Clinical Research on Prognostic Evaluation of Subjects With IPF by Peripheral Blood Biomarkers,Quantitative Imaging Characteristics and Pulmonary Function Parameters

IntroductionIdiopathic pulmonary fibrosis (IPF) is an irreversible and progressive fatal interstitial lung disease with a poor prognosis. The aim of this study is to investigate the predictive value of combined blood biomarkers, pulmonary function and quantitative monitoring by computer-aided diagnosis (CAD) system in IPF patients.MethodsPulmonary baseline function and pathological features of 126 patients with IPF were analyzed using spirometry and chest X-ray. Patients were divided into survival group and non-survival group after 5 years follow-up. The relationships the levels of peripheral blood biomarkers, quantitative imaging characteristics and pulmonary function were analyzed between the two groups.ResultsThe baseline level of serum Krebs von den Lungen-6 (KL-6) and C-X-C motif chemokine 13 (CXCL13) were moderately or highly correlated with annual changes in forced vital capacity (FVC), carbon monoxide diffusing capacity (DLCO), total lung capacity (TLC), total interstitial lung disease (ILD) lesions, and the volume changes of reticular. The baseline level of serum KL-6 was higher than the cut-off value of 800.0 U/ml and baseline level of serum CXCL13 was higher than the cut-off value of 62.0 pg/ml. IPF patients with baseline levels of serum KL-6 and CXCL13 lower than the cut-off value had longer median survival time.ConclusionsSerum KL-6 and CXCL13 may be predictive biomarkers for the outcomes of patients with IPF patients and their baseline levels were related to the progression of pulmonary function and quantitative monitoring by CAD system.     

Clinical impact of the radiological indeterminate for usual interstitial pneumonia pattern on the diagnosis of idiopathic pulmonary fibrosis

BackgroundIdiopathic pulmonary fibrosis (IPF) is a fatal lung disease associated with significant morbidity and mortality. The international clinical practice guidelines for the diagnosis of IPF have recently been revised.MethodsIn this single-center retrospective study conducted between June 2006 and March 2018, 27 patients with a newly classified indeterminate for usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) who had undergone surgical lung biopsy were enrolled at the Japanese Red Cross Medical Center. Clinical and pathological characteristics and prognosis were retrospectively analyzed from patient records.ResultsOn the basis of multidisciplinary discussion (MDD), IPF was diagnosed in six patients (22%), unclassifiable interstitial pneumonia in 5 (19%), chronic hypersensitivity pneumonitis in 10 (37%), collagen vascular disease-associated interstitial lung disease in 5 (19%), and lymphoproliferative disorder in 1 (4%) patient. Ground-glass opacity, peribronchovascular distribution, upper or middle lobe distribution, mosaic attenuation, consolidation patterns, and honeycombing were found on HRCT. Histological UIP or probable UIP was observed in seven patients. The median survival time from the initial visit was 2770 days (92.3 months). There was a significant difference in survival time in the GAP stage and honeycombing on HRCT according to the log-rank test.ConclusionsPatients with an indeterminate for UIP pattern on HRCT were more likely to have non-IPF than IPF through pathological diagnosis and MDD. GAP stage and honeycombing on HRCT may be significant risk factors for all-cause mortality.     

Features of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome compared with idiopathic pulmonary fibrosis

BackgroundThe different characteristics of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome (UIP/pSS) compared with idiopathic pulmonary fibrosis (UIP/IPF) are not fully understood. This study aimed to compare characteristics, prognosis, and treatment responses in these patients.MethodsAmong 129 consecutive patients who underwent surgical lung biopsy to diagnose diffuse lung diseases at Kanagawa Cardiovascular and Respiratory Center between 1998 and 2002, we identified 10 and 19 patients with UIP/pSS and UIP/IPF, respectively. Baseline characteristics, chest high-resolution computed tomography (HRCT) and pathological findings, and the clinical course were compared between the two groups. Responses to immunosuppressive therapy were analyzed by comparing pulmonary function and clinical status before and one year after treatment initiation.ResultsMore patients in the UIP/pSS group tended to be female and older than those in the UIP/IPF group (mean age, 68 years vs. 62 years). In addition, they more commonly exhibited enlarged mediastinal lymph nodes and bronchial wall thickening on HRCT. Pathologically, in the UIP/pSS group, interstitial inflammation, plasma cell infiltration, lymphoid follicles with germinal centers, cysts, bronchiolitis, and pleuritis were significantly more prominent, whereas smooth muscle hyperplasia and fibroblastic foci were milder (all P<0.05). The prognosis was better for UIP/pSS compared with UIP/IPF patients (P=0.01). In addition, immunosuppressive therapy provided better disease control for those with UIP/pSS (83%, 5/6) compared UIP/IPF (7%, 1/15).ConclusionThis study identified distinct clinical, radiological, and pathological characteristics of UIP/pSS compared with UIP/IPF. Immunosuppressive treatment could be a therapeutic option for UIP/pSS.     

Hypersensitivity Pneumonitis and (Idiopathic) Pulmonary Fibrosis Due to Feather Duvets and Pillows

IntroductionExposure to feather bedding may be an unnoticed cause of hypersensitivity pneumonitis (HP) and idiopathic pulmonary fibrosis (IPF). Thus, an in-depth clinical study of the diagnosis of patients with suspected HP and IPF is required in order to determine their etiologies. The objective of the present study is to raise awareness of HP and pulmonary fibrosis due to exposure to feather bedding, and to study the prevalence and describe long-term outcomes.MethodsWe describe a series of 33 patients diagnosed with HP and pulmonary fibrosis due to feather bedding exposure and followed over a 10-year period. The patients were from a subgroup of 127 individuals with HP undergoing in-depth evaluation using a diagnostic protocol at a regional referral center.ResultsEleven (33%) patients were clinically diagnosed with acute HP and 22 (67%) with chronic HP. Ten (45%) chronic HP patients showed a high resolution computed tomography (HRCT) pattern of usual interstitial pneumonia (UIP) with suspected IPF. The prevalence of HP was 6.2/100 000 feather bedding users (compared with 54.6 per 100 000 bird-breeders). The survival rates of patients over the 10-year period was 100% for acute HP and 64% for chronic HP.ConclusionsIn a series of HP patients, the diagnosis was attributed to feather bedding exposure in 26%. UIP pattern on HRCT was present in nearly half of the chronic cases. The survival of patients with chronic HP at ten years was 64%, despite avoiding further exposure.     

The effect of emphysema on lung function and survival in patients with idiopathic pulmonary fibrosis

Background and objective: In this study the prevalence, lung function and prognosis of IPF combined with emphysema were evaluated. Methods: Consecutive patients with usual interstitial pneumonia (UIP) on high‐resolution computed tomography (HRCT), with or without emphysema, were assessed retrospectively. The area of fibrosis in the base of the lungs was assessed by HRCT as minimal (<2 cm from the subpleura), moderate (≥2 cm from the subpleura, <1/3 of the area of the base of the lungs) or severe (≥1/3 of the area of the base of the lungs). Results: Among 660 patients with UIP on HRCT, 221 showed upper‐lobe emphysema. Pulmonary function results for patients with UIP and UIP/emphysema, respectively, were: FVC, 71.8% and 87.1%; FEV1%, 86.7% and 87.9%; and DL_CO, 74.3% and 65.2% of predicted. The relationship between FVC, the extent of fibrosis and survival was investigated in 362 patients with records of pulmonary function tests and no lung cancer at the time of entry into the study. Although the extent of fibrosis was similar between the groups, 71.3% of UIP patients met the lung volume criteria for IPF (FVC <80% of predicted), whereas only 26.5% of UIP/emphysema patients met the lung volume criteria for IPF. Median survival was 7.5 years in the UIP group and 8.5 years in the UIP/emphysema group. Conclusions: Emphysema was a common finding in patients with UIP. Patients with UIP and emphysema had greater lung volumes and better survival compared with those with UIP alone.     

BALF N-acetylglucosaminidase and beta-galactosidase activities in idiopathic pulmonary fibrosis

The lysosomal enzymes N-acetylglucosaminidase (N-ACGA) and beta-galactosidase (beta-gal) are involved in cellular collagen metabolism and may, therefore, be markers of fibrosis in idiopathic interstitial pneumonias, such as idiopathic pulmonary fibrosis (IPF). N-ACGA and beta-gal were analyzed in the bronchoalveolar lavage fluid (BALF) of patients with the histologic pattern of usual interstitial pneumonia (UIP, n=10) and controls (n=9). Cellular distribution in BALF as well as the concentration of TGF-beta a well-known mediator of fibroblast matrix deposition were correlated to the enzyme activities in both groups of patients. We found that both, N-ACGA (UIP: 25.2 nmol/l s +/- 3.4; controls: 73 nmol/l s +/- 1.3) and beta-gal (UIP: 4.7 nmol/l s +/- 0.5; controls: 2.4 nmol/l s +/- 0.3) were elevated significantly in BALF of patients with IPF compared to that of control patients (P<0.003). This increase was paralleled by an increase in neutrophils (IPF: 17.9% +/- 21.8; controls: 5.4% +/- 6.3; P=0.03) and eosinophils (IPF: 2.0% +/- 1.5; controls: 0.2% +/- 0.45; P=0.002) in BALF fluid. In addition, N-ACGA activity correlated closely with lung function (FVC, TLC, and DLCO), transforming growth factor-beta (TGF-beta) in BALF (r=0.77, P=0.008) and activated lymphocytes (r=0.66, P=0.0021). Our findings suggest that measurement of lysosomal enzymes such as N-ACGA may represent a useful indicator of fibrotic activity in IPF.     

Several specific high-resolution computed tomography patterns correlate with survival in patients with idiopathic pulmonary fibrosis

BackgroundEvidence of honeycombing in high-resolution computed tomography (HRCT) is a recognized risk factor for shortened survival in patients with idiopathic pulmonary fibrosis (IPF), but few studies have evaluated the feasibility of exploiting other specific patterns for predicting survival. The aim of this study was to examine the extent of specific HRCT patterns in IPF and determine whether they correlate with clinical features, pulmonary function tests (PFT), and survival.MethodsBoth the presence and extent of specific HRCT patterns, such as traction bronchiectasis, honeycombing, architectural distortion, reticulation, emphysema, and ground glass opacity, in 129 HRCT examinations were scored semi-quantitatively in three zones of each lung. HRCT examinations were also re-classified according to the 2011 and 2018 international statements. Correlations were calculated between the scores of specific HRCT patterns, clinical features, PFT, and patient survival.ResultsThe extent of traction bronchiectasis was found to be an independent risk factor of shortened survival (HR 1.227, P=0.001). Patients with a possible usual interstitial pneumonia (UIP) pattern had a better median survival than the patients with a definite UIP pattern (61 vs. 37 months, P=0.026). The extents of traction bronchiectasis, honeycombing, and architectural distortion displayed an inverse correlation with all PFT values at the time of diagnosis. There were few differences between the radiological classifications of the 2011 and 2018 international statements.ConclusionsWe conclude that several specific HRCT patterns displayed a correlation with shortened survival in IPF; these may help in evaluating the risk of death in IPF patients.     

Pulmonary fibrolysis in a patient with idiopathic pulmonary fibrosis: improvement of clinical and radiological pattern after treatment with pirfenidone

Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive, fibrosing interstitial pneumonia associated with the histologic and/or radiologic pattern of usual interstitial pneumonia (UIP). Nowadays, the high‐resolution computed tomography pattern of “definite UIP” is enough to define a diagnosis of UIP without histological proof. This is pivotal especially in elderly patients with comorbidities. Early recognition of IPF is relevant for its prognostic implication. Some pharmacological strategies have been proposing novel molecules that tend to slow lung function decline, even though without healing fibrosis. We report a case of ex‐heavy smoker male with IPF showing clinical and radiological improvement after 11 months of treatment with Pirfenidone. The definite diagnosis was challenging and it was reached by a multidisciplinary approach.     

Idiopathische Lungenfibrose

The diagnosis of idiopathic pulmonary fibrosis (IPF) is based on a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) in patients not subjected to surgical lung biopsy or specific combinations of HRCT with histopathological patterns after exclusion of known causes of interstitial lung disease. The diagnostic work-up should also include initial lung function and the diagnosis of comorbidities. The interdisciplinary discussion of clinical, radiological and histological data is essential.     

Auscultation of Velcro Crackles is Associated With Usual Interstitial Pneumonia

Auscultation of Velcro crackles has been proposed as a key finding in physical lung examination in patients with interstitial lung diseases (ILDs), especially in idiopathic pulmonary fibrosis (IPF). However, no studies have been carried out to assess the association of Velcro crackles with other clinical variables.We evaluated a cohort of 132 patients, prospectively and consecutively included in our ILD diagnostic program at a tertiary referral center. All patients were auscultated during the physical examination. The patients were divided into 2 groups: “presence” or “nonpresence” of bilateral Velcro crackles.Of all patients assessed, 83 (63%) presented Velcro crackles in the respiratory auscultation. Patients with Velcro crackles usually had more frequently cough and dyspnea at the moment of diagnosis. Forced vital capacity (P = 0.002) and lung diffusion capacity for carbon monoxide (P = 0.04) was lower in these patients. The ILD-GAP index was higher in the group with Velcro crackles (P = 0.01). All patients with usual interstitial pneumonia (UIP) in high-resolution computed tomography and all patients with final IPF diagnosis presented Velcro crackles. In multivariate analysis, the presence of Velcro crackles was independently associated with an UIP pattern.In patients suspected of having ILD, the auscultation of Velcro crackles was associated with UIP, a possibility which must be taken into consideration in early ILD detection in primary care.     

Latin American Registry of Idiopathic Pulmonary Fibrosis (REFIPI): Clinical Characteristics,Evolution and Treatment

IntroductionIdiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and frequently fatal disease. Currently there are national and multinational registries in Europe, United States, Australia and China to better understand the magnitude of the problem and the characteristics of the IPF patients. However, there are no national or regional registries in Latin America, so the objective of this study was to carry out a Latin American registry that would allow the identification of IPF patients in our region.MethodologyA system consisting of 3 levels of control was designed, ensuring that patients met the diagnostic criteria for IPF according to international guidelines ATS/ERS/ALAT/JRS 2011. Demographic, clinical, serological, functional, tomographic, histological and treatment variables were recorded through a digital platform.Results761 IPF patients from 14 Latin American countries were included for analysis, 74.7% were male, with a mean age of 71.9 + 8.3 years. In general there was a long period of symptoms before definitive diagnosis (median 1 year). In functional tests, an average reduction of FVC (70.9%) and DLCO (53.7%) was detected. 72% received at least one antifibrotic drug (pirfenidone or nintedanib) and 11.2% of the patients had an acute exacerbation, of which 38 (45.2%) died from this cause.ConclusionsLike other registries, we found that there is difficulty in the recognition and excessive delay in the diagnosis of IPF in Latin America. Most of the patients in REFIPI received antifibrotics; these were well tolerated and associated with fewer adverse events than those reported in clinical trials.     

Serial 6-month change in forced vital capacity predicts subsequent decline and mortality in Japanese patients with newly diagnosed idiopathic pulmonary fibrosis

BackgroundThe clinical course of idiopathic pulmonary fibrosis (IPF) is characterized by a progressive decline in lung function; however, predicting changes in lung function is difficult. We sought to determine whether the prior 6-month trend in forced vital capacity (FVC) could predict mortality and the subsequent 6-month trend in FVC.MethodsWe retrospectively analyzed consecutive patients with newly diagnosed IPF who underwent serial pulmonary function tests. The immediate two years after the initial evaluation were divided into four terms of six months each and stratified on the basis of presence or absence of a ≥10% relative decline in FVC at six months (declined and stable groups, respectively).ResultsWe included 107 patients with %predicted FVC of 80.8% and %predicted diffusing capacity of the lung for carbon monoxide of 58.9%. In multivariate analysis, a decline in %predicted FVC in the initial six months was found to be an independent prognostic factor (hazard ratio 4.45, 95% confidence interval 2.62–7.56, p < 0.01). Among the 46 terms in which the FVC declined during the initial 1.5-year study period, a decline in FVC was exhibited in 23 (50.0%) of the subsequent terms. Among 231 terms in which FVC remained stable, a decline was observed in 32 (13.9%) of the subsequent terms (relative risk 3.61, p < 0.01). The frequency of FVC decline in each term was 16–27%. FVC was stable or declined in all four terms in 50.5% and 15.9% of cases, respectively.ConclusionsSix-month decline in FVC predicts subsequent FVC change and mortality in IPF patients in the era of antifibrotic agents.     

Interferon gamma-1b as therapy for idiopathic pulmonary fibrosis. An intrapatient analysis

BACKGROUND: The only proven therapeutic option for idiopathic pulmonary fibrosis (IPF) is lung transplantation. It is biologically plausible that interferon gamma-1b (G-IFN) may halt or even reverse the disease process and therefore prove to be an effective medical therapy. We report our results using this medication in a cohort of patients with a wide range of severity of IPF. OBJECTIVES: To determine the impact of G-IFN therapy on the progression of disease in patients with IPF. METHODS: We performed a retrospective analysis of patients' pulmonary function tests (PFTs), specifically the forced vital capacity (FVC) and the single breath diffusing capacity for carbon dioxide (DLCO). Comparisons of these parameters prior to and after the implementation of therapy were made by generating regression slopes by least-squares equations. RESULTS: Twenty-two patients qualified for the analysis. For the FVC, comparison of pre- and post-therapy best-fit regression lines demonstrated a significant difference in favor of G-IFN (p<0.015). For the DLCO, a significant difference in favor of gamma interferon therapy was detected in the advanced group (p<0.03). Seventy percent of the patients showed either stabilization or regression of disease in both their FVCs and their DLCO. CONCLUSION: G-IFN appears to be an effective new therapy for patients with IPF. Salutary effects on the rate of change in the FVC and DLCO were most apparent in patients with advanced disease. With slowing or reversal in loss of lung function, G-IFN may improve longevity and may have utility as a bridge to lung transplantation in those patients who are appropriate candidates.     

Serum amyloid A in patients with idiopathic pulmonary fibrosis

BackgroundSerum amyloid A (SAA) is an apo-lipoprotein (12–14 kDa) produced by the liver in response to proinflammatory cytokines from activated monocytes. The precursor of SAA is an acute-phase protein involved in the pathogenesis of sarcoidosis and has been found to be increased during exacerbation of chronic obstructive pulmonary disease and lung cancer. However, no data are available on SAA levels in patients with idiopathic pulmonary fibrosis (IPF), the most common and severe idiopathic form of interstitial pneumonitis associated with a usual interstitial histological and radiological pattern. The aim of this preliminary study was to evaluate SAA concentration in patients with IPF and to explore its potential use as a clinical biomarker.MethodsSAA levels were determined by enzyme-linked immunosorbent assay in a population of 21 patients with IPF (14 male, aged 64.8 ± 8.1 years) and compared with those in 11 healthy controls (3 male, aged 55 ± 11.3 years). Clinical, functional, and immunological data were collected in a database.ResultsSAA levels were significantly higher in patients with IPF than in controls (p = 0.03). In patients with IPF, statistically significant correlations were found between SAA and HDL cholesterol levels (r = ?0.62, p = 0.05) and FVC % predicted value (r = ?0.52, p = 0.01).ConclusionsSAA is a promising marker of disease severity in patients with IPF. Our preliminary data suggest a potential pathogenetic role of alteration in lipid metabolism in this rare disease.     

Pulmonary dysfunction due to combination of extra-pulmonary causes and alveolar damage is present from first the day of hospital admission in the early phase of acute pancreatitis

BackgroundOnly few studies have attempted to evaluate the pulmonary function in the early phase of acute pancreatitis (AP), although pulmonary dysfunction is the most frequent complication in the early phase of AP. We aimed to evaluate the changes in pulmonary function tests during the early phase of AP.MethodsProspective cohort study including 44 patients (52% men; median age 54 years) admitted with first attack of AP and 22 healthy controls. Patients underwent assessments on day 1, 2, 3, 6, and 10 as well as one month after discharge. Pulmonary function tests included the % predicted: forced expiratory volume during the first second (FEV1), forced vital capacity (FVC), total lung capacity (TLC), diffusion lung capacity (DLCO) and the ratio between DLCO and alveolar volume (DLCO/VA).ResultsIn total, 9% developed severe acute pancreatitis, 7% died, and 14% required treatment at the intensive or semi-intensive care unit. From admission, patients had impaired FEV1, FVC, DLCO, and TLC compared with controls (p < 0.0001 in all analyses). Patients with CRP >150 mg/L had significantly lower lung function tests. One month after discharge, lung function tests improved but patients had lower FEV1 (p = 0.014), FVC (p = 0.022), TLC (p = 0.020), and DLCO (p < 0.001) compared with controls.ConclusionThis study found that patients with AP had evidence of pulmonary impairment from the first day after hospital admission. The impairment lasted several weeks after hospital discharge.     

Prognostic implications of histologic patterns in multiple surgical lung biopsies from patients with idiopathic interstitial pneumonias

STUDY OBJECTIVES: To determine the prevalence and prognostic significance of histologic discordance in multiple lung biopsy specimens obtained from patients investigated for suspected cryptogenic fibrosing alveolitis (CFA)/idiopathic pulmonary fibrosis (IPF). METHODS: and results: Between 1984 and 2001, 64 patients undergoing investigation for CFA/IPF were identified in whom multiple biopsies were performed that showed either a pattern of usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP). These cases were classified into three groups: concordant UIP-UIP (n = 25, 39.1%), discordant UIP-NSIP (n = 8,12.5%), and concordant NSIP-NSIP (n = 31, 48.4%). The discordant UIP group had survival, clinical, and physiologic features similar to those of the concordant UIP group, and prognosis in both concordant and discordant UIP groups was significantly worse than that of the concordant NSIP group (p = 0.02 and p = 0.04, respectively). The age of the concordant UIP group was higher than that of the concordant NSIP group, with the mean age of the discordant group being intermediate. There were no significant differences among the three groups in smoking history, duration of dyspnea, presence or absence of crackles, FVC, diffusion capacity of the lung for carbon monoxide, or PaO(2). CONCLUSIONS: Patients with discordant UIP-NSIP results on multiple biopsies show clinical behavior similar to those with concordant UIP-UIP and should be regarded as having CFA/IPF in the correct clinical context, rather than "idiopathic NSIP" for the purposes of management. Multiple biopsies should be considered in all patients in order to improve the prognostic information provided by lung biopsy.     

The clinical significance of histopathologic subgroups in idiopathic interstitial pneumonia: is surgical lung biopsy essential?

For many years, the clinical entity of idiopathic interstitial pneumonia (IIP) has been a source of confusion for physicians. There has been much debate over the utility of subclassifying this condition histopathologically. It now appears that such classification is useful, and the most important distinction is the presence or absence of usual interstitial pneumonia (UIP). Unlike the other histopathologic subgroups, UIP has a grave prognosis and responds poorly to traditional therapies. To emphasize this clinical difference, the diagnosis of idiopathic pulmonary fibrosis (IPF), once used synonymously with IIP, is now reserved for only those patients with the histopathologic pattern of UIP. Although the gold standard for the diagnosis of IPF/UIP remains surgical lung biopsy, recent studies suggest that careful clinical and radiographic evaluation can identify IPF/UIP with a specificity of 90% or more. In the absence of a clear clinical diagnosis, we recommend pursuing surgical lung biopsy. Knowledge of the underlying histopathology will allow for more accurate prognosis, help guide therapy, and make possible the clinical investigation of novel therapeutic agents for patients with IIP.     

A clinical study of idiopathic pulmonary fibrosis based on autopsy studies in elderly patients

OBJECTIVES: The prevalence of idiopathic pulmonary fibrosis (IPF) tends to be greater in the elderly although the precise outcome is not known. The present study investigated the outcome of IPF in elderly patients whose pathological diagnosis corresponded to usual interstitial pneumonia (UIP) on autopsy findings. METHODS: In a consecutive autopsy series, (n = 4,554 cases), complete information was obtained in 86 cases in which the clinical and pathological diagnosis was IPF and UIP, respectively. RESULTS: Mean subject age was 80.5 years and there were 52 males and 34 females. Median survival time (MST) was 3.5 years overall. Dyspnea ranking by the MRC dyspnea scale (DR) on admission was closely related to the outcome. When MST was compared between DR0 and DR1, or DR2 and DR3, the former patient group survived significantly longer than the latter (p < 0.05). Twenty percent of patients died of severe respiratory failure. Other causes of death were disease progression of IPF (10.5%), bacterial pneumonia (23.3%) and lung cancer (17.4%). The patients in the young-elderly group (age < 75) survived for a significantly longer period than those in the old-elderly group (age > or = 75) (MST; 5.5 vs 2.0 years; p < 0.001). Both lung cancer and pneumonia were significantly more prevalent among IPF patients than among non-IPF patients; these IPF with lung cancer profoundly reflected the smoking history of IPF patients. CONCLUSIONS: The poor outcome in IPF in elderly patients was characterized by more severe dyspnea, advanced age and co-morbidity.     

支气管肺泡灌洗液细胞类型与特发性肺纤维化预后的关联性研究

目的 探讨临床指标、肺功能和BALF中细胞类型与特发性肺纤维化(IPF)患者预后的关系.方法 经临床诊断的43例IPF患者进行肺功能和支气管肺泡灌洗检查.采用Kaplan-Meier检验比较组间生存率,采用Cox比例风险回归方法评价各参数的死亡风险度.结果 IPF患者存在限制性通气功能障碍和弥散功能障碍,FVC占预计值%、肺总量占预计值%和DLCO占预计值%分别为(61±18)%、(54±13)%和(48±14)%.在平均随访30.7个月内,IPF患者诊断后的中位生存期为28.5个月.糖皮质激素和(或)细胞毒类药物治疗不能改变IPF患者的预后.单因素Cox比例风险回归分析结果表明,FVC占预计值%、肺总量占预计值%、DLCO占预计值%、中性粒细胞和嗜酸粒细胞比例是影响IPF患者预后的因素,多因素Cox比例风险回归分析结果表明仅DLCO占预计值%和中性粒细胞比例是影响IPF患者预后的主要因素.结论 IPF患者主要为老年男性,存在限制性通气功能障碍和弥散功能障碍.糖皮质激素和(或)细胞毒类药物不能改变IPF患者的预后.DLCO占预计值%和中性粒细胞比例是影响IPF患者预后的主要因素,其中DLCO占预计值%和IPF患者的预后呈负相关,中性粒细胞比例和IPF患者的预后呈正相关.