Management Of Interstitial Lung Disease Research Articles

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.

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.

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.

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.

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).

Recent Advances in the Treatment of Interstitial Lung Diseases.

Interstitial lung diseases (ILDs) are a group of disorders affecting the parenchymal tissue of the lungs. This disease leads to complications like pulmonary hypertension, heart failure, etc. that can affect patients. The etiological factors, clinical features, investigation methods, and diseases are conditions associated with ILD. The history of these conditions is of great value; any history of environmental and occupational exposure, medications, dust, or any toxic inhalation can be a predisposing factor. The CT scan is the investigation of choice in the case of ILD. This article states the recent advances made in treating interstitial lung diseases. The non-pharmacological and pharmacological management of ILD is discussed in the article. The discussion below concerns newer drugs approved by the FDA and their adverse effects, dosages, and contraindications. Below is a detailed conversation about ILD and the recent advances in treating this disease.

Role of Multidisciplinary Team Meetings in the Diagnosis and Management of Diffuse Parenchymal Lung Diseases in a Tertiary Care Hospital.

Background Decisions on the management of interstitial lung diseases (ILD) and prognostication require an accurate diagnosis. It has been proposed that multidisciplinary team (MDT) meetings for ILD (ILD-MDT) improve these decisions in challenging cases of ILD. However, most studies in this field have been based on the decisions of individual clinicians and there are few reports on the outcomes of the ILD-MDT approach. We therefore describe the experience of the ILD-MDT meetings at our institution. Methods A single-center retrospective review of the electronic health care records of patients discussed in the ILD-MDT meetings at our institution from February 2016 to January 2021 was performed. At out institution, at each ILD-MDT meeting, the referring pulmonologist presents the clinical history and the results of all relevant investigations including serology, blood gas analyses, lung function tests, bronchoscopy, and bronchoalveolar lavage. A radiologist then describes the imaging including serial computed tomography (CT) scans. When available, the findings on lung biopsy are presented by a pathologist. Subsequent discussions lead to a consensus on the diagnosis and further management. Results The study included 121 patients, comprising 71 (57%) males and 76 nonsmokers (62.8%), with a mean age of 65 years (range: 25-93 years). The average number of comorbidities was 2.4 (range: 0-7). Imaging-based diagnoses were usual interstitial pneumonia (UIP)/chronic hypersensitivity pneumonitis (CHP) in 32 (26%) patients, UIP in 20 (17%) patients, probable UIP in 27 (22%) patients, nonspecific interstitial pneumonia in 11 (9%) patients, and indeterminate interstitial lung abnormalities (ILA) in 10 (8%) patients. The most common consensus clinical diagnosis after an ILD-MDT discussion was chronic hypersensitivity pneumonitis/idiopathic pulmonary fibrosis in 17 patients (14%), followed by idiopathic pulmonary fibrosis and connective tissue disease associated interstitial lung disease in 16 patients (13%), CHP in 11 patients (9.1%), and ILA in 10 patients (8.4%). Only a 42 patients (35%) required surgical lung biopsy for confirmation of the diagnosis. Conclusion This study describes the characteristics of the patients discussed in the ILD-MDT meetings with emphasis on their clinical, radiological, and laboratory data to reach a diagnosis and management plan. The decisions on commencement of antifibrotics or immunosuppressive therapy for patients with various ILDs are also made during these ILD-MDT meetings. This descriptive study could help other health care professionals regarding the structure of their ILD-MDT meetings and with discussions about diagnostic and care decisions for diffused parenchymal lung disease patients.

Successful management of interstitial lung disease in dermatomyositis complicated by malignancy: a case-based review.

Dermatomyositis (DM) is associated with interstitial lung disease (ILD) and malignancy. However, the coexistence of ILD and malignancy (DM-ILD-malignancy) is rare, and limited information exists regarding its management. Herein, we report the case of a 70-year-old man who developed DM with rapidly progressive ILD and advanced gastric cancer and provide a literature review of managing DM-ILD-malignancy. The patient presented with typical DM skin rashes and shortness of breath, which worsened within 1month, without muscular symptoms. Additionally, the patient tested negative for myositis-specific autoantibodies (MSAs). Computed tomography revealed ILD and advanced gastric cancer, which was confirmed on endoscopic examination to be a poorly differentiated adenocarcinoma. Although the patient's ILD progressed rapidly, surgical treatment of the cancer was prioritized. Prednisolone (PSL) 0.5mg/kg was initiated 3days before surgery and increased to 1mg/kg at 7days postoperative. Remarkable improvement in the skin rash and ILD was observed, and the PSL dose was tapered without immunosuppressants. A literature review revealed that anti-melanoma differentiation-associated gene 5 and anti-aminoacyl transfer RNA synthetase antibodies are the predominant MSAs in DM-ILD-malignancy, and the optimal treatment should be determined based on several factors, including ILD patterns, and malignancy type and stage. In particular, lung cancer may be a risk factor for the acute exacerbation of ILD, and preceding immunosuppression would be useful. Furthermore, prioritizing surgery for gastric cancer is effective because of its paraneoplastic nature.

The Evolving Concept of the Multidisciplinary Approach in the Diagnosis and Management of Interstitial Lung Diseases.

Interstitial lung diseases (ILDs) are a group of heterogeneous diseases characterized by inflammation and/or fibrosis of the lung interstitium, leading to a wide range of clinical manifestations and outcomes. Over the years, the literature has demonstrated the increased diagnostic accuracy and confidence associated with a multidisciplinary approach (MDA) in assessing diseases involving lung parenchyma. This approach was recently emphasized by the latest guidelines from the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Association for the diagnosis of ILDs. In this review, we will discuss the role, composition, and timing of multidisciplinary diagnosis (MDD) concerning idiopathic pulmonary fibrosis, connective tissue disease associated with ILDs, hypersensitive pneumonia, and idiopathic pneumonia with autoimmune features, based on the latest recommendations for their diagnosis. The integration of clinical, radiological, histopathological, and, often, serological data is crucial in the early identification and management of ILDs, improving patient outcomes. Based on the recent endorsement of transbronchial cryo-biopsy in idiopathic pulmonary fibrosis guidelines, an MDA helps guide the choice of the sampling technique, obtaining the maximum diagnostic performance, and avoiding the execution of more invasive procedures such as a surgical lung biopsy. A multidisciplinary team should include pulmonologists, radiologists, pathologists, and, often, rheumatologists, being assembled regularly to achieve a consensus diagnosis and to review cases in light of new features. The literature highlighted that an MDA is essential to improve the accuracy and reliability of ILD diagnosis, allowing for the early optimization of therapy and reducing the need for invasive procedures. The multidisciplinary diagnosis of ILDs is an ongoing and dynamic process, often referred to as a "working diagnosis", involving the progressive integration and re-evaluation of clinical, radiological, and histological features.

Interstitial lung disease: a decade of progress and hope

The 10th anniversary of The Lancet Respiratory Medicine is an appropriate time to acknowledge advances in the management of interstitial lung disease (ILD) and to contemplate future directions for the field of respiratory medicine, especially for fibrotic ILD, in which developments have revolutionised both our understanding of ILD and the approach to treatment. ILDs have diverse causes and clinical presentations, making diagnosis and management challenging for clinicians. Pulmonary fibrosis, a progressive form of ILD, is characterised by inflammation and fibrosis of the lung parenchyma. 1 Raghu G Remy-Jardin M Richeldi L et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022; 205: e18-e47 Crossref PubMed Scopus (255) Google Scholar Among ILDs, idiopathic pulmonary fibrosis (IPF) is the most common and severe form, with a median survival of 3–5 years from diagnosis, emphasising the importance of ongoing research to reveal the intricacies of disease progression and improve outcomes and quality of life for people with ILD. 2 Khor YH Ng Y Barnes H Goh NSL McDonald CF Holland AE Prognosis of idiopathic pulmonary fibrosis without anti-fibrotic therapy: a systematic review. Eur Respir Rev. 2020; 29190158 Crossref Scopus (30) Google Scholar In the past decade of ILD research, numerous patient-centred achievements have transformed clinical practice and given hope to people with ILD. While acknowledging the past, we should also consider emerging research areas and unanswered questions that have the potential to further transform the field of ILD.

Clinico-Radiological Profile of Patients with Connective Tissue Disease (CTD) Associated Interstitial Lung Disease (ILD) At a Tertiary Care Center in India

Interstitial lung diseases (ILDs) are a group of heterogenous disorders characterized by varying degree of fibrosis in lung [1]. Work-up for connective tissue diseases (CTDs) is an important component of evaluation of a patient with ILD. CTDs affect connective tissue in various organs resulting from poorly controlled autoimmune responses, and associated inflammation. Overall ILD has been documented in up-to 15% of patients with CTD, with rates as high as 26% in Systemic Sclerosis (SS) [2]. Lung involvement contributes to mortality in CTDs, and the management of ILDs depends on the presence and severity of underlying CTDs. Hence it is necessary to understand the clinical presentation of CTD-ILDs

Interstitial lung disease diagnosis and prognosis using an AI system integrating longitudinal data

For accurate diagnosis of interstitial lung disease (ILD), a consensus of radiologic, pathological, and clinical findings is vital. Management of ILD also requires thorough follow-up with computed tomography (CT) studies and lung function tests to assess disease progression, severity, and response to treatment. However, accurate classification of ILD subtypes can be challenging, especially for those not accustomed to reading chest CTs regularly. Dynamic models to predict patient survival rates based on longitudinal data are challenging to create due to disease complexity, variation, and irregular visit intervals. Here, we utilize RadImageNet pretrained models to diagnose five types of ILD with multimodal data and a transformer model to determine a patient’s 3-year survival rate. When clinical history and associated CT scans are available, the proposed deep learning system can help clinicians diagnose and classify ILD patients and, importantly, dynamically predict disease progression and prognosis.

Management of interstitial lung disease in patients with autoimmune disease-related interstitial lung disease.

Interstitial lung disease (ILD) is a common manifestation of systemic autoimmune diseases. A proportion of patients with autoimmune disease associated-ILDs develop progressive pulmonary fibrosis. Regular monitoring of patients with pulmonary fibrosis is recommended to enable prompt detection of progression and initiation or escalation of therapy if needed. However, there is no established algorithm for the treatment of autoimmune disease associated-ILDs. In this article, we present three case studies that demonstrate the challenges in the diagnosis and management of patients with autoimmune disease associated-ILDs and the importance of taking a multidisciplinary approach to their care.