Effects of surgical correction on pulmonary function in pectus excavatum cases

Preoperative and postoperative pulmonary function were compared by spirometry in one hundred and eleven cases, from 1980 to 1989, with pectus excavatum deformity. The patients consisted of 85 males and 26 females, aged 5-41 years. In the preoperative examination, mean values for % vital capacity (%VC), % 1 sec. timed vital capacity (FEV1.0%), % residual volume (%RV), % maximal voluntary ventilation (%MVV) were within normal limits. %VC had a tendency to decrease with increase in the degree of depression of the anterior chest wall. Postoperatively, %VC was significantly dropped, although no significant change was seen in FEV1.0%, %RV and %MVV. Seven of 10 cases with low %VC and four of 6 cases with low %VC and %RV preoperatively demonstrated improvement in %VC and %RV after sternal elevation surgery.     

Value of spirometry in detecting volume restriction in interstitial lung disease patients. Spirometry in interstitial lung diseases

BACKGROUND: Restriction is a typical functional abnormality in interstitial lung disease (ILD) patients, but is not always present, especially in the early stage of the disease. The greater reduction of vital capacity (VC; %pred.) than total lung capacity (TLC; %pred.) is regarded as a typical pattern of lung function disturbances in ILD patients. STUDY OBJECTIVES: To explore the diagnostic value of spirometry in a detection restrictive pattern the relative volume loss assessed by TLC and VC in large series of patients with a diagnosis of ILD referred for lung function tests was evaluated. METHODS: Retrospective, cross-sectional analysis of pulmonary function data was done. The sampleconsisted of 1,173 patients with the diagnosis of different interstitial lung diseases investigated during a period of 5 years. Only patients without airway obstruction (normal FEV1%VC) were included. In all cases spirometry and whole body plethysmography were performed by experienced staff using MasterLab - 'Jaeger' equipment according to ERS standards. Reference values according to ERS guidelines were applied. RESULTS: The mean value of TLC expressed as %pred. was significantly (p < 0.001) lower than VC in all patients (93.7 +/- 18.6 vs. 98.0 +/- 21.4%pred.). The frequency of abnormal (lowered) TLC results was also higher than lowered VC (22.8 vs. 17.8%). Sensitivity of VC reached 69.3% and the positive predictive value was 88.5% in detecting volume restriction as compared to TLC measurement. CONCLUSION: The relative loss of TLC was greater than VC in our large group of patients. Measurement of TLC should be part of functional assessment of ILD patients, irrespective of whether they present or do not present a restrictive pattern in spirometry.     

Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes

BACKGROUND: To investigate whether the better prognosis of interstitial pneumonias associated with collagen vascular disease (CVD) compared with idiopathic interstitial pneumonia (IIP) is due to higher frequency of the nonspecific interstitial pneumonia (NSIP) pattern in CVD, we compared the outcomes of patients from these two groups with the same histopathologic pattern. SUBJECTS: The clinical features and survival of 362 patients (269 with IIP and 93 with CVD) diagnosed using surgical lung biopsy were analyzed. RESULTS: The mean survival of the CVD group (131.0 mo) was longer than that of the IIP group (80.5 mo) (p<0.0001). The patients with usual interstitial pneumonia pattern among the CVD group (n=36) was younger, female, and predominantly nonsmoking compared with the IIP group (n=203). Although baseline lung functions were not significantly different, the CVD group survived longer (mean, 177.0 mo) than the IIP group (mean, 66.9 +/- 6.5 mo; p=0.001). By multivariate analysis, younger age, better pulmonary function, and the presence of a CVD were independent prognostic factors. In NSIP pattern, no significant differences in survival, clinical features, or lung function were found between the two groups. CONCLUSION: Our data suggest that the better prognosis of patients in the CVD group is not solely due to the predominance of the NSIP pattern. The prognosis of patients with the usual interstitial pneumonia pattern in CVD is better than in those with idiopathic pulmonary fibrosis, despite the same pathologic pattern. In contrast, in those with an NSIP pattern, the prognosis is similar in both groups.     

A prospective study of lung cancer in cases of idiopathic interstitial pneumonia (IIP)]

The incidence of lung cancer in 45 patients (37 males and 8 females), who were originally diagnosed as having idiopathic interstitial pneumonia (IIP), was studied prospectively by following them 4 to 10 years. As controls, sex-, age-, and smoking history matched patients with chronic obstructive lung disease (COLD) were also surveyed for the same follow up period. In addition, the differences in clinical findings between those with and without lung cancer were examined. Eight out of 45 patients with IIP (18%) developed lung cancer (adenocarcinoma; 4, small cell carcinoma; 3, squamous cell carcinoma; 2, including one double cancer). All lung cancer patients were male smokers. Looking only at males, 8 out of 37 patients with IIP (21.6%) developed lung cancer. This incidence was significantly higher than that of the male COLD group (2.2%, less than 0.01). In clinical findings, IIP patients with lung cancer had the characteristics of the chronic type of IIP (lack of volume loss on chest radiographic milder dyspnea, not on steroid treatment) when compared with those who did not develop lung cancer.     

AnnexinⅡ在特发性间质性肺炎肺组织中的表达及可能作用的初步探讨

目的检测膜联蛋白Ⅱ（AnnexinⅡ）在特发性间质性肺炎（IIP）中的表达情况,探讨AnnexinⅡ的可能作用机制。方法采用免疫组织化学技术检测IIP及正常肺组织中AnnexinⅡ的表达情况。结果AnnexinⅡ主要表达于肺泡上皮细胞、肺泡间隔纤维细胞的胞浆中。在正常肺组织中,AnnexinⅡ为弥漫的棕褐色强阳性表达,而在IIP肺组织中,为黄色中至弱阳性表达。免疫组织化学染色强度评分结果显示,普通型间质性肺炎组肺组织中AnnexinⅡ的表达强度低于非特异性间质性肺炎组。结论AnnexinⅡ在特发性间质性肺炎中起重要的作用,可能是作为肺纤维化判断预后的重要指标。     

Total respiratory resistance and reactance in patients with diffuse interstitial lung disease

In 54 patients with interstitial lung diseases and no signs of airway obstruction we measured lung volumes, maximal expiratory flows, diffusing capacity (DLCO), total respiratory resistance (Rrs) and reactance (Xrs) between 4 and 26 Hz by means of the forced oscillation technique. In all patients DLCO was less than 75% of the expected value. Patients were classified into two groups depending on total lung capacity (TLC): group A with TLC less than 80% of expected, and group B with TLC of 80% or more. Group A demonstrated a decrease of Xrs especially at low frequencies, with small, not significant changes in Rrs. In the patients in this group with the lowest values of TLC (less than 50%), we observed an increase of Rrs at low frequencies causing a negative frequency dependence of Rrs. In group B no distinct changes of Rrs and Xrs occurred. Canonical correlation analysis between routine lung function data and forced oscillation parameters, showed tight correlations between TLC in absolute value or VC in percent of the predicted value on the one hand and average level of Xrs and average slope of Xrs (and Rrs) vs frequency curves on the other hand. Measurements of lung mechanics in five additional patients and comparison with a model of the respiratory system suggest that the changes of Rrs and Xrs are not explained totally by the observed increase in lung tissue resistance and decrease in lung compliance. The observed changes in Rrs and Xrs are not specific for restrictive lung disorders; similar changes are met also in moderately advanced obstructive diseases.     

A new treatment strategy for advanced idiopathic interstitial pneumonia: living-donor lobar lung transplantation

BACKGROUND: Among patients awaiting cadaveric lung transplantation, patients with idiopathic interstitial pneumonia (IIP) have been demonstrated to have the highest mortality rate. Contraindications to cadaveric lung transplantation include current high-dose systemic corticosteroid therapy because it may increase airway complications and various types of infection. STUDY OBJECTIVES: To analyze the effect of living-donor lobar lung transplantation (LDLLT) for patients with advanced IIP including those receiving high-dose systemic corticosteroids. DESIGN: Retrospective analysis. SETTING: Okayama University Hospital and Okayama Medical Center. PATIENTS: We report on the first nine patients (seven female and two male; age range, 13 to 55 years) with advanced IIP receiving LDLLT. All nine patients had a very limited life expectancy, and eight patients were dependent on systemic corticosteroid therapy as high as 50 mg/d of prednisone. LDLLT was performed under cardiopulmonary bypass using two lower lobes donated by two healthy relatives. RESULTS: There were no airway complications in the 18 bronchial anastomoses. There was one early death (11%) due to severe acute rejection. Eight patients (89%) are currently alive with a follow-up period of 10 to 48 months. Their vital capacity reached 2.03 +/- 0.20 L (mean +/- SEM), 71.4% of predicted at 1 year. All 18 donors have returned to their previous lifestyles. Excised lungs were pathologically diagnosed as usual interstitial pneumonia (UIP) in six cases and fibrotic nonspecific interstitial pneumonia (NSIP) in three cases. CONCLUSIONS: These early follow-up data support the option of LDLLT in patients with advanced IIP, including UIP and fibrotic NSIP, who would die soon otherwise. Current high-dose systemic corticosteroid therapy is not a contraindication in LDLLT.     

HABA (HTLV-I associated bronchiolo-alveolar disorder)]

The presence of antibodies to adult T cell leukemia (ATL) antigen: HTVL-I was studied in patients with chronic interstitial lung diseases such as diffuse panbronchiolitis (DPB) and idiopathic interstitial pneumonia (IIP). Anti-HTLV-I antibody was detected with a high frequency among these diseases (35% in DPB and 7% in IIP) compared with other diseases and healthy controls. We have termed the clinicopathological condition that includes these two disease categories of interstitial lung disease (DPB or IIP) and hematologic disorder (ATL associated with HTLV-I carrier state) HTLV-I associated bronchiolo-alveolar disorder (HABA). At the same time, HTLV-I related reaction (diffuse pattern for MT-1 and/or MT-2) was found except positive reaction of the antibody (granular pattern for MT-1) by immunofluorescent assay. The incidence of HTLV-I related reaction was high in interstitial lung diseases with a rate of 45% in DPB and 53% in IIP. Thus, the total frequency of presence of antibodies and related reactions was 80% in DPB and 60% in IIP. In lung cancer, the frequency was also high, although it was less than in DPB and IIP. We termed cases of anti-HTLV-I antibody positive lung cancer HTLV-I associated lung cancer (HALC). One typical patient with IIP who initially showed HTLV-I related reaction showed a positive antibody reaction 2 years later. Finally he presented with adenocarcinoma with effusion a further 2 years later. In order to examine HTLV-I proviral DNA integration, southern blotting by PCR was performed in patients with HTLV-I related reaction.2+ suggesting one of the causes of DPB.     

Lung transplantation in IIP: A review

The idiopathic interstitial pneumonias (IIP) encompass a large and diverse subtype of interstitial lung disease (ILD) with idiopathic pulmonary fibrosis (IPF) and non‐specific interstitial pneumonia (NSIP) being the most common types. Although pharmacologic treatments are available for most types of IIP, many patients progress to advanced lung disease and require lung transplantation. Close monitoring with serial functional and radiographic tests for disease progression coupled with early referral for lung transplantation are of great importance in the management of patients with IIP. Both single and bilateral lung transplantation are acceptable procedures for IIP. Procedure selection is a complex decision influenced by multiple factors related to patient, donor and transplant centre. While single lung transplant may reduce waitlist time and mortality, the long‐term outcomes after bilateral lung transplantation may be slightly superior. There are numerous complications following lung transplantation including primary graft dysfunction, chronic lung allograft dysfunction (CLAD), infections, gastroesophageal reflux disease (GERD) and airway disease that limit post‐transplant longevity. The median survival after lung transplantation is 4.7 years in patients with ILD, which is less than in patients with other underlying lung diseases. Although long‐term survival is limited, this intervention still conveys a survival benefit and improved quality of life in suitable IIP patients with advanced lung disease and chronic hypoxemic respiratory failure.     

The significance of the heparin precipitable fraction in lung diseases]

The authors recently experienced a case of idiopathic interstitial pneumonia (IIP) that exhibited skin ulcers due to increased heparin precipitable fraction (HPF) in plasma. This case prompted us to investigate the occurrence and significance of HPF in interstitial pneumonia (IP). The subjects included patients with IIP (acute exacerbation 6 cases, chronic active stage 12 cases), IP associated with collagen vascular disease (CVD) (9 cases) and granulomatous lung diseases (7 cases). The data indicated that all of the cases with acute exacerbation of IIP exhibited increased plasma HPF values (218-951 mg/dl) compared to those of normal controls (less than 180 mg/dl). In contrast, the values ranged within normal limits in all of the cases with IP associated with CVD. In a companion study, we measured plasma HPF values in patients with lung cancer, bacterial pneumonia and diffuse panbronchiolitis. It was found that 22% of the subjects showed increased plasma HPF values. We also investigated whether there were correlations between plasma HPF and various inflammatory parameters. The data revealed that there were correlations between HPF and ESR, CRP, alpha 1-globulin, alpha 2-globulin, complement (C3) or fibrinogen. However, there was no correlation between HPF and fibronection. These results suggest that plasma HPF is valuable to evaluate the acute exacerbation of IIP, although the elevation of plasma HPF levels is not specific.     

Late prevalence of respiratory symptoms and pulmonary function abnormalities in Gulf War I Veterans

BACKGROUND: Published reports have documented an increased prevalence of self-reported respiratory symptoms among servicemen deployed during the 1990-1991 Gulf War. We evaluated whether this deployment resulted in long-term adverse respiratory effects. METHODS: A comprehensive medical history was taken and physical and laboratory evaluations, including pulmonary function tests, were performed in 1036 deployed and 1103 nondeployed veterans of the Gulf War. Participants were classified into 5 groups on the basis of their pulmonary function tests findings: normal pulmonary function; nonreversible airway obstruction; reversible airway obstruction; restrictive lung physiology; and small airway obstruction. RESULTS: Deployed veterans were younger, more commonly white, less educated, single, of lower mean family incomes, and more likely to have enlisted than nondeployed veterans. Deployed veterans were also statistically more likely to self-report a history of smoking and wheezing than nondeployed veterans, but comparisons of reported physician visits for pulmonary complaints, pulmonary hospitalizations, numbers of documented episodes of asthma, bronchitis, or emphysema, and pulmonary medications prescribed in the year prior to evaluation did not reveal any differences between deployed and nondeployed veterans. The distribution of pulmonary function test results was identical among deployed and nondeployed veterans. Among both deployed and nondeployed veterans, about 64% had normal pulmonary function, 16% to 18% had nonreversible airway obstruction, 10% to 12.2% had restrictive lung physiology, 6% to 6.7% had small airway obstruction, and the remaining 0.9% to 1.3% had reversible airway obstruction. CONCLUSION: Our findings did not confirm the hypothesis that deployment to the Gulf War in 1990-1991 resulted in an increased prevalence of clinically significant pulmonary abnormalities 10 years later.     

糖皮质激素吸入治疗对哮喘儿童肺功能的影响

目的探讨不同的糖皮质激素吸入治疗的疗程对哮喘儿童肺功能的影响。方法110例初诊时处于发作期的哮喘儿童年龄（6-14岁）,按糖皮质激素吸入治疗的疗程分成3组,A组（半年期组）50例,B组（1年期组）30例和C组（2年期组）30例,分别在用药前及用药后半年、1年、2年测定肺功能。结果三组患儿用药后肺功能均较用药前明显改善（P〈0.001）,其中大气道功能改善最为迅速,半年即达预计值的80%以上,小气道功能障碍持续存在,随疗程的延长,二年期与半年期相比大小气道功能均有显著性改善（P〈0.05）。结论在儿童哮喘防治中,应坚持小剂量糖皮质激素吸入治疗二年以上。     

A comparative study of the prognosis for Japanese patients with idiopathic interstitial pneumonia or BOOP based on histopathologic subsets]

We explored the prognosis for 123 patients with either idiopathic interstitial pneumonia (IIP) or bronchiolitis obliterans organizing pneumonia (BOOP). All patients underwent either open lung biopsy or thoracoscopic lung biopsy procedures. The histopathologic diagnosis of IIP included patients with usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), and desquamative interstitial pneumonia with respiratory bronchiolitis-associated interstitial lung disease. The prognosis was poorest for patients with a histologic diagnosis of UIP, and excellent for those who received a diagnosis of BOOP. Although the prognosis is generally considered to be good for patients with NSIP, some NSIP patients in our study died. Histopathologic diagnosis based on surgical lung biopsy is useful in evaluating the prognosis for patients with IIP.     

Steroid treatment based on the findings of transbronchial biopsy in idiopathic interstitial pneumonia.

This study was performed to find the rationale for administering steroids to patients with idiopathic interstitial pneumonia (IIP), which was unlikely to be usual interstitial pneumonia (UIP) but was not surgically biopsied. Among IIP patients in the file of the departments, nine patients who met the following criteria were selected for this study ("non-UIP" group): 1) transbronchial lung biopsy showed dense mononuclear cell infiltration in thickened alveolar septa; 2) chest radiograph and computed tomography showed irregular linear, reticular or ground-glass opacities with alveolar consolidation without honeycombing in the lung base; and 3) spirometry was performed before and after steroid therapy. Ten patients with pathologically confirmed nonspecific interstitial pneumonia ("NSIP" group) were also selected for the comparison. Baseline values and percentage increase of vital capacity (VC) after steroid therapy were plotted. Steroids improved VC in both groups of patients. After 1 yr of steroid therapy, percentage increase of VC in "non-UIP" was 28.8+/-7.7%, which was not significantly different from that in NSIP (30.0+/-11.7%). One "non-UIP" patient and one NSIP patient died after 6.4 and 4.3 yrs of follow-up, respectively. Patients with idiopathic interstitial pneumonia presenting cellular interstitial pneumonia in transbronchial lung biopsy, in addition to radiographic findings not typical for usual interstitial pneumonia, could expect a beneficial effect of steroids without undergoing surgical biopsy.     

Lung cancer associated with idiopathic interstitial pneumonia

Idiopathic interstitial pneumonia (IIP) is known to have a tendency to be associated with lung cancer. Clinical characteristics of lung cancer associated with IIP are discussed in this article. Thirteen cases of lung cancer associated with IIP were examined from 1975 to 1988. A total of 590 cases of lung cancer and 38 cases of IIP were observed during that period. The 13 cases of lung cancer were found to be associated with IIP during the follow-up observation of our patients. Of these 13 patients, 12 men and a woman, the average age was 68.0 years old. All these 13 cases were ex- or present smokers. Five cases of lung cancer had no symptoms, but were detected by abnormal shadows on chest X-ray. Eight cases were detected symptoms. The duration from the onset of IIP to the onset of lung cancer was 36.5 +/- 23.5 months. Primary sites of lung cancer were distributed as follows. Nine cancers were in the left lung (64%) and five cancers were in the right lung (36%). Three cancers were in the hilar region (21%) and 11 cancers were in the peripheral lung field (79%). The numbers of lung cancers were equal in upper and lower lobe. The most common histological type was squamous cell carcinoma, followed by adenocarcinoma. Most cases were in stage III or IV. Three cases were operated, 4 were treated with chemotherapy, 4 were treated with chemotherapy plus irradiation, and 2 cases were treated by BRMs. The frequency of weight loss or finger clubbing in these patients was higher than in IIP patients without lung cancer.(ABSTRACT TRUNCATED AT 250 WORDS)     

血清抗中性粒细胞胞质抗体阳性肺间质病患者临床及预后分析

目的探究血清抗中性粒细胞胞质抗体(ANCA)阳性的间质性肺疾病患者的临床特点及预后。方法回顾性纳入2006年3月至2016年3月就诊于北京协和医院的间质性肺疾病患者274例,其中男81例,女193例,年龄(53±11)岁,根据诊断分为血清ANCA阳性肺间质病(ANCA-ILD)、结缔组织病相关性肺间质病和自身免疫特征的间质性肺炎(CTD-ILD/IPAF)及特发性间质性肺炎(IIP)组,分析3组患者在临床表现、血清学、肺功能、影像学、生存及复发方面的差异。结果274例患者中ANCA阳性38例(38/274,14%),发病年龄(59±10)岁,随访时间(52±31)个月,死亡7例(7/38,18%)。ANCA阳性患者发病年龄高于CTD-ILD/IPAF[(52±10)岁]和IIP[(53±11)岁,H=19.29,P<0.001],血红蛋白[(129±21)mg/L]低于CTD-ILD/IPAF[(138±15)mg/L]和IIP[(140±19)mg/L,H=8.17,P=0.017],ESR[(46±35)mm/1 h]高于CTD-ILD/IPAF[(26±24)mm/1 h]和IIP[(19±22)mm/1 h,H=19.73,P<0.001],治疗后肺功能FVC改善率(31%)低于CTD-ILD/IPAF(59%)和IIP(39%,χ^2=11.74,P=0.003),胸部CT病变吸收率(61%)低于CTD-ILD/IPAF(82%)和IIP(67%,χ^2=9.23,P=0.010),病死率(18%)高于CTD-ILD/IPAF(6%)和IIP(12%,χ^2=7.16,P=0.028)。结论ANCA阳性患者与其他类型肺间质病患者在临床特征方面存在差异,治疗效果不佳,预后较差。     

Eighty cases of chronic respiratory failure treated with home noninvasive positive pressure ventilation]

We studied the clinical features and efficacy of home noninvasive positive pressure ventilation (NPPV) therapy in 80 patients to ascertain its indications and problems. The causes of chronic respiratory failure were restrictive thoracic diseases of post-tuberculosis sequelae (40 cases) and kyphoscoliosis (9 cases), COPD (8 cases), bronchiectasis (7 cases), and interstitial pneumonia (4 cases). One year survival rate of the patients with post-tuberculosis sequelae was 76% and most of the patients who started NPPV at their acute exacerbation died within several months. About half of the patients of COPD improved their quality of life (QOL) through NPPV. However, their survival rate 3 months later was only 69%. More than half of the patients with bronchiectasis felt that their QOL was improved by NPPV. Most of the patients with interstitial pneumonia died within 3 months indicating that NPPV is less useful for improving QOL of interstitial pneumonia PaCO2, after home NPPV, decreased significantly in the responder group (70.0 +/- 15.4 vs. 57.6 +/- 10.7[SD]Torr, p < 0.05), while PaCO2 in the non-responder group was unchanged (65.4 +/- 12.1 vs. 64.2 - 10.4 [SD] Torr). Body Mass Index (BMI) in the responder group tended to be higher than in the non-responder group. In conclusion, the restrictive thoracic diseases with post-tuberculosis sequelae and kyphoscoliosis are a good indication for NPPV and the therapy is also useful for patients with bronchiectasis who can dispose of their sputum by themselves. Home NPPV is suitable for patients whose PaCO2 decreases through NPPV and whose BMI is relatively high. QOL of interstitial pneumonia barely improves through NPPV, because interstitial pneumonia with hypercapnia is at the terminal stage.     

Usefulness of an exercise pulmonary function test for differential diagnosis or treatment evaluation of restrictive pulmonary diseases

To simplify its applications in patients in poor condition such as cases of idiopathic interstitial pneumonia (IIP), we chose a simple method of exercise test instead of the usual method. The method consisted of the single Master's two-step test and instant blood-gathering without keeping a needle in a vessel. By this method, PaO2 after exercise was estimated approximately 7 torr less than with the usual method, but tendencies of PaO2 changes were accurately observed. In a study on 8 patients with IIP, 20 collagen vascular disease cases with pulmonary changes (CVD), 11 hypersensitivity pneumonitis cases, and 5 sarcoidosis cases, the parameters such as delta PaO2 or delta AaDO2 differentiated the different physiological conditions of the 4 disease groups accurately. However, the exercise test was noted to be less sensitive in terms of differential diagnosis than the at-rest test after a multiple discriminant analysis in the 4 disease groups. For the estimate of patient prognosis delta AaDO2 was most useful in a study of IIP and CVD patients. We conclude that this simple exercise test is useful for the diagnosis or evaluating treatment of such restrictive pulmonary disease.     

Enhanced monocyte chemoattractant protein-3/CC chemokine ligand-7 in usual interstitial pneumonia

Chemokines are increased and may exert effects on both inflammatory and remodeling events in idiopathic pulmonary pneumonia (IIP). Accordingly, we examined the concomitant expression of inflammatory CC chemotactic cytokines or chemokines and their corresponding receptors in surgical lung biopsies obtained at the time of disease diagnosis and pulmonary fibroblasts grown from these biopsies. By gene array analysis, upper and lower lobe biopsies and primary fibroblast lines from patients with usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia, and respiratory bronchiolitis-interstitial lung disease, but not patients without IIP, exhibited CCL7 gene expression. TAQMAN, immunohistochemical, and ELISA analyses confirmed that CCL7 was expressed at significantly higher levels in UIP lung biopsies compared with biopsies from patients with nonspecific interstitial pneumonia, respiratory bronchiolitis-interstitial lung disease, and from patients without IIP. Higher levels of CCL7 were present in cultures of IIP fibroblasts compared with non-IIP fibroblasts, and CCL5, a CCR5 agonist, significantly increased the synthesis of CCL7 by UIP fibroblasts. Together, these data suggest that CCL7 is highly expressed in biopsies and pulmonary fibroblast lines obtained from patients with UIP relative to patients with other IIP and patients without IIP, and that this CC chemokine may have a major role in the progression of fibrosis in this IIP patient group.