strong recommendation)7. Specific conditions:(1) Suspected idiopathic pulmonary fibrosis should be diagnosed by a standardized MDD process. (2) Suspected idiopathic interstitial pneumonia (IIP) should be classified by MDD. If IIP is indicated by imaging or pathology, prioritize identifying potential underlying causes such as connective tissue diseases (CTDs), certain medications, or aspiration.(3) Suspected hypersensitivity pneumonitis should also be diagnosed by MDD. (4) If an ILD patient shows signs suggestive of CTDs, the diagnosis should be made by ILD-MDD with the involvement of rheumatologists. (strong recommendation)8. Unclassifiable ILD: For ILD patients who cannot be clearly classified, create a tailored management plan based on the patient's specific disease features. (strong recommendation)9. Progressive pulmonary fibrosis: Use ILD-MDD to determine whether an ILD patient meets the criteria for progressive pulmonary fibrosis and to discuss a personalized care plan. (strong recommendation).