Interstitial Lung Disease Clinic Research Articles

Older Patients With Interstitial Lung Disease Feature a Distinct Clinical Profile

IntroductionThere are few studies investigating the clinical profile of older patients with interstitial lung disease (ILD), so this study investigated the characteristics of the older population diagnosed with ILD. Material and methodsRetrospective study in a population of new referrals at an ILD clinic from January 2013 to September 2017. Patients over 64 years were selected. Data collection included diseases variables, diagnostic procedures and comorbidities. Gender-age-physiology (GAP) stage, composite physiologic index (CPI) and Charlson index was calculated. Statistical analysis was performed to investigate risk factors associated with survival. ResultsA total of 232 patients were included in this study. Mean age was 76.3 years (SD 6.5). As per protocol, 69.3% completed the initial assessment but this was lower in the elderly group (61.5%). The most frequent diagnosis was unclassifiable ILD (24.1%), followed by ILD associated with connective tissue disease (21.6%), IPF (12.1%) and hypersensitivity pneumonitis (10.3%). During follow-up (36.7 months (SD 28.6)) a significant proportion of patients died (55 cases, 23.7% of the cohort), especially in the late older group (30.4%). Kaplan–Meier curves showed that those over 75 years have a worse survival even when adjusted by covariables (p<0.001). CPI was the only score with statistical significance in a multivariate analysis (HR 1.06. p 0.006). ConclusionsOlder adults with ILD featured a distinct clinical profile. Our findings highlight the need to develop non-invasive biomarkers and specific scores adapted to this age-group.

Real-World Clinical Profile and Safety of Nintedanib in Systemic Sclerosis-Associated Interstitial Lung Disease: A Subgroup Analysis of Interstitial Lung Disease Data From an Interstitial Lung Disease (ILD) Specialty Clinic in India.

Systemic sclerosis (SSc) is a multisystem autoimmune disorder characterized by dysregulated innate and adaptive immunity. Interstitial lung disease (ILD) is a common and serious complication of SSc, often leading to significant morbidity and mortality. Consistent demographic characteristics that aid in the early diagnosis of ILD in SSc are lacking. This study aims to identify clinical and demographic parameters associated with ILD in SSc patients and assess the safety and tolerability of nintedanibwith other immunosuppressants. This study is a subgroup analysis of data from the ILD clinic at All India Institute of Medical Sciences Raipur, collected between January 2022 and January 2024. We assessed the clinical and demographic profiles, high-resolution computed tomography thorax patterns, autoantibody profiles, lung function, and treatments used in the patients. We enrolled 57 patients with SSc-associated ILD. The mean age of the participants was 39.0 ± 11.1 years, with 53 (92.9%) being women. The mean body mass index was 20.4 ± 4.32 kg/m². Dyspnea was the most common symptom, followed by skin tightening and cough. Antinuclear antibody tests were positive in 92.9% of patients, and anti-Scl-70 antibodies were positive in 57.9%. Rheumatoid arthritis-SSc overlap was observed in 15.8% of patients. The mean predicted forced vital capacity was 46.5 ± 19.9%, the mean predicted total lung capacity was 64.5 ± 20.4%, and the mean predicted diffusing capacity for carbon monoxide was 46.2 ± 15.7%. The mean six-minute walk distance was 360.3 ± 81.2 meters, and the mean King's Brief Interstitial Lung Disease score was 63.9 ± 10.7. Common radiological abnormalities included ground-glass opacities in 57.8%, traction bronchiectasis in 43.8%, and honeycombing in 28.07%. The predominant ILD pattern was nonspecific interstitial pneumonia. Patients received a combination of prednisolone (5 mg/day) with mycophenolate mofetil (63.2%), hydroxychloroquine (17.5%), cyclophosphamide (12.3%), and methotrexate (7.02%). Nintedanib, the only antifibrotic used, was administered to 17 (29.8%) patients. ILD is relatively common in SSc, particularly in patients with diffuse cutaneous SSc and those with anti-topoisomerase antibodies. Female patients comprised the predominant population in this study. Patients tolerated mycophenolate mofetil and cyclophosphamide well. Nintedanib was the only antifibrotic used, and all patients tolerated the combination of antifibrotics and immunosuppressants well. Early diagnosis is crucial to slow disease progression and preserve lung function. Our results highlight the need for vigilant screening in high-risk groups and suggest that MMF, cyclophosphamide, and nintedanib can be safely incorporated into treatment regimens, offering a potential strategy to improve patient outcomes.

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.

Antifibrotics in the Management of Rheumatoid Arthritis-Associated Interstitial Lung Disease: Prospective Real-World Experience From an Interstitial Lung Disease Clinic in India.

Interstitial lung disease (ILD) in rheumatoid arthritis (RA) is a serious complication with varied prevalence ranging from 4% to as high as 68%, with varied presentation. Immunosuppressants and antifibrotics are used in the management of RA ILD. The clinicodemographic profile and presentation in our country need to be further explored. We assessed the efficacy and safety profile of antifibrotic drugs in combination with immunosuppressants among RA ILD patients. A prospective observational study was conducted in the Interstitial Lung Disease (ILD) Clinic in the Department of Pulmonary Medicine, All India Institute of Medical Sciences Raipur, India, between January 2022 to January 2023. RA patients with dyspnea and chronic cough were referred to us for evaluation of ILD. Patients underwent clinical examination, complete lung function study including spirometry, single breath diffusion capacity for carbon monoxide (DLCO), six-minute walk test, and high-resolution computed tomography of the thorax. Quality of life was assessed using the King's Brief Interstitial Lung Disease (KBILD) questionnaire. Two hundred eighteen RA patients were evaluated and out of these, 43 (20.8%) had features of ILD on high-resolution computed tomogram (HRCT) thorax. Twenty-six (2.18%) met the inclusion criteria for starting antifibrotics. The mean ± SD. age of the patients was 52.96 ± 14.04 and the majority (77%) were females. Fourteen (53.38%) patients had usual interstitial pneumonia (UIP)/probable UIP pattern and 12 (46.22%) had nonspecific interstitial pneumonia (NSIP) patterns on HRCT. Out of 26 patients, 24 (92.3%) were started on antifibrotics. Fourteen (53.8%) patients were on nintedanib and 10 (38.4%) were on pirfenidone. The mean ± SD forced vital capacity (FVC)% predictedwas 62.5 ± 20.04.The mean ± SD. The DLCO percentage predicted was 54.4 ± 22.8. Twenty-two (84.6%) patients did not experience any side effects. The mean ± SD. KBILD score was 59.9 ± 11.17 and was similar in both sexes. In our study, the prevalence of RA ILD was nearly 20.8% and more common in females. Twenty-four (2%) patients were included for antifibrotic treatment. There was an improvement in lung function at the end of six months, but the change was not significant. All patients tolerated antifibrotics well without any serious adverse events.

Impact of a multidisciplinary interstitial lung disease clinic on healthcare utilization

RATIONALEA minimal amount is known of the impact that a multidisciplinary interstitial lung disease (ILD) clinic with dedicated nursing support may have on healthcare utilization. OBJECTIVES The objective of this study is to determine if there is a reduction in healthcare utilization including emergency room (ER) visits, hospitalizations, and hospitalization costs in the year after a patient is assessed in the ILD clinic. METHODS This retrospective study evaluated the number of ER visits and hospitalizations 1 year before and after being seen in an ILD clinic. For those with hospitalizations, length of stay (LOS) and cost of each stay were collected. Pre- and post-ILD clinic outcomes were compared using Mann-Whitney and Wilcoxon rank sum test. MEASUREMENTS AND MAIN RESULTSA total of 202 patients were screened and 140 included in the analysis. Mean age was 66 years (±12), and 47% were female. Mean forced vital capacity percentage predicted was 77% (±22). There was no significant difference in the pre- and post-ILD clinic mean number of ER visits per patient (p = 0.33) nor hospitalizations (p = 0.91). LOS was shorter in the post-ILD clinic period (10.8 ± 10.5 days) versus pre-ILD clinic visit (18.8 ± 25.4 days), although not statistically significant (p = 0.30). Pre-ILD clinic visit mean hospitalization cost was $24,881.89 (±35,817.48) and post-ILD clinic visit was $16,751.68 (±16,549.74) although not statistically different (p = 0.66). CONCLUSIONS We found no statistically significant difference in ER visits, hospitalizations or hospitalization costs post-ILD multidisciplinary clinic assessment in our ILD cohort. However, there was a trend toward lower overall healthcare utilization and cost in the year post-ILD clinic.

Predictive factors for dose reduction and discontinuation of nintedanib in fibrotic interstitial lung diseases: Real life data

Nintedanib, an intracellular inhibitor targeting multiple tyrosine kinases, has emerged as a standard treatment for various fibrotic lung diseases. Despite its efficacy, side effects such as nausea, diarrhea, and hepatotoxicity often lead to dose reduction or discontinuation. In this retrospective analysis at an university hospital's interstitial lung disease clinic, we aimed to identify baseline characteristics associated with dose adjustment or treatment discontinuation. Of the 58 patients included, 41.4% maintained the full nintedanib dose, while 31.0% required dosage reduction, and 27.6% discontinued treatment due to adverse events, predominantly gastrointestinal and hepatotoxic effects. Multivariate analysis revealed body surface area (BSA) as an independent and significant baseline risk factor (adjusted Odds Ratio [aOR] 0.22), suggesting a 78% decreased chance of requiring dose modification for every decimal point increase in BSA. A BSA cutoff of ≤1.73 m [2] exhibited a sensitivity of 73% and specificity of 91.7%, with significant impact on one-year survival under full-dose treatment (p < 0.001). Lower BSA was associated with early onset adverse effects, particularly gastrointestinal, supporting the need for regular clinical monitoring. The study emphasizes the importance of recognizing baseline factors to ensure the safety and tolerability of nintedanib, thereby preventing the progression of pulmonary fibrosis. These findings contribute to the evolving understanding of nintedanib management in fibrotic interstitial lung diseases, guiding clinicians in personalized treatment approaches.

The Clinical Frailty Scale for Risk Stratification in Patients With Fibrotic Interstitial Lung Disease

Previous studies have shown the importance of frailty in patients with fibrotic interstitial lung disease (ILD). Is the Clinical Frailty Scale (CFS) a valid tool to improve risk stratification in patients with fibrotic ILD? Patients with fibrotic ILD were included from the prospective multicenter Canadian Registry for Pulmonary Fibrosis. The CFS was assessed using available information from initial ILD clinic visits. Patients were stratified into fit (CFS score 1-3), vulnerable (CFS score 4), and frail (CFS score 5-9) subgroups. Cox proportional hazards and logistic regression models with mixed effects were used to estimate time to death or lung transplantation. A derivation and validation cohort was used to establish prognostic performance. Trajectories of functional tests were compared using joint models. Of the 1,587 patients with fibrotic ILD, 858 (54%) were fit, 400 (25%) were vulnerable, and 329 (21%) were frail. Frailty was a risk factor for early mortality (hazard ratio, 5.58; 95%CI, 3.64-5.76, P< .001) in the entire cohort, in individual ILD diagnoses, and after adjustment for potential confounders. Adding frailty to established risk prediction parameters improved the prognostic performance in derivation and validation cohorts. Patients in the frail subgroup had larger annual declines in FVC %predicted than patients in the fit subgroup (-2.32; 95%CI,-3.39 to-1.17 vs-1.55; 95%CI,-2.04 to-1.15, respectively; P= .02). The simple and practical CFS is associated with pulmonary and physical function decline in patients with fibrotic ILD and provides additional prognostic accuracy in clinical practice.

Risk factors associated with COVID-19 pneumonia in Chinese patients with pre-existing interstitial lung disease during the SARS-CoV-2 pandemic.

In China, the emergence of a nationally widespread epidemic infection of severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) has appeared within a month since December 7, 2022. To evaluate the risk factors for suffering from coronavirus disease 2019 (COVID-19) pneumonia due to infection with SARS-CoV-2 in different kinds of interstitial lung disease (ILD) patients with diverse immunizations, we conducted this retrospective study on 525 patients with ILDs who underwent regular follow-up in our ILD clinic. Among them, 128 ILD patients (24.4%) suffered from COVID-19 pneumonia after SARS-CoV-2 infection. Patients were older with a male predominance in the pneumonia group than in the nonpneumonia group (65.0 ± 10.0 yearsvs. 56.4 ± 11.7 years, p < 0.001, 55.5% vs. 39.5%, p = 0.002, respectively). Connective tissue disease-associated ILD(CTD-ILD) (25%), idiopathic pulmonary fibrosis(23.4%), and interstitial pneumonia with autoimmune features(21.1%) were the main pre-existing ILDs in the pneumonia group. In Cox multivariable analysis, only male sex and corticosteroid use were risk factors for COVID-19 pneumonia after infection. Two or threedoses of vaccination were a protective factor for pre-existing ILD patients suffering from COVID-19 pneumonia. More than twodoses of vaccination were strongly recommended for pre-existing ILD patients, particularly for males who were administered corticosteroids.

Patient and Physician Assessments of Clinical Status: A Mixed-methods Study of Interstitial Lung Disease

The SARS-CoV-2 pandemic necessitated novel health care delivery for patients with interstitial lung disease (ILD), including reduced in-person appointments and physiologic testing to minimize transmission. Clinicians often have been required to rely on patients' subjective assessments of their clinical status during phone follow-up appointments. It is unknown how accurate a patient's self-assessment is compared with that of their physician during an in-person evaluation. Are patients' self-assessments of their clinical status in agreement with their physicians' assessments, and are telemedicine vsin-person visits acceptable? Patients were enrolled prospectively from the University of Calgary ILD clinic. Participants were asked by phone before the in-person appointment and after the appointment to rate their clinical status on a five-point Likert scale. Physicians then rated the patient's clinical status after the appointment on a similar five-point Likert scale, masked to patient responses. Patients and physicians were asked if an in-person appointment was necessary or if telemedicine would have sufficed. Clinical variables associated with physician assessments were assessed. Fifty patients with mean age of 67 ± 11.8 years participated. Mean time since last follow-up was 5.0 ± 3.0months. No correlation was found between the preclinical patient self-assessment and postclinical physician assessment (P= .18; κ= 0.28). Correlation of postclinical assessment was statistically significant (P< .001), with moderate agreement (κ= 0.49). Physicians thought telephone visits were acceptable for 58%of appointments, whereas only 12%of patients preferred telephone visits. Physician's assessment of clinical status seemed to be driven by change in diffusion capacity of the lungs for carbon monoxide (P= .039). Telemedicine may improve access to care for patients during pandemic management, in rural communities, and for those with impaired mobility. Despite these benefits, our data support that patients and physicians may not agree on determination of clinical status and that patients generally prefer in-person patient-physician interactions.

Low Rates of Reproductive Counseling Documentation in Women With Interstitial Pneumonia With Autoimmune Features.

Women with interstitial pneumonia with autoimmune features (IPAFs), a subset of interstitial lung disease (ILD), are at risk for pregnancy complications. Family planning discussions improve pregnancy outcomes in women with ILD. The objective of this study was to evaluate the documentation of reproductive counseling in IPAF female patients of childbearing age by pulmonary and rheumatology providers at an academic medical center. We conducted a medical record review study of pulmonary and rheumatology encounters in reproductive-aged women with IPAF to evaluate documentation of family planning discussions and contraceptive use. We used nonparametric measures of association and logistic regression to evaluate the relationship between patient characteristics and the presence of reproductive counseling documentation by providers. Thirty-one women met IPAF classification and were ≤50 years at initial ILD clinic visit. Twenty-five (81%) of these women had risk factors for adverse pregnancy outcomes. Ten women (32%) had a record of reproductive counseling during any visit with their pulmonary provider. Of the 21 patients who also saw a rheumatology provider, 12 (57%) women had a record of reproductive counseling during any visit with their rheumatology provider. No baseline characteristics were associated with odds of reproductive counseling documentation. Neither pulmonary nor rheumatology providers consistently discussed family planning/contraceptive use with reproductive-aged women with IPAF. There was a trend for rheumatology providers to discuss reproductive issues with IPAF patients more frequently than pulmonary providers. Efforts should focus on educating providers about the need for reproductive counseling in women with IPAF of childbearing age.

A review of the challenges, learnings and future directions of home handheld spirometry in interstitial lung disease

BackgroundPatients with interstitial lung disease (ILD) require regular physician visits and referral to specialist ILD clinics. Difficulties or delays in accessing care can limit opportunities to monitor disease trajectory and response to treatment, and the COVID-19 pandemic has added to these challenges. Therefore, home monitoring technologies, such as home handheld spirometry, have gained increased attention as they may help to improve access to care for patients with ILD. However, while several studies have shown that home handheld spirometry in ILD is acceptable for most patients, data from clinical trials are not sufficiently robust to support its use as a primary endpoint. This review discusses the challenges that were encountered with handheld spirometry across three recent ILD studies, which included home spirometry as a primary endpoint, and highlights where further optimisation and research into home handheld spirometry in ILD is required. bodyRate of decline in forced vital capacity (FVC) as measured by daily home handheld spirometry versus site spirometry was of primary interest in three recently completed studies: STARLINER (NCT03261037), STARMAP and a Phase II study of pirfenidone in progressive fibrosing unclassifiable ILD (NCT03099187). Unanticipated practical and technical issues led to problems with estimating FVC decline. In all three studies, cross-sectional correlations for home handheld versus site spirometry were strong/moderate at baseline and later timepoints, but longitudinal correlations were weak. Other issues observed with the home handheld spirometry data included: high within-patient variability in home handheld FVC measurements; implausible longitudinal patterns in the home handheld spirometry data that were not reflected in site spirometry; and extreme estimated rates of FVC change.ConclusionsHome handheld spirometry in ILD requires further optimisation and research to ensure accurate and reliable FVC measurements before it can be used as an endpoint in clinical trials. Refresher training, automated alerts of problems and FVC changes, and patient support could help to overcome some practical issues. Despite the challenges, there is value in incorporating home handheld spirometry into clinical practice, and the COVID-19 pandemic has highlighted the potential for home monitoring technologies to help improve access to care for patients with ILD.

ADVANCED-CARE PLANNING IN PULMONARY HYPERTENSION AND INTERSTITIAL LUNG DISEASE: NEEDS ASSESSMENT AND DEVELOPMENT OF A QUALITY IMPROVEMENT PROCESS

ADVANCED-CARE PLANNING IN PULMONARY HYPERTENSION AND INTERSTITIAL LUNG DISEASE: NEEDS ASSESSMENT AND DEVELOPMENT OF A QUALITY IMPROVEMENT PROCESS

Post COVID-19 Chronic Parenchymal Lung Changes.

Introduction: Persistent parenchymal lung changes are an important long-term sequela of COVID-19. There are limited data on this COVID-19 infection sequela characteristics and trajectories. This study aims to evaluate persistent COVID-19-related parenchymal lung changes 10 weeks after acute viral pneumonia and to identify associated risk factors.Methods: This is a retrospective case-control observational study involving 38 COVID-19 confirmed cases using nasopharyngeal swab reverse transcriptase-polymerase chain reaction (RT-PCR) at King Abdullah Medical City (KAMC) Hospital, Makkah. Patients were recruited from the post-COVID-19 interstitial lung disease (ILD) clinic. Referral to this clinic was based on the pulmonology consultant’s assessment of hospitalized patients suspected of developing COVID-19-related ILD changes during hospitalization.Results: Thirty-eight patients with parenchymal lung changes were evaluated at the ILD clinic. Nineteen patients who had persistent parenchymal changes 10 weeks after the acute illness (group 1) were compared with 19 control patients who had accelerated clinical and/or radiological improvement (group 2). Group 1 was found to have the more severe clinical and radiological disease, with a higher peak value of inflammatory biomarkers. Two risk factors were identified, neutrophil-lymphocyte ratio (NLR) > 3.13 at admission increases the odds ratio (OR) of chronic parenchymal changes by 6.42 and 5.92 in the univariate and multivariate analyses, respectively. Invasive mechanical ventilation had a more profound effect with ORs of 13.09 and 44.5 in the univariate and multivariate analyses, respectively.Conclusion: Herein, we found that only receiving invasive mechanical ventilation and having NLR >3.13 at admission were strong risk factors for persistent parenchymal lung changes. Neither the clinical severity of the acute illness nor the radiological one is found to predict this outcome. None of the medications received during the acute illness were found to alter the risk for this post-COVID-19 infection sequelae.

Provider Perspectives on and Access to Palliative Care for Patients With Interstitial Lung Disease

Interstitial lung disease (ILD) results in profound symptom burden and carries high mortality. Palliative care (PC) is dedicated to improving quality of life in patients with serious illness. Early PC provision improves rates of advance care planning and symptom management in patients with ILD. What are the current perspectives on PC among ILD providers, and what are the barriers to PC in ILD specialty centers? A 24-question electronic survey was disseminated to providers at the 68 Pulmonary Fibrosis Foundation Care Centers across the United States from October 2020 to December2020. The survey was completed by 128 participants representing all 68 Pulmonary Fibrosis Foundation Care Center Network sites. Most respondents were physicians. Most providers exhibit good knowledge of, feel comfortable assessing a patient's readiness for, and agree with the need for PC for patients with ILD. Providers are most likely to refer to PC at objective disease and/or symptomatic progression rather than at initial diagnosis. In comparison with providers who report referring their patients to PC, providers who report rare referral are more likely to cite lack local PC availability (P< .01) and less likely to feel comfortable discussing prognosis/disease trajectory (P= .03) or feel it is important to address advance directives in ILD clinic (P= .02). There is a lack of standardized measures used to assess specific symptoms, overall symptom burden, or health-related quality of life across institutions. Discordance exists between self-reported and actual access to local inpatient and outpatient PC services. Most ILD providers use PC and are comfortable discussing PC. Barriers to PC identified in this survey include the following: perceived lack of local access to PC, lack of systematic tools to assess symptom burden, lack of established optimal timing of PC referral, and unclear need for specialized PC delivery.

DEDICATED INTERSTITIAL LUNG DISEASE CLINIC IMPROVES CARE FOR VETERANS WITH IDIOPATHIC PULMONARY FIBROSIS

TOPIC: Diffuse Lung Disease TYPE: Original Investigations PURPOSE: Idiopathic Pulmonary Fibrosis (IPF) is an Interstitial Lung Disease (ILD) with significant morbidity and mortality. Guidelines recommend frequent monitoring for disease progression, co-morbidities, and early referral to pulmonary rehabilitation and palliative care1. Previous studies have shown improved survival in patients with ILD cared for in dedicated ILD clinics in tertiary care centers2. In May 2019, a dedicated weekly ILD clinic was established at the Hunter Holmes McGuire Veterans Affairs Medical Center. We performed a retrospective chart review to assess whether the ILD clinic improved adherence to guideline-based metrics in veterans with IPF. METHODS: A list of all patients on antifibrotic therapy, reflecting most individuals with IPF at our medical center, was obtained from the pharmacy. We then performed a chart-based retrospective review comparing the frequency of guideline-recommended monitoring and discussions performed for patients seen in ILD clinic compared to the general pulmonary clinic. The ILD clinic consisted of a dedicated Pulmonologist and Respiratory Therapist. Each new patient seen in the clinic completed an extensive medical, exposure and family history. A 6-minute walk test (6MWT) and bedside spirometry was attempted every patient visit. Charts from May 1, 2019 to March 31, 2020 were reviewed, reflecting the period from when ILD clinic was initiated until usual clinic operations were halted due to the COVID-19 pandemic. RESULTS: A total of 20 IPF patients were identified; 7 were followed in the ILD clinic while the remaining 13 were seen in general pulmonary clinic. Individuals followed in the ILD clinic had closer monitoring of end points used to monitor for disease progression, such as spirometry and 6MWT, with both occurring every 6 months in 85.7% of patients. In the general pulmonary clinic, 46% had PFTs every 6 months while none had 6MWT every 6 months. Of the IPF patients seen in the ILD clinic, 100% underwent screening for sleep apnea, gastroesophageal reflux disease and hypoxia, while 46.2%, 84.6% and 76.9% respectively were screened in general pulmonary clinic. ILD clinic patients also had discussions regarding pulmonary rehab and goals of care, 100% and 42.9% of the time compared to 23% and 38.5% in those seen in general pulmonary clinic. CONCLUSIONS: ILD clinics, regardless of their association with a tertiary care center, can lead to improved monitoring of disease progression and co-morbidities in patients with IPF. Such dedicated care can also lead to more referrals to pulmonary rehab and palliative care. The key limitations to this study are the sample size and the retrospective design. In addition, we may not have captured patients with IPF who were not considered to be candidates for anti-fibrotic therapy. CLINICAL IMPLICATIONS: With minimal associated cost, dedicated ILD clinics, even those located in small healthcare systems, can improve care provided to patient’s with IPF.1) Idiopathic pulmonary fibrosis: diagnosis and treatment. International Consensus Statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 2000; 161: 646–6642)Povitz M, Li L, Hosein K, Shariff S, Mura M. Implementing an interstitial lung disease clinic improves survival without increasing health care resource utilization. Pulm Pharmacol Ther. 2019 Jun;56:94-99. doi: 10.1016/j.pupt.2019.03.012. Epub 2019 Mar 29. PMID: 30930173. DISCLOSURES: No relevant relationships by Angelique Gross, source=Web Response No relevant relationships by Michael L'Heureux, source=Web Response No relevant relationships by Angela Love, source=Web Response No relevant relationships by Alexandru Spulber, source=Web Response

THE FAULT IN OUR MOSAIC PATTERNS: PULMONARY HYPERTENSION MISSED AND TREATED AS HYPERSENSITIVITY PNEUMONITIS

TOPIC: Pulmonary Vascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Mosaic Pattern refers to areas of variable lung attenuation seen on Computed Tomography (CT) of the chest. The pattern is a nonspecific finding and can be associated with airway, infiltrative lung, or pulmonary vascular diseases[1]. We present a case of exertional dyspnea with mosaic pattern on high resolution CT (HRCT) that was initially misdiagnosed and treated as hypersensitivity pneumonitis (HP) and found later to be caused by pulmonary arterial hypertension. CASE PRESENTATION: A 70-year-old Caucasian female presented with worsening exertional dyspnea after percutaneous coronary intervention. HRCT of the chest done at an outside office showed mosaic pattern and few tiny bilateral nodules with no fibrotic changes that was interpreted as possible chronic hypersensitivity pneumonitis. She was started on prednisone 40mg daily with minimal improvement in symptoms and referred to our Interstitial Lung Disease (ILD) Clinic for further work up and treatment. Upon interviewing the patient, she reported previous intake of anorexigenics (dextroamphetamine) in her 20's for 2 months. Review of the HRCT study showed parenchymal changes were not consistent with any specific ILD pattern and no exaggeration of mosaic pattern with expiratory cuts suggesting the presence of pulmonary hypertension and absence of significant air trapping. Prednisone was tapered off, and serologic work up showed no evidence of connective tissue disease. 6-minute walk test revealed severe reduction in exercise capacity with patient covering 38m which was 12% of predicted distance, and spirometry showed moderate-severe restrictive defect. Her echocardiography showed normal LV systolic function with ejection fraction (55-65%), and her ventilation-perfusion (VQ) scan was negative for any embolic disease. The patient was admitted to the hospital for hypervolemia and after adequate diuresis, a detailed hemodynamic study by right heart catheterization for the assessment of pulmonary hypertension was done. Results showed increased mean pulmonary artery pressure (mPAP) (31 mmHg) at rest; however, during limited exercise challenge which she could not complete, pulmonary artery occlusion pressure (PAOP) increased to 23 mmHg suggesting decreased left atrial/ventricular compliance. She was started on sildenafil and discharged with close follow up. DISCUSSION: Air-trapping in airway diseases causing relative hyperemia of healthy lungs, pulmonary vascular diseases causing relative oligemia in hypo-perfused lungs, and parenchymal disease presenting as patchy ground-glass opacities, all can give a mosaic pattern on CT[1]. Infiltrative diseases and airway diseases are reliably differentiated as the cause of mosaic attenuation on CT scan, but vascular diseases are often misinterpreted.[2] CONCLUSIONS: Careful review of HRCT with a chest radiologist in an advanced lung disease center is necessary to avoid misdiagnoses and unnecessary treatments. REFERENCE #1: Stern, Eric J., et al. "CT mosaic pattern of lung attenuation: distinguishing different causes.” AJR. American journal of roentgenology 165.4 (1995) REFERENCE #2: Worthy, Sylvia A., et al. "Mosaic attenuation pattern on thin-section CT scans of the lung: differentiation among infiltrative lung, airway, and vascular diseases as a cause.” Radiology205.2 (1997) DISCLOSURES: No relevant relationships by Roberto Bernardo, source=Web Response No relevant relationships by Salim Daouk, source=Web Response No relevant relationships by Ahmad Hassan, source=Web Response No relevant relationships by Abed Madanieh, source=Web Response No relevant relationships by Chirin Orabi, source=Web Response

Diagnosis of myositis-associated interstitial lung disease: Utility of the myositis autoantibody line immunoassay

ObjectivesThe detection of myositis autoantibodies (MA) in patients with interstitial lung disease (ILD) has major implications for diagnosis and management, especially amyopathic and forme frustes of idiopathic inflammatory myositis-associated ILD (IIM-ILD). Use of the MA line immunoblot assay (MA-LIA) in non-rheumatological cohorts remains unvalidated. We assessed the diagnostic performance of the MA-LIA and explored combined models with clinical variables to improve identification of patients with IIM-ILD. MethodsConsecutive patients referred to a specialist ILD clinic, with ILD-diagnosis confirmed at multidisciplinary meeting, and MA-LIA performed within six months of baseline were included. Pre-specified MA-LIA thresholds were evaluated for IIM-ILD diagnosis. ResultsA total 247 ILD patients were included (IIM-ILD n = 12, non-IIM connective tissue disease-associated ILD [CTD-ILD] n = 52, idiopathic interstitial pneumonia [IIP] n = 115, other-ILD n = 68). Mean age was 64.8 years, with 45.3% female, mean FVC 75.5% and DLCO 59.2% predicted. MA were present in 13.8% overall and 83.3% of IIM-ILD patients. The most common MA in IIM-ILD and non-IIM ILD patients were anti-Jo-1 (prevalence 40%) and anti-PMScl (29.2%) autoantibodies respectively. The pre-specified low-positive threshold (>10 signal intensity) had the highest discriminative capacity for IIM-ILD (AUC 0.86). Combining MA-LIA with age, gender, clinical CTD-manifestations and an overlap non-specific interstitial pneumonia/organising pneumonia pattern on HRCT improved discrimination for IIM-ILD (AUC 0.96). ConclusionThe MA-LIA is useful to support a diagnosis of IIM-ILD as a complement to multi-disciplinary ILD assessment. Clinical interpretation is optimised by consideration of the strength of the MA-LIA result together with clinical and radiological features of IIM-ILD.

Fine crackles on chest auscultation in the early diagnosis of idiopathic pulmonary fibrosis: a prospective cohort study

IntroductionIdiopathic pulmonary fibrosis (IPF) is an interstitial lung disease (ILD) with a poor prognosis. Early diagnosis and treatment of IPF may increase lifespan and preserve quality of life. Chest CT...

Understanding cough in interstitial lung disease: a cross-sectional study on the adequacy of treatment.

Cough is a common symptom in interstitial lung disease (ILD), often leading to treatment dissatisfaction for patients and physicians. To identify the prevalence and subjective adequacy of control of cough in patients with ILD. A cross-sectional study of patients with ILD attending a tertiary ILD clinic in Perth was undertaken using a pre-designed questionnaire that patients were invited to complete when attending clinic. Cough severity and impact on quality of life were assessed using a visual analogue scale and the validated Leicester cough questionnaire. Participants were asked to list triggers of their cough and strategies or medications trialled to control cough. Of 164 respondents, 118 (72%) had cough, with prevalence common in all ILD subtypes. A lower forced vital capacity (FVC) was found in the cough group versus non-cough group (74.6 ± 18.7 vs 87.0 ± 15.9, P-value < 0.0001). Common reported triggers were lung irritants, exertion and doing routine daily activities. Avoidance of triggers was a common strategy to control cough. A high prevalence of non-ILD causes of cough was recorded in both groups. A variety of medications had been trialled, including anti-fibrotics, immunosuppression drugs, inhalers and proton pump inhibitors, with moderate benefit reported by 18% of participants. Cough is prevalent in ILD but is not adequately suppressed. Cough has a significant impact on quality of life, leading patients to adopt their own strategies to control their cough. More research is needed to understand cough mechanisms in ILD and the interplay of other potential co-pathologies.

Travel Distance to Subspecialty Clinic and Outcomes in Patients with Fibrotic Interstitial Lung Disease.

Rationale: Early access to subspecialty care is associated with improved outcomes for patients with fibrotic interstitial lung disease (ILD). Access to ILD care may be limited for patients living far from subspecialty clinics. Objectives: To test the hypothesis that greater travel distance to access ILD clinical care would be associated with more severe disease at time of referral and worse clinical outcomes. Methods: Patients with fibrotic ILD were recruited from a multicenter national pulmonary fibrosis registry. Residential postal codes were geocoded to estimate travel distance from the home to the clinic. Travel distance was dichotomized at ⩽70 km (near) and >70 km (far). Demographics and disease severity at the initial referral, changes in lung function, and the risk of death or lung transplant were analyzed in unadjusted and adjusted models for their association with travel distance. Results: The cohort included 1,162 patients, of whom 856 lived near to their ILD clinic and 306 lived far from their ILD clinic. Patients residing farther from their clinic were younger, more likely to have smoked, had a greater 6-minute-walk distance, and had lower composite risk scores than patients residing closer to their clinic. In models adjusted for age, sex, and baseline forced vital capacity, patients from farther away had a greater risk of death or lung transplant than patients residing closer (hazard ratio, 1.52; 95% confidence interval [CI], 1.10-2.11), a finding predominantly driven by patients with connective tissue disease-related ILD (hazard ratio, 2.14; 95% CI, 1.16-3.94). Conclusions: Patients with fibrotic ILD with a longer travel distance to their ILD clinic had better prognostic indices at baseline but had a higher risk of death or lung transplant in the total cohort and in patients with connective tissue disease-related ILD. Assuming that disease epidemiology and severity are distributed evenly across geographic regions, these findings raise important questions about equitable access to patient care in large healthcare regions with centralized subspecialty programs.