Form Of Idiopathic Interstitial Pneumonia Research Articles

Interstitial lung diseases in persons infected with human immunodeficiency virus

Diagnosis of interstitial lung diseases (ILD) in individuals infected with the human immunodeficiency virus (HIV) remains poorly understood.The purpose of the work was to demonstrate approaches to the diagnosis of ILD in HIV-infected patients with different levels of CD4 lymphocytes. An analysis of literature data and clinical observations of ILD in HIV-infected individuals (n = 2) whose CD4 lymphocyte count was 677 (37.5%) and 1 (0.21%) cells/μl are presented. The patients were observed and treated for a long time by pulmonology specialists, including anti-tuberculosis chemotherapy. The patient with CD4 lymphocyte count of 677 cells/μl in the absence of Mycobacterium tuberculosis in the sputum had an established diagnosis of idiopathic pulmonary fibrosis with the progressive development of respiratory failure and progression of the computed tomographic pattern from large areas of “ground glass” to the formation of reticular changes and “honeycomb” lung within 2 years. The patient with CD4 count of 1 cell/μl was diagnosed with nonspecific interstitial pneumonia based on characteristic clinical manifestations of increasing respiratory failure against the background of diffuse interstitial pneumofibrosis in the absence of bacterial excretion, the effects of antimycobacterial, antibacterial and antifungal treatment for 7 months, and excluding other causes of lung damage.Conclusion. The development of interstitial fibrosing pulmonary process of non-infectious etiology in HIV-infected patients with different states of T-helper immunity has been demonstrated. After excluding opportunistic infections, the influence of other factors, and concomitant diseases, these patients were diagnosed with various forms of idiopathic interstitial pneumonia. However, existing reports of the direct damaging effects of the virus on the pulmonary parenchyma also suggest the possibility of HIV-associated interstitial pulmonary fibrosis.

Survival differences in rheumatoid arthritis interstitial lung disease and idiopathic pulmonary fibrosis may be explained by delays in presentation: results from multivariate analysis in a monocentric UK study

Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) has a better prognosis compared to idiopathic pulmonary fibrosis (IPF). Recent data suggest that antifibrotics are effective in slowing progression across both groups. Hence, we designed this study to investigate the similarities and differences between these groups of patients. This is a retrospective cohort study examining baseline data, progression and outcomes in patients with RA-ILD and IPF prior to antifibrotic use in the Coventry ILD database. Ethics approval was obtained from the University Hospital Coventry and Warwickshire NHS Trust. Statistical analysis was performed using R software and Cox’s proportional hazards technique was used for survival analysis. We identified 131 cases, including 49 patients with IPF, 34 patients with RA-ILD and 48 patients with other forms of idiopathic interstitial pneumonia. At baseline, there were significant differences in the groups with RA-ILD patients being significantly younger (65.7 vs 72.4 years), had preserved lung volumes (FVC 95% vs 84.7%) and higher gas transfer (61.5% vs 48.2%) compared to IPF patients. 5-year survival was better for RA-ILD compared to IPF (87.5% vs 40.4%, p = 0.0042). Univariate analysis revealed gas transfer, FVC, age, sex and phenotype (IPF or RA-ILD) were all significant predictors, but multivariate analysis revealed that gas transfer and age were both significantly associated with prognosis, whereas sex, FVC or phenotype were not significant. This study suggests that the difference between RA-ILD and IPF prognosis may be due to demographics and early diagnosis rather than the diseases behaving differently. This has important management implications.

The Reliability of Ultrasound in Diagnosis of Idiopathic Pulmonary Fibrosis

Abstract Background Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause. IPF is the most common and severe form of idiopathic interstitial pneumonia, with an unpredictable and variable clinical course, and is associated with an extremely poor prognosis. Objective To determine role of Ultrasound in diagnosis of idiopathic pulmonary fibrosis. Patients and Methods This cross-sectional study was conducted at tertiary care hospital at Ain Shams University hospitals from May 2021 till November 2021 and performed on total 60 patients diagnosed with idiopathic pulmonary fibrosis and had previous clinical features, documented high-resolution computed tomography (HRCT) scan findings and comparing it with lung ultrasound findings. Results Consequently, B-lines number and Pleural lines thickness statistically had significant high diagnostic performance in the diagnosis of IPF with cut-off of ≥ 3.0 which indicates that B-lines number ≥3.0 and Pleural lines thickness ≥3.0 mm had perfect sensitivity and LR+, while Pleural lines interruption had perfect specificity. B-lines number ≥3.0 had highest diagnostic accuracy, Youden’s index and kappa. Our research study revealed that B-lines number and Pleural lines thickness statistically were significantly higher in multiple zones spread cases (p value<0.001). Pleural lines interruption statistically was significantly more frequent in multiple zones spread cases. Conclusion As evident from the current study, this study introduced and thoroughly evaluated lung US features, which showed a high correlation with the high-resolution CT findings. Chest ultrasound could constitute an additional monitoring tool for IPF, which is a rapidly progressive disease, usable by the pulmonologist at the patient’s bedside or during a consultation, easily available, non-irradiating, achievable in a short time and immediately informative, for a trained sonographer.

A Real-World Prognosis in Idiopathic Pulmonary Fibrosis: A Special Reference to the Role of Antifibrotic Agents for the Elderly.

Idiopathic pulmonary fibrosis (IPF) is the most common and severe form of idiopathic interstitial pneumonia, and its prevalence increases with age. In the era of pre-antifibrotic agents, the median survival time of Japanese patients with IPF is 35 months, with a 5-year survival rate in western countries ranging from 20% to 40%. The prevalence of IPF is highest in elderly patients aged ≥75 years; however, the efficacy and safety of long-term use of pirfenidone and/or nintedanib are not fully understood. This study aimed to determine the efficacy and safety of the sole use of antifibrotic agents (pirfenidone or nintendanib) for IPF in the elderly. We retrospectively reviewed patients with IPF who were diagnosed and treated with either pirfenidone or nintedanib in our hospital between 2008 and 2019. We excluded patients with the subsequent use of both antifibrotic agents. We examined the survival probability and frequency of acute exacerbation, with focus on long-term use (≥1 year), elderly patients (≥75 years of age), and disease severity. We identified 91 patients with IPF (male to female ratio: 63 to 28, age 42 to 90 years). The numbers of patients with disease severity classified by JRS (I/II/III/IV) and GAP stage (I/II/III) were (38/6/17/20) and (39/36/6), respectively. The survival probabilities were comparable between the elderly (n = 46) and non-elderly groups (n = 45, p = 0.877). After the initiation of antifibrotic agents, the cumulative incidence ratio of acute exacerbation of IPF was significantly lower in the early stage (GAP stage I, n = 20) than in the progressive stage of disease (GAP stages II and III, n = 20, p = 0.028). A similar trend was noted in the JRS disease severity classification (I, II vs. III, IV) (n = 27 vs. n = 13, p = 0.072). In the long-term treatment (≥1 year) group (n = 40), the survival probabilities at 2 and 5 years after treatment initiation were 89.0% and 52.4%, respectively, which did not reach the median survival rate. Even in elderly patients (≥75 years of age), antifibrotic agents demonstrated positive effects on survival probability and the frequency of acute exacerbation. These positive effects would be improved for earlier JRS/GAP stages or long-term use.

Update on cryptogenic organizing pneumonia.

Cryptogenic organizing pneumonia (COP) is a form of idiopathic interstitial pneumonia that results from the pulmonary reaction to various unidentified injuries. Secondary organizing pneumonia is diagnosed when the triggering factor has been identified; it is mainly caused by infections, toxic substance exposure, drugs, connective tissue diseases, malignancies, autoimmune diseases, bone marrow, or organ transplantation, and radiotherapy. There has been an increase in the number of reports of drug-induced organizing pneumonia (OP). New biological therapies, interferon, monoclonal antibodies, anti-interleukin antibodies, and PD1/PDL-1 inhibitors may induce this specific pulmonary reaction. The classical form of COP is usually subacute and does not manifest as severe disease. Patients maintain sufficient respiratory function, and treatment with steroids is usually effective. Several specific forms of OP (e.g., the cicatricial variant or acute fibrinous type) have distinct clinical and histological features, require higher doses of immunosuppressive drugs, and have a worse prognosis. In the era of administering steroid-sparing therapies for the treatment of interstitial lung diseases, connective tissue dases, and other conditions, it is important to emphasize this type of therapy for patients with COP.

Magnetic liposome as a dual-targeting delivery system for idiopathic pulmonary fibrosis treatment

Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonia, where M2 macrophages play an irreplaceable role in the anti-inflammatory progress. Targeting M2 macrophages and regulating their polarization may be a potential treatment strategy for IPF. Herein, we designed a magnetic liposome based dual-targeting delivery system for the IPF treatment, constructed by mannose-modified magnetic nanoparticles (MAN-MNPs) loaded on the surface of the liposome (MAN-MNPs@LP). The delivery system is capable of responding to a static magnetic field (SMF) and then recognizing in situ of M2 macrophages through the mannose receptor-dependent internalization. Firstly, a series of physical and chemical assays were used to characterize these nanoparticles. Subsequently, magnetic liposomes accumulation in the damaged lung with/without mannose modification and SMF were compared by in vivo imaging system. Finally, the reduction of M2 macrophages and inhibition of their polarization confirmed that the development of IPF was retarded due to the in situ release of encapsulated dexamethasone (Dex) in lungs under the SMF. Further investigation demonstrated that the expression of α-SMA and collagen deposition was reduced. Altogether, this dual-targeting delivery system can effectively deliver Dex into M2 macrophages in the lung, making it a novel and promising therapeutic system for the IPF treatment.

ОПЫТ ВЕДЕНИЯ ПАЦИЕНТКИ С ИДИОПАТИЧЕСКИМ ПЛЕВРОПАРЕНХИМАТОЗНЫМ ФИБРОЭЛАСТОЗОМ: ОСОБЕННОСТИ ДИАГНОСТИКИ И ЛЕЧЕНИЯ

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonia the etio¬logy of which is often unknown, and the course of the disease is usually rapidly progressive. The disease occurs with equal frequency in both men and women. Its onset usually is observed in the second half of life. Complaints are nonspecific: patients suffer from dry cough, shortness of breath, low body mass. A distinctive feature of IPPFE is the involvement of the apices of the lungs and development of platythorax, which can be easily detected in these patients during computed tomography. There is no specific treatment, but there is an evidence of positive experience with the use of antifibrotic drugs.

The method of probe-based confocal laser endomicroscopy in the diagnosis of idiopathic interstitial pneumonias

The diagnosis of idiopathic interstitial pneumonia (IIP) remains one of the most challenging issues in pulmonology. The article demonstrates the capabilities of the method of probe-based confocal laser endomicroscopy (pCLE) in diagnosing various forms of IIPs and provides comparisons with CT and the histological picture. According to the data obtained, it is difficult to distinguish most IIPs from each other using pCLE. Among the specific signs, a large number of alveolar cells in pCLE in a nonsmoking patient with desquamative interstitial pneumonia is not typical for other IIP in nonsmokers. Also, pCLE can be used to differentiate between IIP and other conditions, in particular, malignant lesions.

Diagnosis and management of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a form of idiopathic interstitial pneumonia that currently carries a poor prognosis and remains incurable, though antifibrotic therapy can slow disease progression. This article provides an overview of its diagnosis, treatment and the management of co‐morbidities.

Evaluation of Proteasome Inhibitors in the Treatment of Idiopathic Pulmonary Fibrosis.

Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonia, and it has a worse prognosis than non-small cell lung cancer. The pathomechanism of IPF is not fully understood, but it has been suggested that repeated microinjuries of epithelial cells induce a wound healing response, during which fibroblasts differentiate into myofibroblasts. These activated myofibroblasts express α smooth muscle actin and release extracellular matrix to promote matrix deposition and tissue remodeling. Under physiological conditions, the remodeling process stops once wound healing is complete. However, in the lungs of IPF patients, myofibroblasts re-main active and deposit excess extracellular matrix. This leads to the destruction of alveolar tissue, the loss of lung elastic recoil, and a rapid decrease in lung function. Some evidence has indicated that proteasomal inhibition combats fibrosis by inhibiting the expressions of extracellular matrix proteins and metalloproteinases. However, the mechanisms by which proteasome inhibitors may protect against fibrosis are not known. This review summarizes the current research on proteasome inhibitors for pulmonary fibrosis, and provides a reference for whether proteasome inhibitors have the potential to become new drugs for the treatment of pulmonary fibrosis.

Role of nanoparticles loaded with pirfenidone after bleomycin-induced lung fibrosis in adult male albino rats: a histological study

Background Lung fibrosis is a severe form of idiopathic interstitial pneumonia. Targeting cells is a good approach in drug delivery. Pirfenidone is an antifibrotic drug ‘used’ for the treatment of idiopathic pulmonary fibrosis. Aim The aim of the present work was to study the effect of nanoparticles (niosomes) loaded with pirfenidone on bleomycin-induced lung fibrosis in adult male albino rats with different histological techniques. Material and methods A total of 40 rats were divided into six groups: group I, control (five rats); group II pirfenidone treated (five rats); group III, encapsulated-pirfenidone treated (five rats); group IV, bleomycin treated (five rats); group V, bleomycin-pirfenidone treated (10 rats), which was then subdivided into two subgroups, that is, subgroup Va (five rats) and subgroup Vb (five rats); and group VI, bleomycin-encapsulated-pirfenidone treated (10 rats), which was then subdivided into two subgroups, that is, subgroup VI a (five rats) and subgroup VIb (five rats). Lung specimens were stained using hematoxylin and eosin-stained, Masson’s trichrome, anti-transforming growth factor beta 1 (TGF-β1), and toluidine blue for light microscopic examination and transmission electron microscopy. Assessments of mean area % of the stained collagen fibers and intensity of TGF-β1 and the mean thickness of blood–air barrier of ultrathin sections were performed and then subjected to statistical analysis. Results Encapsulated pirfenidone-treated subgroups showed more patent alveoli, sacs, and ducts; patent bronchioles; blood vessels; and more type I pneumocytes and less type II. The blood–air barrier had thin basal lamina. There were better results in collagen deposition and TGF-β1 in lung tissues with encapsulated pirfenidone treatment. Conclusion Encapsulated pirfenidone resulted in better improvement than using pirfenidone only.

Dissecting the Role of Mesenchymal Stem Cells in Idiopathic Pulmonary Fibrosis: Cause or Solution.

Idiopathic pulmonary fibrosis (IPF) is one of the most aggressive forms of idiopathic interstitial pneumonias, characterized by chronic and progressive fibrosis subverting the lung’s architecture, pulmonary functional decline, progressive respiratory failure, and high mortality (median survival 3 years after diagnosis). Among the mechanisms associated with disease onset and progression, it has been hypothesized that IPF lungs might be affected either by a regenerative deficit of the alveolar epithelium or by a dysregulation of repair mechanisms in response to alveolar and vascular damage. This latter might be related to the progressive dysfunction and exhaustion of the resident stem cells together with a process of cellular and tissue senescence. The role of endogenous mesenchymal stromal/stem cells (MSCs) resident in the lung in the homeostasis of these mechanisms is still a matter of debate. Although endogenous MSCs may play a critical role in lung repair, they are also involved in cellular senescence and tissue ageing processes with loss of lung regenerative potential. In addition, MSCs have immunomodulatory properties and can secrete anti-fibrotic factors. Thus, MSCs obtained from other sources administered systemically or directly into the lung have been investigated for lung epithelial repair and have been explored as a potential therapy for the treatment of lung diseases including IPF. Given these multiple potential roles of MSCs, this review aims both at elucidating the role of resident lung MSCs in IPF pathogenesis and the role of administered MSCs from other sources for potential IPF therapies.

Knockdown of long non‑coding RNA DLEU2 suppresses idiopathic pulmonary fibrosis by regulating the microRNA‑369‑3p/TRIM2 axis.

Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonia with an increasing incidence. In the present study, Genome Expression Omnibus (GEO) datasets (GSE10667, GSE24206 and GSE32537) were applied to identify lncRNA DLEU2 in IPF. Through prediction using starBase, TargetScan, miRTarBase and miRDB, tripartite motif containing 2 (TRIM2) and prostaglandin F2 receptor inhibitor (PTGFRN) were found to be upregulated in IPF. DLEU2 expression, the mRNA expression of TRIM2 and PTGFRN, and miR-369-3p expression in A549 cells and lung tissues were detected by RT-qPCR. The protein expression of TRIM2 and PTGFRN in lung tissues and A549 cells was detected by western blot analysis. The proliferation and migration of A549 cells was respectively detected by CCK-8 assay and wound healing assay. The expression of collagen I, α-smooth muscle actin (SMA) and E-cadherin was detected by immunofluorescence assay in A549 cells, and collagen I expression was detected by immunohistochemistry assay in lung tissues. The expression of collagen I, α-SMA and E-cadherin was also detected by western blot analysis in A549 cells and lung tissues. Dual-luciferase reporter assay was used to confirm the association between DLEU2 and miR-369-3p, and miR-369-3p and TRIM2. As a result, DLEU2 expression was found to be upregulated in IPF and in transforming growth factor (TGF)-β1-stimulated A549 cells. The silencing of DLEU2 inhibited the TGF-β1-induced proliferation, migration and epithelial-mesenchymal transition (EMT) of A549 cells and bleomycin (BLM)-induced pulmonary fibrosis in mice. TRIM2 expression was increased and miR-369-3p expression was decreased in the lung tissues of mice with BLM-induced fibrosis and in TGF-β1-stimulated A549 cells. DLEU2 directly targeted miR-369-3p. The effect of the silencing of DLEU2 on TGF-β1-stimulated A549 cells was suppressed by the silencing of miR-369-3p. TRIM2 was the target protein of miR-369-3p. On the whole, the present study demonstrates that the silencing of DLEU2 suppressed IPF by upregulating miR-369-3p expression and downregulating TRIM2 expression.

Pneumothorax in Patients with Idiopathic Pleuroparenchymal Fibroelastosis: Incidence, Clinical Features, and Risk Factors

Background: Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare form of idiopathic interstitial pneumonia that is characterized by predominantly upper lobe pleural and subpleural lung parenchymal fibrosis. Pneumothorax is one of the major respiratory complications in PPFE patients; however, its clinical features are poorly understood. Objective: We aimed to investigate the complication of pneumothorax in patients with idiopathic PPFE. Methods: A retrospective multicenter study involving 89 patients who had been diagnosed with idiopathic PPFE was conducted. We investigated the cumulative incidence, clinical features, and risk factors of pneumothorax after the diagnosis of idiopathic PPFE. Results: Pneumothorax developed in 53 patients (59.6%) with 120 events during the observation period (41.8 ± 35.0 months). The cumulative incidence of pneumothorax was 24.8, 44.9, and 53.9% at 1, 2, and 3 years, respectively. Most events of pneumothorax were asymptomatic (n = 85; 70.8%) and small in size (n = 92; 76.7%); 30 patients (56.6%) had recurrent pneumothorax. Chest drainage was required in 23 pneumothorax events (19.2%), and a persistent air leak was observed in 13 (56.5%). Patients with pneumothorax were predominantly male and frequently had pathological diagnoses of PPFE and prior history of pneumothorax and corticosteroid use; they also had significantly poorer survival than those without pneumothorax (log-rank test; p = 0.001). Multivariate analysis revealed that a higher residual volume/total lung capacity ratio was significantly associated with the development of pneumothorax after the diagnosis. Conclusion: Pneumothorax is often asymptomatic and recurrent in patients with idiopathic PPFE, leading to poor outcomes in some cases.

Integrated analysis of the molecular mechanisms in idiopathic pulmonary fibrosis.

Rationale: Idiopathic pulmonary fibrosis (IPF) is one of the most aggressive forms of idiopathic interstitial pneumonia. Some miRNAs may be associated with IPF and may affect the occurrence and development of IPF in various pathways. Many miRNAs and genes that may be involved in the development of IPF have been discovered using chip and high throughput technologies.Methods: We analyzed one miRNA and four mRNA databases. We identified hub genes and pathways related to IPF using GO, KEGG enrichment analysis, gene set variation analysis (GSVA), PPI network construction, and hub gene analysis. A comprehensive analysis of differentially expressed miRNAs (DEMs), predicted miRNA target genes, and differentially expressed genes (DEGs) led to the creation of a miRNA-mRNA regulatory network in IPF.Results: We found 203 DEGs and 165 DEMs that were associated with IPF. The findings of enrichment analyses showed that these DEGs were mainly involved in antimicrobial humoral response, antimicrobial humoral immune response mediated by antimicrobial peptide, extracellular matrix organization, cell killing, and organ or tissue specific immune response. The VEGFA, CDH5, and WNT3A genes overlapped between hub genes and the miRNA-mRNA regulatory network. The miRNAs including miR-199b-5p, miR-140-5p, miR-199a-5p, miR-125A-5p, and miR-107 that we predicted would regulate the VEGFA, CDH5, and WNT3A genes, which were also associated with IPF or other fibrosis-related diseases. GSVA indicated that metabolic processes of UTP and IMP, immune response, regulation of Th2 cell cytokine production, and positive regulation of NK cell-mediated immunity are associated with the pathogenesis and treatment of IPF. These pathways also interact with VEGFA, CDH5, and WNT3A.Conclusion: These findings provide a new research direction for the diagnosis and treatment of IPF.

Emerging cellular and molecular determinants of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonia, is a progressive, irreversible, and typically lethal disease characterized by an abnormal fibrotic response involving vast areas of the lungs. Given the poor knowledge of the mechanisms underpinning IPF onset and progression, a better understanding of the cellular processes and molecular pathways involved is essential for the development of effective therapies, currently lacking. Besides a number of established IPF-associated risk factors, such as cigarette smoking, environmental factors, comorbidities, and viral infections, several other processes have been linked with this devastating disease. Apoptosis, senescence, epithelial-mesenchymal transition, endothelial-mesenchymal transition, and epithelial cell migration have been shown to play a key role in IPF-associated tissue remodeling. Moreover, molecules, such as chemokines, cytokines, growth factors, adenosine, glycosaminoglycans, non-coding RNAs, and cellular processes including oxidative stress, mitochondrial dysfunction, endoplasmic reticulum stress, hypoxia, and alternative polyadenylation have been linked with IPF development. Importantly, strategies targeting these processes have been investigated to modulate abnormal cellular phenotypes and maintain tissue homeostasis in the lung. This review provides an update regarding the emerging cellular and molecular mechanisms involved in the onset and progression of IPF.

Advances in Targeted Therapy for Progressive Fibrosing Interstitial Lung Disease.

Progressive fibrosing interstitial lung disease (PF-ILD) has been redefined as a new clinical syndrome that shares similar genetics, pathophysiology, and natural history to idiopathic pulmonary fibrosis (IPF). IPF is the most common form of idiopathic interstitial pneumonias, which is progressive in nature and is associated with significant mortality. Therapies targeting an inflammatory and/or immune response have not been consistently effective or well tolerated in patients with IPF. The two antifibrotic drugs approved for IPF treatment, nintedanib and pirfenidone, have been shown to reduce lung function decline in PF-ILD. Novel uses of antifibrotic therapy are emerging due to a paucity of evidence-based treatments for multiple ILD subtypes. In this review, we describe the current body of knowledge on antifibrotic therapy and immunomodulators in PF-ILD, drawing from experience in IPF where appropriate.

Desquamative interstitial pneumonia: a systematic review of its features and outcomes

Desquamative Interstitial Pneumonia (DIP) is a rare form of idiopathic interstitial pneumonia (IIP). Data on clinical features, aetiology, prognosis and effect of treatment strategies are limited. We aimed to collect all published cases to better characterise DIP. A systematic literature search was performed for all original cases of adult patients with histopathologically-confirmed DIP. Individual patient data were extracted and summarised. We included 68 individual cases and 13 case series reporting on 294 cases. Most common presenting symptoms were dyspnoea and cough. Pulmonary function showed a restrictive pattern (71%) with decreased diffusion capacity. We found a high incidence (81%) of ever smoking in patients with DIP and 22% of patients had other (occupational) exposures. Characteristic features on high-resolution computed tomography (HRCT) scan were bilateral ground-glass opacities with lower lobe predominance (92%). Treatment and duration of treatment widely varied. Initial response to treatment was generally good, but definitely not uniformly so. A significant proportion of patients died (25% of individual cases) or experienced a relapse (18% of individual cases). DIP remains an uncommon disease, frequently but not always related to smoking or other exposures. Furthermore, DIP behaves as a progressive disease more often than generally thought, possibly associated with different underlying aetiology.

Analysis of the clinical characteristics of 176 patients with pathologically confirmed cryptogenic organizing pneumonia.

BackgroundCryptogenic organizing pneumonia (COP), is a form of idiopathic interstitial pneumonia (IIP) and is diagnosed by clinical-radiologic-pathologic (CRP) diagnosis. To summarize the clinical, imaging, pathological, and prognostic characteristics of 176 patients with confirmed COP.MethodsPatients who were diagnosed with organizing pneumonia (OP) by lung biopsy between January 1, 2000, and December 31, 2013, in our hospital were retrospectively analyzed and followed up until 12/31/2017. COP was confirmed by CRP diagnosis.ResultsA total of 1,346 OP cases were identified including 1,170 (86.9%) secondary OP cases (31 cases were originally misdiagnosed as COP but later confirmed as secondary OP during follow-up) and 176 (13.1%) cases of COP. The 176 patients with COP presented with no specific clinical symptoms and chest CT revealed diverse imaging features, such as patchy ground-glass opacity (112/176, 63.6%), consolidation (100/176, 56.8%), nodules (70/176, 39.8%), and fibrous stripes (69/176, 39.2%). The majority of patients (65.3%, 115/176) presented mixed patterns, and 12 (6.8%) showed a reversed halo sign. Of the 176 patients, 83 patients had unilateral lesions (ULs) and did not undergo glucocorticoid therapy. Of the 93 patients with bilateral lesions (BLs), 3 underwent complete resection and were radically cured. The other 90 patients underwent partial resection; of these patients, 37 cases achieved spontaneous remission, and 53 were treated with glucocorticoid therapy. After steroid therapy was reduced or stopped, 35 (66%) patients experienced recurrence. Overall, the 5-year survival rate for the COP patients in our study was 98.3%.ConclusionsOur study showed that the majority of the patients pathologically diagnosed as OP had secondary OP (86.9%), with COP accounting for only 13.9% of cases. CRP diagnosis and continuous follow-up may be the key for the accurate diagnosis of COP.

Pirfenidone is a cardioprotective drug: Mechanisms of action and preclinical evidence

Myocardial fibrosis is an endogenous response to different cardiac insults that may become maladaptive over time and contribute to the onset and progression of heart failure (HF). Fibrosis is a direct and indirect target of established HF therapies, namely inhibitors of the renin-angiotensin-aldosterone system, but its resilience to therapy warrants a search for novel, more targeted approaches to myocardial fibrosis.Pirfenidone is a drug approved for idiopathic pulmonary fibrosis, a severe form of idiopathic interstitial pneumonias. Pirfenidone is a small synthetic molecule with high oral bioavailability, exerting an antifibrotic activity, but also anti-oxidant and anti-inflammatory effects. These effects have been attributed to the inhibition of several growth factors (in particular transforming growth factor-β, but also platelet-derived growth factor and beta fibroblast growth factor), matrix metalloproteinases, and pro-inflammatory mediators (such as interleukin-1β and tumour necrosis factor-α), and possibly also an improvement of mitochondrial function and modulation of lymphocyte activation. Given the activation of similar profibrotic pathways in lung and heart disease, the crucial role of fibrosis in several cardiac disorders, and the wide spectrum of activity of pirfenidone, this drug has been evaluated with interest as a potential treatment for cardiac disorders. In animal studies, pirfenidone has shown cardioprotective effects across different species and in a variety of models of cardiomyopathy. In the present review we summarize the pharmacological characteristics of pirfenidone and the data from animal studies supporting its cardioprotective effects.