Treatment Of Interstitial Lung Disease Research Articles

Treatment patterns and patient journey in progressive pulmonary fibrosis: a cross-sectional survey

BackgroundFor patients with interstitial lung diseases (ILDs) presenting with a progressive pulmonary fibrosis (PPF) phenotype, current knowledge of disease characteristics at diagnosis, patient journey, and treatment is limited. This study aimed to describe demographics and clinical experiences of patients presenting with PPF in a European real-world setting.MethodsData were analysed from the Adelphi Real World PPF-ILD Disease Specific Programme™, a cross-sectional survey of pulmonologists and rheumatologists in five European countries (France, Germany, Italy, Spain, United Kingdom) and internal medicine specialists (France) from April to October 2022. Physicians provided data for up to 12 consecutive patients with physician-confirmed ILD with a progressive phenotype other than idiopathic pulmonary fibrosis. Analyses were descriptive.ResultsOverall, 265 physicians reported on 1,335 patients. Mean (standard deviation) age at survey date was 60.4 (11.6) years, 91.2% were white, 58.1% female, 44.0% non-smokers. Most patients (63.3%) first consulted a primary care physician. There was a mean delay of 7.8 (22.7) months between first ILD symptom and healthcare professional visit, and another 7.7 (12.8) months to ILD diagnosis. At survey date, 47.7% of patients had physician-reported moderate ILD, 42.3% had mild ILD and 10.0% had severe ILD. Disease progression was reported in the 12 months prior to the survey for 19.5% of patients; of these, progression was based on worsening symptom in 27.3% and lung function decline in 25.8%. For patients experiencing symptoms prior to ILD diagnosis (72.8%), the most common symptoms were dyspnoea on exertion (80.5%) and cough (57.8%). Overall, 17.4% of patients were misdiagnosed prior to ILD diagnosis, with chronic obstructive pulmonary disease suspected in 39.2% of them. The most frequent comorbidities were anxiety (16.9%) and gastroesophageal reflux (15.5%). Although 77.8% of patients were receiving treatment for ILD at survey date, 15.6% of patients had never been prescribed treatment for ILD.ConclusionsThis real-world study expands our understanding of patients, diagnostic delays and treatment gaps experienced by patients diagnosed with PPF in Europe. There was a mean delay of 15.5 months between first ILD symptoms and ILD diagnosis. Given the progressive nature of PPF, diagnostic delay may lead to poor outcomes, including shorter survival.Trial registrationN/a.

EVALI SYNDROME IN A PATIENT WITH HYPERSENSITIVITY PNEUMONITIS

Smoking electronic cigarettes, or vaping, is considered by many to be a safe alternative to smoking tobacco. It was only relatively recently that reports began to appear about the development of lung damage caused by electronic cigarettes - EVALI syndrome. Currently, the morphological manifestations of this syndrome have not been studied sufficiently due to the small number of cases described in the literature. These facts, as well as the fact that the changes that occur are not specific, and may vary in different patients, make it difficult to diagnose the disease in a timely manner. We have described a clinical case of death in a patient who has repeatedly received inpatient treatment for interstitial lung disease, who was admitted to the pulmonology department with signs of decompensated respiratory failure after prolonged and intensive use of electronic cigarettes. The autopsy revealed and substantiated clinical and morphological signs of chronic hypersensitive pneumonitis in combination with EVALI syndrome. The article also provides a brief literature review of the literature on EVALI and hypersensitive pneumonitis with their diagnostic criteria. Like EVALI, hypersensitive pneumonitis is difficult to recognize and diagnose by doctors, which is often associated with low awareness, as well as the absence of pathognomonic symptoms.

Chronic Respiratory Diseases: Innovations in Treatment and Management

Chronic Respiratory Diseases (CRDs) include diseases of the airways and the lung and are among the major causes of morbidity and mortality worldwide. The major CRDs include asthma, chronic obstructive pulmonary disease (COPD), pulmonary sarcoidosis, pneumoconiosis and interstitial lung diseases (ILDs). The common symptoms of CRDs include wheezing, chest tightness, shortness of breath and cough [1]. These diseases affect millions of people across the globe and have significant impacts on patients’ quality of life and healthcare. Over the past few decades, however, improvement in the pharmacotherapy has provided a considerable progress. For example, biologics have given a new insight in the management of severe asthma by modulating certain inflammatory processes. Medications like omalizumab, dupilumab, and Mepolizumab are aimed at adjusting the immune response and significantly decrease the frequency of exacerbations and increase overall lung function. In addition, innovations in the field of stem cell therapy and tissue engineering are targeted to repair or replace diseased lung tissue, thus, being aimed at treating the origin of the diseases rather than simply alleviating the symptoms. Furthermore, Nanoparticles can be orally given, intravenously injected or inhaled using nanoparticle aerosols. Nanoparticles can also be used in the molecular imaging of chronic lung diseases such as COPD. Advanced inhalers, developed with sensors, can monitor the usage of medication and provide feedback in real-time to ensure compliance to prescriptions and other salient treatment plans. COPD pulmonary rehabilitation programs of exercise and education together with psychological support for individuals have been documented to enhance quality of life. The use of AI and a machine learning system has now moved to the ability to forecast disease exacerbations and provide individualized recommendations for treatment of CRDs. As a result, people with COPD receive care through various healthcare professions like doctors, nurses, and physiotherapists, which may have diverse roles, such as prescribing medications, supporting their self‐management or patient education, or delivering exercise training. The aim of an Integrated Disease Management (IDM) programme is that various aspects of care through which healthcare providers are working in coordination to deliver improved and optimum care to patients. Similarly Advancements in the treatment of ILD are being conducted, and recently, some new FDA-approved drugs have been advised for the management of patients. Among these are Nintedanib is a tyrosine kinase inhibitor, Pirfenidone an anti-fibrotic and anti-thrombotic agent and Treprostinil is an analogue of prostacyclin. These drugs have opened a new path for the treatment of ILD and have improved the recovery outcomes of patients [2]. Rituximab has also been found to be effective in more than half of patients treated in a small prospective open-label trial of refractory pulmonary disease in sarcoidosis[3]. Overall, there is a renewed emphasis on CRD management as a burgeoning field. With the advancement in the research in the personalized, proactive and integrative strategy the efforts made will lay down new avenues of better health outcomes for millions of people suffering from the CRDs. The future of respiratory health is bright as it is a growing field that has no sign of discontinuing its steady development and pushing for the comforts and breakthroughs for many patients across the globe.

Nintedanib in the treatment of interstitial lung disease in patients with systemic connective tissue diseases

Nintedanib in the treatment of interstitial lung disease in patients with systemic connective tissue diseases

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.

Treatment of interstitial lung disease in systemic sclerosis: guidelines and new clinical trial results.

Interstitial lung disease (ILD) is the leading cause of death in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). The American College of Rheumatology (ACR), in conjunction with the American College of Chest Physicians (CHEST), recently published clinical practice guidelines for the treatment of adults with systemic autoimmune rheumatic disease-associated ILD, including SSc-ILD. Herein, we summarize evidence from randomized trials evaluating the safety and efficacy of pharmacologic therapies for the treatment of SSc-ILD. In this review, we present findings from recent randomized controlled trials in SSc-ILD. The pharmacologic therapies discussed include immunosuppressive medications (mycophenolate, cyclophosphamide, rituximab, and tocilizumab) and antifibrotic medications (nintedanib and pirfenidone). Randomized trials provide an evidence base for the SSc-ILD treatment recommendations put forth in the ACR/CHEST Guidelines for the treatment of ILD in people with systemic autoimmune rheumatic diseases. These guidelines will help inform clinical practice and highlight areas in which further research is needed.

Potential of phosphodiesterase 4B inhibitors in the treatment of interstitial lung disease associated with autoimmune diseases.

Patients with autoimmune disease-related interstitial lung disease may develop pulmonary fibrosis, which may become progressive. Progressive pulmonary fibrosis (PPF) is associated with poor outcomes. Antifibrotic therapies have shown efficacy as treatments for PPF in patients with autoimmune diseases, but new treatments are needed to slow or halt disease progression. Phosphodiesterases (PDEs) are enzymes that mediate the hydrolysis of cyclic adenosine monophosphate (cAMP) and cyclic guanosine monophosphate (cGMP). Pre-clinical data suggest that preferential inhibition of PDE4B has the potential to slow the progression of pulmonary fibrosis by inhibiting inflammatory and fibrotic pathways, with a lower risk of gastrointestinal adverse events than associated with pan-PDE4 inhibitors. Nerandomilast (BI 1015550) is a preferential PDE4 inhibitor that has demonstrated anti-inflammatory and antifibrotic effects in pre-clinical studies. In a phase II trial in patients with idiopathic pulmonary fibrosis, nerandomilast (given alone or on top of background antifibrotic therapy) prevented a decrease in lung function over 12 weeks with an acceptable safety and tolerability profile. The phase III FIBRONEER-ILD trial is evaluating the efficacy and safety of nerandomilast, given alone or on top of nintedanib, in patients with PPF, including PPF associated with autoimmune diseases. In this article, we review the potential of PDE4B inhibition in the treatment of ILD associated with autoimmune diseases, including the pre-clinical and early clinical data available to date.

Update on systemic sclerosis

The updates to the European recommendations and the German guidelines for the treatment of systemic sclerosis are expected shortly, which are very good evidence-based guidelines for all those treating the disease; however, there are still disease manifestations with insufficient studies and current study results that were published after the review of the literature for the guidelines and might be of interest to the reader. The aim of this work is to provide an overview of the publications in the last year that are interesting from the authors' point of view. The aim is to provide practically relevant information on the current state of knowledge that can supplement the guidelines. The pathogenesis of systemic sclerosis (SSc) is becoming better understood in its interplay between environmental factors and the development of autoantibodies. There have also been overviews of the manifestation and prognosis of cardiac involvement in the last year. The American Thoracic Society issued the first guidelines for the treatment of interstitial lung disease in SSc. There are an increasing number of studies that suggest that disease-modulating combination therapies, such as rituximab and mycophenolate mofetil (MMF) are beneficial. Work addressing the involvement of joints suggests that inflammatory changes are common. Current options for the treatment of gastrointestinal involvement are presented. The diagnosis and treatment of systemic sclerosis is making progress and many symptoms and complications are treatable. Nevertheless, much remains to be done to improve the quality of life of the patients.

Physicians\' Perspectives and Practice Patterns in India on the Diagnosis and Treatment of Interstitial Lung Disease (IN-ILD Survey)

Physicians\' Perspectives and Practice Patterns in India on the Diagnosis and Treatment of Interstitial Lung Disease (IN-ILD Survey)

2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases.

We provide evidence-based recommendations regarding the treatment of interstitial lung disease (ILD) in adults with systemic autoimmune rheumatic diseases (SARDs). We developed clinically relevant population, intervention, comparator, and outcomes questions. A systematic literature review was then performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A panel of clinicians and patients reached consensus on the direction and strength of the recommendations. Thirty-five recommendations were generated (including two strong recommendations) for first-line SARD-ILD treatment, treatment of SARD-ILD progression despite first-line ILD therapy, and treatment of rapidly progressive ILD. The strong recommendations were against using glucocorticoids in systemic sclerosis-ILD as a first-line ILD therapy and after ILD progression. Otherwise, glucocorticoids are conditionally recommended for first-line ILD treatment in all other SARDs. This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the treatment of ILD in people with SARDs.

2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases.

We provide evidence-based recommendations regarding the treatment of interstitial lung disease (ILD) in adults with systemic autoimmune rheumatic diseases (SARDs). We developed clinically relevant population, intervention, comparator, and outcomes questions. A systematic literature review was then performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A panel of clinicians and patients reached consensus on the direction and strength of the recommendations. Thirty-five recommendations were generated (including two strong recommendations) for first-line SARD-ILD treatment, treatment of SARD-ILD progression despite first-line ILD therapy, and treatment of rapidly progressive ILD. The strong recommendations were against using glucocorticoids in systemic sclerosis-ILD as a first-line ILD therapy and after ILD progression. Otherwise, glucocorticoids are conditionally recommended for first-line ILD treatment in all other SARDs. This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the treatment of ILD in people with SARDs.

Assessing Patient Values and Preferences to Inform the 2023 American College of Rheumatology/American College of Chest Physicians Interstitial Lung Disease Guidelines.

Patient engagement is critical to clinical practice guideline (CPG) development. This work presents our approach to ascertaining patients' values and preferences to inform the American College of Rheumatology guidelines for screening, monitoring, and treatment of interstitial lung disease (ILD) in people with systemic autoimmune rheumatic diseases (SARDs). We conducted a cross-sectional qualitative study of a purposefully sampled Patient Panel using a modified content analytic approach. The study team reviewed text transcripts from the Patient Panel discussion to identify themes and develop a clustered thematic schema. Twenty-one patients (75% women) participated, with a mean age of 53 years (range 33-73). Patients had one or more SARDs: systemic sclerosis (38%), Sjögren disease (38%), idiopathic inflammatory myopathy (33%), rheumatoid arthritis (24%), and mixed connective tissue disease (10%). We identified 10 themes in 4 thematic clusters: communication, screening and monitoring, treatment goals, and treatment adverse effects. Patients prioritized recognizing ILD symptoms, importance of ILD screening and close monitoring, goals of survival and improving quality of life, and willingness to accept treatment risks provided that there is close communication with providers. Patient representatives shared patients' priorities and insight at the Voting Panel meeting, influencing multiple guideline recommendations. Patient engagement fosters a holistic approach to CPG development, leading to recommendations aiming for the best clinical outcomes while prioritizing outcomes important for patients. The patient-identified themes played a critical role in ILD guideline development and provide core elements for shared decision-making as clinicians make management and therapeutic decisions with patients with SARD-associated ILD.

Clinical Experience of Liu Huawei, a Famous Chinese Medicine Practitioner, in the Treatment of Interstitial Lung Disease by Applying the "Five Elements of qi-hua Theory"

Clinical Experience of Liu Huawei, a Famous Chinese Medicine Practitioner, in the Treatment of Interstitial Lung Disease by Applying the "Five Elements of qi-hua Theory"

Abstract PO5-12-12: Implementation of a comprehensive monitoring protocol for the prevention and treatment of interstitial lung disease in patients undergoing treatment with trastuzumab deruxtecan

Abstract Background: In the DESTINY-BREAST studies, Trastuzumab deruxtecan (T-DXd) demonstrated significant antitumor activity in heavily pretreated patients (pts) with HER2-positive and HER2-low advanced breast cancer (ABC). T-DXd is associated with an increased risk of interstitial lung disease (ILD), which occurs in 10-15% of patients with a 2.2% rate of fatal events. Increased monitoring for ILD within DESTINY-BREAST03 showed a reduction in grade 3-5 events. Patients with a history of clinically significant lung disease were excluded from this study, however this may not be reflective of the standard community patient population treated with T-DXd. There are currently no standardized monitoring protocols for ILD/pneumonitis for pts treated with T-DXd. We conducted a retrospective chart review to assess the benefit of implementing an ILD monitoring protocol in a cohort of patients receiving treatment with T-DXd at the Duke Cancer Institute. Methods: Patients with HER2-positive or HER2-low ABC who received ≥ five cycles of T-DXd between Jan 1, 2020- April 30, 2022 were included. Imaging with CT chest ILD protocol and pulmonary function testing with diffusing capacity for carbon monoxide (DLCO) were performed at baseline prior to initiation of T-DXd, prior to cycle 3 and cycle 5 in conjunction with ILD chest CT and then every 6 weeks to monitor for ILD. DLCO was corrected for hemoglobin. Clinical symptoms including cough, shortness of breath, dyspnea, and new or worsening respiratory symptoms were recorded. For pts that experienced more than a 10% decrease in corrected DLCO (DLCOc), a pulmonology consult was recommended. DLCOc was reviewed in combination with chest imaging and clinical history to evaluate for ILD and guidance was provided for continuation or cessation of T-DXd. Results: A total of 33 patients with HER2-positive (N=27) and HER2-low (N=6) ABC were monitored as per the predefined ILD monitoring protocol. There were 5 confirmed cases of ILD/pneumonitis within the pt cohort. 16 patients (48%) experienced DLCOc decreases >10% warranting a pulmonology consult or further review. Upon further assessment, ILD/pneumonitis was ruled out based on chest imaging and/or asymptomatic presentation in 11 patients and therapy continued without treatment delay or discontinuation. There were 5 confirmed cases of ILD/pneumonitis within the patient cohort. 3 of the 5 patients diagnosed with ILD experienced DLCOc decreases ≥10%, while 2 of the 5 diagnosed patients had DLCOc changes of < 10% but had concern for ILD on ILD/chest CT and were diagnosed after pulmonology consult. Additional results to be presented at meeting. Conclusion: For patients receiving T-DXd, implementation of a comprehensive protocol to monitor and assess for ILD secondary to treatment may allow patients with suspected ILD to begin treatment sooner or prevent permanent discontinuation by excluding ILD diagnosis. While PFT monitoring can be helpful for some patients to monitor for ILD, continuous PFT monitoring is not recommended for all patients given inconsistency. However, baseline PFTs prior to initiation and with suspected ILD in combination with imaging warrants further investigation. The use of a multidisciplinary team including pulmonologists experienced with T-DXd-induced ILD may assist in diagnosing or excluding ILD and allow for continuation of T-DXd therapy. Citation Format: Heather Moore, Kelly Westbrook, Thomas Bagwell, Scott Shofer, Carey Anders, Amy Guisinger, Olivia White, Susan Dent. Implementation of a comprehensive monitoring protocol for the prevention and treatment of interstitial lung disease in patients undergoing treatment with trastuzumab deruxtecan [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 PO5-12-12.

Imaging Innovations in the Screening, Diagnosis, and Monitoring of Systemic Autoimmune Disease-Related Interstitial Lung Disease

Interstitial lung disease (ILD) is a common and serious complication of systemic autoimmune rheumatic diseases (SARD). The prevalence and prognosis of ILD vary depending on the type and severity of the underlying SARD. Recently, two drugs have been approved by the U.S. Food and Drug Administration (FDA) for the treatment of ILD in SARD, and the American College of Rheumatology (ACR) has issued guidelines for the screening and monitoring of these patients. Therefore, it is essential to detect and manage ILD in SARDs as early as possible. High-resolution CT of the chest is the current gold standard test for diagnosis of ILD, yet, it is not uniformly performed as an initial diagnostic test. This review discusses the latest advances in imaging techniques for the diagnosis, assessment, and follow-up of SARD-ILDs.

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.

Interstitial lung disease and associated factors in patients with Sjögren's syndrome.

Interstitial lung disease (ILD) is a common pulmonary manifestation of Sjögren's syndrome (SjS) and associated with an increased risk of death. Early detection and treatment of ILDs and knowing the risk factors are very important for prognosis in rheumatic diseases. This study was performed to determine ILD and associated factors in patients with SjS. Four hundred three SjS patients were evaluated in this cross-sectional cohort study. Clinical, laboratory, serological, and imaging features were compared of patients with and without pulmonary involvement. Logistic regression analyses were used to identify risk factors for lung involvement and to identify independent risk factors. Thirty-five (8.7%) of SjS patients had ILD and 368 (91.3%) had no ILD. The presence of Raynaud's phenomenon was significantly more common in ILD. The geriatric age group over the age of 65years (OR 8198; 95% CI 3788-17,742; p < 0.001), Raynaud's phenomenon (OR 17,852; 95% CI 6155-51,779; p < 0.001), and smoking (OR 3598; 95% CI 1495-8657; p = 0.003) were risk factors to be associated for ILD in the multivariable analysis. The most common abnormality was non-specific interstitial pneumonia in 20 patients (57.1%) and usual interstitial pneumonia in 15 (42.9%) patients. The distribution of male patients compared to female patients was higher in patients with lung involvement than in patients without lung involvement. This may be related to older age, higher smoking rate, and longer nicotine consumption in men. Age, smoking, and severity of lung involvement are more important than inflammation status and autoantibodies for prognosis.

A Case of Successful Immunosuppressive Therapy for Interstitial Lung Disease Associated with Sjögren's Syndrome With Double-positive Anti-SS-A and Anti-centromere Antibodies.

Sjögren's syndrome (SS) can present with extraglandular organs, such as interstitial lung disease (ILD). Anti-SS-A antibody is frequently found in SS cases, whereas anti-centromere antibody (ACA) is detected in some SS cases. Notably, the anti-SS-A and ACA double-positive cases exhibited distinct features with a higher prevalence of ILD. However, there have so far been no reports on the treatment of ILD in anti-SS-A and ACA double-positive cases. We herein present a case of ILD with anti-SS-A and ACA double-positive SS that was successfully treated with immunosuppressive therapy. Our case suggests the potential efficacy of immunosuppressive therapy for this poorly understood condition.

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.

The Role of Nailfold Capillaroscopy in Evaluating Patients with Interstitial Lung Disease Related to Connective Tissue Disease.

Capillaroscopy is a non-invasive and safe imaging method that allows the evaluation of the microcirculation of the small vessels of the skin. The method's main advantage is the early detection of microvascular changes that may occur in certain connective tissue diseases (CTDs). Today, the presence of specific autoantibodies and capillaroscopic findings are generally accepted and emerge as a powerful diagnostic tool for detecting underlying CTDs in patients with Raynaud's phenomenon. The role of capillaroscopy has also been investigated in patients with CTD and interstitial lung disease (ILD). In these patients, lung involvement is considered one of the most severe complications, potentially leading to significant morbidity and mortality. So far, studies have shown an association of the scleroderma pattern in capillaroscopy with lung involvement in Scleroderma patients. Although there are studies on the association of capillary findings in patients with other CTDs, further efforts are needed to evaluate this technique and produce high-performance algorithms in the early detection of involvement and the progression of (CTD) related ILD (CTD-ILD). The present study aims to perform capillaroscopy in CTDILD patients with different imaging patterns and to correlate the method's findings with those found in high-resolution computed tomography, pulmonary tests, and the immunological profile of patients. Furthermore, the impact of ILD treatment on the capillaroscopic findings will be evaluated.