Chronic Idiopathic Interstitial Pneumonias Research Articles

Native lung surgery after single lung transplantation: clinical characteristics and outcomes.

Single lung transplantation (SLT) is a viable option for patients with end-stage pulmonary parenchymal and vascular diseases. However, various diseases can occur in native lungs after SLT. Between January 2000 and December 2021, 35 patients underwent cadaveric SLT and survived for more than 30days in our hospital. Among these 35 patients, 10 required surgery for diseases that developed in their native lungs. The clinical characteristics of these 10 patients and the outcomes of native lung surgery (NLS) were investigated. Among these ten patients, the indications for lung transplantation were chronic obstructive pulmonary disease and idiopathic interstitial pneumonia in three patients each, and lymphangioleiomyomatosis and collagen vascular disease-related interstitial pneumoniain two patients each. The causes of NLS included pneumothorax (n = 4), primary lung cancer (n = 2), native lung hyperinflation (n = 2), and pulmonary aspergilloma (n = 2). The surgical procedures were pneumonectomy (n = 7), lobectomy (n = 2), and alveolar-pleural fistula repair (n = 1). Only one postoperative complication, empyema, was treated with antibiotics. The 5-year overall survival rates after transplantation with and without NLS were 70.0% and 80.0%, respectively, and did not differ to a statistically extent (p = 0.56). NLS is an effective treatment option for diseases that develop in the native lungs after SLT.

Hemosiderin-Laden Macrophages in Bronchoalveolar Lavage: Predictive Role for Acute Exacerbation of Idiopathic Interstitial Pneumonias.

Background Hemosiderin-laden macrophages (HLMs) have been identified in the bronchoalveolar lavage fluid (BALF) of patients with idiopathic pulmonary fibrosis (IPF). This retrospective study examined the ability of HLMs in BALF to predict the acute exacerbation (AE) of chronic idiopathic interstitial pneumonias (IIPs). Methods Two hundred and twenty-one patients with IIP diagnosed by bronchoscopy were enrolled in the study (IPF, n = 87; IIPs other than IPF, n = 134). Giemsa stain was used to detect HLMs in BALF specimens. Prussian blue stain was used to quantify HLMs in BALF, and a hemosiderin score (HS) was given to the specimens containing HLMs. Results Twenty-four patients had a positive HS (range: 7‒132). The receiver-operating characteristic curve analysis identified the cutoff HS value for predicting the AE of IIPs to be 61.5. Seven cases had a higher HS (≥61.5) and 214 had a lower HS. AE occurred significantly earlier in the higher HS group (4/7 cases) than in the lower HS group (41/214 cases) during a median observation period of 1239 days (log-rank test, p = 0.026). Multivariate Cox proportional hazard regression analysis showed that a higher HS was a significant predictor of AE in addition to IPF, percent predicted forced vital capacity, and modified Medical Research Council score. The C-statistics for the prediction of AE did not significantly improve by all the above parameters with HS as compared without HS. Conclusions A higher HS was a significant predictor of AE in IIPs but did not significantly improve the predictive ability of other parameters.

Lung function trajectory in progressive fibrosing interstitial lung disease.

Proposed criteria for progressive fibrosing interstitial lung disease (PF-ILD) have been linked to increased mortality risk, but lung function trajectory after satisfying individual criteria remains unknown. Because survival is rarely employed as the primary end-point in therapeutic trials, identifying PF-ILD criteria that best predict subsequent change in forced vital capacity (FVC) could improve clinical trial design. A retrospective, multicentre longitudinal cohort analysis was performed in consecutive patients with fibrotic connective tissue disease-associated ILD (CTD-ILD), chronic hypersensitivity pneumonitis and idiopathic interstitial pneumonia at three US centres (test cohort) and one UK centre (validation cohort). 1-year change in FVC after satisfying proposed PF-ILD criteria was estimated using joint modelling. Subgroup analyses were performed to determine whether results varied across key subgroups. 1227 patients were included, with CTD-ILD predominating. Six out of nine PF-ILD criteria were associated with differential 1-year change in FVC, with radiological progression of fibrosis, alone and in combination with other features, associated with the largest subsequent decline in FVC. Findings varied significantly by ILD subtype, with CTD-ILD demonstrating little change in FVC after satisfying most PF-ILD criteria, while other ILDs showed significantly larger changes. Findings did not vary after stratification by radiological pattern or exposure to immunosuppressant therapy. Near-term change in FVC after satisfying proposed PF-ILD criteria was heterogeneous depending on the criterion assessed and was strongly influenced by ILD subtype. These findings may inform future clinical trial design and suggest ILD subtype should be taken into consideration when applying PF-ILD criteria.

P16‐48: The prognostic marker of polymyxin B‐immobilized fiber column‐direct hemoperfusion in patients who developed acute exacerbation of chronic fibrosing idiopathic interstitial pneumonia

P16‐48: The prognostic marker of polymyxin B‐immobilized fiber column‐direct hemoperfusion in patients who developed acute exacerbation of chronic fibrosing idiopathic interstitial pneumonia

Pulmonary hypertension in fibrosing idiopathic interstitial pneumonia: Uncertainties, challenges and opportunities

Pulmonary hypertension is a serious complication of chronic fibrosing idiopathic interstitial pneumonia (PH-fIIP) leading to greater morbidity and mortality. The pathophysiologic basis for PH in fIIP is not completely understood, but microvascular rarefaction may play a key role. Severe hypoxemia and reduced diffusion capacity are characteristic. Doppler echocardiography has limited diagnostic utility and right heart catheterization is required to confirm the diagnosis. Lung volumes can be minimally affected, and radiographic findings can be subtle, making the distinction from pulmonary arterial hypertension challenging. Several randomized controlled trials of pulmonary arterial hypertension targeted therapies have recently been completed. Endothelin-receptor antagonists have shown either no benefit or harm. Sildenafil may have some favorable short-term effects but does not appear to impact long-term outcomes. Riociguat treatment increased hospitalizations and mortality. A recent trial of inhaled treprostinil demonstrated improved exercise capacity, but the impact on long-term morbidity and mortality are unknown. Currently, the only viable option for improved survival is lung transplantation. Early referral is imperative to optimize post-transplant outcomes.

Familial pulmonary fibrosis - a case report

Introduction: Idiopathic pulmonary fibrosis (IPF), a chronic progressive idiopathic interstitial pneumonia (IIP), occasionally, IPF occurs in families. IPF is associated with an estimated survival of 20%–50% at 5 years. Familial pulmonary fibrosis (FPF) can be defined as the occurrence of at least two cases of some type or fibrous interstitial pneumonia in members of the same biological family. Is important to note that the forms of interstitial lung diseases that affect relatives not necessarily need to be the same. Adults with FPF are essentially indistinguishable from patientes with sporadic IPF in terms of clinical presentation, radiographic findings and histopathology, except that those with FPF tend to present it earlier.
 Objective(s): To describe the clinical, functional, and radiological features of index cases of familial pulmonary fibrosis (FPF). Case overview: Male, 72 years old, seeks pulmonology service in October 16th, 2019 with cough, sputum and sinus pain. Denied dyspnea or other respiratory symptoms. Non-smoker, no alcoholism history, related physical activity 3 times per week and no environmental exposures. Treatment for sinusitis was indicated with return in 2 weeks. On return, he was asymptomatic. Unfortunately, he said that his 74-year old middle brother was hospitalized with severe dyspnea, mMRC 4, SpO2 91% using oxygen 4L in cateter and was diagnosed with non-specific interstitial pneumonia (NSIP). The CT scan revealed a tomographic pattern of NSIP, with a fibrous evolution form and emphysema. Also, he has a familiar history of an older brother who died 5 years ago, at the age of 80, with pulmonary fibrosis, and a CT scan usual intersticial pneumonia (UIP) pattern. The pulmonary auscultation had: a preserved vesicular murmur, mild bilateral “velcro-like” crackles in pulmonary basis, heard during middle to late inspiration, no wheezes. The patient didn’t accept to be submitted to invasive procedures, as bronchoscopy or surgical lung biopsy, because didn’t have respiratory symptoms (although the importance of these exams has been explained). The diagnosis of familial pulmonary fibrosis can be established when, despite having different tomographic patterns, siblings have in common the fibrous feature, as in this case. Even if the younger brother doesn’t have fibrosis yet, with the diagnosis confirmed by the other two brothers, the antifibrotic medication should be started.

Characteristics of patients with chronic idiopathic interstitial pneumonia undergoing repeated respiratory-related hospitalizations: A retrospective cohort study.

Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung disease. Chronic idiopathic interstitial pneumonia (c-IIP) is a group of lung diseases consisting mainly of fibrotic IIPs, and IPF is a type of c-IIP. Some patients with c-IIP undergo respiratory-related hospitalizations (RHs). With the hypothesis that the characteristics of patients who undergo RHs are related to the number of hospitalizations, we reviewed and investigated the RHs of patients with c-IIP. We retrospectively examined the data of patients with c-IIP who were admitted to Kindai University Hospital between January 2008 and December 2018 for respiratory-related causes. During the study period, 243 patients with c-IIP were hospitalized a total of 544 times because of respiratory-related causes. The most common reasons for the first RH were acute exacerbation (48.1%) followed by pulmonary infection (32.5%). The most frequent reason for subsequent RHs was pulmonary infection. The in-hospital and 90-day mortality rate of patients with pulmonary infection increased with increasing numbers of RHs. Patients with multiple RHs had significantly worse long-term survival than patients hospitalized a single time. Pulmonary infection was the most frequent reason for repeated RHs. The proportion of all patients hospitalized for pulmonary infection at each RH increased with increasing numbers of RHs, along with the mortality rate of patients with pulmonary infections. Furthermore, repeated RHs were associated with poor survival.

Neumonías intersticiales idiopáticas

Idiopathic interstitial pneumonias (IIPs) are a group of parenchymal lung diseases of unknown aetiology and with similar clinical, radiological and functional features. They are classified as chronic (idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia), smoking-related (respiratory bronchiolitis-associated interstitial lung disease and desquamative interstitial pneumonia), acute/subacute (cryptogenic organizing pneumonia and acute interstitial pneumonia), and rare (lymphoid interstitial pneumonia and pleuroparenchymal fibroelastosis).

Occupational and Environmental Risk Factors for Chronic Fibrosing idiopathic Interstitial Pneumonia in South Korea.

We studied the association of occupational and environmental agents with chronic fibrosing idiopathic interstitial pneumonia (IIP) in South Korea. We recruited 92 patients with chronic fibrosing IIP and 92 matched controls who had normal chest radiograph findings by age and gender. We used a structured exposure questionnaire to evaluate potential occupational and environmental risk factors for chronic fibrosing IIP, with adjustments for age, smoking, and clinical risk factors. We used conditional logistic regression models to analyze associations with chronic fibrosing IIP adjusted for age, smoking and clinical risk factors. Exposure to stone, sand, or silica significantly increased the risk of chronic fibrosing IIP (odds ratio = 5.01; 95% confidence interval, 1.07-24.21) CONCLUSIONS:: Our findings indicate that exposure to stone, sand, and silica might constitute a risk factor for developing chronic fibrosing IIP in the Korean population.

Impact of lymphocyte differential count\u2009>\u200915% in BALF on the mortality of patients with acute exacerbation of chronic fibrosing idiopathic interstitial pneumonia

BackgroundChronic fibrosing idiopathic interstitial pneumonia (CFIIP) has a potential risk of acute exacerbation (AE). However, the usefulness of cellular analysis of bronchoalveolar lavage fluid (BALF) has never been evaluated. This study aimed to evaluate the impact of the lymphocyte differential count > 15% in BALF on the mortality of patients with AE of CFIIP.MethodsWe retrospectively analysed 37 patients with AE of CFIIP who underwent BAL on admission. Patients were divided into two groups: one group consisting of those with a lymphocyte differential count > 15% and the other consisting of those with a lymphocyte differential count ≤ 15%. We compared the 90-day mortality between the two groups as the primary outcome, using the two-tailed log-rank test.ResultsThe median follow-up duration was 6.9 months. Twenty-four patients had a lymphocyte differential count > 15%. The 90-day mortality was significantly higher in the group with a lymphocyte differential count ≤ 15% than in the group with a lymphocyte differential count > 15% (long rank test, p = 0.003). In the multivariate analysis a lymphocyte differential count > 15% was shown to be an independent favourable prognostic factor for 90-day mortality (HR: 0.125; 95% CI: 0.0247–0.589; p = 0.009).ConclusionsA lymphocyte differential count > 15% in BALF may be associated with favourable outcomes in patients with AE of CFIIP.

Classification of idiopathic interstitial pneumonias using anti\u2013myxovirus resistance-protein 1 autoantibody

Chronic fibrosing idiopathic interstitial pneumonia (IIP) can be divided into two main types: idiopathic pulmonary fibrosis (IPF), a steroid-resistant and progressive disease with a median survival of 2–3 years, and idiopathic non-specific interstitial pneumonia (INSIP), a steroid-sensitive and non-progressive autoimmune disease. Although the clinical courses of these two diseases differ, they may be difficult to distinguish at diagnosis. We performed a comprehensive analysis of serum autoantibodies from patients definitively diagnosed with IPF, INSIP, autoimmune pulmonary alveolar proteinosis, and sarcoidosis. We identified disease-specific autoantibodies and enriched KEGG pathways unique to each disease, and demonstrated that IPF and INSIP are serologically distinct. Furthermore, we discovered a new INSIP-specific autoantibody, anti–myxovirus resistance-1 (MX1) autoantibody. Patients positive for anti-MX1 autoantibody constituted 17.5% of all cases of chronic fibrosing IIPs. Notably, patients rarely simultaneously carried the anti-MX1 autoantibody and the anti–aminoacyl-transfer RNA synthetase autoantibody, which is common in chronic fibrosing IIPs. Because MX1 is one of the most important interferon-inducible anti-viral genes, we have not only identified a new diagnostic autoantibody of INSIP but also obtained new insight into the pathology of INSIP, which may be associated with viral infection and autoimmunity.

Antitussive effect of carcainium chloride in patients with chronic cough and idiopathic interstitial pneumonias: A pilot study

BackgroundCough is a common presenting symptom in patients with idiopathic interstitial pneumonia (IIP); it is often disabling, and lacks effective treatment. Studies in animals suggest that carcainium chloride, a quaternary derivative of the local anesthetic lidocaine, is able to inhibit experimentally induced cough by a mechanism of action distinct from that of lidocaine. ObjectiveTo determine the effectiveness of aerosolised carcainium chloride (VRP700) in controlling cough in patients with IIP. MethodsEight female patients (mean age 71 years) with IIP were investigated in a double blind, randomised, placebo controlled crossover, adaptive contingency study design (EudraCT Number 2010-021350-19). The study consisted of a screening visit to assess the eligibility of patients, and two separated (48–72 h) study days. On the two study days, patients were randomised to receive either nebulized VRP700 (1.0 mg/kg) on the first study visit followed by nebulised placebo (sodium chloride 0.9%) on the second visit, or placebo on the first visit followed by VRP700 on the second visit. The primary endpoint was cough frequency over a 4-h assessment period; secondary endpoints were subjective cough-related level of discomfort as assessed by a visual analogue scale (VAS) and the subjective response to treatment as assessed by a quality of life question. Safety (ECG, spirometry, urine and blood tests) and adverse events occurring during the trial were also investigated. ResultsIn all patients both VRP700 and placebo decreased cough frequency; however, mean decreases in cough frequency after treatment with VRP700 were significantly (P < 0.001) higher than with placebo. Similarly, mean reductions in VAS score were significantly (P < 0.001) higher after treatment with VRP 700 compared with placebo. All but one patient indicated that they felt better after receiving VRP700. No adverse events were reported during the study, nor were any changes in ECG variables, spirometry, urine and blood tests noted. ConclusionThe results of this exploratory study indicate that nebulised VRP700 improved cough and quality of life in hospitalised IIP patients with no significant side effects. A larger trial is warranted to assess these promising results.

Acute and subacute idiopathic interstitial pneumonias.

Idiopathic interstitial pneumonias (IIPs) may have an acute or subacute presentation, or acute exacerbation may occur in a previously subclinical or unrecognized chronic IIP. Acute or subacute IIPs include acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP), acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) and AE-NSIP. Interstitial lung diseases (ILDs) including connective tissue disease (CTD) associated ILD, hypersensitivity pneumonitis, acute eosinophilic pneumonia, drug-induced lung disease and diffuse alveolar haemorrhage need to be differentiated from acute and subacute IIPs. Despite the severe lack of randomized controlled trials for the treatment of acute and subacute IIPs, the mainstream treatment remains corticosteroid therapy. Other potential therapies reported in the literature include corticosteroids and immunosuppression, antibiotics, anticoagulants, neutrophil elastase inhibitor, autoantibody-targeted treatment, antifibrotics and hemoperfusion therapy. With regard to mechanical ventilation, patients in recent studies with acute and subacute IIPs have shown better survival than those in previous studies. Therefore, a careful value-laden decision about the indications for endotracheal intubation should be made for each patient. Noninvasive ventilation may be beneficial to reduce ventilator associated pneumonia.

Combined pulmonary fibrosis and emphysema (CPFE): an entity different from emphysema or pulmonary fibrosis alone.

Chronic obstructive pulmonary disease (COPD) and idiopathic interstitial pneumonias (IIP), with different radiological, pathological, functional and prognostic characteristics, have been regarded as separate entities for a long time. However, there is an increasing recognition of the coexistence of emphysema and pulmonary fibrosis in individuals. The association was first described as a syndrome by Cottin in 2005, named "combined pulmonary fibrosis and emphysema (CPFE)", which is characterized by exertional dyspnea, upper-lobe emphysema and lower-lobe fibrosis, preserved lung volume and severely diminished capacity of gas exchange. CPFE is frequently complicated by pulmonary hypertension, acute lung injury and lung cancer and prognosis of it is poor. Treatments for CPFE patients with severe pulmonary hypertension are less effective other than lung transplantation. However, CPFE has not yet attracted wide attention of clinicians and there is no research systematically contrasting the differences among CPFE, emphysema/COPD and IIP at the same time. The authors will review the existing knowledge of CPFE and compare them to either entity alone for the first time, with the purpose of improving the awareness of this syndrome and exploring novel effective therapeutic strategies in clinical practice.

Low-normal lung volume correlates with pulmonary hypertension in fibrotic idiopathic interstitial pneumonia: computer-aided 3D quantitative analysis of chest CT.

We investigated whether the lung volume determined on CT, especially the volume of the normal lung, is correlated with mean pulmonary artery pressure (PAP) in patients with chronic fibrosing idiopathic interstitial pneumonia (IIP). The subjects were 40 patients with IIP who underwent right heart catheterization (RHC) and chest CT. Thirty-three patients (82.5%) were smokers or former smokers. Using a computer-aided system, the lungs in the 3D CT images were automatically categorized pixel-by-pixel with gaussian histogramnormalized correlations, and the relative volume of each lesion to the CT lung volume was calculated as "normal(%)," "ground-glass opacities(%)," "consolidation(%)," "emphysema(%)," and "fibrosis(%)." The relationship between each "volume(%)" and pulmonary hypertension was evaluated using logistic regression analysis. ROC curves were constructed to assess the predictive value of these CT-based volumes in the identification of pulmonary hypertension. Sixteen patients had pulmonary hypertension at rest (mean PAP > 25 mm Hg on RHC). Emphysema constituted more than 10% of the CT lung volume in 13 patients. On multivariate analysis of each volume(%), normal(%) was significant for detecting pulmonary hypertension (odds ratio, 0.92; 95% CI, 0.86-0.96; p = 0.02). On ROC analysis, the AUC of normal(%) was 0.849 (0.731-0.967). The relative CT volume of any single lesion was of limited value in predicting pulmonary hypertension in patients with pulmonary fibrosis and emphysema. In these patients, normal(%), measured by a 3D computer-aided system, was correlated with pulmonary hypertension measured by RHC.

Diagnosis and management of idiopathic pulmonary fibrosis: French practical guidelines.

Idiopathic pulmonary fibrosis (IPF) is the most frequent chronic idiopathic interstitial pneumonia in adults. The management of rare diseases in France has been organised by a national plan for rare diseases, which endorsed a network of expert centres for rare diseases throughout France. This article is an overview of the executive summary of the French guidelines for the management of IPF, an initiative that emanated from the French National Reference Centre and the Network of Regional Competence Centres for Rare Lung Diseases. This review aims at providing pulmonologists with a document that: 1) combines the current available evidence; 2) reviews practical modalities of diagnosis and management of IPF; and 3) is adapted to everyday medical practice. The French practical guidelines result from the combined efforts of a coordination committee, a writing committee and a multidisciplinary review panel, following recommendations from the Haute Autorité de Santé. All recommendations included in this article received at least 90% agreement by the reviewing panel. Herein, we summarise the main conclusions and practical recommendations of the French guidelines.

Pleuroparenchymal Fibroelastosis: Associations and Underlying Conditions

Pleuroparenchymal fibroelastosis (PPFE) is a clinicopathologic entity characterized by clinical presentation suggestive of a chronic idiopathic interstitial pneumonia, radiologic features of pleural and parenchymal involvement accentuated in the upper lobes, and a constellation of histologic findings including visceral pleural fibrosis and prominent fibroelastosis of the subpleural lung parenchyma. While the large majority of cases of PPFE have been considered idiopathic, development of PPFE in post-bone marrow transplant patients and in lung transplant recipients has recently been reported. Further, with the growing number of idiopathic PPFE cases reported in the literature, interesting patterns of association between PPFE and various clinical conditions – including prior treatment for malignancy, autoimmunity, recurrent infections, and vascular compromise/ischemia – are beginning to emerge. These associations, reviewed here, may offer clues into the pathogenesis of this rare condition. Keywords: Chronic lung allograft dysfunction, graft versus host disease, interstitial lung disease, pleuroparenchymal fibroelastosis, pulmonary fibrosis, pulmonary upper lobe fibrosis, restrictive allograft syndrome.

Expression of toll-like receptor 2 and 4 is increased in the respiratory epithelial cells of chronic idiopathic interstitial pneumonia patients

Idiopathic interstitial pneumonia (IIP) is characterized by chronic interstitial inflammation and fibrosis. Although mounting evidence has suggested that toll-like receptor (TLR) 2 and TLR4 are involved in the pathogenesis of non-infectious lung injury invitro and in mouse models, their roles in human IIP remain unknown. To address this issue, we investigated the expression patterns of TLR2 and TLR4 by immunohistochemistry in resected lung tissues from patients with usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP). Type II pneumocytes, bronchial epithelial cells (BECs), and alveolar macrophages accounted for the majority of TLR2- and TLR4-expressing cells in both UIP and NSIP. The numbers of TLR2 and TLR4-positive respiratory epithelial (RE) cells, including type II pneumocytes and BECs, were significantly greater in UIP and NSIP than in the control. In particular, the numbers of TLR2-positive RE cells were much greater in UIP than in NSIP. The intensities of TLR2 and TLR4 expression in type II pneumocytes were also significantly stronger in UIP and NSIP than in the control. A comparison of the TLR expression patterns between the fibroblastic and fibrotic areas in UIP indicated that the numbers TLR2 and TLR4-positive RE cells were similar in fibroblastic areas, whereas the TLR2-positive RE cells outnumbered the TLR4-positive RE cells in the fibrotic areas. This study demonstrates that RE cells over-express TLR2 and TLR4 in the lungs of IIP patients. These findings suggest that high expression of TLRs may contribute to the pathogenesis of human IIP.

The morphology and molecular bases of damage to the stem cell niche of respiratory acini in idiopathic interstitial pneumonias

The authors present the material of their study of the morphological and molecular biological features of damage to the stem cell niches (SCN) in the respiratory acini of the lung and the significance of their occurring changes in the pathogenesis of chronic idiopathic interstitial pneumonias (IIP), including idiopathic pulmonary fibrosis (IPF), desquamative interstitial pneumonia (DIP), cryptogenic organizing pneumonia (COP) with bronchiolitis obliterans (BO), and nonspecific interstitial pneumonia (NSIP). The study was performed using open transthoracic (n=181) and transbronchial (n=71) lung biopsies from 194 patients (118 cases (61%) with IPF, 35 (18%) with NSIP, 23 (12%) with DIP, 18 (9%) with COP + BO). The serial paraffin sections were stained with hematoxylin and eosin and van Gieson's picrofuchsin and immunohistochemical reactions were carried out to detect MMP-1, MMP-2, MMP-7, Apo-Cas ("Novocastra", 1:100), vimentin (Vimentin) ("LabVision" 1:100), SMA ("LabVision", 1:100), TGF-β, TNF-α, CD34, Ost-4, and CD117 ("Dako", 1:50), CD68, and EMA ("Dako", 1:100). Biotinylated anti-mouse and anti-rabbit immunoglobulin antibodies ("Dako" LSAB + KIT, PEROXIDASE) were used as secondary antibodies. All the quantitative and semi-quantitative data obtained were processed by variation statistics. The compared IIPs were shown to differ in the site and degree of initial and secondary respiratory acinus damages caused by the aggressiveness of an inflammatory infiltrate and the spread of a lesion to different SCN areas involved in the regeneration of lung tissue. The mesenchymal cell with myofibroblast differentiation, which is probably associated with a mesenchymal stem cell, as evidenced by Oct-4, Vimentin, SMA, CD117, and CD34 expression by these cells, may be considered to be a marker cell of deep SCN damage. The author state that the clinical course and degree of morphological changes in IPP directly depend on the severity and depth of damage to the SCN areas of the respiratory acinus.

Lymphocyte subsets in bronchoalveolar lavage fluid from patients with idiopathic interstitial pneumonia

Lymphocyte activation may be involved in interstitial inflammatory processes in the lungs. We analyzed lymphocytes in bronchoalveolar lavage fluid obtained from 15 patients with idiopathic interstitial pneumonia (IIP) and from 7 controls. Clinical criterial were used to divide the patients with IIP into two groups: acute (n = 5) and chronic (n = 10). Lymphocytes in the bronchoalveolar lavage fluid were analyzed with a flow-cytometric two-color system. Differential cell counts showed that percentages and numbers of lymphocytes were significantly higher in patients with acute IIP (44.2 +/- 25.2%, 12.3 +/- 8.18 x 10(4)/ml) than in controls (8.31 +/- 3.66%, 0.69 +/- 0.39 x 10(4)/ml, p < 0.05) and in patients with chronic IIP (8.44 +/- 6.11%, 1.58 +/- 1.08 x 10(4)/ml, p < 0.05). In particular, percentages and numbers of CD8+ S6F1+ lymphocytes, which are regarded as activated cytotoxic T lymphocytes were markedly higher in patients with acute IIP (32.3 +/- 16.0%, 39.6 +/- 37.1 x 10(3)/ml), than in controls (6.31 +/- 1.69%, 0.42 +/- 0.25 x 10(3)/ml, p < 0.05) and in patients with chronic IIP (10.6 +/- 6.16%, 1.88 +/- 1.42 x 10(3)/ml, p < 0.05). These data suggest that patients with acute IIP differ from those with chronic IIP in the percentage and the number of lymphocytes in bronchoalveolar lavage fluid, and that activated cytotoxic T lymphocytes may play an important role in the pathogenesis of acute IIP.