Group Of Idiopathic Interstitial Pneumonias Research Articles

КРИПТОГЕННАЯ ОРГАНИЗУЮЩАЯСЯ ПНЕВМОНИЯ (КЛИНИЧЕСКИЙ ПРИМЕР, ОБЗОР ЛИТЕРАТУРЫ)

Despite the widespread introduction of CT into clinical practice, the diagnosis of cryptogenic organizing pneumonia is still an urgent task of modern radiology. Radiological diagnosticians and clinicians have significant difficulties in diagnosis due to rather low frequency of this disease and nonspecific clinical manifestations. Cryptogenic organizing pneumonia is a disease which sharply worsens the quality of life in case of untimely diagnosis and belongs to the group of idiopathic interstitial pneumonias. The article is devoted to the clinical case of a patient with verified cryptogenic organizing pneumonia. CT of the chest organs, along with clinical data, plays a key role in the accurate diagnosis of organizing pneumonia.

Nailfold capillaroscopy findings of interstitial pneumonia with autoimmune features.

We evaluated nailfold capillaroscopy (NFC) of interstitial pneumonia with autoimmune features (IPAF) and compared it with that of patients with connective tissue disease-interstitial lung disease (CTD-ILD) and idiopathic interstitial pneumonia (IIP). Patients with newly diagnosed as ILD were evaluated using NFC. Baseline demographic, clinical, serological, and high-resolution CT findings were collected. NFC was semi-quantitatively scored with six domains ranging from 0 to 18. In addition, the overall patterns (scleroderma/non-scleroderma patterns) were determined. A total of 81 patients (31 with CTD-ILD, 18 with IPAF, and 32 with IIP) were included. The non-specific interstitial pneumonia pattern was the most common ILD pattern in the CTD-ILD and IPAF groups, whereas the usual interstitial pneumonia pattern was the most common in the IIP group. The semi-quantitative score of the CTD-ILD group was higher than that of the IPAF or IIP groups (5.8 vs 4.2 vs 3.0, p < 0.001, respectively). Giant capillaries and haemorrhages were more frequently present in the CTD-ILD and IPAF groups than in the IIP group. A scleroderma pattern was present in 27.8% of the IPAF group, whereas none of the IIP patients showed a scleroderma pattern. NFC findings may be useful in classifying patients with ILD into CTD-ILD/IPAF/IIP.

Host factors and characteristics of hospitalized patients with pneumocystis jirovecii pneumonia

Objectives: To describe the underlying diseases, microbiologic examination and severity of hospitalized patients with Pneumocystis jirovecii pneumonia (PJP) in a tertiary Chinese hospital. Methods: We conducted a retrospective analysis of 485 identified PJP patients who were admitted to our hospital between January 2013 and December 2021. Results: Among the 485 enrolled PJP cases, there were 237 males and 248 females, aging (53.3±16.2) years (range from 14 y to 88 y). They were divided into 8 subgroups with variable underlying diseases. There were 209 cases with connective tissue diseases(CTD), 27 cases with non-hematologic malignancies, 38 cases with hematologic malignancies, 81 cases with kidney diseases, 33 cases with idiopathic interstitial pneumonia(IIP), 30 cases infected with human immunodeficiency virus (HIV), and 42 cases with miscellaneous underlying diseases. In the CTD group, there was more females than males, while male patients were predominant in both the malignant and the HIV groups. The Pneumocystis was identified in 44.95%(218/485) sputum samples and 92.01%(265/288) bronchoscopic samples. Pneumocystis asci were observed at direct microscopic examination with Grocott's methenamine silver stain in 4.95%(24/485)sputum samples and 9.72%(28/288)bronchoscopic samples. Pneumocystis DNA fragments were identified by PCR analysis in 43.09%(209/485)sputum samples and 90.63%(261/288)bronchoscopic samples. Among the 8 groups, cytomegaviremia and respiratory failure were most common in the HIV-infected PJP group, but the rates of mechanic ventilation, intensive care unit (ICU) admission and death were the lowest. There were less PJP patients in the IIP group (IIP-PJP) who received mechanic ventilation and admitted to ICU than the other groups except HIV-infected PJP group. However, the mortality rate was highest for the IIP-PJP group. Conclusions: CTD was the most common predisposed underlying disease for our enrolled PJP cases. Cytomegaviremia and respiratory failure were common in HIV-infected PJP patients, but the prognosis of HIV-PJP was slightly better than the others. The disease was more severe, rapidly progressive and fatal in the IIP-PJP group.

PD-L1 Expression in Patients with Idiopathic Pulmonary Fibrosis.

Background: Idiopathic pulmonary fibrosis (IPF) is the most common and severe form within the group of idiopathic interstitial pneumonias. It is characterized by repetitive alveolar injury in genetically susceptible individuals and abnormal wound healing, leading to dysregulated bronchiolar proliferation and excessive deposition of extracellular matrix, causing complete architectural distortion and fibrosis. Epithelial-to-mesenchymal transition is considered an important pathogenic event, a phenomenon also observed in various malignant neoplasms, in which tumor cells express programmed death-ligand one (PD-L1). The aim of this study was to assess the presence of PD-L1 in patients with IPF and other interstitial lung diseases (ILDs). Method: Patients with a clinically and radiologically suspected idiopathic interstitial pneumonia or other ILDs undergoing transbronchial cryobiopsy to confirm the diagnosis at the Department of Respiratory Diseases and Allergy, Aarhus University Hospital, were included in this prospective observational study. Cellular membrane PD-L1 expression in epithelial cells was determined using the DAKO PD-L1 IHC 22C3 PharmDx Kit. Results: Membrane-bound PD-L1 (mPD-L1) was found in twelve (28%) of the forty-three patients with IPF and in five (9%) of the fifty-five patients with other ILDs (p = 0.015). When adjusting for age, gender and smoking status, the odds ratio of having IPF when expressing mPD-L1 in alveolar and/or bronchiolar epithelial cells was 4.3 (CI: 1.3–14.3). Conclusion: Expression of mPD-L1 in epithelial cells in the lung parenchymal zones was detected in a consistent subgroup of patients with IPF compared to other interstitial pneumonias. Larger studies are needed to explore the role of mPD-L1 in patients with IPF.

Differential diagnosis of idiopathic pulmonary fibrosis

The review is devoted to the urgent problem of modern pulmonology: the differential diagnosis of idiopathic pulmonary fibrosis (ILF). ILF occupies a special place among many interstitial lung diseases for a number of reasons: 1) it is a deadly disease; 2) early diagnosis and adequate antifibrotic therapy significantly extend the life expectancy of patients; 3) anti-inflammatory drugs (corticosteroids) and cytostatics with ILF that are widely used in other forms of interstitial lung diseases are ineffective and accelerate the progression of the process; 4) the commonality of the main clinical signs (increasing respiratory failure) of various interstitial lung diseases. The list of respiratory diseases with which ILF should be differentiated is huge, and if with diffuse lung lesions of a known nature (disseminated pulmonary tuberculosis, pneumoconiosis, etc.) with a certain experience/qualification, the diagnosis is relatively simple, then the isolation of ILF from the group of idiopathic interstitial pneumonias always represents certain difficulties. The main methods used in the diagnosis of ILF are summarized taking into account current international and national recommendations.

Predictors of erectile dysfunction among male patients with idiopathic interstitial pneumonias

BackgroundSexual dysfunction is a neglected area among patients with idiopathic interstitial pneumonias (IIPs). Hence, the aim of this study is to identify the prevalence of erectile dysfunction (ED) and its predictors in male patients with IIPs.Patients and methodsA total 65 male patients (45 IIPs and 20 controls) were assessed; clinical data, testosterone levels, pulmonary function tests, arterial blood gases, and self-reported questionnaires on erectile function.ResultsThe prevalence of ED was 66.7 and 11.1% of them presented with severe ED. The mean age of the patients was 33.4±5.9 years. Of the patients, 60% were smokers and 17.8% were nonsmokers. Regarding comorbid manifestations, 26.7% was hypertensive, 51.1% had diabetes mellitus, 42.2% with dyslipidemia, and 17.8% had ischemic heart disease. The mean duration since the diagnosis of IIPs was 7.46±2.77 years, whereas the mean time since evolution of ED was 2.17±1.3 years. A significant decrease in mean arterial oxygen tension (PaO2) and oxygen saturation (SaO2) were observed in the IIPs group. Also, the mean diffusion lung capacity for carbon monoxide (DLCO%) and testosterone level were decreased. A significant decrease in testosterone level among patients with severe diffusion defect was observed (P=0.000). The most predictors for ED were PaO2 (≤60 mmHg), SaO2 (≤88%), and DLCO≤60%.ConclusionED is a common problem in patients with IIPs. Thus, physicians should keep in mind that IIPs patients need a comprehensive sexual evaluation. This is the first study to report that low PaO2, SaO2, and DLCO≤60% were the predictor risk factors.

Unique expression profiles of mucin proteins in interstitial pneumonia-associated lung adenocarcinomas.

In order to clarify idiopathic interstitial pneumonia (IIP)-associated lung adenocarcinoma (LADC), we herein focused on the expression profiles of mucin proteins, the most common cellular differentiation markers. The expression of the mucin (MUC) 1, MUC2, MUC3B, MUC4, MUC5AC, MUC5B, MUC6, MUC7, MUC9, and MUC21 proteins was examined immunohistochemically and their levels were semi-quantified in 80 IIP-associated LADCs and 106 non-IIP LADCs. LADCs were divided into low and high expressers based on thresholds obtained from the receiver operating characteristic (ROC) curves of each mucin protein. Low expressers of MUC1, MUC7, and MUC21 and high expressers of MUC4, MUC5AC, MUC5B, and MUC9 were dominant in the IIP group. Multivariate analyses confirmed that the correlations between mucin expression profiles and IIP-associated LADCs were independent of putative confounding factors, such as smoking, gender, histological types, and cytological types. Thus, the expression profiles of these mucin proteins significantly differed between the IIP and non-IIP groups. IIP-associated LADCs appear to have unique cellular differentiation features and they may develop through a distinct histogenetic pathway. This is the first study to demonstrate that IIP-associated LADCs have unique mucin expression profiles.

Diagnostic value of KL-6 in idiopathic interstitial pneumonia.

Idiopathic interstitial pneumonia (IIP) can induce type II alveolar epithelial cell proliferation and pulmonary basement membrane damage and subsequent release of Krebs von den Lungen-6 antigen (KL-6) to the bloodstream. This study investigated the diagnostic and prognostic value of serum KL-6 levels for IIP. One hundred five patients with lung disease were divided into IIP (n=75) and non-IIP groups (n=30) according to pathological and computed tomography findings. Serum KL-6 levels were evaluated in blood samples from all subjects. Nineteen IIP group patients were also subjected to a longitudinal study of disease progression and serum KL-6 levels over time. Serum KL-6 levels were significantly higher in the IIP group vs. the non-IIP group [1,096.0 (565.0-1,544.0) vs. 226.0 (173.5-346.5) U/mL; P<0.01]. Within the IIP group, serum KL-6 levels differed significantly between patients with and without concomitant disease or pulmonary infection (Z=-2.475, P=0.013). In a receiver operating characteristic (ROC) curve analysis, the area below the curve for serum KL-6 was 0.911 [95% confidence interval (CI): 0.847-0.975, P<0.001], indicating a good diagnostic performance for IIP, with a cut-off level of 485 U/mL, sensitivity of 85.33%, specificity of 90.00%, positive predictive value (PPV) of 95.52%, negative predictive value (NPV) of 71.05%, and Kappa value of 0.70. Accordingly, the serum KL-6 and clinical diagnostic results were consistent. Moreover, in the longitudinal study, the serum KL-6 levels differed significantly from before to after treatment in patients with exacerbated or improved disease (P=0.004 and P=0.043, respectively), whereas no obvious changes were observed in patients with stable disease (P=0.692). The serum KL-6 level is a valuable and significant diagnostic marker of IIP and a useful predictor of clinical prognosis.

Clinical characteristics of patients with anti-aminoacyl-tRNA synthetase antibody positive idiopathic interstitial pneumonia.

Anti-aminoacyl-tRNA synthetase (ARS) antibodies have been detected in patients with polymyositis/dermatomyositis (PM/DM) and are especially correlated with interstitial lung disease (ILD). The aim of this study was to clarify the clinical features of patients with anti-ARS antibody positive idiopathic interstitial pneumonias (IIPs). Patients were classified into three groups: 1) IIP with anti-ARS antibodies (ARS(+)IIP), 2) IIP without anti-ARS antibodies (ARS(-)IIP), and 3) PM/DM-associated ILD with anti-ARS antibodies (ARS(+)PM/DM-ILD). Clinical characteristics were compared retrospectively between the ARS(+)IIP group and the ARS(-)IIP group or ARS(+)PM/DM-ILD group. Eighteen ARS(+)IIP, 284 ARS(-)IIP, and 20 ARS(+)PM/DM-ILD patients were enrolled. The ARS(+)IIP group was significantly older and the male sex was predominant, had a lower prevalence of signs of connective tissue disease, differences in HRCT findings and patterns, and higher KL-6 levels compared to the ARS(+)PM/DM-ILD group. The findings in the bronchoalveolar lavage fluid (BALF) showing lymphocytosis and a lower CD4/CD8 ratio were similar between the two groups. However, the ARS(+)IIP group had significantly lower percentage of sputum, higher prevalence of mechanic's hand, higher KL-6 levels, lower percentage of vital capacity in the pulmonary function test, and lower CD4/CD8 ratio in BALF, compared to the ARS(-)IIP group. The present study demonstrated that features of pulmonary involvement were similar to those in the ARS(+)PM/DM-ILD group; however, some differences including HRCT findings and higher KL-6 levels suggest that ARS(+)IIP has severe ILD compared with ARS(+)PM/DM-ILD. Further prospective studies with a larger number of patients will elucidate the exact role of anti-ARS antibodies in IIPs.

Inhibition of Cell Apoptosis and Amelioration of Pulmonary Fibrosis by Thrombomodulin

Pulmonary fibrosis is the terminal stage of a group of idiopathic interstitial pneumonias, of which idiopathic pulmonary fibrosis is the most frequent and fatal form. Recent studies have shown that recombinant human thrombomodulin (rhTM) improves exacerbation and clinical outcome of idiopathic pulmonary fibrosis, but the mechanism remains unknown. This study evaluated the mechanistic pathways of the inhibitory activity of rhTM in pulmonary fibrosis. Transgenic mice overexpressing human transforming growth factor-β1 that develop spontaneously pulmonary fibrosis, and wild-type mice treated with bleomycin were used as models of lung fibrosis. rhTM was administered to mice by i.p. injection or by the intranasal route. Therapy with rhTM significantly decreased the concentration of high mobility group box1, interferon-γ, and fibrinolytic markers, the expression of growth factors including transforming growth factor-β1, and the degree of lung fibrosis. rhTM significantly suppressed apoptosis of lung epithelial cells in invivo and invitro experiments. The results of the present study demonstrated that rhTM can inhibit bleomycin-induced pulmonary fibrosis and transforming growth factor-β1-driven exacerbation and progression of pulmonary fibrosis, and that apart from its well-recognized anticoagulant and anti-inflammatory properties, rhTM can also suppress apoptosis of lung epithelial cells.

Year in review 2016: Interstitial lung disease, pulmonary vascular disease, pulmonary function, paediatric lung disease, cystic fibrosis and sleep.

Year in review 2016: Interstitial lung disease, pulmonary vascular disease, pulmonary function, paediatric lung disease, cystic fibrosis and sleep.

Effect of collagen vascular disease-associated interstitial lung disease on the outcomes of lung cancer surgery

PurposeThis study compared the effect of collagen vascular disease-associated interstitial lung disease (CVD-ILD) with that of idiopathic interstitial pneumonias (IIPs) on the outcomes of lung cancer surgery.MethodsThis study retrospectively reviewed the medical records of patients who underwent surgery for non-small cell lung cancer (NSCLC) and compared the data of 16 patients with CVD-ILD with those of 70 patients with IIPs. The patterns of interstitial lung disease (ILD) on chest computed tomography were classified into usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP) patterns.ResultsThe numbers of UIP and NSIP patterns were 10 (62.5%) and 6 (37.5%) patients in CVD-ILD group, and 62 (88.6%) and 8 (11.4%) patients in IIPs group, respectively. A postoperative acute exacerbation (AE) appeared in 1 patient (6.3%) in the CVD-ILD group and 6 patients (8.6%) in the IIPs group. No significant differences in the incidence of postoperative AE and mortalities were observed between the two groups. The five-year overall survival rates of the CVD-ILD and IIPs groups were 37.5 and 49.2%, respectively.ConclusionsSurgery for NSCLC in CVD-ILD patients appear to cause no increase in postoperative AE and mortality in comparison to that seen in IIPs patients. Similar to IIPs, CVD-ILD might therefore affect the prognosis of resected NSCLC.

The pathological features of idiopathic interstitial pneumonia-associated pulmonary adenocarcinomas.

To investigate the pathological features of idiopathic interstitial pneumonia (IIP)-associated pulmonary adenocarcinoma. Surgically resected adenocarcinomas associated with IIP (the IIP group) and adenocarcinomas without IIP (the non-IIP group) were subjected to analysis. Adenocarcinomas in the IIP group were subdivided into two groups: one group included tumours connected to bronchiolar metaplasia in honeycomb lesions (the H-IIP group), and the other included tumours unrelated to honeycomb lesions (the NH-IIP group). Histomorphological appearance and immunohistochemical expression were compared among the H-IIP group, the NH-IIP group, and the non-IIP group. Most of the tumour cells in the H-IIP group had a tall, columnar shape that showed similar features to proximal bronchial epithelium, whereas tumour cells in the NH-IIP group and the non-IIP group had a club-like shape that showed similar features to respiratory bronchiolar/alveolar epithelium. Adenocarcinomas in the H-IIP group tended to be negative for thyroid transcription factor-1 (TTF-1) and positive for hepatocyte nuclear factor-4α (HNF-4α). The frequency of EGFR mutations was significantly lower in adenocarcinomas in the H-IIP group, although the frequencies of KRAS and ALK mutations did not differ among the three groups. Idiopathic interstitial pneumonia-associated pulmonary adenocarcinomas, especially those arising from honeycomb lesions, have distinct pathological features.

Pemetrexed for advanced non-small cell lung cancer patients with interstitial lung disease.

BackgroundNon-small cell lung cancer (NSCLC) patients with interstitial lung disease (ILD) need to be approached carefully given the high incidence of pulmonary toxicity. Pemetrexed (PEM) is the key drug for the treatment of NSCLC. However, its safety, especially with respect to the exacerbation of ILD, and efficacy in NSCLC patients with ILD have yet to be established.MethodWe investigated the safety and efficacy of PEM monotherapy in NSCLC patients with or without idiopathic interstitial pneumonia (IIPs). The medical charts of these patients were retrospectively reviewed.ResultsTwenty-five patients diagnosed as having IIPs (IIPs group) and 88 patients without ILD (non-ILD group) were treated with PEM monotherapy at Juntendo University Hospital between 2009 and 2013. In the IIPs group, 12 patients were found to have usual interstitial pneumonitis (UIP) on chest computed tomography (CT) (UIP group) and the other 13 patients showed a non-UIP pattern on chest CT (non-UIP IIPs group). Three patients in the IIPs group (2 in the UIP group and 1 in the non-UIP IIPs group) and 1 in the non-ILD group developed pulmonary toxicity during treatment (3.5% overall, 12.0% in the IIPs group versus 1.1% in the non-ILD group). Moreover, all 3 patients in the IIPs group died of pulmonary toxicity. Overall survival tended to be longer in the non-ILD group than in the IIPs group (p = 0.08). Multivariate analyses demonstrated that IIPs was the only significant independent risk factor for PEM-related pulmonary toxicity.ConclusionWe found that the incidence of PEM-related pulmonary toxicity was significantly higher amongst NSCLC patients with IIPs than among those without IIPs. Particular care must be taken when administering PEM to treat NSCLC patients with IIPs.

Update for Pathologists on Idiopathic Interstitial Pneumonias

Idiopathic interstitial pneumonias are a subset of diffuse pulmonary interstitial diseases classified by international consensus in 2002 as idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis interstitial lung disease, desquamative interstitial pneumonia, and lymphoid interstitial pneumonia. Each is associated with a characteristic histopathologic pattern. In 2011, updated consensus guidelines were released for diagnosis and management of idiopathic pulmonary fibrosis. The entire group of idiopathic interstitial pneumonias is currently undergoing refinement, with updates expected in a forthcoming consensus classification. Many of these recent and anticipated changes are relevant to pathologists. To review international consensus guidelines for diagnosis of idiopathic pulmonary fibrosis and other idiopathic interstitial pneumonias and to discuss recent and expected future classification updates. Published peer-reviewed literature and personal experience of the authors. Diagnosis of idiopathic interstitial pneumonias by multidisciplinary discussion among clinicians, radiologists, and pathologists is now strongly encouraged. Diagnosis of idiopathic pulmonary fibrosis no longer requires surgical lung biopsy; high-resolution computed tomography is an acceptable surrogate. In the context of clinical trials, pathologists are being asked to assign levels of confidence for histologic diagnosis of usual interstitial pneumonia in patients with idiopathic pulmonary fibrosis. Acute exacerbation of idiopathic pulmonary fibrosis is now accepted and should be considered when acute lung injury is superimposed on a background of usual interstitial pneumonia. The updated classification of idiopathic interstitial pneumonias will include a separate category for rare entities, including lymphoid interstitial pneumonia and idiopathic pleuroparenchymal fibroelastosis.

Nicht spezifische interstitielle Pneumonie

Non-specific interstitial pneumonia (NSIP) belongs to the group of idiopathic interstitial pneumonias (IIP). However, NSIP can also be found in several other diseases. For example, the NSIP pattern is most commonly found in interstitial lung disease due to connective tissue disease. In this review, the definition and classification, aetiology, pathogenesis and histology, clinical symptoms, serological markers, lung function parameters, radiographic signs, treatment, and prognosis of NSIP are presented. Idiopathic NSIP as a distinct form of NSIP will be discussed separately.

Serum biomarkers in idiopathic pulmonary fibrosis

Within the group of Idiopathic Interstitial Pneumonias (IIPs), above all Idiopathic Pulmonary Fibrosis (IPF) poses a considerable diagnostic and therapeutic problem. Although genetic profiling indicates that IPF, Non Specific Interstitial Pneumonia (NSIP), and chronic hypersensitivity pneumonitis (HP) are distinctly different diseases, in every day practice these diseases can be difficult to tell apart. Furthermore, treatment of these diseases is notoriously difficult. Serum biomarkers reflect our understanding of the underlying pathogenesis and potentially fulfill a role in establishing a diagnosis, prognosis and therapy. While no single biomarker is currently able to accurately predict the presence or absence of an IIP, a composite of several markers holds promise for the future. Several biomarkers, such as KL-6, surfactant proteins and circulating fibrocytes, appear to contribute to our insight into disease progression and prognosis. It is however uncertain whether these markers give us additional information to common diagnostic tests and their value has as yet to be validated for every day practice. Fortunately, the potential of biomarkers is increasingly recognized and biomarker data are prospectively gathered in current placebo-controlled therapeutic trials.

Interstitial lung disease: new challenges and evolving phenotypes

There have been major changes in the field of interstitial lung disease (ILD) in the past decade, mostly in disease classification and diagnostic processes, and in the role of high-resolution computed tomography (HRCT) of the chest in assessment of diagnosis and prognosis. In the present issue of the European Respiratory Review , Harari and Caminati [1] review the current evidence related to ILD. This editorial emphasises some of the most important challenges in idiopathic pulmonary fibrosis (IPF) as well as some of the recent and changing concepts among the many phenotypes of ILD. IPF is the most severe of the chronic ILDs. One major advance has been made by the better delineating of the disease and the well-adopted international definition of IPF that allowed large clinical trials to be conducted. As some inconsistencies remain in the classification of the idiopathic interstitial pneumonias, an international collaborative task force has begun working under the auspices of the American Thoracic Society and the European Respiratory Society on a possible revision of the current classification. Among many other issues, the task force will have to address the still ongoing debate of usual interstitial pneumonia versus nonspecific pneumonia and their frequently overlapping features; the recently individualised syndrome of combined pulmonary fibrosis and emphysema [2]; and new histopathological patterns that might correspond to clinically relevant entities ( e.g. acute fibrinous and organising pneumonia, smoking-related interstitial fibrosis [3], etc .) [4]; and will question the adequacy of maintaining, in the group of idiopathic interstitial pneumonias, entities that are mostly due to environmental exposure or tobacco smoking and therefore not idiopathic ( e.g. desquamative interstitial pneumonia) or that are characterised by prominent alveolar, rather than interstitial, changes in histopathology ( e.g. organising pneumonia). Several messages emerge from the review by H …

Role of alveolar macrophages in the pathogenesis of idiopathic interstitial pneumonia

To clarify role of alveolar macrophages in the pathogenesis of idiopathic interstitial pneumonia (IIP), we studied (1) the localization and expression of monocyte chemoattractant protein-1 (MCP-1) in IIP by immunohistochemistry and in situ hybridization. (2) the role of MCP-1 in macrophage recruitment to be lung, and (3) the clinical usefulness of measuring MCP-1. (1) In IIP, MCP-1 was observed in cuboidal and flattened metaplastic epithelial cells, alveolar macrophages, and vascular endothelial cells. In contrast, no epithelial cells from patients without IIP stained positive for MCP-1, although alveolar macrophages and vascular endothelial cells were labeled. MCP-1 production by epithelial cells in IIP may be caused by the metaplastic nature of the epithelial cells and may be a main cause of the irreversible progression of IIP. (2) MCP-1 levels in bronchoalveolar lavage fluid (BALF) were significantly higher in the IIP, the interstitial pneumonia due to collagen vascular disease (IP-CVD) and sarcoidosis groups than in normal controls. In the IIP group, the MCP-1 level was significantly higher than in any other patient groups. In all three groups of patients, the monocyte chemotactic activity in BALF correlated positively with the MCP-1 levels in BALF, and were neutralized by anti-MCP-1. (3) BALF MCP-1 levels were significantly higher than serum MCP-1 levels in the IIP group, and they were lower in the IP-CVD and non-specific interstitial pneumonia groups. Serum MCP-1 levels reflected the activity of interstitial lung diseases, especially during treatment with corticosteroids. These results indicate (1) that MCP-1 plays a significant role in the recruitment of monocytes into the lung in IIP, (2) that measuring MCP-1 levels both in BALF and in serum may help discriminate IIP from other types of interstitial lung diseases, and (3) that monitoring the serum MCP-1 level may be useful in estimating the activity of interstitial lung diseases.

Variable prevalence of pulmonary hypertension in patients with advanced interstitial pneumonia

Pulmonary hypertension may occur in patients with interstitial pneumonia and is associated with increased mortality. We sought to determine the prevalence of pulmonary hypertension in sub-groups of patients with interstitial pneumonia and to investigate possible associations between pulmonary vascular hemodynamics and pulmonary function. The presence or absence of pulmonary hypertension was assessed in 70 patients with advanced interstitial pneumonia who underwent right heart catheterization. The associations of pulmonary hypertension with clinical characteristics and pulmonary function tests were analyzed. The prevalence of pulmonary hypertension in patients with idiopathic interstitial pneumonia was 29% vs 64% in patients with connective tissue disease-interstitial pneumonia (p = 0.013). African American patients had a significantly higher prevalence of pulmonary hypertension in the entire study population (81% vs 22%, p < 0.001) and in the idiopathic interstitial pneumonia group (70% vs 19%, p < 0.01). Regression analyses revealed no association between mean pulmonary artery pressure (mPAP) and forced vital capacity or mPAP and diffusion capacity of the lung for carbon monoxide in the entire cohort or in sub-groups of patients. African American patients and patients with connective tissue disease-interstitial pneumonia had a high prevalence of pulmonary hypertension. Non-African American patients with advanced idiopathic interstitial pneumonia (including idiopathic pulmonary fibrosis) had a low prevalence of pulmonary hypertension.