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PubMed1996

Incidence of hepatitis C virus infection in Italian patients with idiopathic pulmonary fibrosis.

BACKGROUND: A viral cause of idiopathic pulmonary fibrosis (IPF) was recently suggested by a Japanese study in which a high prevalence of anti-hepatitis C virus (HCV) antibodies was detected. A subsequent British study failed to confirm these results. METHODS: Antibodies to HCV were evaluated in 60 patients with IPF, 130 patients with non-interstitial lung disease, and in 4614 blood donors. HCV-RNA and HCV genotypes were evaluated in the anti-HCV positive patients with IPF. Anti-HCV antibodies were evaluated by ELISA and confirmed by recombinant immunoblotting assay (RIBA). HCV-RNA and genotypes were detected by reverse transcriptase polymerase chain reaction (PCR). RESULTS: Eight patients with IPF had anti-HCV antibodies detected by ELISA (13.3%). In the blood donor control group the prevalence of HCV antibodies was lower (0.3%). In patients with non-interstitial lung disease HCV antibody prevalence was 6.1%. In all eight patients with IPF found to be anti-HCV positive by ELISA, HCV antibodies were also detected by RIBA. Furthermore, all were HCV-RNA positive by PCR assay. HCV genotypes were identified in four of these eight patients. In all four genotype II was present and in two it was associated with genotype III and/or genotype IV. In the remaining four cases the genotype was not identified. CONCLUSION: Italian patients with IPF show an increased prevalence (approximately 13%) of HCV infection and viral replication, but the prevalence of anti-HCV antibodies does not differ from other lung diseases.

Adult↗
PubMed2004

Mechanisms of pulmonary fibrosis.

Tissue injury evokes highly conserved, tightly regulated inflammatory responses and less well-understood host repair responses. Both inflammation and repair involve the recruitment, activation, apoptosis, and eventual clearance of key effector cells. In this review, we propose the concept of pulmonary fibrosis as a dysregulated repair process that is perpetually "turned on" even though classical inflammatory pathways may be dampened or "switched off." Significant regional heterogeneity, with varied histopathological patterns of inflammation and fibrosis, has been observed in individual patients with idiopathic pulmonary fibrosis. We discuss environmental factors and host response factors, such as genetic susceptibility and age, that may influence these varied manifestations. Better understanding of the mechanisms of lung repair, which include alveolar reepithelialization, myofibroblast differentiation/activation, and apoptosis, should offer more effective therapeutic options for progressive pulmonary fibrosis.

Humans↗
PubMed2000

Single versus bilateral lung transplantation for idiopathic pulmonary fibrosis: a ten-year institutional experience.

OBJECTIVE: Between July 1988 and July 1998, we performed 433 lung transplants. Forty-five patients had idiopathic pulmonary fibrosis, and operations for these patients included 32 single lung transplants and 13 bilateral sequential lung transplants. This study reviews this experience and compares single lung transplantation and bilateral lung transplantation for pulmonary fibrosis. METHODS: We performed a retrospective review, including inpatient hospital charts, outpatient clinic records, and telephone contact with patients to verify current health status. RESULTS: Perioperative mortality was 4 (8.9%) patients. One patient underwent redo bilateral lung transplantation for reperfusion injury and graft failure after single lung transplantation. The median hospitalization was 22 days. Actuarial survival at 1 and 5 years was 75.5% and 53.5%, respectively, which was not significantly different from our survival for all recipients (85.5% and 56.4%, respectively). Seventeen (41%) of 41 operative survivors have died. Late causes of death included obliterative bronchiolitis with respiratory failure (9), malignancy (3), and cytomegalovirus pneumonitis (2). Hospital mortality was 3 (9.4%) of 32 after single lung transplantation and 1 (7.7%) of 13 after bilateral lung transplantation. There was no difference between single and bilateral lung transplantation with regard to hospital stay. Four (12.5%) of the 32 patients undergoing single lung transplantation required tracheostomy, whereas 3 (23%) of 13 recipients undergoing bilateral lung transplantation required tracheostomy. CONCLUSION: Single or bilateral lung transplantations offer viable therapy for patients with pulmonary fibrosis. We demonstrate no benefit of bilateral over single lung transplantation for patients with this diagnosis. Survival after transplantation appears better than that of historic control subjects receiving standard medical care at other institutions.

Female↗
PubMed2004

The effect of pulmonary fibrosis on survival in patients with hypersensitivity pneumonitis.

PURPOSE: To determine the effect of pulmonary fibrosis on survival in an unselected group of patients with hypersensitivity pneumonitis. METHODS: We identified 72 patients with hypersensitivity pneumonitis confirmed by surgical lung biopsy in the database of the Clinical Interstitial Lung Disease Program at the National Jewish Medical and Research Center. All biopsy specimens were scored according to the presence or absence of fibrosis. Comparisons were made between patients with (fibrotic group) and without (nonfibrotic group) pathologic fibrosis. Vital status was ascertained and Kaplan-Meier curves were plotted. Cox regression analysis was used to determine predictors of survival. RESULTS: Forty-six patients were classified as fibrotic and 26 as nonfibrotic. Twenty-nine percent had exposure to a bird antigen, 33% had exposure to a microbial antigen, and 38% had unknown exposure. Patients with fibrosis were significantly older, showed greater restrictive lung physiology, and had greater all-cause and respiratory mortality. Median survival in fibrotic patients was 7.1 years, which was significantly less than survival in those without fibrosis. In an age-adjusted regression analysis, antigen class, symptom duration, and lung function had no effect on survival. Only the presence of pathologic fibrosis was predictive of increased mortality (hazard ratio = 6.01; 95% confidence interval: 1.68 to 21.45; P = 0.006). CONCLUSION: Pulmonary fibrosis is associated with diminished survival in patients with hypersensitivity pneumonitis.

Alveolitis, Extrinsic Allergic↗
PubMed2004

Hermansky-Pudlak syndrome type 4 in a patient from Sri Lanka with pulmonary fibrosis.

Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by oculocutaneous albinism and a platelet storage pool deficiency. Some patients also develop fatal pulmonary fibrosis and some have granulomatous colitis. Six human genes HPS1, ADB3A, HPS3, HPS4, HPS5, and HPS6 have been identified as cause of the six known subtypes of HPS. While there exist nearly 500 Puerto Rican and non-Puerto Rican HPS-1 patients, very few HPS-4 patients have been reported, and most of these have not been described in detail. We now delineate the clinical characteristics of an HPS-4 patient homozygous for a novel HPS-4 mutation, P685delC. The patient, the first individual with HPS reported from Sri Lanka, had severe pulmonary fibrosis, typical of HPS-1 disease, without granulomatous colitis. We conclude that pulmonary fibrosis occurs as part of HPS-4 and that HPS should be considered in all ethnic groups.

Albinism, Oculocutaneous↗
PubMed1999

Increased intensity of lung infiltrates at the side of lung cancer in patients with lung cancer associated with pulmonary fibrosis.

It has been reported that lung cancer is frequently associated with idiopathic pulmonary fibrosis (IPF). The purpose of this study was to compare the intensity of lung infiltrates between the side associated with lung cancer and the side without lung cancer. Twenty-three patients (24 lung cancers) with primary lung cancer associated with pulmonary fibrosis were retrospectively evaluated. Chest CT findings were evaluated by three expert radiologists using the intensity scores. In 16 of the 23 patients, it was possible to compare the intensity of lung infiltrates between both sides of the lungs. As a result, increased intensity at the side in which lung cancer developed was demonstrated in 12 of 16 patients (75%). In the remaining four patients, intensity of lung infiltrates was the same in both lungs. In operated patients as well as autopsied patients, it was possible to evaluate the pathological findings of lung tissues around cancer cells. This study clearly demonstrates that the intensity of lung infiltrates increased at the side in which lung cancer developed.

Adenocarcinoma↗
PubMed2001

Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy.

Idiopathic pulmonary fibrosis is a progressive and usually fatal lung disease characterized by fibroblast proliferation and extracellular matrix remodeling, which result in irreversible distortion of the lung's architecture. Although the pathogenetic mechanisms remain to be determined, the prevailing hypothesis holds that fibrosis is preceded and provoked by a chronic inflammatory process that injures the lung and modulates lung fibrogenesis, leading to the end-stage fibrotic scar. However, there is little evidence that inflammation is prominent in early disease, and it is unclear whether inflammation is relevant to the development of the fibrotic process. Evidence suggests that inflammation does not play a pivotal role. Inflammation is not a prominent histopathologic finding, and epithelial injury in the absence of ongoing inflammation is sufficient to stimulate the development of fibrosis. In addition, the inflammatory response to a lung fibrogenic insult is not necessarily related to the fibrotic response. Clinical measurements of inflammation fail to correlate with stage or outcome, and potent anti-inflammatory therapy does not improve outcome. This review presents a growing body of evidence suggesting that idiopathic pulmonary fibrosis involves abnormal wound healing in response to multiple, microscopic sites of ongoing alveolar epithelial injury and activation associated with the formation of patchy fibroblast-myofibroblast foci, which evolve to fibrosis. Progress in understanding the fibrogenic mechanisms in the lung is likely to yield more effective therapies.

Anti-Inflammatory Agents↗
PubMed2001

Steroids in idiopathic pulmonary fibrosis: a prospective assessment of adverse reactions, response to therapy, and survival.

PURPOSE: We evaluated the risk and potential benefit of high-dose corticosteroid therapy in patients with idiopathic pulmonary fibrosis. SUBJECTS AND METHODS: We prospectively studied 41 patients with previously untreated, biopsy-proven idiopathic pulmonary fibrosis. Before treatment, we calculated clinical, radiographic, and physiologic severity-of-illness scores for each patient. We scored high-resolution computerized tomographic (CT) scans for ground glass and interstitial opacity. We determined the extent of cellular infiltration, interstitial fibrosis, desquamation, and granulation in open lung biopsy samples. Patients were monitored monthly for steroid-related side effects, response to therapy at 3 months, and mortality. RESULTS: All patients experienced at least one steroid-induced side effect. Eleven (27%) patients were nonresponders, 11 (27%) were responders, and 19 (46%) remained stable. Of the 19 patients who died during a mean (+/- SD) follow-up of 3.3 +/- 2.3 years, 8 (42%) lost weight during the initial 3 months of steroid therapy; only 3 (14%) of the 22 patients still living (P = 0.08) experienced weight loss. In a multivariate analysis, greater fibrosis (hazard ratio [HR] = 1.4 per unit increase; 95% confidence interval [CI]: 1.0 to 1.9; P = 0.03) and cellularity (RR = 1.9 per unit increase; 95% CI: 1.3 to 2.8; 3, P <0.001) in the biopsy sample and whether a patient was classified as a responder (RR = 0.4 versus nonresponder; 95% CI: 0.2 to 1.0; P = 0.05) or stable (RR = 0.2 versus nonresponder; 95% CI: 0.1 to 0.6, P <0.001) after steroid therapy were associated with mortality. CONCLUSION: Corticosteroid treatment for idiopathic pulmonary fibrosis is associated with substantial morbidity. Patients who remain stable or respond to corticosteroid therapy have better survival than those who fail to respond. Whether this difference reflects an effect of treatment or less severe disease can be determined only in a randomized trial.

Adrenal Cortex Hormones↗
PubMed2003

STAT1 activation and STAT1-dependent immune-response gene ICAM-1 expression in alveolar macrophages of rats suffered from interstitial pulmonary fibrosis.

AIM: To investigate the role of signal transducer and activator of transcription 1(STAT(1)) in alveolar macrophages (AMs) from rats with bleomycin-induced pulmonary fibrosis. METHODS: Fifty adult female Wistar rats were randomly divided into two groups: One group was intratracheally instilled with bleomycin (BLM), while another group with 9 g/L NaCl solution (NS). The kinetic change of STAT(1) activation and intercellular adhesion molecule-1(ICAM-1) expression in AMs was examined by Western blot and immunohistochemical staining, respectively. RESULTS: (1)STAT(1) was rarely activated in AMs of NS group. After bleomycin treatment, the STAT(1) activation of AMs was significantly increased on day 1, climaxed on day 7, and then gradually decreased, but remaining significantly higher than that of NS group on day 28 (P<0.05). (2)There was only a few cells ICAM-1 higher than that positively stained in AMs of NS group. After intratracheal instillation of bleomycin, the number of ICAM-1 positive stained cells was significantly increased on day 1, reached its peak values on day 7, and then gradually decreased, and still higher than that of NS group on day 28 (P<0.05).(3)There was a significant correlationship between STAT(1) activation and ICAM-1 expression in AMs (r =0.913, P<0.01). Further more, the ICAM-1 expression in AMs was significantly correlated with the severity of inflammation in lung tissues (r =0.947, P<0.01). CONCLUSION: STAT(1) was abnormally activated in AMs of rats with bleomycin-induced interstitial pulmonary fibrosis (IPF). The abnormal STAT(1) activation may play a role in the pathogenesis of acute alveolitis and pulmonary fibrosis.

Animals↗
PubMed2006

Overexpression of the transcription factor GATA-3 enhances the development of pulmonary fibrosis.

Recent studies have demonstrated that Th2 cytokines, such as interleukin-4 and interleukin-13, enhance fibrotic processes by activating fibroblast proliferation and collagen production, whereas interferon-gamma, a Th1 cytokine, inhibits these processes. Th1 and Th2 cells both differentiate from common T precursor cells, with transcription factor GATA-3 a key regulator of Th2 differentiation. In the present study, therefore, we examined the effects of GATA-3 overexpression on the development of pulmonary fibrosis in a mouse model. Wild-type C57BL/6 mice and GATA-3-overexpressing (GATA-3-tg) mice of the same background were intratracheally treated with bleomycin. The survival rate after bleomycin was significantly decreased in GATA-3-tg mice compared with wild-type mice. The degree of pulmonary fibrosis was much greater in GATA-3-tg mice than in wild-type mice 28 days after bleomycin treatment. Lung interferon-gamma concentration was significantly decreased in GATA-3-tg mice compared with wild-type mice by 7 days after either saline or bleomycin treatment. The concentration of transforming growth factor-beta, a fibrogenic cytokine, was significantly higher in GATA-3-tg mice than in wild-type mice. Exogenous administration of interferon-gamma to GATA-3-tg mice improved the degree of pulmonary fibrosis and thus increased survival. These results indicate that overexpression of GATA-3 enhances the development of pulmonary fibrosis, possibly by reducing interferon-gamma levels in the lung.

Animals↗
PubMedDate not supplied

Lung function in children and adolescents with idiopathic interstitial pulmonary fibrosis.

Lung function of 65 patients who had idiopathic interstitial pulmonary fibrosis (IIPF) that had been treated with prednisone was evaluated by tests of ventilatory function, lung mechanics, and gas exchange at rest and during exercise. Ages on initial investigation ranged from 5 to 20 years. In 35 of 65 patients the studies were repeated an average of four times over a period of 1 to 9 years. Results of the first testing were as follows: vital capacity (VC)-significantly reduced in all patients; inspiratory capacity (IC)-significantly reduced in all patients; total lung capacity (TLC)-reduced in 91%; functional residual capacity (FRC)-reduced in 31%; residual volume (RV)-reduced in 6%; elastic recoil of the lungs (Pstl)-significantly increased in 97% at 100% TLC, significantly increased in 52% at 90% TLC, reduced in 68% at 60% TLC; 7) static compliance (Cst)-reduced in 83%; 8) dynamic compliance (Cdyn)-reduced in 88%; 9) specific airway conductance at FRC level (Gaw/TGVex)-significantly increased in 50%; 10) maximum expiratory flow rates at 60% TLC (Vmax 60% TLC, in TLC/s)-significantly reduced in 33%; 11) upstream airway conductance (Gus 60% TLC, in TLC/s/cm H2O)-reduced in 32%; 12) diffusing capacity of the lungs for carbon monoxide (DLco) related to body-surface area-abnormal in 58% (when corrected for lung size, i.e., DLco/TLC, abnormal in only 8%); 13) PaO2 at rest and after 6 minutes submaximal exercise-reduced in 25% and 63%, respectively. Changes in lung function that occurred with growth were assessed in terms of percentages of predicted values. Results showed that the VC and IC remained significantly reduced. An actual reduction of TLC, FRC, RV, breathing frequency, DLCO, and Pstl at 100% and 90% TLC was observed. Increases were seen in Pstl at 60% TLC, Gaw/TGVex, Vmax, and Cst. Indices of lung elasticity suggested that regions of fibrosis and emphysema had become present. Smaller patients were also noted to have stiffer lungs.

Adolescent↗
PubMed2004

Human insulin-like growth factor-IA expression in transgenic mice promotes adenomatous hyperplasia but not pulmonary fibrosis.

Insulin-like growth factor-I (IGF-I) has been implicated in postnatal alveolar development, pulmonary fibrosis, and non-small cell lung cancer. To further investigate the role of IGF-I, we created a line of transgenic mice in which alveolar type II epithelial cells express human IGF-IA under the control of the surfactant protein C promoter. We determined the effect of pulmonary overexpression of human IGF-IA on 1) pulmonary inflammation and fibrosis in response to intratracheal instillation of bleomycin, 2) premalignant pulmonary adenomatous hyperplasia, and 3) adenoma formation. Transgenic expression of human IGF-IA had no effect on baseline gross lung pathology, cellularity of bronchoalveolar lavage, or total lung collagen content. In addition, there were no significant differences between transgenic mice and nontransgenic littermate controls in the development of pulmonary inflammation or pulmonary fibrosis in response to intratracheal bleomycin instillation. However, pulmonary expression of human IGF-IA in older mice (>12 mo) significantly increased the incidence of premalignant adenomatous hyperplastic lesions compared with littermate controls without affecting adenoma formation. These findings suggest that increased expression of human IGF-IA in alveolar air spaces does not affect the development of pulmonary fibrosis but promotes premalignant changes in the alveolar epithelium.

Adenoma↗
PubMedDate not supplied

Immunophenotyping of lymphocytes in the lung interstitium and expression of osteopontin and interleukin-2 mRNAs in two different murine models of pulmonary fibrosis.

In the surfactant protein C/tumor necrosis factor (SP-C/TNF) transgenic mouse, the TNF-alpha transgene is overexpressed in type II pneumocytes. Pulmonary lymphocytic infiltration develops which is followed by fibrotic changes including accumulation of fibroblasts and deposition of extracellular matrix. We hypothesized that lymphocytes played a role in the development of pulmonary fibrosis in this model. Lymphocytes were recovered from the interstitium of the lung and analyzed by flow cytometry. The absolute number of lymphocytes recovered from transgenic mice were approximately four times of that in littermates. Flow cytometric analysis showed the presence of gamma delta T cells and B1 cells in the former group but these cells were almost absent in the lung of non-transgenic littermates. We also studied lymphocytes accumulating in the lung during bleomycin (BLM)-induced pneumopathy. Serial analyses showed a progressive increase of CD4/CD8 ratio after injection of BLM, reaching a peak at day 14, then decreased to the normal level by day 48. Northern blot analysis of the lung showed an enhanced expression of interleukin (IL)-2 and osteopontin (OPN) mRNA in those two models of pulmonary fibrosis. Expansion of clonal alpha beta T cells as detected by reverse transcriptase-polymerase chain reaction/single strand conformation polymorphism (RT-PCR/SSCP) suggests involvement of antigen-driven mechanisms in the development of pulmonary fibrosis.

Animals↗
PubMed2002

Low fasting serum triglyceride and high free fatty acid levels in pulmonary fibrosis: a previously unreported finding.

OBJECTIVE: The authors describe here the occurrence of low fasting serum triglyceride (TG) and high free fatty acid (FFA) levels in pulmonary fibrosis, a finding that has never been reported before. PATIENTS AND METHODS: TGs were measured in: (a) 44 patients (3 male and 41 female; mean age SEM: 63.06 +/- 4.04 years) who have been hospitalized in the authors' department in the past 2 years for chronic interstitial or fibrosing pulmonary disease (FP); (b) 20 patients (4 male and 16 female; mean age: 69.80 +/- 2.96 years) affected by various nonfibrosing pneumopathies or bronchopathies (NFP); (c) 110 control subjects (CS), composed of 15 male and 95 female consecutive patients (mean age: 67.52 +/- 1.52 years) hospitalized in 1999 for minor nonlung-related illnesses. In 24 FP and 36 CS patients, FFA have also been measured. All subjects investigated had no clinical or laboratory evidences of liver, kidney, or thyroid diseases, nor a history of hormonal treatment, alcohol consumption, smoking, or dietary disorders. None of the subjects investigated was under treatment with lipid lowering agents or with other drugs that could affect the levels of TG or FFA or their measurement (including ascorbic acid). RESULTS: Compared with CS, the FP group showed a 61% reduction of TG (0.64 +/- 0.03 vs 1.63 +/- 0.08 mmol/L; P <.001), whereas no significant difference was observed between NFP and CS groups. Serum FFA in FP showed a 63% increase compared with CS (P <.01). In the FP group, TGs were also low in 5 patients with type 2 diabetes (0.76 +/- 0.11 mmol/L, -53%, P <.001) and in 6 obese (body mass index [BMI] > 29 kg/m2) subjects (0.51 +/- 0.03, -69%, P <.01), although it is known that TGs are often elevated in diabetes and obesity. CONCLUSIONS: Chronic interstitial or pulmonary fibrosis is associated with low TG and high FFA levels. Although it is known that the lung contains lipoprotein lipase and is a significant source of plasma postheparin lipase activity, the mechanisms of this change remain to be clarified. Since insulin-like growth factor-I (IGF-I) is known to lower TG and increase FFA, a role could be played by the enhanced production of IGF-I that has been reported to occur in pulmonary fibrosis.

Aged↗
PubMed1999

[Effects of metalloproteinases on pulmonary fibrosis by bleomycin in rats].

OBJECTIVE: Matrix metalloproteinases (MMPs) are the mediators for dynamic equilibrium of extracellular matrix. The purpose was to investigate the effects of metalloproteinases on the development of pulmonary fibrosis. METHODS: The expression and activity of metalloproteinases of alveolar macrophage, and fibroblasts were evaluated on rats with pulmonary fibrosis induced by bleomycin. RESULTS: The activity of collagenase was low in the macrophage and fibroblast in control group, 20.4 x 10(-7) U/cell and 30.6 x 10(-7) U/cell respectively. There was an increase in the first and fourth week groups of bleomycin rats. The highest level of activity of alveolar macrophages was 258.0 x 10(-7) U/cell in first week group. A peak of enzymatic activity was found in 4th week group (289.0 x 10(-7) U/cell). The mRNA of MMP-1, MMP-2 were expressed at lower levels in the normal lung tissue and there was an increase in first week, and fourth week group of bleomycin rats with a peak in one week group. CONCLUSIONS: The regulation of metalloproteinases might play an important role in the development of pathological processes in pulmonary fibrosis.

Animals↗
PubMed2006

[Changes of matrix metalloproteinases activities in pulmonary fibrosis rats].

OBJECTIVE: To observe the changes of matrix metalloproteinases (MMPs) activities in pulmonary fibrosis rats. METHODS: Eighty male SD rats were randomly divided into sham group (n = 40) and bleomycin group (BLM, n = 40), in which SD rats were injected with a single intratracheal dose of sham saline or bleomycin respectively. On day 1, 3, 7, 14, and 28 following bleomycin or saline instillation, rats were randomly killed, and serum from abdominal aorta, alveolar fluid from the bronchoalveolar lavage, and the lung homogenate were collected and then stored at -80 degrees C. MMPs activity was determined by zymography. RESULTS: Compared with sham group, the levels of MMP-9 in all samples were augmented. MMP-9 activities in the serum were highest on day 3 than those on day 1 and day 7, and in lung tissue homogenate were highest on day 7; however, no significant differences were found between BLM group and sham group on day 14 and day 28; and that of bronchoalveolar lavage fluid (BALF) was highest on day 7 than those on day 1 and day 14, while no significant difference existed between BLM group and sham group on day 28. Serum MMP-2 level did not change from day 1 to day 28, while the level of BALF MMP-2 began to increase after day 14, even on day 28. Lung tissue homogenate MMP-2 level began to increase early on day 3 and continued to day 28. CONCLUSION: The sources and effects of MMP-2 and MMP-9 differ in BLM-induced rat pulmonary fibrosis.

Animals↗
PubMed2003

[Evaluation of clinical diagnostic guidelines for idiopathic pulmonary fibrosis].

OBJECTIVES: To analyze the international consensus statement on diagnostic criteria for idiopathic pulmonary fibrosis. METHODS: All patients diagnosed of any interstitial lung disease by means of open lung biopsy since 1980 were included. The patients' clinical records were examined to determine whether they fulfilled the diagnostic criteria, and their biopsies were reviewed to find those with the usual interstitial pneumonia pattern. We calculated sensitivity, specificity, positive and negative predictive values and likelihood ratios for the diagnostic criteria in the consensus statement. Afterwards, we performed the analyses again using only one of the two conditions for fulfilling the function criterion. RESULTS: Of 39 patients enrolled in the study, 17 had idiopathic pulmonary fibrosis. Specificity and positive predictive value were both 100%, but sensitivity was 41.2% and negative predictive value was 68.7%. The likelihood ratio for a negative result was 0.59. In the second analysis, sensitivity was 64.7% and negative predictive value was 78.5%, while specificity and positive predictive value remained unchanged. The likelihood ratio for a negative result was 0.35. CONCLUSIONS: The criteria are sufficiently specific to allow us to diagnose confidently when the criteria are fulfilled. However, a slight change in the function criterion should be considered in order to increase diagnostic yield.

Adolescent↗