Case Of Idiopathic Pulmonary Fibrosis Research Articles

TO STUDY CLINICORADIOLOGICAL PROFILE OF IDIOPATHIC PULMONARY FIBROSIS (IPF) PATIENTS

Background - The term "interstitial lung disease" (ILD), or diffuse parenchymal lung disease (DPLD), represents a group of about 200 distinct disorders. Idiopathic pulmonary brosis (IPF) is one of the most severe forms of IIP. This study aims to observe clinicoradiological prole of idiopathic pulmonary brosis patients. A cross-sectional observational study (inpatients and out Methods- patients) visiting the Department of Respiratory Medicine, Gandhi Medical College, Bhopal for the period of 18 months from November 2019 to April 2021, diagnosed to have Idiopathic Pulmonary Fibrosis (IPF) by HRCT who meet the inclusion and exclusion criteria. The incidence of IPF is higher in males as Resultcompared to females. It appears mainly between the fth and seventh decades of life ,the mean age of IPF was 58.86 ± 11.93, and the maximum number of patients came under the age of 51–60 years .In this study, breathlessness and cough were the predominant symptoms in denitive UIP. The severity of breathlessness is higher in denitive UIP. In our study every patient with IPF, we found to have an abnormal chest xray. Hence, chest xray should be the rst investigation in any suspected case of IPF. The commonest chest radiographic abnormality in patients with IPF is peripheral reticular opacity, most marked at the bases. In this study, the most common HRCT Chest ndings in IPF patients were reticulations, subpleural and basal involvement, and brosis. In this study, the most common pattern of idiopathi Conclusion- c pulmonary brosis was denitive UIP and most common age group was 51–60 years old and IPF reveals a male predominance. Clubbing was found in 39.4% of patient's maximum with denitive UIP (28.8%). On auscultation, the most common ndings are ne crackles and the most common ndings on HRCT are reticulations So, the nal diagnosis of IPF is a multidisciplinary approach. Further study is needed with a larger sample size to understand the disease in detail.

A Case of Idiopathic Pulmonary Fibrosis Diagnosed with Progression Post-COVID-19 Pneumonia

A Case of Idiopathic Pulmonary Fibrosis Diagnosed with Progression Post-COVID-19 Pneumonia

A clinical case of idiopathic pulmonary fibrosis against the background of comorbid pathology

Idiopathic pulmonary fibrosis (IPF) is one of the most common diseases in the group of interstitial lung diseases, which is characterized by persistent progression and poor prognosis. Over the past decade, experts have made significant progress in developing a diagnostic algorithm for IPF patients. This algorithm includes analysis of clinical, laboratory, and instrumental data, primarily the results of high-resolution computed tomography (HRCT). Precise adherence to the diagnostic algorithm and correct interpretation of HRCT data are prerequisites for IPF diagnosis.Specialists of the Tomsk region have developed routing of patients with suspected IPF. The presented clinical case is a successful example of adhering to this algorithm. Wide implementation of modern diagnostic algorithms into diagnosis and treatment of IPF and quality improvement of imaging methods, primarily HRCT, carried out as a part of the differential diagnosis, open up prospects for early diagnosis of this pathology. A timely prescribed antifibrotic therapy (nintedanib, pirfenidone) in IPF allows to slow down pathological progression and improves the prognosis.

Идиопатический легочный фиброз в практике врача-терапевта

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic progressive fibrosing interstitial pneumonia of unknown etiology that affects only the lungs and is associated with the histological and / or radiological pattern of common interstitial pneumonia. The clinical substrate for IPF is progressive dyspnea and dry cough. The characteristic auscultatory sign of IPF is inspiratory crepitus. Fibrosis is an important morphological component of IPF. The leading CT sign of IPF is a “honeycomb” lung (local air cysts located subpleurally and having similar sizes from 2–3 to 10 mm with clearly delineated walls). Mostly people over 60 years old are ill, often smokers, or with a history of smoking. In 60% of patients, the course of IPF is progressive, death within 5 years in 40% of cases. In 30–50% of cases, IPF requires the exclusion of other forms of interstitial lung disease. To date, it has been established that the basic drugs in the treatment of IPF with proven efficacy are nintedanib and pirfenidone. This article presents a clinical case of idiopathic pulmonary fibrosis in a 63-year-old patient who was hospitalized in the pulmonology department. The given example justifies the need for increased vigilance among pulmonologists and general practitioners when interpreting the clinical manifestations of the disease.

Feline Idiopathic Pulmonary Fibrosis

Background: Feline idiopathic pulmonary fibrosis is an irreversible disease that is rarely reported in veterinary clinical routine, with this case as the second reported in Brazil. This study aimed to describe a case of idiopathic pulmonary fibrosis in a domestic cat.Case: A 10-year-old female cat with a history of respiratory distress was treated at the Veterinary Hospital of the State University of Santa Cruz (HV-UESC). The first signs were observed three months before clinical care, characterized mainly by dyspnea that was more pronounced during nighttime. There was no history of previous illnesses or prior drug use. The cat lived with two other domiciled cats, did not have access to the street, and had restricted access to the terrace of the building, from where other animals could have possibly entered. Physical examination showed a poor body condition with a temperature of 37°C and dehydration estimated at 9%.The animal was observed to remain in an orthopedic position, with tachypnea (109 mvm), wheezing through the mouth, and severe expiratory dyspnea. Pulmonary auscultation detected fine discontinuous adventitious noises. There were no changes in the cardiac function in terms of rhythm, frequency, or auscultation. Oxygen therapy and slow administration of 0.9% NaCl solution were performed; moreover, blood was collected for complete blood count, in which no abnormalities were observed. Thoracic radiography was performed on the cat, and the results showed a mixed pulmonary pattern characterized by bronchiectasis, thickening of the bronchiolar wall, and an unstructured interstitial pattern throughout the pulmonary area. Shortly after the beginning of the clinical care, the animal died due to respiratory arrest and was necropsied. The main macroscopic finding was pulmonary edema. Lung samples were subjected to microscopic evaluation, which revealed extensive multifocal areas of alveolar septa thickening characterized by smooth muscle hyperplasia, hypertrophy associated with intense fibroplasia, type II pneumocyte hyperplasia, and discrete intra-alveolar and interstitial lymphohistiocytic inflammatory infiltrate. Based on the clinical, radiographic, macroscopic, and histopathological findings, the diagnosis of idiopathic pulmonary fibrosis was established. Discussion: This is the second case of feline idiopathic pulmonary fibrosis reported in Brazil. The observations in the physical examination, namely, the orthopedic positioning, dyspnea, and mouth breathing, are characteristic of respiratory distress and showed the severity of the lesions in the respiratory system. The clinical manifestation of idiopathic pulmonary fibrosis in cats reveals that gas exchange had already been compromised, which indicates an advanced stage of disease. In addition to respiratory signs, systemic signs such as apathy, anorexia, weight loss, and dehydration also confirm the severity of the condition. Because of the severity of the clinical condition at the time of the clinical care, it was not possible to adopt a more aggressive therapeutic approach, and the animal eventually died. Taken together, the clinical, radiographic, macroscopic, and histopathological findings led to the diagnosis of idiopathic pulmonary fibrosis. Literature states that the diagnosis of idiopathic pulmonary fibrosis requires clinical, radiographic, and histopathological findings consistent with the alterations observed in this cat. Much remains to be understood with regard to idiopathic pulmonary fibrosis in felines, since the currently available information is sparse and divergent. The description of these cases is extremely important to increase the available knowledge and to improve the prognosis and therapy for this serious disease.

A Case of Idiopathic Pulmonary Fibrosis with Different Radiological Involvement and Slow Course

A Case of Idiopathic Pulmonary Fibrosis with Different Radiological Involvement and Slow Course

An experience of therapy with nintedanib in patients with idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is the most common clinical variant of idiopathic interstitial pneumonia. Pathophysiology of IPF involves recurrent damage of the alveolar epithelium with fibroblast excessive proliferation as aberrant reparative activity of the lung interstitium. Currently, a treatment approach to this disease has been revised. Given new knowledge on pathogenesis of IPF, an international evidencebased clinical guideline published in 2015 recommends antifibrotic agents as the basic therapy of IPF. The only antifibrotic agent registered in Russia is niintedanib. A clinical case of IPF with the first experience of therapy with nintedanib in Omsk region has been reported in the article.

Idiopathic pulmonary fibrosis in a captive lion (Panthera leo): case report

Spontaneous idiopathic pulmonary fibrosis (IPF) was observed in a captive felid. Very little is known of this condition in captive wildlife. Conventional diagnostic tools including histological techniques were often employed in the diagnosis. This communication presents the first case of idiopathic pulmonary fibrosis in a captive lion in Ibadan, Nigeria. The lion was recumbent and inactive with random episodes of dyspnoea. On necropsy, the carcass was in a poor body condition. Grossly, the lung was non-collapsed, mottled and rubbery to firm in texture with numerous fine nodules (1–3 mm diameter). Cytology showed abundance of mononuclear cells. There was diffuse alveolar damage, interstitial and intra-alveolar fibrosis and marked proliferation of fibroblasts with multiple myofibroblastic foci in interstitium and airspaces. The cause of IPF could not be easily ascertained; however, it is known that certain environmental factors and exposures to allergens increase the risk in man and cats. This report would further improve the screening of wildlife health.

A case of idiopathic pulmonary fibrosis treated with clarithromycin

A case of idiopathic pulmonary fibrosis treated with clarithromycin

P277 The Incidence Of Lung Cancer In People With Idiopathic Pulmonary Fibrosis And Connective Tissue Disease Associated Pulmonary Fibrosis In The Uk: A Population Based Study

Introduction Studies have suggested that lung cancer is more common in people with idiopathic pulmonary fibrosis (IPF). However, there is limited information on the risk of lung cancer in individuals with connective tissue disease associated pulmonary fibrosis (CTD-PF). The aim of this study was to compare the incidence of lung cancer in people with IPF and CTD-PF with that of the general population. Methods Using electronic primary care records from The Health Improvement Network (THIN), we identified incident cases of IPF and CTD-PF between 2000 and 2011. For every case of IPF or CTD-PF, up to 4 general population controls matched on age, sex and general practice were randomly selected. We conducted a matched cohort analysis to estimate rate ratios for lung cancer in cases of IPF and CTD-PF compared with matched controls, adjusting for smoking habit. Results Our study population consisted of 3266 incident cases of IPF, 494 cases of CTD-PF and 14,463 matched general population controls. The majority (64.1%) of people with IPF were male, while most (55.3%) of those with CTD-PF were female. Individuals with CTD-PF were also younger at time of diagnosis compared to people with IPF (mean age at diagnosis 69.0 vs. 74.2 years; p Conclusion Individuals with IPF and CTD-PF are at an increased risk of lung cancer, which cannot be fully explained by smoking habit. With the increasing use of new therapies that may prolong the median survival in individuals with lung fibrosis, our findings raise the possibility these patients may represent a suitable population for lung cancer screening.

Idiopathic pulmonary fibrosis and coronary artery disease


 
 
 
 Idiopathic pulmonary fibrosis (IPF) is defined as a chronic fibrosing interstitial disease of unknown cause, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP); it generally progresses into respiratory failure and death. Although progression of the disease is the most common cause of death, there are increasing reports of its association with other pathologies has been reported: e.g., IPF patients seem more susceptible to cardiovascular diseases. Therefore, other pathologies might also influence the natural course. In this paper, we describe a case of IPF and coronary artery disease (CAD). We emphasize the importance of cardiopulmonary exercise test (CPET) as a useful procedure to monitor disease progression in IPF patients. We also stress the importance of a careful analysis of variables measured for an accurate interpretation of the clinical picture and an improvement of the clinical management of patients. Moreover, we suggest that a careful assessment of CPET parameters may additionally help in the early detection of high cardiovascular ischemic risk.
 
 
 

The Peripheral Blood Transcriptome Identifies the Presence and Extent of Disease in Idiopathic Pulmonary Fibrosis

RationalePeripheral blood biomarkers are needed to identify and determine the extent of idiopathic pulmonary fibrosis (IPF). Current physiologic and radiographic prognostic indicators diagnose IPF too late in the course of disease. We hypothesize that peripheral blood biomarkers will identify disease in its early stages, and facilitate monitoring for disease progression.MethodsGene expression profiles of peripheral blood RNA from 130 IPF patients were collected on Agilent microarrays. Significance analysis of microarrays (SAM) with a false discovery rate (FDR) of 1% was utilized to identify genes that were differentially-expressed in samples categorized based on percent predicted DLCO and FVC.Main Measurements and ResultsAt 1% FDR, 1428 genes were differentially-expressed in mild IPF (DLCO >65%) compared to controls and 2790 transcripts were differentially- expressed in severe IPF (DLCO >35%) compared to controls. When categorized by percent predicted DLCO, SAM demonstrated 13 differentially-expressed transcripts between mild and severe IPF (< 5% FDR). These include CAMP, CEACAM6, CTSG, DEFA3 and A4, OLFM4, HLTF, PACSIN1, GABBR1, IGHM, and 3 unknown genes. Principal component analysis (PCA) was performed to determine outliers based on severity of disease, and demonstrated 1 mild case to be clinically misclassified as a severe case of IPF. No differentially-expressed transcripts were identified between mild and severe IPF when categorized by percent predicted FVC.ConclusionsThese results demonstrate that the peripheral blood transcriptome has the potential to distinguish normal individuals from patients with IPF, as well as extent of disease when samples were classified by percent predicted DLCO, but not FVC.

A Spectrum of Severe Familial Liver Disorders Associate with Telomerase Mutations

BackgroundTelomerase is an enzyme specialized in maintaining telomere lengths in highly proliferative cells. Loss-of-function mutations cause critical telomere shortening and are associated with the bone marrow failure syndromes dyskeratosis congenita and aplastic anemia and with idiopathic pulmonary fibrosis. Here, we sought to determine the spectrum of clinical manifestations associated with telomerase loss-of-function mutations.Methodology/Principal FindingsSixty-nine individuals from five unrelated families with a variety of hematologic, hepatic, and autoimmune disorders were screened for telomerase complex gene mutations; leukocyte telomere length was measured by flow fluorescence in situ hybridization in mutation carriers and some non-carriers; the effects of the identified mutations on telomerase activity were determined; and genetic and clinical data were correlated. In six generations of a large family, a loss-of-function mutation in the telomerase enzyme gene TERT associated with severe telomere shortening and a range of hematologic manifestations, from macrocytosis to acute myeloid leukemia, with severe liver diseases marked by fibrosis and inflammation, and one case of idiopathic pulmonary fibrosis but not with autoimmune disorders. Additionally, we identified four unrelated families in which loss-of-function TERC or TERT gene mutations tracked with marrow failure, pulmonary fibrosis, and a spectrum of liver disorders.Conclusions/SignificanceThese results indicate that heterozygous telomerase loss-of-function mutations associate with but are not determinant of a large spectrum of hematologic and liver abnormalities, with the latter sometimes occurring in the absence of marrow failure. Our findings, along with the link between pulmonary fibrosis and telomerase mutations, also suggest a common pathogenic mechanism for fibrotic diseases in which defective telomere repair plays important role.

A Case of Idiopathic Pulmonary Fibrosis Exacerbated by Anti-cancer Chemotherapy Complicated with Central Type Squamous Cell Carcinoma of the Lung

A Case of Idiopathic Pulmonary Fibrosis Exacerbated by Anti-cancer Chemotherapy Complicated with Central Type Squamous Cell Carcinoma of the Lung

Pulmonary nocardiosis during immunosuppressive therapy for idiopathic pulmonary fibrosis

Nocardiosis is a subacute or chronic suppurative infection caused by Nocardia species. Although it is more common in immunocompromised hosts, idiopathic pulmonary fibrosis (IPF) has not been recognized as a predisposing factor for nocardial infection. We report a case of IPF, in which pulmonary nocardiosis developed during treatment with prednisolone and cyclophosphamide. The risk of pulmonary nocardiosis may be increased in cases of IPF on immunosuppressive therapy. Since IPF often accompanies lung carcinoma, it is important to correctly differentiate nocardiosis from carcinoma.

Pulmonary nocardiosis during immunosuppressive therapy for idiopathic pulmonary fibrosis

Nocardiosis is a subacute or chronic suppurative infection caused by Nocardia species. Although it is more common in immunocompromised hosts, idiopathic pulmonary fibrosis (IPF) has not been recognized as a predisposing factor for nocardial infection. We report a case of IPF, in which pulmonary nocardiosis developed during treatment with prednisolone and cyclophosphamide. The risk of pulmonary nocardiosis may be increased in cases of IPF on immunosuppressive therapy. Since IPF often accompanies lung carcinoma, it is important to correctly differentiate nocardiosis from carcinoma.

Neumomediastino espontáneo y fibrosis pulmonar idiopática

The association between pulmonary fibrosis and pneumomediastinum is infrequent. Computed tomography is the suited method for the detection of small pneumomediastinum, which is difficult to detect by chest radiography. A case of idiopathic pulmonary fibrosis, with frequent and violent cough, with pneumomediastinum and cervical subcutaneous emphysema, without pneumothorax, probably caused by rupture of bullae is presented. The outcome was favourable with symptomatic treatment.

Idiopathic Pulmonary Fibrosis (IPF) Necessitating Therapeutic Midtrimester Abortion: A Case Report

A 35-year old multiparous lady was admitted at 19 weeks of pregnancy with rapid onset of dyspnoea which progressed to grade III in 2 months. She was diagnosed as a case of idiopathic pulmonary fibrosis (IPF). She had a vital capacity of 1.25 l, 46.5% of predicted and PaO2 of 60 mmHg at rest, dropping to 35 mmHg on mild exercise testing. She did not respond to prednisolone 40 mg daily given orally for one month. In view of lack of improvement, therapeutic abortion was carried out at 24 weeks by abdominal hysterotomy combined with tubal ligation. Subsequent to termination of pregnancy, her clinical status and pulmonary function improved markedly and she had only grade I dyspnoea 30 months later without corticosteroids. Effect of pregnancy on interstitial lung disorders especially IPF is not yet clear because of extreme rarity of their association. Therapeutic abortion should be seriously considered in patients who cannot increase their oxygen consumption 3 times normal without uncorrectable hypoxaemia.

Idiopathic Pulmonary Fibrosis

A case of idiopathic pulmonary fibrosis with multiple areas of mismatch on ventilation-perfusion lung imaging in the absence of pulmonary embolism is presented. Idiopathic pulmonary fibrosis is one of the few nonembolic diseases producing a pulmonary ventilation-perfusion mismatch. In this condition, chest radiographs may not detect the full extent of disease, and xenon-133 ventilation imaging may be relatively insensitive to morbid changes in small airways. Thus, when examining patients with idiopathic pulmonary fibrosis, one should be aware that abnormal perfusion imaging patterns without matching ventilation abnormalities are not always due to embolism. In this setting, contrast pulmonary angiography is often needed for accurate differential diagnosis.