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PubMed2001

Is it necessary to treat all patients with idiopathic pulmonary fibrosis?

BACKGROUND AND AIM OF THE WORK: To investigate the clinical course of untreated patients with idiopathic pulmonary fibrosis (usual interstitial pneumonia) (IPF/UIP). METHODS: Forty-three patients with IPF/UIP, divided into two groups. Group I consisted of 29 patients treated at diagnosis, while Group II comprised 14 patients who did not receive treatment. The indication of treatment was established whenever patients referred to a significant progression of the degree of dyspnea during the year prior to diagnosis. RESULTS: At diagnosis, patients from Group I had lower FVC (mean +/- SEM, 56+/-3% vs 73+/-3%) (p = 0.0004) and a greater extent of ground glass pattern in high resolution CT scan (18+/-4% vs 4+/-1%) (p = 0.004) than those from Group II. In group I, a follow-up study was carried out on 26 patients for 24+/-4 months. Thirteen of these 26 patients (50%) died 11+/-4 months after the initial assessment. Serial pulmonary functional tests were performed on 19 patients. Thirteen patients from Group II were followed up for 23+/-3 months. Seven of these 13 patients were treated 12+/-3 months after the diagnosis because of progression of the disease. The remaining 6 patients remained untreated and with the disease stable at the end of the follow-up, representing 15% (6 out of 39) of the whole study group. No patients from this group died during the follow-up. At the end of the follow-up, there were no differences in lung function changes between treated patients (19 from Group I and 7 from Group II), and the 6 untreated patients. CONCLUSIONS: Some patients with IPF/UIP remain stable for extended periods of time without treatment.

Aged↗
PubMed1991

Prognosis of idiopathic pulmonary fibrosis in patients with mucous hypersecretion.

In order to determine the prognosis of patients with chronic idiopathic pulmonary fibrosis (IPF), we evaluated clinical, laboratory, and bronchoalveolar lavage (BAL) data at the onset of IPF in 25 patients who survived beyond 1 yr (nine women and 16 men, 59 +/- 3 yr of age, mean +/- SE). When the patients were divided into two groups according to whether they had or did not have mucous hypersecretion, 11 patients with hypersecretion (Group A) had a poorer survival rate (6 yr) than did 14 patients without hypersecretion (Group B) (10 yr) (p less than 0.01). Further, there was a significant negative correlation between sputum volume and the duration of survival in 25 patients (r = -0.55, p less than 0.01). Before glucocorticoid treatment, we also found significantly larger numbers of neutrophils (17%) and eosinophils (5%) in differential cell counts of bronchoalveolar lavage fluid (BALF) in Group A than in Group B (neutrophils, 1%; eosinophils, 0.6%) (p less than 0.05 each). Chest radiographic findings and other laboratory data including pulmonary function tests did not correlate with the survival rate. These findings suggest that mucous hypersecretion as well as neutrophils and eosinophils in BALF are among the determinants of prognosis in patients with chronic IPF.

Aged↗
PubMed1994

Lung monocyte chemoattractant protein-1 gene expression in bleomycin-induced pulmonary fibrosis.

Recent studies indicate that monocyte chemoattractant protein-1 (MCP-1) may play an important role in pulmonary inflammation. In vitro studies show that a number of cell types are capable of producing MCP-1. In this study, MCP-1 expression in lungs of rats with bleomycin (BLM)-induced pulmonary fibrosis is examined to evaluate its cellular origin and potential role in pathogenesis. Lung fibrosis was induced in male Fisher 344 rats by endotracheal injection on day 0. On selected days after injection, lungs were harvested for in situ and Northern hybridization analyses for MCP-1 mRNA expression, immunochemical and histochemical analyses for MCP-1 protein expression, and identification of cell type. Northern analysis revealed significant elevation in lung MCP-1 mRNA expression beginning on day 3 post-BLM treatment, increasing to a peak on day 7, and then decreasing toward control levels after day 21. In situ hybridization combined with histochemical staining with chromotrope 2R indicate that most of the cells expressing MCP-1 mRNA at these time points are primarily eosinophils. A few scattered reactive fibroblasts, some mononuclear cells, epithelial cells, and cells of certain blood vessel walls also express this mRNA. Increased MCP-1 protein expression also was found to be predominantly within and adjacent to eosinophils. The eosinophils expressing this mRNA were found predominantly within areas of active fibrosis. The kinetics of increase in the number of cells expressing significant MCP-1 mRNA in lung sections paralleled that for MCP-1 mRNA expression, as assessed by Northern analysis. These results, for the first time, demonstrate that MCP-1 is up-regulated significantly in this rat animal model, and that infiltrating eosinophils represent the major cellular source for this increased MCP-1 expression.

Animals↗
PubMed1993

Outcome of subjects with idiopathic pulmonary fibrosis who fail corticosteroid therapy. Implications for further studies.

To evaluate the outcome of subjects with idiopathic pulmonary fibrosis (IPF) whose conditions clinically deteriorate while receiving corticosteroid therapy, we studied 12 of these subjects (7 male, 5 female) who received subsequent therapy with intravenous (IV) pulse cyclophosphamide (CPX). Seven of the 12 study subjects died during the course of therapy. Six of these subjects died of respiratory failure, and one died of cholecystitis. Among those who died, the mean age at diagnosis was 63 years compared with 57 years in those who have continued to survive (p = 0.29). Smoking status and pack-years of cigarette smoking were similar between those subjects who died and those who continue to survive. However, subjects who died received CPX for a mean of 6 months, while subjects still living have received CPX for a mean of 16 months (p = 0.01). Subjects who died were given a CPX a mean of 64 months after the onset of symptoms, compared with a mean of 50 months for subjects who are still alive (p = 0.57). Interestingly, there were no significant differences in measures of pulmonary function between living and dead subjects. In fact, measures of lung function and gas exchange remained stable in both groups throughout the period of observation. These data suggest that (1) measures of lung function may not be a reliable indicator of patient mortality in end-stage IPF, and (2) while not statistically significant, these data raise the possibility that duration of symptomatic disease may play a role in the outcome of IPF patients receiving alternative therapeutic agents after failure of corticosteroid therapy. In future intervention trails, controlling entry criteria for duration of disease may prove helpful in determining the effects of these agents on the disease process. These data do not permit a determination of the effect of CPX in patients with IPF.

Administration, Oral↗
PubMed1980

Collagen concentration and rates of synthesis in idiopathic pulmonary fibrosis.

To define the biochemical correlates of the apparent morphologic increase in lung interstitial collagen in idiopathic pulmonary fibrosis (IPF), collagen content was quantitated, and the morphologic degree of fibrosis was assessed in 9 patients with IPF and 6 control subjects. There were no significant differences in the collagen content among patients with IPF compared with that of control subjects. In addition, there was no correlation between collagen content and the morphologic assessment of the degree of fibrosis. Analysis of the collagen content from multiple sites made from base to apex of postmortem material from 3 patients with IPF and 2 control subjects demonstrated a wide, but similar, variation in collagen content in both groups; no definite pattern of anatomic distribution was found in either the patients with IPF or the control subjects. Furthermore, the rates of collagen and noncollagen protein synthesis in explants of lung of patients with IPF demonstrated no significant differences compared with those of the control subjects. The results of this study are consistent with the concept that IPF is a disease of an alteration in quality, form, and location of collagen rather than simply a disease of increased interstitial collagen.

Adult↗
PubMed1992

Idiopathic pulmonary fibrosis. Quantitative assessment of lung pathology. Comparison of a semiquantitative and a morphometric histopathologic scoring system.

The objective of this study was to determine the accuracy of a semiquantitative method of assessing the relative degree of cellularity and fibrosis compared with a morphometric analysis of specific histopathologic features in idiopathic pulmonary fibrosis (IPF). Morphometric analysis was performed on biopsy tissue from 20 patients. Morphometry showed that approximately 70% of parenchymal tissue was abnormal: 35% cellular consolidation and fibrosis, 20% honeycomb changes, 10% thick alveolar septa, and 5% small airways within abnormal parenchymal tissue. The prominent components of the abnormal parenchymal tissue were extracellular fibers, an abundance of interstitial cells, and epithelial cell hyperplasia. Correlation between four groupings of pathologic features, identified by a semiquantitative analysis, and each of the components of the parenchymal lesions showed significant correlations between (1) the fibrotic or reparative factor (alveolar wall metaplasia, fibrosis, honeycombing, and smooth muscle and vascular changes) and components of honeycomb lesions (extracellular fibers, interstitial cells, and epithelial injury and repair), and (2) the inflammatory and exudative factor and extracellular fibers in the lesions. These results support that the scoring system used by the pathology panel provides an accurate assessment of pathologic features useful in the assessment of the extent and severity of the histopathologic lesions of IPF.

Biopsy↗
PubMed1998

Mapping of topoisomerase II alpha epitopes recognized by autoantibodies in idiopathic pulmonary fibrosis.

Autoantibodies against DNA topoisomerase II alpha have been identified in the sera of patients with idiopathic pulmonary fibrosis (IPF). To map topoisomerase II autoepitopes, we tested by ELISA and immunoblotting the IPF anti-topoisomerase II-positive sera against a series of recombinant proteins which covered the full length of topoisomerase II alpha. Specific patterns of reactivity were observed, indicating the existence of multiple epitopes on topoisomerase II, either highly complex or conformational/discontiguous or conformational/contiguous ones. The latter resided in amino acid residues 854-1147 and 1370-1447. A detailed analysis of these regions was undertaken, but we were not able to pinpoint a sequential peptide-sized epitope, or any significant homology with foreign pathogens. Further, we observed a significant correlation between the progression from a contiguous to a quaternary/tertiary structure-dependent autoepitope and the disease duration but not with the disease severity. Therefore, this result supports the hypothesis that anti-topoisomerase II autoreactivity evolves following an antigen-driven process.

Adult↗
PubMed1999

[The therapeutic effect of ligustrazin and salvia miltiorrhiza on the gene expression of alpha1 (I) and alpha1 (III) procollagen in rat pulmonary fibrosis].

OBJECTIVE: To evaluate the effect of ligustrazin and salvia miltiorrhiza on collagen gene expression in rat pulmonary fibrosis (PF). METHODS: The tested animals were divided into 7 groups: normal control, untreated animal groups (day 7, 14 and 29), Ligustrazin, Salvia Miltiorrhiza and hydrocortisone groups. Treatment was started from day 15 to day 28. HE stain and in situ hybridization with alpha(1) (I) and alpha(1) (III) procollagen (PC) cDNA probes were applied to rat lungs on day 29. The results were quantified by a feature analysor. RESULTS: All the three drugs were effective, ligustrazin the best (P < 0.01), salvia miltiorrhiza the second (P < 0.01). In untreated groups both alpha(1) (I) and alpha(1) (III) PC mRNA reached the maximum on day 7. Alpha(1) (I) PC mRNA still kept high on day 29 while alpha(1) (III) PC mRNA had decreased to normal. In ligustrazin group alpha(1) (I) PC mRNA decreased to normal (P > 0.05) but in salvia miltiorrhiza and hydrocortisone groups it was still above normal level (P < 0.01). In all groups alpha(1) (III) PC mRNA had decreased to normal on day 29. CONCLUSIONS: Inhibitive effect of ligustrazin on alpha(1) (I) PC mRNA may play an important role in treating PF. The effect of salvia miltiorrhiza is less than ligustrazin.

Animals↗
PubMed1988

The effect of cigarette smoke on bleomycin-induced pulmonary fibrosis in hamsters.

To investigate the effect of cigarette smoke on the development of bleomycin (BLM)-induced pulmonary fibrosis in hamsters, four experimental groups were studied: a control group (C), a cigarette smoke-inhaled group (T), a BLM-administered group (B), and a cigarette smoke-inhaled plus BLM-administered group (TB). Groups T and TB were exposed to sidestream smoke of cigarettes for 30 min/day, 5 days/wk. Groups B and TB were administered 0.5 mg BLM hydrochloride per 100 g body weight endotracheally once on day 30 (Day 0) after housing start. Quantitative morphometry of the lungs revealed that Group TB showed less lung fibrotic change compared with Group B, but based on qualitative observation, the fibrotic lesions of Group TB were intermingled with slight emphysematous changes. Neutrophils in bronchoalveolar lavage fluid were remarkably increased in both the groups, with a peak on Day 1, but the increase in Group TB lasted longer. Alveolar macrophages were increased in both smoking groups (T and TB) compared to the non-smoking groups (C and B). These results suggest that cigarette smoke reduces BLM-induced lung fibrotic changes; however, it simultaneously causes derangement of alveolar architecture. The persistence of increased neutrophils in the early phase after BLM accompanied by exposure to cigarette smoke may play an important role in the mechanism by which smoke ameliorates the effect of BLM.

Animals↗
PubMed1985

Comparison of cell profiles of bronchial and bronchoalveolar lavage fluids between normal subjects and patients with idiopathic pulmonary fibrosis.

The cell profiles of bronchial and bronchoalveolar lavage fluids (BLF and BALF) of patients with idiopathic pulmonary fibrosis (IPF) were compared with those of normal volunteers (NV) and age-matched control patients (CP), to characterize the cell profiles of the bronchoalveolar region in normals and patients with IPF. In BALF of nonsmokers from both control groups (NV and CP), alveolar macrophages (AM) were predominant and the percentage of neutrophil leukocytes and that of eosinophil leukocytes below 1% of the total cells. The percentage of neutrophils and that of bronchial epithelial cells were higher in BLF than in BALF of both control groups. Of the immune and inflammatory cells in BLF, the mean percentage of neutrophils was 12% in NV group and 42% in CP group. The percentage of neutrophils and that of eosinophils in BALF were higher in IPF group than in CP group, but the percentage of neutrophils in BLF of IPF group was comparable to that of CP group. In the IPF group, the percentage of neutrophils in BALF was lower than that in BLF. These results indicated that even in healthy subjects, a considerable number of neutrophils are present in the bronchial region and that the cell profile of the lavage fluid of the bronchoalveolar tree changes depending on the method of lavage. Presumably the higher percentage of neutrophils in BALF of patients with IPF is partly due to derangements of the alveolar structure, because the amount of saline infused into this region is limited.

Adult↗
PubMed2005

A critical assessment of treatment options for idiopathic pulmonary fibrosis.

BACKGROUND: To date, no management approach has proven to be efficacious for the treatment of idiopathic pulmonary fibrosis (IPF). Consequently, therapeutic options remain controversial and confusing for many clinicians. We sought to formally review available evidence on treatment options for IPF and to have a diverse panel of physicians rate the "appropriateness," "inappropriateness," or "uncertainty" of some of the available therapeutic options. METHODS: The RAND/UCLA Appropriateness Method was used to review and rate multiple clinical scenarios for the treatment of IPF. The panel was composed of nine physicians from geographically diverse areas who received a systematic review on the risks and benefits of commonly used treatments for IPF as background. RESULTS: A total of 324 clinical scenarios were rated: 25% as appropriate; 39%, uncertain; and 36%, inappropriate. The panel disagreed about 12% of the therapy indications in the final ratings, falling from 26% in the first-round ratings. CONCLUSIONS: Key themes emerged from the consensus process. Lacking evidence for a definitive therapy, it was considered most appropriate to enroll eligible patients in clinical trials and refer eligible patients for transplant evaluation. For patients without access to clinical trials, the committee was not unanimous regarding treatment recommendations. It was considered inappropriate for patients with a confident diagnosis of IPF to be treated with corticosteroids as the sole agent: corticosteroids should be used in conjunction with azathioprine. With progressive disease despite such combination use, there was agreement for the use of interferon gamma-1b in patients unwilling or unable to participate in available clinical trials.

Aged↗
PubMed1999

Immunoglobulins and cellular constituents of the BAL fluid of patients with sulfur mustard gas-induced pulmonary fibrosis.

STUDY OBJECTIVE: The acute heavy exposure to sulfur mustard gas can lead to pulmonary fibrosis (PF). This study was performed to determine the cellular and protein content of BAL fluid in 24 patients with sulfur mustard gas-induced PF. PATIENTS: Twenty-four veterans with sulfur mustard gas-induced PF and 18 nonexposed veterans serving as control subjects were enrolled into the study. MEASUREMENTS: Chest roentgenograms, pulmonary function tests (PFTs), tests for carbon monoxide diffusing capacity of the lung (DLCO), high-resolution CT scans of the chest, BAL via fiberoptic bronchoscopy, analyses of BAL fluids for cellular and protein constituents, and determinations of serum albumin and Ig levels were performed in all cases. A transbronchial lung biopsy was done in all patients following BAL. RESULTS: Neutrophilic alveolitis was the predominant feature. Neutrophils (p = 0.0001) and eosinophils (p = 0.0001) were the predominant cell types in the BAL fluid of patients with PF. There was a strong correlation between the BAL fluid neutrophil count (p = 0.76; p = 0.0003) or its percentage (p = 0.77; p = 0.0003) and the degree of fibrosis. Of the BAL fluid Ig levels, only the IgG level in the study group was significantly higher than the IgG level of the control group (p = 0.0001). Of the PFT physiologic parameters, only the percentage of DLCO showed a significant correlation with the degree of fibrosis (p = -0.76; p < 0.001). CONCLUSION: The cellular constituents of BAL fluid in patients with sulfur mustard gas-induced PF are very similar to the cellular constituents seen in patients with idiopathic PF, and this finding indicates the presence of an ongoing active alveolitis in PF.

Adult↗
PubMed1989

Pulmonary fibrosis induced in the hamster by amiodarone and desethylamiodarone.

Associated with amiodarone use is pneumonitis which may progress to life-threatening pulmonary fibrosis. Desethylamiodarone, a metabolite, whose role in the etiology of amiodarone-induced pulmonary toxicity has been unclear, also possesses antiarrhythmic activity and could potentially be used as an antiarrhythmic drug itself. We have used a single intratracheal administration of equimolar amounts of amiodarone or desethylamiodarone (1.83 mumol) to male golden Syrian hamsters to investigate the fibrogenicity of desethylamiodarone. Animals were terminated at 1, 7, 14, 21, and 28 days post-treatment, and toxicity was assessed by measurement of lung hydroxyproline content and by histological techniques. Amiodarone and desethylamiodarone significantly increased lung hydroxyproline content over vehicle control animals by 21 days (33 and 58% respectively). While amiodarone-treated lungs had hydroxyproline contents similar to control levels at 28 days, desethylamiodarone-treated lungs remained elevated (44% over control values). Quantitative histologic examination revealed that lungs from desethylamiodarone-treated animals displayed a greater toxic effect, while trichrome staining confirmed the increased deposition of interstitial collagen in these same animals. These results may be due to the higher affinity of the lung for desethylamiodarone and thus a prolonged exposure. The findings indicate that, in the hamster, both compounds are toxic by this route and that desethylamiodarone is not a nontoxic metabolite. Further, use of desethylamiodarone as an antiarrhythmic agent may not be devoid of the adverse effects associated with amiodarone.

Amiodarone↗
PubMed1989

Cellular events in alveolitis and the evolution of pulmonary fibrosis.

"Alveolitis", as opposed to "pneumonia" sensu strictiori, is a term used to denote diffuse inflammatory changes of the pulmonary parenchyma, excluding those that result from local bacterial, fungal or other extracellular microbial growth. The various types of alveolitis are classified according to their histological characteristics and range from "luminal phagocytic" or "mural lymphoplasmacellular" and "exudative" to "fibrosing" alveolitis. In this overview, various exogenous and endogenous causes of different types of alveolitis, and the cellular events in their pathogenesis are briefly discussed to illustrate the complex mechanisms involved. Particular emphasis is placed on the possible transition from diffuse exudative to fibrosing alveolitis. It appears that pulmonary fibrosis, which is usually patchy rather than truly diffuse, does not have a uniform pathogenesis. Besides the possibility of a certain degree of a diffuse fibrosis three major pathways are evident: (1) granulation tissue budding into alveolar lumina (luminal fibrosis) (2) exudate incorporation into alveolar walls (mural fibrosis) and--at least equally important--(3) so-called collapse (atelectatic) induration (obliterative-interseptal fibrosis), a process that has largely been neglected so far.

Air Pollutants↗
PubMedDate not supplied

[Bi-apical pulmonary fibrosis during ankilosing spondylitis: A report on 5 cases (author's transl)].

The authors describe 5 cases of bi-apical pulmonary fibrosis occurring during the course of ankylosing spondylitis. In 4 patients the lesions were advanced and detected during pneumological investigations. In the 5th patient, the lesions were at an early stage and were discovered during a retrospective study of 100 case-reports of patients with ankylosing spondylitis. Radiological signs were morphologically similar: localized pulmonary lesions of the two apices, of the retractile type and with a fibrous hilar framework, bullous images that were more or less extensive, raising of the two hili with compensatory distention at both bases, and pleural lesions consisting of thickening of both pleurae at the apices. The mean age of the patients at the time these lesions appear is 60 years, and they occur about 20 years after the onset of the articular disorder. These fibro-bullous lesions can be complicated by aspergillosis (20%) of pneumothorax (8%). The principal differential diagnosis is advanced tuberculous disease in the lung apices, and this explains why these patients have often been prescribed antituberculosis therapy.

Adult↗