Common Interstitial Lung Disease Research Articles

Immunomodulatory treatment of interstitial lung disease.

Interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) have an array of immunomodulatory treatment options compared with IPF, due to their inflammatory component. However, there is a relative paucity of guidance on the management of this heterogeneous group of diseases. In ILDs other than IPF, immunosuppression is the cornerstone of therapy, with varying levels of evidence for different immunomodulatory agents and for each specific ILD. Classification of ILDs is important for guiding treatment decisions. Immunomodulatory agents mainly include corticosteroids, mycophenolate mofetil (MMF), azathioprine, methotrexate, cyclophosphamide and rituximab. In this review, the available evidence for single agents in the most common ILDs is first discussed. We then reviewed practical therapeutic approaches in connective tissue disease–related ILD and interstitial pneumonia with autoimmune features, scleroderma-related ILD, vasculitis and dermatomyositis with hypoxemic respiratory failure, idiopathic non-specific interstitial pneumonia, hypersensitivity pneumonitis sarcoidosis, fibrosing organizing pneumonia and eosinophilic pneumonia. The treatment of acute exacerbations of ILD is also discussed. Therapy augmentation in ILD is dictated by the recognition of progression of disease. Criteria for the evaluation of progression of disease are then discussed. Finally, specific protocol and measures to increase patients’ safety are reviewed as well, including general monitoring and serologic surveillance, Pneumocystis jirovecii prophylaxis, patients’ education, genetic testing for azathioprine, MMF serum levels and cyclophosphamide administration protocols. Immunomodulatory therapies are largely successful in the management of ILDs and can be safely managed with the application of specific protocols, precautions and monitoring.

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.

Management of patients with fibrosing interstitial lung diseases.

PurposeThis article summarizes the appropriate use and pharmacology of treatments for fibrosing interstitial lung diseases, with a specific focus on the antifibrotic agents nintedanib and pirfenidone.SummaryThe interstitial lung diseases are a heterogenous group of parenchymal lung disorders with a common feature—infiltration of the interstitial space with derangement of the normal capillary-alveolar anatomy. Diseases characterized by fibrosis of the interstitial space are referred to as the fibrosing interstitial lung diseases and often show progression over time: idiopathic pulmonary fibrosis is the most common fibrotic interstitial lung disease. Historically, therapies for fibrosing lung diseases have been limited in number, questionable in efficacy, and associated with potential harms. Food and Drug Administration (FDA) approval of the antifibrotic agents nintedanib and pirfenidone for idiopathic pulmonary fibrosis in 2014 heralded an era of reorganization of therapy for the fibrotic interstitial lung diseases. Subsequent investigations have led to FDA approval of nintedanib for systemic sclerosis–associated interstitial lung disease and interstitial lung diseases with a progressive phenotype. Although supportive care and pulmonary rehabilitation should be provided to all patients, the role(s) of immunomodulators and/or immune suppressing agents vary by the underlying disease state. Several agents previously used to treat fibrotic lung diseases (N-acetylcysteine, anticoagulation, and pulmonary vasodilators) lack efficacy or cause harm.ConclusionWith the introduction of effective pharmacotherapy for fibrosing interstitial lung disease, pharmacists have an increasingly important role in the interdisciplinary team managing these patients.

Hypersensitivity Pneumonitis: Diagnostic and Therapeutic Challenges.

Hypersensitivity pneumonitis (HP) is one of the most common interstitial lung diseases (ILD), that presents unique challenges for a confident diagnosis and limited therapeutic options. The disease is triggered by exposure to a wide variety of inciting antigens in susceptible individuals which results in T-cell hyperactivation and bronchioloalveolar inflammation. However, the genetic risk and the pathogenic mechanisms remain incompletely elucidated. Revised diagnostic criteria have recently been proposed, recommending to classify the disease in fibrotic and non-fibrotic HP which has strong therapeutic and outcome consequences. Confident diagnosis depends on the presence of clinical features of ILD, identification of the antigen(s), typical images on high-resolution computed tomography (HRCT), characteristic histopathological features, and lymphocytosis in the bronchoalveolar lavage. However, identifying the source of antigen is usually challenging, and HRCT and histopathology are often heterogeneous and not typical, supporting the notion that diagnosis should include a multidisciplinary assessment. Antigen removal and treating the inflammatory process is crucial in the progression of the disease since chronic persistent inflammation seems to be one of the mechanisms leading to lung fibrotic remodeling. Fibrotic HP has a few therapeutic options but evidence of efficacy is still scanty. Deciphering the molecular pathobiology of HP will contribute to open new therapeutic avenues and will provide vital insights in the search for novel diagnostic and prognostic biomarkers.

The improved clinical course of persistent tachypnea of infancy with inhaled bronchodilators and corticosteroids.

Persistent tachypnea of infancy (PTI) is the most common interstitial lung disease in young children. As no standardized therapeutic guidelines exist, different pharmaceuticals are used to treat PTI;inhaled corticosteroids (ICS) and bronchodilators being mostly used. This observation assessed the effectiveness of bronchodilators and ICS in children with PTI enrolled in the children's interstitial lung diseases (chILD)-EU Register. Symptomatic children with PTI were observed according to a predetermined stepwise protocol including bronchodilators as the first choice treatment (6 weeks). In patients with incomplete response, additionally,ICS was given (12 weeks).Signs, symptoms, and pulmonary function were evaluated at three time points: at baseline, 6 (±1) weeks after initiation of bronchodilators, and 12 (±1) weeks after bronchodilators/ICS. Thirty-one children (median age: 44 months, interquartile range [IQR]: 15-67) were included. The therapy was associated with a significant reduction of tachypnea (53.3% of patients, p = 0.02), exercise intolerance (52.2% of patients, p < 0.001), chest retractions (43.8% of patients, p = 0.04), and crackles (29.2% of patients, p = 0.02). Also, a significant improvement in forced expiratory volume in 1 s (FEV1 ) (median z score: -2.21 vs. -0.47, p = 0.03), residual volume (RV) (median z score 5.28 vs. 1.07, p = 0.007), RV% total lung capacity (TLC) (median z score: 6.05 vs. 1.48, p = 0.01), sRaw (median z score: 6.6 vs. 4.64, p = 0.01), R5 (median z score: 1.27 vs. 0.31, p = 0.009), and R5-R20 (median: 0.58 vs. 0.26 kPa/(l/s), p = 0.002) was demonstrated. Inhaled bronchodilators and ICS may exert a positive effect on the severity of symptoms and pulmonary function test (PFT) in symptomatic children with PTI. However, a randomized control trial should be conducted to confirm their effectiveness.

An update on interstitial lung disease.

Interstitial lung diseases are a complex group of conditions that cause inflammation and scarring of the lung interstitium. This article discusses the diagnosis and management of common interstitial lung diseases including idiopathic pulmonary fibrosis, hypersensitivity pneumonitis, connective tissue disease associated-interstitial lung disease, sarcoidosis and drug-induced interstitial lung disease. A multidisciplinary approach to diagnosis of interstitial lung disease is the gold standard; key history and examination features, blood panel, pulmonary function tests, high resolution computed tomography imaging, and when required bronchoalveolar lavage and lung biopsy results are discussed to reach a multidisciplinary consensus diagnosis. Advances, including the development of the disease-modifying anti-fibrotic medications nintedanib and pirfenidone, continue to shape the future management of interstitial lung disease. A holistic approach to the care of patients with interstitial lung disease is paramount, as they often have a high symptom burden and considerable palliative care needs.

Mise au point : pneumopathies d’hypersensibilité

Hypersensitivity Pneumonitis (HP) is a common immune-mediated interstitial lung disease (ILD) induced by repeated exposure to environmental antigens in susceptible individuals. The most commonly known forms are bird fancier's disease and farmer's lung. However, the antigens involved are widely diverse. Therefore, the list of causes of HP is frequently expanding. HP seems to be under-diagnosed owing to its highly heterogeneous presentation in both the non-fibrotic and fibrotic subtypes and could represent up to 15% of all ILDs encountered in clinical practice. However, the recognition of HP cases is essential to ensure appropriate therapy for the patient. Home health care workers’ intervention is sometimes critical in this context. In case of confirmed exposure, the diagnosis could be considered with high confidence if the high-resolution computed tomography (HR-CT) shows a typical HP pattern associated with a lymphocytosis over 30% in the broncho-alveolar lavage (BAL). In all other situations, the patients should undergo further investigations and additional histopathological sampling should be considered and submitted to a multidisciplinary team discussion. After diagnosis, antigenic eviction is the rule whenever possible. Corticosteroid treatment is the first-line medical treatment for severe forms and aims to prevent the development of fibrosis. Anti-fibrotic therapy is now an option for patients with progressive ILD and failure of immunomodulatory/immunosuppressive therapies.

English

BACKGROUND Interstitial lung disease (ILD) is an unpredictable diffuse parenchymal lung disease, which involves interstitium of lung (tissue around the alveoli of the lungs). High resolution computed tomography (HRCT) is one of the confirmatory, easily accessible methodology for the conclusion and follow up assessment of interstitial lung disease. We wanted to study the normal HRCT patterns found with interstitial lung disease and contrastingly different HRCT designs and clinical information in differential determination of pulmonary fibrosis. We also wanted to study the different patterns of interstitial lung disease on high resolution computed tomography and thereby provide accurate diagnosis and management to the patients. METHODS The study was a hospital based prospective, cross sectional study. In the present study, total fifty patients referred from Department of Medicine and Department of Pulmonary Medicine of GCS Medical College having suspicion of interstitial lung disease were studied from April 2019 to September 2019. All patients underwent HRCT thorax on 16 slice Siemens computerised tomography (CT) scan machine in recumbent position utilising usual HRCT protocol. Lung abnormalities were noticed and classified for explicit diagnosis of interstitial lung pathologies. RESULTS Most of the patients (N = 25) were found to be in the age group of 50 - 80 years (17 female &amp; 8 male). Progressive dyspnoea (N = 47; 94 %) was the most common chief complaint. The most common form of interstitial lung disease was usual interstitial pneumonia (UIN) (N = 18; 36 %) in our study. Acute interstitial pneumonia (AIP) (N = 7; 14 %) and non-specific interstitial pneumonia, NSIP (N = 7; 14 %) were the next common interstitial lung diseases. CONCLUSIONS The most well-known interstitial lung disease seen in our examination was usual interstitial pneumonia. Cases of interstitial lung disease are on the rise. Interstitial lung disease should be ruled out in all patients with progressive dyspnoea, particularly when there are no obvious or known causes of dyspnoea. Clinical and laboratory findings, pulmonary function tests, history of exposure along with HRCT workup is indispensable for the identification or exclusion of interstitial lung disease. HRCT is also useful for the follow-up study. KEYWORDS Acute Interstitial Pneumonia (AIP), High Resolution Computed Tomography (HRCT), Interstitial Lung Disease (ILD), Nonspecific Interstitial Pneumonia (NSIP), Usual Interstitial Pneumonia (UIP)

Dopamine receptor agonists ameliorate bleomycin-induced pulmonary fibrosis by repressing fibroblast differentiation and proliferation

Idiopathic pulmonary fibrosis (IPF) is the most common fatal interstitial lung disease, with limited therapeutic options. The abnormal and uncontrolled differentiation and proliferation of fibroblasts have been confirmed to play a crucial role in driving the pathogenesis of IPF. Therefore, effective and well-tolerated antifibrotic agents that interfere with fibroblasts would be an ideal treatment, but no such treatments are available. Remarkably, we found that dopamine (DA) receptor D1 (D1R) and DA receptor D2 (D2R) were both upregulated in myofibroblasts in lungs of IPF patients and a bleomycin (BLM)-induced mouse model. Then, we explored the safety and efficacy of DA, fenoldopam (FNP, a selective D1R agonist) and sumanirole (SMR, a selective D2R agonist) in reversing BLM-induced pulmonary fibrosis. Further data showed that DA receptor agonists exerted potent antifibrotic effects in BLM-induced pulmonary fibrosis by attenuating the differentiation and proliferation of fibroblasts. Detailed pathway analysis revealed that DA receptor agonists decreased the phosphorylation of Smad2 induced by TGF-β1 in primary human lung fibroblasts (PHLFs) and IMR-90 cells. Overall, DA receptor agonists protected mice from BLM-induced pulmonary fibrosis and may be therapeutically beneficial for IPF patients in a clinical setting.

Prevalence and prognostic impact of physical frailty in interstitial lung disease: A prospective cohort study.

Physical frailty is associated with increased mortality and hospitalizations in older adults. We describe the prevalence of physical frailty and its prognostic impact in patients with a spectrum of fibrotic interstitial lung disease (ILD). Patients with fibrotic ILD at the McMaster University ILD programme were prospectively followed up from November 2015 to March 2020. Baseline data were used to classify patients as non-frail (score=0), pre-frail (score=1-2) or frail (score=3-5) based on modified Fried physical frailty criteria. The association between physical frailty and mortality was assessed using time-to-event models, adjusted for age, sex, lung function and diagnosis using the ILD Gender-Age-Physiology (ILD-GAP) score. We included 463 patients (55% male, mean [SD] age 68 [11] years); 82 (18%) were non-frail, 258 (56%) pre-frail and 123 (26%) frail. The most common ILD diagnoses were idiopathic pulmonary fibrosis (n=183, 40%) and connective tissue disease-associated-ILD (n=79, 17%). Mean time since diagnosis was 2.7 ± 4.6 years. There were 56 deaths within the median follow-up of 1.71 (interquartile range [IQR] 1.24, 2.31) years. Both frail and pre-frail individuals had a higher risk of death compared to those categorized as non-frail at baseline (adjusted hazard ratio [aHR] 4.14, 95% CI 1.27-13.5 for pre-frail and aHR 4.41, 95% CI 1.29-15.1 for frail). Physical frailty is prevalent in patients with ILD and is independently associated with an increased risk of death. Assessment of physical frailty provides additional prognostic value to recognized risk scores such as the ILD-GAP score, and may present a modifiable target for intervention.

ThOracic Ultrasound in Idiopathic Pulmonary Fibrosis Evolution (TOUPIE): research protocol of a multicentric prospective study

IntroductionIdiopathic pulmonary fibrosis (IPF) is the most common and severe interstitial lung disease (ILD). It is a progressive disease that requires a regular follow-up: clinical examination, pulmonary function testing (PFT)...

Clinical, functional, and computed tomography findings in a cohort of patients with neuroendocrine cell hyperplasia of infancy.

Neuroendocrine cell hyperplasia of infancy (NEHI) is one of the most common interstitial lung diseases in children. Both the etiology and pathophysiological mechanisms of the disease are still unknown. Prognosis is usually favorable; however, there are significant morbidities during the early years of life. To describe the clinical course, infant pulmonary function tests and computed tomography (CT) findings in a cohort of patients with NEHI in Argentina. This is a observational multicenter cohort study of children diagnosed with NEHI between 2011 and 2020. Twenty patients participated in this study. The median age of onset of symptoms was 3 months and the median age at diagnosis was 6 months. The most common clinical presentation was tachypnea, retractions and hypoxemia. The chest CT findings showed central ground glass opacities and air trapping. Infant pulmonary function tests revealed an obstructive pattern in 75% of the cases (10/12). Most patients (75%) required home oxygen therapy for 17 months (interquartile range 12-25). In 85% of them, tachypnea and hypoxemia spontaneously resolved between the second and third years of life. In this cohort, the first symptoms appeared during the early months of life. The typical clinical, CT, and functional findings allowed the diagnosis without the need of a lung biopsy. Although most patients required home oxygen therapy, they showed a favorable evolution.

Distribution of interstitial lung diseases in a tertiary care centre of South India

Background: The term interstitial lung diseases (ILD) refer to a broad category of lung diseases rather than a specific disease entity. True prevalence of ILD is difficult to estimate and it may vary according to the geography, environment, occupation etc. Aim and objectives were to find out the distribution of interstitial lung disease (ILD) subtypes in a tertiary care centre. Methods: A descriptive study was conducted between August 2016 and November 2018 in a tertiary care centre in Puducherry, South India. Baseline demographic details, clinical symptoms, signs, radiological findings (chest radiograph and HRCT), pathological findings, and physiological findings were taken into consideration and diagnosis of type of interstitial lung disease was made using multidisciplinary discussion. Statistical analysis was done using SPSS 19.0 version.Results: A total of 150 patients were recruited of which 70.5% were females. Most common ILD subtype in our study was connective tissue disease associated ILD – 97 patients (65%) followed by idiopathic pulmonary fibrosis (IPF)–31 patients (22%). Most common type of CTD ILD observed in our study was progressive systemic sclerosis (46%) followed by mixed connective tissue disease (24%). The most common HRCT finding was NSIP pattern and most commonly observed physiological abnormality was moderate restriction and moderate diffusion impairment. Conclusions: Connective tissue disease-associated ILD was the most common ILD found in our study amongst south Indian population. This suggests that the distribution of ILD would vary depending on the geographical area and the environmental exposure which was in contrast with the Indian ILD registry.

LncRNA SNHG16 promotes pulmonary fibrosis by targeting miR-455-3p to regulate the Notch2 pathway

BackgroundIdiopathic pulmonary fibrosis (IPF) is the most common interstitial lung diseases with a poor prognosis. Long non-coding RNAs (lncRNAs) have been reported to be involved in IPF in several studies. However, the role of lncRNA SNHG16 in IPF is largely unknown.MethodsFirstly, experimental pulmonary fibrosis model was established by using bleomycin (BML). Histology and Western blotting assays were used to determine the different stages of fibrosis and expression of several fibrosis biomarkers. The expression of SNHG16 was detected by quantitative real-time polymerase chain reaction (qRT‐PCR). EdU staining and wound-healing assay were utilized to analyze proliferation and migration of lung fibroblast cells. Molecular mechanism of SNHG16 was explored by bioinformatics, dual-luciferase reporter assay, RNA immunoprecipitation assay (RIP), and qRT-PCR.ResultsThe expression of SNHG16 was significantly up-regulated in bleomycin-(BLM) induced lung fibrosis and transforming growth factor-β (TGF-β)-induced fibroblast. Knockdown of SNHG16 could attenuate fibrogenesis. Mechanistically, SNHG16 was able to bind and regulate the expression of miR-455-3p. Moreover, SNHG16 also regulated the expression of Notch2 by targeting miR-455-3p. Finally, SNHG16 could promote fibrogenesis by regulating the expression of Notch2.ConclusionTaken together, our study demonstrated that SNHG16 promoted pulmonary fibrosis by targeting miR-455-3p to regulate the Notch2 pathway. These findings might provide a novel insight into pathologic process of lung fibrosis and may provide prevention strategies in the future.

Buccal Hydromorphone Syrup for Managing Dyspnea in Idiopathic Pulmonary Fibrosis.

Dyspnea is a highly distressing symptom that characterizes idiopathic pulmonary fibrosis (IPF), a common idiopathic interstitial lung disease (ILD) with a high symptom burden, poor quality of life, and early mortality. Though opioids are mentioned in guidelines for dyspnea management, guidance on how and when to initiate opioids is lacking. Different pharmacologic strategies are needed to address different types of dyspnea (baseline, incident, and crisis). Due to a longer onset of action, the oral route (swallowed) may be less effective for prevention of incident dyspnea or for rapid relief of crisis dyspnea, prompting the use of alternative drug delivery strategies for self-management. We inadvertently discovered the efficacy of buccal administration of low dose, low volume hydromorphone oral syrup for dyspnea management in ILD, which has not been previously reported in the literature. We describe our approach to dyspnea assessment and management in IPF, including use of the Multidimensional Dyspnea Scale (MDDS), a novel instrument yet to be validated that we developed to better identify and categorize dyspnea into the types experienced by a patient with IPF over the course of a day. We then describe how buccal hydromorphone oral syrup is initiated and titrated for dyspnea management in 3 patients at different points in their disease trajectory. Buccal hydromorphone oral syrup is effective for dyspnea management across the spectrum of IPF. When integrated into a patient-centered algorithm for symptom assessment and management, it allows for rapid and easy self-management of dyspnea by patients and their caregivers.

English

BACKGROUND Diffuse interstitial lung disease is on the rise in recent times. This could be attributed to number of causes / reasons. Environmental pollution is one of the important causes for the upswing. Increased exposure to inorganic dust, toxic fumes, certain drugs (gold, amiodarone, antibiotics etc.) and radiation can also cause ILD (Interstitial Lung Disease). METHODS This is a hospital based prospective observational study, conducted in the Department of Radiodiagnosis, NSCB Medical College and Hospital, Jabalpur. This study comprised of a total of 50 patients clinically diagnosed interstitial lung disease who had undergone x-ray chest and HRCT (High-Resolution Computed Tomography) Chest examination. RESULTS Incidence of interstitial lung disease was maximum in the middle age group of 51 – 60 years (38 %) with male predominance (58 %). Smoking habit was seen to be associated with 52 % of the cases suggesting association. 8 patients (16 %) had developed diffuse lung disease secondary to connective tissue disorder (rheumatoid arthritis, progressive systemic sclerosis and polymyositis). A confident diagnosis was made on the basis of chest radiograph alone in 11 patients (29 %). Among cases evaluated by HRCT Chest, 41 (82 %) cases showed specific patterns of ILD and nine cases (18 %) showed nonspecific patterns and were classified under unclassified IIP (Idiopathic Interstitial Pneumonia). Out of 41 cases, the most common ILD reported was IPF (20) followed by cases of idiopathic NSI, 8 cases of collagen vascular diseases, 2 cases suggestive of COP / BOOP, and smoking related ILDs were seen in 4 cases, and 2 cases of sarcoidosis were seen in the study. Occupational- and radiation-induced-ILD were seen in one case each. CONCLUSIONS Chest radiography is the initial imaging investigation in the evaluation of diffuse interstitial lung disease. HRCT is the imaging modality of choice to assess interstitial lung disease. It can detect lesion even when the chest radiograph is normal. HRCT can confirm the location, extent of disease, and pattern of disease. HRCT helps in the confident diagnosis of ILD as compared to chest radiography. Thus, it avoids lung biopsy in a large number of patients. HRCT is also useful in assessing the disease reversibility. Follow up of patients is better done by HRCT. KEYWORDS HRCT, Interstitial Lung Disease, Chest X-Ray, Ground Glass Opacity, Honeycombing

Ethnic influence on the phenotype of French patients with systemic sclerosis.

Systemic sclerosis (SSc) is a rare multisystem autoimmune disorder. It has a worldwide distribution but geographical and ethnic influences are poorly known. The aim of the study was to compare demographic characteristics and frequency of internal organ system involvement of Black SSc patients to those of White SSc patients in France. Patient population included 425 SSc patients recruited at Cochin Hospital in Internal medicine and Rheumatology departments. Data were collected at the baseline visit, each Black patient was matched with 2 to 3 White controls from the same department. One hundred and five Black patients and 320 White were included. Demographic comparison highlighted an older age for the White patients (48.66±14.87 vs 39.56±10.79, P<0.0001). Phenotypic comparison showed more severe skin involvement for Black patients: they had more often diffuse skin involvement than White patients (69.2% vs. 44.7%, P<0.0001) with a higher baseline modifiedRodnan skin score (15.8 vs. 11.3, P<0.001). Comparisons also showed more active ulcers (46.5% vs. 21.6%, P<0.001) and more common interstitial lung disease (73.7% vs. 43%, P<0.0001) for Black patients. Auto-antibody testing showed that White patients were more likely to harbor anti-centromere antibodies (ACA) (26.6% vs. 9%, P<0.001) whereas Black patients were more likely to have anti-U1RNP antibody (24.6% vs. 6.2%, P<0.0001). In this population recruited in a disease referral center, Black patients had more severe skin and lung involvements with lower prevalence of ACA as compared to White patients, supporting a more severe phenotype.

Complications in Idiopathic Pulmonary Fibrosis: Focus on Their Clinical and Radiological Features.

Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease with uncertain origins and pathogenesis; it represents the most common interstitial lung disease (ILD), associated with a pathological pattern of usual interstitial pneumonitis (UIP). This disease has a poor prognosis, having the most lethal prognosis among ILDs. In fact, the progressive fibrosis related to IPF could lead to the development of complications, such as acute exacerbation, lung cancer, infections, pneumothorax and pulmonary hypertension. Pneumologists, radiologists and pathologists play a key role in the identification of IPF disease, and in the characterization of its complications—which unfortunately increase disease mortality and reduce overall survival. The early identification of these complications is very important, and requires an integrated approach among specialists, in order to plane the correct treatment. In some cases, the degree of severity of patients having IPF complications may require a personalized approach, based on palliative care services. Therefore, in this paper, we have focused on clinical and radiological features of the complications that occurred in our IPF patients, providing a comprehensive and accurate pictorial essay for clinicians, radiologists and surgeons involved in their management.

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.

Derivation and Validation of a Diagnostic Prediction Tool for Interstitial Lung Disease

Interstitial lung disease (ILD) results in high morbidity and health-care utilization. Diagnostic delays remain common and often occur in nonpulmonology settings. Screening for ILD in these settings has the potential to reduce diagnostic delays and improve patient outcomes. This study sought to determine whether a pulmonary function test (PFT)-derived diagnostic prediction tool (ILD-Screen) could accurately identify incident ILD cases in patients undergoing PFT in nonpulmonology settings. Clinical and physiologic PFT variables predictive of ILD were identified by using iterative multivariable logistic regression models. ILD status was determined by using a multi-reader approach. An ILD-Screen score was generated by using final regression model coefficients, with a score≥ 8 considered positive. ILD-Screen test performance was validated in an independent external cohort and applied prospectively to PFTs over 1 year to identify incident ILD cases at our institution. Variables comprising the ILD-Screen were age, height, total lung capacity, FEV1, diffusion capacity, and PFT indication. The ILD-Screen showed consistent test performance across cohorts, with a sensitivity of 0.79 and a specificity of 0.83 when applied prospectively. A positive ILD-Screen strongly predicted ILD (OR, 18.6; 95%CI, 9.4-36.9) and outperformed common ILD clinical features, including cough, dyspnea, lung crackles, and restrictive lung physiology. Prospective ILD-Screen application resulted in a higher proportion of patients undergoing chest CT imaging compared with a historical control cohort (74%vs56%, respectively; P= .003), with a significantly shorter median time to chest CT imaging (5.6 vs21.1months; P< .001). The ILD-Screen showed good test performance in predicting ILD across diverse geographic settings and when applied prospectively. Systematic ILD-Screen application has the potential to reduce diagnostic delays and facilitate earlier intervention in patients with ILD.