Management Of Interstitial Lung Disease Research Articles

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.

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.

Abstract P1-11-05: Provider perceptions of DESTINY-Breast04, HER2-low directed treatment, and interstitial lung disease

Abstract Background: Human epidermal growth factor receptor 2 (HER2)-targeted therapies are an established treatment for patients with HER2-positive breast cancer, however, these therapies have not proven effective in the HER2-negative setting. Until recently, HER2 status was used to guide treatment decisions based on a binary classification of positive or negative. A new pathological category, HER2-low, has emerged as a subtype of interest within the breast cancer treatment landscape. HER2-low status is defined as a HER2 immunohistochemistry score of 1+ or 2+ and a negative in-situ hybridization result. DESTINY-Breast04 (DB04) was the first trial to evaluate a HER2-targeted agent within the metastatic HER2-low breast cancer setting. The anti-HER2 agent trastuzumab deruxtecan (T-DXd) demonstrated promising clinical activity in HER2-low expressing tumors. However, development of T-DXd-related interstitial lung disease (ILD) remains a concern when using this therapy. This survey-based study aimed to evaluate community oncologists’ perceptions of the DB04 data, HER2-low directed treatment, and management of ILD. Methods: U.S.-based oncologists (n=83) convened at two live meetings in June 2022 to review clinical updates presented at ASCO 2022. Participant characteristics and demographic data were collected via an online survey prior to the respective meetings. Perceptions/reactions to clinical updates were captured in real-time via electronic keypad. Data were summarized using descriptive statistics. Results: Among respondents, 83.1% identified as community providers, with an average experience of 20.7 years in practice. On average, participants reported that 88.2% of their time is allocated towards direct patient care, with roughly 18 patients seen per clinic day. Nearly half of respondents (49.4%) reported awareness of HER2-low as a distinct pathological category prior to the presentation of DB04 at ASCO 2022, however, less than 10% of respondents had previously used this sub-category to determine therapy. Increased T-DXd-related ILD, which occurred in 12% of trial participants, was cited as the greatest limitation of the DB04 trial by over one-third (37.3%) of respondents. After reviewing real-world evidence data of ILD incidence in metastatic breast cancer, nearly one-third (31%) of respondents reported that their observed ILD rates are less than DB04, but more (36%) said that ILD can be hard to quantify because patients are not always symptomatic. When asked if the ILD rate associated with T-DXd would limit their selection of this agent for their patients with breast cancer, approximately one-quarter (24.1%) of respondents indicated that they would reserve T-DXd use for patients without symptomatic pulmonary disease. However, the majority of respondents (60.2%) indicated that they would not limit their use of T-DXd based on ILD rates, with most (55.4%) opting for a risk-management approach involving increased monitoring for the development of ILD-related adverse events. Conclusions: Advancements in assay interpretation have made it possible to differentiate gradients of HER2 expression, creating a space for pathological sub-categories within a formerly binary paradigm. Among providers who reported awareness of HER2-low as a distinct pathological sub-category, few had used this as a benchmark to guide their treatment decisions prior to the presentation of DB04 at ASCO 2022. Newer anti-HER2 agents, such as T-DXd, provide a potential new standard of care for patients with HER2-low expressing tumors. Despite the concern of ILD rates associated with T-DXd use, the majority of providers do not view this as a limiting factor due to the ability to closely monitor patients for the development of adverse events coupled with appropriate provider/patient education. Citation Format: Brooke Leon, Robert Bone, Yolaine Jeune-Smith, Bruce Feinberg. Provider perceptions of DESTINY-Breast04, HER2-low directed treatment, and interstitial lung disease [abstract]. In: Proceedings of the 2022 San Antonio Breast Cancer Symposium; 2022 Dec 6-10; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2023;83(5 Suppl):Abstract nr P1-11-05.

Treatable traits in interstitial lung diseases: a call to action

Despite growing interest in the management of interstitial lung diseases (ILDs) and many clinical trials, treatment options for ILDs remain scarce. This reality stems from variability in disease pathogenesis across ILD subtypes, unpredictable natural history, and heterogeneous treatment responses. 1 Kokosi MA Margaritopoulos GA Wells AU Personalised medicine in interstitial lung diseases: number 6 in the Series “personalised medicine in respiratory diseases” edited by Renaud Louis and Nicolas Roche. Eur Respir Rev. 2018; 27170117 Crossref PubMed Scopus (10) Google Scholar Some data suggest that diverse forms of ILD might share pathogenetic and biological pathways and could therefore be amenable to similar treatment strategies. 1 Kokosi MA Margaritopoulos GA Wells AU Personalised medicine in interstitial lung diseases: number 6 in the Series “personalised medicine in respiratory diseases” edited by Renaud Louis and Nicolas Roche. Eur Respir Rev. 2018; 27170117 Crossref PubMed Scopus (10) Google Scholar , 2 Hoffmann-Vold AM Weigt SS Saggar R et al. Endotype-phenotyping may predict a treatment response in progressive fibrosing interstitial lung disease. EBioMedicine. 2019; 50: 379-386 Summary Full Text Full Text PDF PubMed Scopus (29) Google Scholar This evidence points to the existence of treatable traits—specific disease characteristics that are clinically relevant and modifiable through pharmacological or non-pharmacological interventions—that cut across traditional diagnostic categories. 3 McDonald VM Fingleton J Agusti A et al. Treatable traits: a new paradigm for 21st century management of chronic airway diseases: Treatable Traits Down Under International Workshop report. Eur Respir J. 2019; 531802058 Crossref Scopus (110) Google Scholar

Integrating morphology and treatable traits into the management of ILD

Interstitial lung diseases (ILDs) are typically classified on the basis of their underlying causes; however, most ILDs can present with a multitude of morphological patterns on chest imaging or lung biopsy, and each pattern can similarly result from one of several underlying causes. Usual interstitial pneumonia (UIP) is one of these common patterns of pulmonary fibrosis that is typically associated with a poor prognosis. Although usually corresponding to a clinical diagnosis of idiopathic pulmonary fibrosis (IPF), both the overall pattern of UIP and its distinguishing features (eg, honeycombing) are also recognised in other ILD diagnoses, such as fibrotic hypersensitivity pneumonitis and connective tissue disease-associated ILD.

Influence of thoracic radiology training on classification of interstitial lung diseases

IntroductionInterpretation of high-resolution CT images plays an important role in the diagnosis and management of interstitial lung diseases. However, interreader variation may exist due to varying levels of training and expertise. This study aims to evaluate interreader variation and the role of thoracic radiology training in classifying interstitial lung disease (ILD). MethodsThis is a retrospective study where seven physicians (radiologists, thoracic radiologists, and a pulmonologist) classified the subtypes of ILD of 128 patients from a tertiary referral center, all selected from the Interstitial Lung Disease Registry which consists of patients from November 2014 to January 2021. Each patient was diagnosed with a subtype of interstitial lung disease by a consensus diagnosis from pathology, radiology, and pulmonology. Each reader was provided with only clinical history, only CT images, or both. Reader sensitivity and specificity and interreader agreements using Cohen's κ were calculated. ResultsInterreader agreement based only on clinical history, only on radiologic information, or combination of both was most consistent amongst readers with thoracic radiology training, ranging from fair (Cohen's κ: 0.2–0.46), moderate to almost perfect (Cohen's κ: 0.55–0.92), and moderate to almost perfect (Cohen's κ: 0.53–0.91) respectively. Radiologists with any thoracic training showed both increased sensitivity and specificity for NSIP as compared to other radiologists and the pulmonologist when using only clinical history, only CT information, or combination of both (p < 0.05). ConclusionsReaders with thoracic radiology training showed the least interreader variation and were more sensitive and specific at classifying certain subtypes of ILD. Summary sentenceThoracic radiology training may improve sensitivity and specificity in classifying ILD based on HRCT images and clinical history.

Descriptive study of the role of ultrasound in the evaluation of patients with interstitial lung disease associated with autoimmune connective tissue disorders

Background and Aim: High-resolution computed tomography (HRCT) of the chest has been the conventionally accepted modality of radiological investigation utilized in the evaluation and management of interstitial lung diseases (ILDs). The aim of this study was to compare the utilization of lung ultrasound (LUS) as a radiological modality versus HRCT scan of the chest, in the diagnosis of cases of ILD in autoimmune connective tissue disease (AICTD) patients at a tertiary care center in Northern India. Methods: In this descriptive study, 42 consecutive diagnosed cases of AICTD with clinical risk of ILD were included, between July 2016 and March 2019, attending the rheumatology outpatient department of CH WC. They were assessed with lung ultrasonography and HRCT chest. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of LUS were estimated considering HRCT as the reference method. Results: Of these 42 patients, 30 (71.4%) had abnormal LUS findings. HRCT findings suggestive of ILD were seen in 31 (73.8%). Considering HRCT as gold standard, LUS resulted in 01 false-positive and 02 false-negative results. Sensitivity and specificity of LUS with respect to HRCT were 93.55% and 90.91%, respectively. Positive predictive value (PPV) and negative predictive value (NPV) of LUS were calculated at 89.38% and 94.51%, respectively. In 91.6% (11/12) patients with nonspecific interstitial pneumonitis pattern on HRCT, B-lines were found to be numerous and compact (<3 mm distance between B lines). On the other hand, compact B lines were seen in only 21.1% (4/19) cases showing HRCT patterns suggestive of usual interstitial pneumonitis (UIP)/likely UIP, with a significant difference between the two (P = 0.0002). Conclusions: LUS is as nearly sensitive and specific as HRCT chest in the detection of ILD and has the distinct advantage of being without radiation exposure.

Validity and repeatability of cardiopulmonary exercise testing in interstitial lung disease

BackgroundCardiopulmonary exercise testing (CPET), and its primary outcome of peak oxygen uptake (VO2peak), are acknowledged as biomarkers in the diagnostic and prognostic management of interstitial lung disease (ILD). However, the validity and repeatability of CPET in those with ILD has yet to be fully characterised, and this study fills this evidence gap.MethodsTwenty-six people with ILD were recruited, and 21 successfully completed three CPETs. Of these, 17 completed two valid CPETs within a 3-month window, and 11 completed two valid CPETs within a 6-month window. Technical standards from the European Respiratory Society established validity, and repeatability was determined using mean change, intraclass correlation coefficient and typical error.ResultsEvery participant (100%) who successfully exercised to volitional exhaustion produced a maximal, and therefore valid, CPET. Approximately 20% of participants presented with a plateau in VO2, the primary criteria for establishing a maximal effort. The majority of participants otherwise presented with secondary criteria of respiratory exchange ratios in excess of 1.05, and maximal heart rates in excess of their predicted values. Repeatability analyses identified that the typical error (expressed as percent of coefficient of variation) was 20% over 3-months in those reaching volitional exhaustion.ConclusionThis work has, for the first time, fully characterised how patients with ILD respond to CPET in terms of primary and secondary verification criteria, and generated novel repeatability data that will prove useful in the assessment of disease progression, and future evaluation of therapeutic regimens where VO2peak is used as an outcome measure.

Management of Interstitial Lung Diseases: A Consensus Statement of the Indian Chest Society and National College of Chest Physicians (India)

Management of Interstitial Lung Diseases: A Consensus Statement of the Indian Chest Society and National College of Chest Physicians (India)

Costs of Pharmacological and Non-Pharmacological Interventions in Interstitial Lung Disease Management in Germany

Introduction: Interstitial lung diseases (ILDs) are associated with a high economic burden, yet prospective data of the German healthcare system are sparse. Objective: We assessed average ILD-related costs of pharmacological and non-pharmacological (hospitalizations, outpatient, rehabilitation, physiotherapy, and medical aids) interventions in ILD. Methods: We used data from the multicenter, observational, prospective Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases registry to evaluate adjusted per capita costs and cost drivers for ILD-related healthcare costs over 4 years, using generalized estimating equation regression models. Results: Idiopathic pulmonary fibrosis (IPF) had the highest annual pharmacological costs >EUR 21,000, followed by connective tissue disease-associated ILD (CTD-ILD) averaging EUR 6,000. Other idiopathic interstitial pneumonias and hypersensitivity pneumonitis averaged below EUR 2,400 and sarcoidosis below EUR 400. There were no significant differences in pharmacological costs over time. Trends in non-pharmacological costs were statistically significant. At year 1, CTD-ILD had the highest costs (EUR 7,700), while sarcoidosis had the lowest (EUR 2,547). By year 4, these declined to EUR 3,218 and EUR 232, respectively. Regarding cost drivers, the ILD subtype had the greatest impact with 75 times higher pharmacological costs in IPF and 4 times higher non-pharmacological costs in CTD-ILD, compared to the reference. Pulmonary hypertension (PH) and gastroesophageal reflux disease (GERD) triggered higher pharmacological costs, and higher values of forced vital capacity % predicted were associated with lower pharmacological and non-pharmacological costs. Conclusion: Stabilizing lung function and reducing the impact of PH and GERD are crucial in reducing the economic burden of ILD. There is an urgent need for effective treatment options, especially in CTD-ILD.

PULMONARY LIMITED SERONEGATIVE GRANULOMATOSIS WITH POLYANGIITIS VASCULITIS

PULMONARY LIMITED SERONEGATIVE GRANULOMATOSIS WITH POLYANGIITIS VASCULITIS

Anna-Maria Hoffmann-Vold: building bridges in systemic sclerosis.

“A lot of my focus is on building the bridge between basic science and clinical science,” says Anna-Maria Hoffmann-Vold, rheumatologist at Oslo University Hospital in Norway. Hoffmann-Vold leads research in inflammatory and fibrotic diseases and also runs a clinic for patients with systemic sclerosis and connective tissue disease-associated interstitial lung disease, noting the integral connection between the two. “Without basic evidence, what we do in the clinic is not really evidence-based. And on the other hand, if you develop anything in the lab, it doesn't help if it's not implemented into the clinics,” she says. Collaborative working is also a priority, uniting people who are “often in totally different worlds” but are travelling down the same road. With experience and persistence, Hoffmann-Vold builds the bridge piece by piece. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statementsThrough a robust modified Delphi process developed by a diverse panel of experts, the first evidence-based consensus statements were established on guidance for the identification and medical management of systemic sclerosis-associated ILD. Full-Text PDF

3. Recent Progress in Management of Interstitial Lung Disease Associated with Connective Tissue Diseases

3. Recent Progress in Management of Interstitial Lung Disease Associated with Connective Tissue Diseases

YKL-40 and KL-6 Levels in Serum and Sputum of Patients Diagnosed With Hypersensitivity Pneumonitis

YKL-40 (chitinase 3-like-1) and Krebs von den Lungen-6 (KL-6) are 2 promising biomarkers that may have an important role in the management of interstitial lung diseases (ILD). The aim of this study was to investigate the values of KL-6 and YKL-40 as biomarkers in the diagnosis and prognosis of patients with hypersensitivity pneumonitis (HP). A cross-sectional study conducted in 49 patients diagnosed with HP due to exposure to birds (n= 32) or fungi (n= 17), 48 patients with other ILD, and 67 healthy volunteers. Patients with HP were divided into fibrotic and nonfibrotic. Serum and sputum YKL-40 and KL-6 levels were determined using commercial enzyme-linked immunosorbent assay kits. Receiver operating characteristic (ROC) curves were used to determine the sensitivity and specificity of both biomarkers for the diagnosis of HP. Pulmonary function tests were performed in patients during follow-up. KL-6 and YKL-40 levels were significantly higher in serum of patients with HP exposed to birds with a fibrotic pattern than in controls (P < .0001 and .0055, respectively). Serum KL-6 levels were also significantly higher in patients with fibrotic HP exposed to fungi compared with the control group (P= .0001). In patients with HP exposed to fungi, sputum KL-6 and YKL-40 levels were higher in those with a fibrotic pattern (P= .0289 and .016, respectively). ROC analysis showed that the range between 55-121 ng/mL for serum YKL-40 levels and 346-1441 U/mL for serum KL-6 levels had the best sensitivity and specificity for discriminating between patients with HP, healthy controls, and patients with idiopathic pulmonary fibrosis (IPF). In patients with HP, serum KL-6 levels correlated negatively with total lung capacity (r=-0.485; P= .0103) and diffusing capacity of the lungs for carbon monoxide (r=-0.534; P= .0002) at 12 months. Both KL-6 and YKL-40 proteins seem to be capable of distinguishing patients with HP from healthy individuals and from patients with IPF. Their sensitivity and specificity confirm their potential role as biomarkers. KL-6 may also be a predictor of disease progression.

Physiotherapy management of interstitial lung disease.

Physiotherapy management of interstitial lung disease.