Common Interstitial Lung Disease Research Articles

Current Diagnosis and Management of Hypersensitivity Pneumonitis.

Hypersensitivity Pneumonitis (HP) one of the most common interstitial lung diseases (ILDs) is characterized by exposure to an inhaled inciting antigen that leads to a host immunologic reaction determining interstitial inflammation and architectural distortion. The underlying pathogenetic mechanisms are unclear. The absence of international shared diagnostic guidelines and the lack of a “gold-standard” test for HP combined with the presence of several clinical and radiologic overlapping features makes it particularly challenging to differentiate HP from other ILDs, also in expert contests. Radiology is playing a more crucial role in this process; recently the headcheese sign was recognized as a more specific for chronic-HP than the extensive mosaic attenuation. Several classification proposals and diagnostic models have been advanced by different groups, with no prospective validation. Therapeutic options for HP have been limited to antigen avoidance and immunosuppressant drugs over the last decades. Several questions about this condition remain unanswered and there is a need for more studies.

Surgical treatment of lung cancer patients with idiopathic pulmonary fibrosis

Lung cancer is the leading cause of death in malignant tumors. Surgery is one of the main treatments for early stage lung cancer. Idiopathic pulmonary fibrosis (IPF), one of the most common interstitial lung disease, is a chronic, progressive lung disease, characterized by insidious onset and progressive deterioration. It is reported that there are common pathways in idiopathic pulmonary fibrosis and lung cancer, and patients with IPF have a higher risk of lung cancer than the general population, whose prevalence was 3.34 times than that of the general population. The incidence of acute exacerbations of IPF and postoperative mortality was significantly higher in lung cancer patients complicated with IPF than those without IPF. The long-term outcomes of patients with IPF are dramatically worse than those without IPF. It was concluded that lobectomy has been still the standard surgical procedure for such patients, but sublobectomy should be taken as potential alternative choice for patients with high risk after preoperative evaluation. Moreover, further studies should be conducted on the prevention and treatment of acute exacerbation of IPF after surgery.

TPA promotes the proliferation of lung fibroblasts and activates the Wnt/β-catenin signaling pathway in idiopathic pulmonary fibrosis

ABSTRACT Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, irreversible and the most common fatal interstitial lung disease, which is characterized by damaged alveolar structure, the massive proliferation of fibroblasts and deposition of extracellular matrix (ECM). While the pathogenesis of IPF remains unclear, it has been clearly established that the excessive proliferation of lung fibroblasts is the most direct cause of fibrogenesis. Numerous proliferating fibroblasts form fibrous foci and secrete a large amount of ECM to aggravate the process of pulmonary fibrosis. Tissue plasminogen activator (tPA) is a kind of serine protease, its main function is to activate zymogens into active enzymes involved in fibrinolysis. Our study found tPA functioned as a cytokine to promote the proliferation of lung fibroblasts through intracellular signaling events involving Erk1/2, p90RSK, GSK-3β phosphorylation, and cyclinD1 induction. We also uncovered that tPA indirectly activated the Wnt/β-catenin signaling pathway by regulating the GSK-3β phosphorylation level. It’s well-known that Wnt/β-catenin signaling pathway plays an important role in the pathogenesis of pulmonary fibrosis, in which the accumulation of β-catenin in the cytoplasm is an important signal of the activation of Wnt/β-catenin signaling pathway. Our study unveiled that tPA can serve as a cytokine involved in Wnt/β-catenin signaling pathway and be implicated in pulmonary fibrosis.

Interstitial Lung Disease: Update on the Role of Computed Tomography in the Diagnosis of Idiopathic Pulmonary Fibrosis.

Idiopathic pulmonary fibrosis is the commonest interstitial lung disease. Radiologists play an essential role in making an accurate diagnosis, and this is necessary given its prognostic implications and potential use of antifibrotic therapies. This review highlights the radiologic features and imaging classification of fibrosis with reference to recently updated international guidelines and emphasizing the importance of the multidisciplinary team in idiopathic pulmonary fibrosis diagnosis and, in particular, when pathological sampling is required.

Hypersensitivity pneumonitis recognised in a single pulmonary unit, between 2005 and 2015 - comparison with recently proposed diagnostic criteria.

Hypersensitivity pneumonitis (HP) is the third most common interstitial lung disease after idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Pathogenesis of HP is related to repeated exposure to inhaled environmental antigens that sensitise the susceptible, genetically predisposed persons. The aim of the present retrospective study was to summarise the diagnostic methods used in consecutive patients with HP, recognised in a single pulmonary unit, between 2005 and 2015, and to compare them with current diagnostic criteria. 135 patients, 68 males, 67 females, median age 53 years (18-75 years), entered the study. Chest CT features characteristic of HP were defined as: mosaic attenuation of lung parenchyma, air trapping and/or ill-defined centrilobular nodules. Lymphocytosis in BAL was defined as ≥ 30%. Median time from first symptoms to diagnosis was 12 months. The exposure to one or more allergens was found in 94% of patients, chest CT features characteristic of HP have been reported in 87%, BAL lymphocytosis - in 86%. According to recent diagnostic criteria - in 54% of patients, clinical diagnosis of HP was confident, in 16% - probable, in 26% - possible and in 4% - unlikely. The confirmation of HP with lung biopsy has been obtained in 36% of non-confident cases (16% of the study group). HP diagnosis was confirmed according to current diagnostic criteria in 70% of patients diagnosed between 2005 and 2015. Contradictions to lung biopsy have been the main reason for inability to confirm HP in non-confident cases.

Long-Term Effects and Adverse Events of Nintedanib Therapy in Idiopathic Pulmonary Fibrosis Patients with Functionally Advanced Disease.

Idiopathic pulmonary fibrosis (IPF) is one of the most common interstitial lung diseases with limited survival. The effect of IPF therapy in patients with severely impaired lung function is not well established. The aim of this study was to characterize IPF patients with a forced vital capacity (FVC) < 50% (group 1) and FVC 50-60% predicted (group 2) and analyze the effect and adverse events of nintedanib in Hungarian patients diagnosed between April 2015 and July 2017. The impact of nintedanib therapy on lung function, survival, and adverse events was analyzed longitudinally. Twenty-two out of 103 patients were included in the analysis (group 1: N = 10; male/female = 6:4, age 62.6 ± 10.8years and group 2: N = 12; male/female = 3:9, age 65.7 ± 11.6years). Eighteen patients were treated with nintedanib (8 in group 1, 10 in group 2); treatment stabilized lung function in 42% and 50%, respectively, in the two groups. Median survival was 444days for group 1 and 476days for group 2. Adverse events were less common than in clinical trials; dose reduction was necessary in three cases, drug discontinuation in two cases. No differences between groups were identified regarding clinical parameters and radiological pattern; however, hypertension as comorbidity was more common in group 1 patients. Nintedanib therapy was effective and well tolerated even among patients with severely impaired lung function. Longitudinal follow-up confirmed high mortality in patients with very severe and severe IPF; however, median survival was meaningful as it exceeded 1year in both groups.

Evaluation and management of Idiopathic Pulmonary Fibrosis.

Idiopathic Pulmonary Fibrosis (IPF) is a most common progressive interstitial lung disease (ILD) of unknown etiology, although majority of patients are elderly male smokers. The main pathogenesis is aberrant recovery of epithelial injury and collagen deposition. Fibrotic nonspecific interstitial pneumonia, connective tissue disease (CTD) especially rheumatoid arthritis (RA) associated ILD, and chronic hypersensitivity pneumonia(CHP) are important differential diagnosis. Main symptoms are non-productive cough and progressive exertional dyspnea. Crucial physical findings are scalene muscle hypertrophy, bibasilar fine crackles, and finger clubbing. The serum markers such as lactate dehydrogenase (LDH) and Krebs von den Lungen-6 (KL-6) are sensitive for ILD detection and activity. Both pulmonary function test (PFT) and the 6-minute walk test (6MWT) are useful tool for evaluation of disease progression of IPF. Serial changes of forced vital capacity (FVC) and 6MWT distance predict mortality in IPF effectively. Recently published international IPF guidelines highlight the importance of chest high resolution computed tomography (HRCT) findings such as honeycombing, traction bronchiectasis (TBE), and sub-pleural reticular opacity. IPF is chronic and progressive; therefore, tracking disease behavior is crucial. Unifying clinical, physiological, and imaging information over time is useful. With regard to its management, two anti-fibrotic drugs such as pirfenidone and nintedanib have been available. These drugs can slow the decline of FVC and prevent acute exacerbation (AE). In this review, I outline the clinical characteristics of IPF, physiological, imaging, pathological findings and review diagnosis process and management.

Niclosamide alleviates pulmonary fibrosis in vitro and in vivo by attenuation of epithelial-to-mesenchymal transition, matrix proteins & Wnt/β-catenin signaling: A drug repurposing study

Drug repurposing off late has been emerging as an inspiring alternative approach to conventional, exhaustive and arduous process of drug discovery. It is a process of identifying new therapeutic values for a drug already established for the treatment of a certain condition. Our current study is aimed at repurposing the old anti-helimenthic drug Niclosamide as an anti-fibrotic drug against pulmonary fibrosis (PF). PF is most common lethal interstitial lung disease hallmarked by deposition of extracelluar matrix and scarring of lung. Heterogenous nature, untimely diagnosis and lack of appropriate treatment options make PF an inexorable lung disorder. Prevailing void in PF treatment and drug repositioning strategy of drugs kindled our interest to demonstrate the anti-fibrotic activity of Niclosamide. Our study is aimed at investigating the anti-fibrotic potential of Niclosamide in TGF-β1 induced in vitro model of PF and 21-day model of Bleomycin induced PF in vivo respectively. Our study results showed that Niclosamide holds the potential to exert anti-fibrotic effect by hampering fibroblast migration, attenuating EMT, inhibiting fibrotic signaling and by regulating WNT/β-catenin signaling as evident from protein expression studies. Our study findings can give new directions to development of Niclosamide as an anti-fibrotic agent for treatment of pulmonary fibrosis.

The value of serum precipitins against specific antigens in patients diagnosed with hypersensitivity pneumonitis - retrospective study.

IntroductionHypersensitivity pneumonitis (HP) is the third most common interstitial lung disease, and is often under-recognized, especially in patients who are not aware of their occupational or environmental contact with organic antigens. The aim of the present study was to assess the results of serum specific IgG antibodies (ssIgG) in HP patients and their correlation with clinical data.Material and methods128 HP patients, median age 53 years, participated in the study. The control group consisted of 102 patients with interstitial lung diseases (ILDs) other than HP. Assessment of pretreatment ssIgG to thermophilic actinomycetes and protein antigens from bird droppings (pigeons, hens, ducks, parrots, turkeys) was performed by double diffusion in agar gel according to Ouchterlony method.ResultsPositive precipitins were obtained in 57% of all HP patients and in 61% of those exposed to above mentioned antigens. Positive results in the control group were obtained in 7% of patients. Sensitivity of ssIgG in HP group was 0.57 and specificity 0.93. Precipitins to at least one bird antigen was confirmed in 64% of HP patients exposed to birds. Precipitins to thermophilic bacteria were found in 29% of HP patients exposed to hay or hay products.ConclusionsThe results of the study indicate that ssIgG against birds’ allergens were the valuable diagnostic tool in HP patients. Low-rate of confirmation of ssIgG to thermophilic bacteria in patients exposed to hay or hay products indicate that other microorganisms, most likely molds, could be responsible for the disease development.

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.

Interstitial Lung Disease and Pulmonary Fibrosis: A Practical Approach for General Medicine Physicians with Focus on the Medical History.

Interstitial lung disease (ILD) and pulmonary fibrosis comprise a wide array of inflammatory and fibrotic lung diseases which are often confusing to general medicine and pulmonary physicians alike. In addition to the myriad of clinical and radiologic nomenclature used in ILD, histopathologic descriptors may be particularly confusing, and are often extrapolated to radiologic imaging patterns which may further add to the confusion. We propose that rather than focusing on precise histologic findings, focus should be on identifying an accurate etiology of ILD through a comprehensive and detailed medical history. Histopathologic patterns from lung biopsy should not be dismissed, but are often nonspecific, and overall treatment strategy and prognosis are likely to be determined more by the specific etiology of ILD rather than any particular histologic pattern. In this review, we outline a practical approach to common ILDs, highlight important aspects in obtaining an exposure history, clarify terminology and nomenclature, and discuss six common subgroups of ILD likely to be encountered by general medicine physicians in the inpatient or outpatient setting: Smoking-related, hypersensitivity pneumonitis, connective tissue disease-related, occupation-related, medication-induced, and idiopathic pulmonary fibrosis. Accurate diagnosis of these forms of ILD does require supplementing the medical history with results of the physical examination, autoimmune serologic testing, and chest radiographic imaging, but the importance of a comprehensive environmental, avocational, occupational, and medication-use history cannot be overstated and is likely the single most important factor responsible for achieving the best possible outcomes for patients.

Clinicoradiological and demographic pattern in diffuse parenchymal lung diseases: An observational study

Introduction: Diffuse parenchymal disease is a group of lung disease affecting alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissue. It includes many diseases like IPF, Sarcoidosis, BOOP, COP, LAM etc. it is still under diagnosed entirety. Many patients die due to lack of awareness and investigating modalities. Aims and objective: This study is to see Demographic pattern of DPLD, its clinical pattern and radiological findings in different type of ILDs. Study Type: It is a Prospective Observational Study done over a period of 1 year. Material & Method: Patients studied over20 years of age and having respiratory symptoms. We excluded patients of other diseases like COPD, Tuberculosis etc. Total 75 patients were diagnosed as a case of DPLD by clinical findings, and investigations like X ray chest, HRCT thorax, Bronchoscopy. Results: Most common ILD (Interstitial lung Disease) is IPF 40.0% followed by Sarcoidosis etc. Most common clinical symptom was breathlessness (100.00%) in IPF, Fever & Chest Pain are common in Granulomatous type. Conclusion: DPLD (Diffuse Paranchymal lung Disease) is a chronic lung disease characterized by progressive decline in lung function. IPF is most common in DPLD and leads to fast deterioration of lung function. HRCT (High Resolution computed tomography) is very important to assess type of disease. Spirometry, Broncoscopy are important for diagnosis of ILD and lung function.

Idiopathic pulmonary fibrosis: a clinical update.

Idiopathic pulmonary fibrosis (IPF) is the commonest interstitial lung disease (ILD) and is characterised by progressive scarring of the lungs. It typically presents in the sixth or seventh decade.1 The prognosis of IPF is poor and is worse than most cancers, with a median survival from diagnosis of 2.5–3.5 years.1 In 2012, over 5000 people died of IPF in the UK accounting for 1% of all deaths and 4.6% of respiratory deaths.2 On average, a general practice of 10 000 patients will see 2–3 new cases every 3 years,3 although this is likely to be an underestimate, contrasting with 500 per 10 000 for chronic obstructive pulmonary disease (COPD). The rising incidence and prevalence means that GPs will be pivotal in the early detection and long-term management of patients with IPF. Patients with IPF typically present first to their GP. Distinguishing these symptoms from more common conditions such as COPD can be challenging. A history of persistent progressive breathlessness and/or cough in a patient >45 years who may, but not exclusively, have velcro crackles on auscultation and finger clubbing should warrant further management with radiological imaging (chest X-ray and high-resolution computed tomography [Figure 1]) and/or referral to an IPF specialist. …

Diagnosis and differential diagnosis of pulmonary sarcoidosis

Pulmonary sarcoidosis is one of the most common interstitial lung diseases. The prevalence in Russia is 10-20 per 100 thousand population. Overwhelmingly it is found among working age population, and the peak of morbidity is 30-40 years. In half of the cases, sarcoidosis occurs in oligosymptomatic form. According to research conducted by our scientists, the adverse course of the disease with exacerbations and progressive decrease in pulmonary function found in 27,3% of patients. The diagnosis is based on clinical, X-ray and histological examination. Material for histological study was prepared depending on the extent of the process. Preference is given to minimally invasive methods of biopsy: transbronchial puncture of intrathoracic lymph nodes, peripheral lymph nodes, bone marrow aspiration, skin sarcoid items, etc. In the case of the intrathoracic localization of the disease, the treatment of choice is videothoracoscopy. The detection of epithelioid cell granuloma without necrosis confirms the diagnosis of sarcoidosis. First of all it is necessary to exclude tuberculosis, oncohematological diseases, disseminated lung diseases, combined with alveolitis syndrome and mediastinal lymphadenopathy. The primary diagnosis should be carried out under conditions of a multifunctional clinic. The verification of the pulmonary sarcoidosis should be held in close cooperation of different specialists: pulmonologists, radiologists, thoracic surgeons, pathologists, etc. Further treatment of the patient should be implemented by the pulmonologist in the place of residence. A clinical case of late diagnosis of diffuse diseases of connective tissue, which at its beginning started under the mask of sarcoidosis is demonstrated. The main mistake that led to deep disability of the patient, was absence of timely histological verification of the diagnosis.

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.

Spontaneous Pneumomediastinum in Connective Tissue Diseases

SESSION TITLE: Systemic Disease and Pulmonary Manifestations SESSION TYPE: Original Investigation Poster PRESENTED ON: Wednesday, November 1, 2017 at 01:30 PM - 02:30 PM PURPOSE: Spontaneous pneumomediastinum (SP) is an uncommon manifestation of connective tissue diseases (CTD) but has been associated with the diagnosis of dermatomyositis/polymyositis (DM/PM). We aimed to explore this relationship by identifying consecutive adult patients with CTD and SP and analyzing the presenting features and clinical course associated with this complication. METHODS: Using a computer-assisted search, we retrospectively identified and reviewed the medical records of all CTD patients diagnosed with SP between January 1997 and December 2016. We extracted data regarding demographics, clinical manifestations and treatment of the underlying CTD. We also gathered information regarding clinical-radiological presentation and treatment of SP, and its outcome. Chest CT studies were reviewed to investigate the presence and describe the pattern of the underlying interstitial lung disease (ILD). RESULTS: We identified 27 patients affected by CTD who developed SP (8 cases of DM/PM, 6 rheumatoid arthritis, 6 systemic sclerosis, 4 overlap syndromes, 2 systemic lupus erythematosus and 1 unclassifiable CTD). Median age at SP presentation was 53 (range 18-82) years and 59% were women. Sixty-two per cent of patients were never smokers, 27% were former smokers and 11% were current smokers. SP occurred before the diagnosis of CTD in 3 patients while SP led to the diagnosis of CTD in 5 additional patients; remaining 19 patients (70%) had a known CTD including associated ILD in all but one patient. SP-related symptoms were present in 58% of patients and included dyspnea (46%), followed by chest pain (27%) and cough (12%); 42% were asymptomatic. The precipitating factors included pulmonary function testing in 31% of patients, severe coughing due to exacerbation or respiratory infection in 15%, vomiting in 8%, air travel in 8%; in 46% no potential precipitating factors were identified. Seventy-eight per cent of patients were on immunosuppressant therapy at the time of SP onset. The initial diagnosis of SP was achieved by chest radiography in 20% of cases, chest CT in 64% and CT pulmonary angiography in 16%. Concomitant pneumothorax was present in 4 cases (17%). Only 2 cases (8%) of SP occurred in the absence of CTD-associated interstitial lung disease (ILD). The most common ILD pattern on chest CT was NSIP in 45%, followed by BOOP superimposed on NSIP in 18%, unclassifiable ILD in 23%, and possible UIP in 5%. SP was treated with observation alone in 23 cases (88%). Eleven patients (42%) were hospitalized. Chest tube drainage was instituted in one of 4 patients with pneumothorax. The median hospital stay was 11 (range 1-20) days. There were no deaths attributable to SP nor were there any relapses of SP in a median follow up of 14 (range 0-183) months. The cumulative survival at 1 and 2 years was 56% and 44% respectively. CONCLUSIONS: SP is an uncommon manifestation of CTD related ILD but sometimes it can lead to the diagnosis of CTD. SP is associated with a relatively benign course in patients with CTD. CLINICAL IMPLICATIONS: SP is an uncommon manifestation seen in patients with CTD and can usually be managed conservatively with observation alone. DISCLOSURE: The following authors have nothing to disclose: Federica De Giacomi, Misbah Baqir, Christian W. Cox, Jay Ryu No Product/Research Disclosure Information

Lung inflammation after bleomycin treatment in mice: Selection of an accurate normalization strategy for gene expression analysis in an ex-vivo and in-vitro model

Pulmonary fibrosis (PF) is the most common and aggressive interstitial lung disease, characterized by a patchy development of fibrosis leading to progressive destruction of the normal lung architecture which is preceded by an inflammatory process. Gene expression studies are important to understand the development of PF but the accuracy and reproducibility of Real-Time PCR depend on appropriate normalization strategies. This study aimed to analyze the expression variability of eight commonly used reference genes during the initial inflammatory phase of bleomycin-induced PF in a mouse model and to verify whether the selected reference genes could be applied to an in-vitro model of BLM-treated primary murine lung fibroblasts. Wild-type C57BL/6 mice (n=40) were used. Real-Time PCR was carried out on lung tissue of mice either BLM (BLM-tm) or physiological solution-treated (PSS-tm), and in primary lung fibroblasts, isolated from healthy C57BL/6 mice. Histological analysis was performed to confirm the inflammation development. During inflammation, the most stable genes resulted: PPIA, HPRT-1 and SDHA for both models; the normalization strategy was tested analyzing mRNA expression of PTX-3 and TNF-α which resulted up-regulated both in ex-vivo and in-vitro with respect to PSS-tm/fibroblasts. Histological analysis supported the results. This study identified a new set of reference genes expressed both in the in-vitro and ex-vivo models. A higher expression of both markers in BLM-tm with respect to PSS-tm indicated that BLM might lead to increased PTX-3 local production by a co-regulation with TNF-α at lung level.

High resolution computed tomography in the evaluation of interstitial lung diseases: Imaging perspective revisited with review of literature

Aims and Objectives: The study was designed with a broad objective of studying the imaging findings of the common interstitial lung diseases and to assess the severity of the disease and determine the reversibility capability of the pulmonary parenchymal damage in ILD’s. Patients and Methods: This was a prospective, observational study in which we evaluated 50 patients suspected of having interstitial lung diseases based on radiographic or clinical findings, by high resolution computed tomography (HRCT) over a period of one year. Results: Out of the 50 patients, majority of them had idiopathic pulmonary fibrosis (n=16) followed by interstitial pulmonary involvement in patients of rheumatoid arthritis (n=13), scleroderma (n=10) and systemic lupus erythematosis (n=9). The less common ILD’s included were Occupational lung disease (n=1) and Mycosis fungoides (n=1). Conclusion: HRCT of the lung in cases of the suspected interstitial lung diseases forms an invaluable tool for accurate and early identification and in conjunction with the clinical findings can obviate the need of lung biopsy in diagnosis of ILD’s.

High-resolution computed tomography and magnetic resonance imaging protocols in the diagnosis of fibrotic interstitial lung disease: overview for "non-radiologists".

Fibrotic interstitial lung diseases (ILDs) include a number of pulmonary disorders characterized by infiltration of inflammatory cells in lung parenchyma and fibrosis resulting in decreased lung compliance. Idiopathic pulmonary fibrosis (IPF) represents the most common ILD. ILDs can be divided in two anatomo-pathological and radiographic patterns: usual interstitial pneumonitis (UIP) and non-specific interstitial pneumonitis (NSIP). The different radiological features of UIP and NSIP are discussed. The American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association guidelines for the diagnosis and management of IPF have identified several characteristic high-resolution computed tomography (HRCT) features of UIP. However, even if these guidelines recommend to avoid surgical lung biopsy in case of confident UIP diagnosis on HRCT, they present some limitations, the most important of which is represented by interobserver agreement. Magnetic resonance imaging (MRI) can be considered as a radiation-free alternative to HRCT for several lung diseases. However, the clinical value of MRI for IPF diagnosis remains to be proven. (Sarcoidosis Vasc Diffuse Lung Dis 2017; 34: 300-306).

Heterologous Matrix Metalloproteinase Gene Promoter Activity Allows In Vivo Real-time Imaging of Bleomycin-Induced Lung Fibrosis in Transiently Transgenized Mice.

Idiopathic pulmonary fibrosis is a very common interstitial lung disease derived from chronic inflammatory insults, characterized by massive scar tissue deposition that causes the progressive loss of lung function and subsequent death for respiratory failure. Bleomycin is used as the standard agent to induce experimental pulmonary fibrosis in animal models for the study of its pathogenesis. However, to visualize the establishment of lung fibrosis after treatment, the animal sacrifice is necessary. Thus, the aim of this study was to avoid this limitation by using an innovative approach based on a double bleomycin treatment protocol, along with the in vivo images analysis of bleomycin-treated mice. A reporter gene construct, containing the luciferase open reading frame under the matrix metalloproteinase-1 promoter control region, was tested on double bleomycin-treated mice to investigate, in real time, the correlation between bleomycin treatment, inflammation, tissue remodeling and fibrosis. Bioluminescence emitted by the lungs of bleomycin-treated mice, corroborated by fluorescent molecular tomography, successfully allowed real time monitoring of fibrosis establishment. The reporter gene technology experienced in this work could represent an advanced functional approach for real time non-invasive assessment of disease evolution during therapy, in a reliable and translational living animal model.