Spectrum Of Interstitial Lung Disease Research Articles

Clinical-Epidemiological Profile and Distribution of Interstitial Lung Diseases: A Tertiary Care Centre Experience from North India

Background: The Interstitial Lung Disease (ILD) India registry, which included more than 1000 patients across 27 centers of the country, reported Hypersensitivity Pneumonitis (HP) to be the most common ILD in India, followed by Connective Tissue Disease-related ILD (CTD-ILD) and Idiopathic Pulmonary Fibrosis (IPF). This study was carried out to evaluate whether the spectrum of ILDs at our center conforms to the national data. Aims: To evaluate the clinical and epidemiological profile, distribution of various ILDs, and any association of the type of ILD with clinical parameters. Materials and methods: A prospective, observational study was carried out at a tertiary care center in North India, where patients having symptoms and radiological features suggestive of ILD were evaluated for clinical, radiological features and spirometric indices. Results: A total of 83 patients with ILD were evaluated. The mean age was 53.76 ± 13.07 years. The male-to-female ratio was 0.97. The mean FEV1 was 1.41 ± 0.38 L, mean FVC was 1.57 ± 0.43 L and the mean FEV1/FVC was 90.26 ± 8.99%. The mean 6 MWD was 270.85 ± 110.52 m. Reticulation and honeycombing were the most common radiological features. IPF was the most common ILD (28.9%). Conclusion: IPF was the most common ILD at our center. A statistically significant association was found between type of ILD and age, gender, past ATT intake, HTN, clubbing, and FEV1/FVC%.

Acute Exacerbation of Interstitial Lung Disease: Definitions, Epidemiology, Prognosis and Management.

Abstract Acute exacerbation of interstitial ling disease (AE-ILD) denotes a rapid deterioration of respiratory function often leading to ICU admission due to severe hypoxemia. Definitions encompass the diverse spectrum of interstitial lung diseases and specific criteria for AE-ILD, originally established for idiopathic pulmonary fibrosis (IPF) and later expanded to other interstitial lung diseases. The incidence varies among the various interstitial lung disease subtypes, with around 10% of interstitial lung disease patients experiencing acute exacerbations within the initial two years of diagnosis. Clinical risk factors encompass advanced interstitial lung disease, compromised pulmonary function, and coexisting conditions. Prognostic markers include baseline pulmonary parameters, blood markers, and a newly developed Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) staging system. However, due to the scarcity of randomized studies, optimal treatment strategies remain uncertain. Current guidelines propose supportive care and oxygen therapy, with cautious use of corticosteroids in AE-IPF. Antibiotics are employed to manage infections, with procalcitonin-guided therapy showing potential. Management involves exposure elimination, targeted antibiotics, and antacid and antifibrotic use. For severe cases, mechanical ventilation, extracorporeal membrane oxygenation (ECMO), and even transplantation are considered. To conclude, AE-ILD poses formidable challenges and high mortality rates. Given the lack of standardized approaches, the pursuit of randomized trials is vital to enhance the understanding and management of AE-ILD, optimizing patient outcomes within the ICU setting. Keywords: acute exacerbation, interstitial lung disease, idiopathic pulmonary fibrosis, intensive care unit, definition.

ERS International Congress 2023: highlights from the Interstitial Lung Diseases Assembly.

This article summarises a selection of scientific highlights in the field of interstitial lung diseases (ILDs) presented at the International Congress of the European Respiratory Society in 2023. Translational and clinical studies focused on the whole spectrum of ILDs, from (ultra)rare ILDs to sarcoidosis, ILDs associated with connective tissue disease and idiopathic pulmonary fibrosis. The main topics of the 2023 Congress presentations were improving the diagnostic process of ILDs, better prediction of disease course and investigation of novel treatment options.

Spectrum of Interstitial Lung Disease as Seen on Computed Tomography in a Tertiary Centre

Spectrum of Interstitial Lung Disease as Seen on Computed Tomography in a Tertiary Centre

Immune-related interstitial lung disease induced by different immune checkpoint inhibitors regimens: A real-world study from 2014 to 2022 based on FAERS databases

This study further approaches immune-related interstitial lung disease adverse event in patients undergoing immune checkpoint inhibitor (ICI) monotherapy, ICI plus chemotherapy and ICI plus anti-VEGF therapy in the postmarketing period. Reports for ICI-related interstitial lung disease adverse event from the FDA Adverse Event Reporting System (FAERS) database between 2014 and 2022 were analysed in this study. The reporting odds ratio (ROR) and Bayesian confidence propagation neural networks of information components (IC) were computed to identify disproportionate reporting of ICI-related interstitial lung disease. 44,964,609 records were extracted from the FAERS database, with 9150 records for interstitial lung disease after ICI treatment. Men had a slightly higher reporting frequency than women (63.07% vs. 25.69%). The morbidity rate (2.05%) of acute respiratory distress syndrome was low, the fatality rate (67.55%) was the highest, the time to onset was relatively short. Within 3 months, the cumulative proportion of ICI-related interstitial lung disease records was 75.03%. The ICI plus anti-VEGF therapy group had the lowest frequency of interstitial lung disease adverse events compared to the ICI monotherapy group and the ICI plus chemotherapy group (IC025 = 1.72, IC025 = 3.21, IC025 = 3.22). Moreover, ICI plus anti-VEGF therapy group had the narrowest spectrum of interstitial lung disease among these three therapeutic regimens. This study showed substantial characteristics of a spectrum of interstitial lung disease adverse events after different ICI regimens. Notably, ICI plus anti-VEGF therapy might be a treatment method that can to some extent control ICI-related interstitial lung disease. These data provide some important information for clinicians to weigh the risks and benefits of different ICI regimens.

Prognostic Predictive Characteristics in Patients With Fibrosing Interstitial Lung Disease: A Retrospective Cohort Study.

Background: Limited data are available regarding the entire spectrum of interstitial lung disease with a progressive fibrosing feature. We investigated the prevalence and prognostic predictive characteristics in patients with PF-ILD. Methods: This retrospective cohort study included patients with fibrosing ILD who were investigated between 1 January 2015 and 30 April 2021. We recorded clinical features and outcomes to identify the possible risk factors for fibrosing progression as well as mortality. Results: Of the 579 patients with fibrosing ILD, 227 (39.21%) met the criteria for progression. Clubbing of fingers [odds ratio (OR) 1.52, 95% confidence interval (CI) 1.03 to 2.24, p = 0.035] and a high-resolution computed tomography (HRCT)-documented usual interstitial pneumonia (UIP)-like fibrotic pattern (OR 1.95, 95% CI 1.33 to 2.86, p = 0.001) were risk factors for fibrosis progression. The mortality was worse in patients with PF with hypoxemia [hazard ratio (HR) 2.08, 95% CI 1.31 to 3.32, p = 0.002], in those with baseline diffusion capacity of the lung for carbon monoxide (DLCO) % predicted <50% (HR 2.25, 95% CI 1.45 to 3.50, p < 0.001), or in those with UIP-like fibrotic pattern (HR 1.68, 95% CI 1.04 to 2.71, p < 0.001). Conclusion: Clubbing of fingers and an HRCT-documented UIP-like fibrotic pattern were more likely to be associated with progressive fibrosing with varied prevalence based on the specific diagnosis. Among patients with progressive fibrosing, those with hypoxemia, lower baseline DLCO% predicted, or UIP-like fibrotic pattern showed poor mortality.

Spectrum of interstitial lung diseases and their association with pulmonary hypertension.

Interstitial lung diseases (ILDs) are an intriguing group of pulmonary disorders, which still require the study of epidemiological, genetic, pathophysiological, clinical, and radiological parameters. Pulmonary hypertension (PH) is an underreported complication in interstitial lung diseases which is associated with worse outcome. In our study, we have reported the spectrum of ILDs and estimated the prevalence of pulmonary hypertension among these subjects at a tertiary care centre. A cross-sectional study was performed in which demographical, clinical, radiological, and histological data of subjects with ILD, attending the department of Respiratory Medicine in the University was collected from 1st September 2018 to 31st August 2019. Serological tests were done wherever indicated. Standard criteria along with multidisciplinary opinion were needed to arrive at the final diagnosis. All subjects were screened for pulmonary hypertension via 2-D echocardiography. Mean pulmonary artery pressure ≥20 mmHg was used to define PH. In the defined period, 239 subjects were enrolled (58% females, n=141; mean age 52.38±13.40 years). A tissue diagnosis was obtained in 34% cases. The most common ILD was hypersensitivity pneumonitis (32.2%), followed by autoimmune-ILD (31.4%), idiopathic pulmonary fibrosis (IPF) (15.9%) and sarcoidosis (12.6%), non-IPF idiopathic interstitial pneumonitis (2.1%) and rest 21 (5.9%) subjects were diagnosed as other types of ILD. Pulmonary hypertension was seen in 46.0% of subjects.

Performance of Lung Ultrasound for Monitoring Interstitial Lung Disease.

In this study, we sought to assess the validity of lung ultrasound (LUS) during the follow-up of patients with a wide spectrum of interstitial lung diseases (ILDs). Twenty-four patients (13 males, 11 females; mean age ± SD, 65.4 ± 14.3 years; age range, 40-84 years) with a diagnosis of ILDs who were admitted to the Interstitial Lung Disease Unit were prospectively enrolled. Patients were examined with a 56-lung intercostal space LUS protocol in lateral decubitus position, at baseline, 6-months, and 1-year. The LUS score was defined as the sum of B-lines counted in each intercostal space. All patients underwent complete pulmonary function tests at baseline and follow-up time-points. High-resolution computed tomography (HRCT) was performed at baseline and during follow-up, according to personalized patients' needs. All HRCT studies were graded according to the Warrick scoring system (WS). Pooled data analysis showed a significant correlation between WS and LUS scores (P< .001). For separate time-point analysis, a significant correlation between LUS scores and WS was found at baseline (P< .001) and 1 year (P= .005). LUS scores negatively correlated with alveolar volume (VA) (P< .046) and diffusing capacity for carbon monoxide (DLCO) (P< .001) at 6 months and with transfer coefficient of the lung for carbon monoxide (KCO) (P< .031) and DLCO (P= .002) at 12-months. A multivariate regression model showed DLCO to be an independent predictor of LUS score at 1 year (P= .026). Our results highlight the validity and potential applicability of LUS for disease monitoring in a wide spectrum of ILDs.

Spectrum of Interstitial Lung Disease on High Resolution Computed Tomography- A Cross-Sectional Analysis- A Case of Mitotically Active Cellular Fibroma

Spectrum of Interstitial Lung Disease on High Resolution Computed Tomography- A Cross-Sectional Analysis- A Case of Mitotically Active Cellular Fibroma

Spectrum of interstitial lung disease in rheumatic diseases

Spectrum of interstitial lung disease in rheumatic diseases

Clinical and radiological spectrum of interstitial lung disease in a north Indian state: A single centre study

Background: The present study was carried out with an aim of understanding the types of Interstitial lung disease (ILD) and their radiological patterns in the north Indian state of Jammu and Kashmir. Methods: The present study was a prospective observational study conducted over a period of 2 years in Sher-i-Kashmir Institute of Medical Sciences- Medical College, Bemina, Srinagar. All consecutive patients referred to the department for High Resolution CT scan (HRCT) of the chest with suspected diagnosis of ILD based on clinical features, spirometry and chest X-ray findings, who gave consent for the study were included. Clinical, biochemical, serological and radiological parameters were assessed and the diagnosis of various ILDs was made based on standard international criteria. Results: A total 122 patients of ILD were enrolled over a period of 2 years in the present study. Mean age of the patients was 63.4 ± 19.7 years and majority (59.8%) were males. 41.8% patients were current or previous smokers. Mean symptom duration before diagnosis was 3.1 ± 1.7 years. Exertional breathlessness (98.4%) and cough (94.3%) were the most common symptoms. Hypertension (70.5%) and gastroesophageal reflux disease (62.3%) were the most frequent comorbidities. On HRCT thorax ground glass opacities (52.5%) were the most common finding, followed by interlobular (39.3%) and intralobular (34.4%) septal thickening. Diffuse pattern of lung involvement was seen in 41.8% and predominant upper/middle lobe involvement in 33.6%. Idiopathic pulmonary fibrosis (29.5%) was the most common form of ILD, followed by Nonspecific interstitial pneumonia (23.8%) and hypersensitivity pneumonitis (15.6%). Gujjar lung, a unique form of ILD in Kashmir, was diagnosed in 2.5% patients. Conclusion: The present study is the first from the state of Jammu and Kashmir assessing the radiological spectrum and types of ILD in this part of country. It provides a useful insight into the clinical and radiological patterns of ILD in our population and highlights the need for larger prospective multicentre registries to assess the true incidence and prevalence of ILD in the country.

The interstitial lung disease spectrum under a uniform diagnostic algorithm: a retrospective study of 1,945 individuals.

BackgroundReported data on the disease spectrum of interstitial lung diseases (ILDs) of China are sparse and varied. We aimed to investigate the spectrum of ILDs and the distribution of diagnostic methods under a uniform diagnosis.MethodsThis retrospective study enrolled ILDs cases from Guangzhou Institute of Respiratory Health (GIRH). All cases were classified into specific subgroups of ILDs according to updated guidelines.ResultsA total of 1,945 subjects were enrolled from January 2012 to December 2017. The mean age was 57.9 years, and 1,080 (55.5%) patients were male. The most common subtype of ILDs was idiopathic pulmonary fibrosis (IPF, 20.3%), followed by interstitial pneumonia with autoimmune features (IPAF, 17.9%), connective tissue disease associated ILD (CTD-ILD, 18.3%) and unclassifiable idiopathic interstitial pneumonia (UIIP, 14.7%). A total of 818 (42.1%) patients underwent lung biopsy in order to obtain a histological diagnose. TBLB was performed in 565 (29.0%) patients, eleven of whom underwent SLB because TBLB failed to obtain lung samples. SLB was performed in 213 (11.0%) patients and TBCB was performed in 51 (2.6%) patients. Among them, histological results were considered clinically helpful in 722 (88.3%) cases, and provided definitive histopathological diagnoses in 368 cases. Surgical lung biopsy (SLB) was performed in 213 (10.9%) subjects, while 115 (54.0%) cases were performed among the idiopathic interstitial pneumonia (IIP). Among SLB cases in IIP, the highest rate of SLB was desquamative interstitial pneumonia/respiratory bronchiolitis-interstitial lung disease (DIP/RB-ILD, 10/10), lymphoid interstitial pneumonia (LIP, 9/9), followed by cryptogenic organizing (COP, 18/26), nonspecific interstitial pneumonia (NSIP, 22/53), IPF (43/395), UIIP (13/285).ConclusionsIPF was the most common ILDs in our ILD center, followed by IPAF, CTD-ILD and UIIP. Histological information may help to establish diagnostic algorithm in ILD.

The Spectrum of Interstitial Lung Disease Associated with Autoimmune Diseases: Data of a 3.6-Year Prospective Study from a Referral Center of Interstitial Lung Disease and Lung Transplantation.

Interstitial lung disease (ILD) may occur in patients with a rheumatic autoimmune disease (AD), increasing their risk of morbidity and mortality. However, little is known about the prevalence of AD in patients diagnosed with an ILD. In this prospective study, we determined the spectrum of ILD associated with AD (AD-ILD) among patients sent for assessment to a single clinic of ILD and lung transplantation from a referral center between May 2016 and December 2019. ILD diagnosis was made by pneumologists based on clinical and radiological findings and pulmonary function test abnormalities. All patients with ILD were also assessed by experienced rheumatologists. During the period of assessment, 338 patients were diagnosed with ILD. Among them, 32.8% fulfilled definitions for an AD. Most cases with AD-ILD had a diagnosis of rheumatoid arthritis (27.0%), systemic sclerosis (26.1%) or anti-synthetase syndrome (17.1%). Interestingly, 18% of the patients with AD-ILD were diagnosed as having an interstitial pneumonia with autoimmune features. Antinuclear antibodies and non-specific interstitial pneumonia were the most frequent positive autoantibodies and radiological pattern found in AD-ILD patients, respectively. In conclusion, our study indicates that a high number of ILD patients have a related AD. Consequently, close collaboration among rheumatologists and pneumologists is needed.

Etiologic spectrum of interstitial lung diseases in Chinese children older than 2\u2009years of age

BackgroundChildhood interstitial lung diseases (ILD) (chILD) refer to a rare heterogeneous group of disorders. Global collaborations have been working on the etiologies and classification scheme of chILD. With the development of medical technologies, some new diseases were identified to be associated with chILD and its etiologic spectrum is expanding. The aim of this study is to describe the etiologic spectrum of chILD in children older than 2 years of age and summarize the approaches to diagnosis of chILD.MethodsWe made a retrospective analysis of children older than 2 years of age with chILD who referred to Beijing Children’s Hospital from 21 provinces all over China from 2013 to 2018. After excluding pulmonary infection, congenital heart disease, bronchopulmonary dysplasia, bronchiolitis obliterans and bronchiectasis, 133 patients were included and categorized by etiology. Clinical manifestations, high-resolution computed tomography, laboratory data, genetic data and pathologic findings were all collected and reviewed.ResultsSystemic disease associated ILD were the most common causes, accounting for 49.6% of the patients, followed by alveolar structure disorder-associated ILD (27%), exposure related ILD (13.5%), and disorders masquerading as ILD (3.8%). In systemic disease associated ILD, in addition to common etiologies such as vasculitis (10.5%) and connective tissue diseases (9.0%), primary immunodeficiency diseases (PID) associated ILD (9.8%), interstitial pneumonia with autoimmune features (6.8%), and metabolic diseases (6.8%) were not rarely found. Some newly reported etiologies such as STING–associated vasculopathy with onset in infancy, COPA syndrome and STAT3 mutation were included in PID associated ILD. Genetic tests contributed to 15% of the diagnoses which mainly distributed in PID associated ILD, metabolic diseases and surfactant dysfunction disorders, and contributed to the final diagnoses more than lung biopsies (13.5%) and biopsies of rashes or other tissues (12%).ConclusionsThis study first demonstrated an etiologic spectrum of chILD in Chinese children older than 2 years of age and summarized the approaches to diagnosis. The etiologic spectrum of chILD is expanding with more genetic etiologies being recognized.

The evolving spectrum of interstitial lung disease in myositis—Management pearls

Idiopathic inflammatory myopathies (IIMs) are characterized by muscle inflammation caused by exacerbated autoimmunity reactions. Patients with IIMs also have extramuscular lesions, such as skin rash, arthritis, interstitial lung disease (ILD), and cardiomyopathy. ILD is one of the leading causes of mortality in patients with IIMs. Thus, physicians need to manage patients with IIM-associated ILD (myositis-ILD) appropriately to prevent the development and progression of ILD. Predictive factors for morality should be considered at the time of making decisions on therapeutic strategies for myositis-ILD. There have been numerous prognostic factors associated with mortality or pulmonary dysfunction. According to the latest research, which contains the large database analysis enrolling 499 incident cases of myositis-ILD with the comprehensive measurement of myositis-specific autoantibodies (MSAs), the presence of anti-melanoma differentiation-associated gene 5 antibody, age ≥60 years, C-reactive protein ≥1 mg/dL, and pulse saturation oxygen

Clinical and genetic spectrum of interstitial lung disease in Chinese children associated with surfactant protein C mutations

BackgroundMutations in the surfactant protein C gene (SFTPC) result in interstitial lung disease (ILD). Our objective was to characterize clinical and genetic spectrum of ILD in Chinese children associated with SFTPC mutations.MethodsSix Chinese children with ILD heterozygous for SFTPC mutations were included. Candidate genes responsible for surfactant dysfunction were sequenced by next-generation sequencing. Subclones of SFTPC with novel mutations were generated and transiently transfected into A549 cells. The functional characterization of mutant surfactant protein C (SP-C) was evaluated by Western blotting and immunofluorescence.ResultsThe age of onset ranged from 7 days to 15 months. All cases required supplemental oxygen. Failure to thrive (5/6) was the most significant extra-pulmonary manifestation. Hydroxychloroquine was given as the long-term treatment of lung disease in four patients and two of them responded well. Three mutations were identified in six patients: four with I73T, one with D105G, one with Y113H. Mutations in three patients were inherited and three arised de novo. Western blotting revealed totally different band patterns between mutant SP-C (D105G and Y113H) and the wildtype. Immunofluorescence showed mutant SP-C (D105G) was scarcely trafficked to lamellar bodies but localized well to early endosomes, which was in marked contrast to the wildtype protein.ConclusionSFTPC mutations were an important cause of childhood ILD in Chinese population. I73T was a common SFTPC mutation in Chinese ILD children associated with surfactant protein C mutations.

An observational study of RA-ILD at tertiary care hospital in eastern India

Introductions: The spectrum of interstitial lung diseases (ILDs) have mainly been reported from the developed countries; data from developing countries is sparse and conflicting. The aim of this study is to describe the distribution of various RA-ILDs from a tertiary care hospital. Methods: This is an analysis of prospectively collected clinical and demographic data of consecutive subjects with ILDs with special reference to RA-ILD from a single tertiary care medical center. The diagnosis of the specific subtype of ILD was made according to standard criteria for various ILDs.Results: A total of 100 subjects with mean age and standard deviation 52.21 ± 14.41 and 37.0% male and 63.0% female were enrolled during the study period (March 2017 to December 2018). Cough was the most common symptoms 100.0% followed by 28.0%, shortness of breath (SOB) 98.0%, Wheezing 32.0%, Chest Pan (Pleuritic) 41.0% subjects had a restrictive defect on spirometry It was found that 61% subjects had pallor, 40% subjects had clubbing, 42% subjects had edema, 65% subjects had arthalgia (S/L), 37% subjects had skin manifestations and 14% subjects had mucosal involment. Total in16 no. of RA-ILD cases (9) male (7) female having UIP pattern n-11 & NSIP pattern in n-5 cases.Conclusion: RA-ILD among the CTD-ILDs was the most common ILDs seen at a tertiary center in earthern India similar to the spectrum reported from developed countries. More studies are required from developing countries to ascertain the spectrum of ILDs in different geographic locales.

Spectrum of interstitial lung diseases at a tertiary center in a developing country: A study of 803 subjects.

BackgroundThe spectrum of interstitial lung diseases (ILDs) have mainly been reported from the developed countries; data from developing countries is sparse and conflicting. The aim of this study is to describe the distribution of various ILDs from a developing country.MethodsThis is an analysis of prospectively collected clinical, radiological and histological data of consecutive subjects (age >12 years) with ILDs from a single tertiary care medical center. The diagnosis of the specific subtype of ILD was made according to standard criteria for various ILDs.ResultsA total of 803 subjects (mean age, 50.6 years; 50.2% women) were enrolled between March 2015 to February 2017 of which 566 (70.5%) were diagnosed during the study period (incident cases). Sarcoidosis (42.2%), idiopathic pulmonary fibrosis (IPF, 21.2%), connective tissue disease (CTD)-related ILDs (12.7%), hypersensitivity pneumonitis (10.7%), and non-IPF idiopathic interstitial pneumonias (9.2%) were the most common ILDs. The spectrum of ILDs was not significantly different (p = 0.87) between incident and prevalent cases. A histopathological specimen was obtained in 49.9% of the subjects yielding a histologically confirmed diagnosis in 40.6%. A diagnostic procedure was not performed in 402 subjects; the most common reasons were presence of definite usual interstitial pneumonia pattern on high resolution computed tomography and patients’ unwillingness to undergo the procedure.ConclusionSarcoidosis, IPF and CTD-ILDs were the most common ILDs seen at a tertiary center in northern India similar to the spectrum reported from developed countries. More studies are required from developing countries to ascertain the spectrum of ILDs in different geographic locales.

The Spectrum of Interstitial Lung Disease in Connective Tissue Disease.

Interstitial lung disease (ILD) is a common cause of morbidity and mortality in patients with connective tissue disease (CTD). In a minority of patients the ILD may be the presenting (or only) manifestation of an underlying CTD. Diagnosis of CTD-related ILD relies on a multidisciplinary team including pulmonologists, pathologists, radiologists, and rheumatologists, as the imaging and pathologic findings may be indistinguishable from idiopathic interstitial pneumonias. Moreover, many patients with ILD are suspected of having an underlying CTD but do not meet all of the necessary criteria for a specific disorder. This article provides a pattern-based approach to the imaging of CTD-related ILD and also reviews relevant clinical, pathologic, and serologic data that radiologists should be familiar with as part of a multidisciplinary team.

Classic spectrum of interstitial lung diseases on HRCT

Classic spectrum of interstitial lung diseases on HRCT