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PubMed1997

Features of idiopathic pulmonary fibrosis with organizing pneumonia.

To characterize the clinical features of patients with idiopathic pulmonary fibrosis (IPF) having organizing pneumonia (OP), we retrospectively reviewed the clinical charts, chest X-rays, CT scans, and transbronchial lung biopsy (TBLB) specimens of patients with IPF. Patients with IPF and OP had a subacute onset of symptoms (within 2 months) (87.5%), leukocytosis (> 10,000/mm3) (62.5%), and a strong C-reactive protein (CRP) reaction (> 3+) (75%). Some of these features were distinctly different from those of IPF patients without OP (subacute onset of symptoms 0%, leukocytosis 0%, strong CRP reaction 16.7%). In the patients with IPF with OP, A-aDo2 and semiquantitative scores of chest X-ray abnormalities improved significantly after prednisolone treatment. Those abnormalities improved only slightly in the patients with IPF without OP. Diffusing capacity remained decreased and abnormal interstitial infiltration persisted, even after prednisolone treatment in the patients with IPF with or without OP. Clinical features of IPF patients with OP differed from those of patients with IPF without OP. IPF patients with OP showed good clinical response to corticosteroid therapy. These findings warrant further study of the presence of OP in TBLB specimens in predicting corticosteroid responsiveness and prognosis of patients with IPF.

Cryptogenic Organizing Pneumonia↗
PubMed1986

Lomustine (CCNU)-induced pulmonary fibrosis.

A case of progressive pulmonary interstitial fibrosis following adjuvant lomustine (CCNU)-5-fluorouracil therapy for a colon carcinoma is reported. A close relationship between lomustine assumption and lung damage seems to emerge from the clinical, radiologic and histologic findings presented. On the basis of this experience and previous available data from literature, pulmonary toxicity must be regarded as a possible adverse effect of lomustine therapy.

Adult↗
PubMed2000

Curcumin inhibition of bleomycin-induced pulmonary fibrosis in rats.

Curcumin, an anti-inflammatory, antioxidant, was evaluated for its ability to suppress bleomycin (BLM)-induced pulmonary fibrosis in rats. A single intratracheal instillation of BLM (0.75 U 100(-1) g, sacrificed 3, 5, 7, 14 and 28 days post-BLM) resulted in significant increases in total cell numbers, total protein, and angiotensin-converting enzyme (ACE), and alkaline phosphatase (AKP) activities in bronchoalveolar lavage fluid. Animals with fibrosis had a significant increase in lung hydroxyproline content. Alveolar macrophages from BLM-administered rats elaborated significant increases in tumour necrosis factor (TNF)-alpha release, and superoxide and nitric oxide production in culture medium. Interestingly, oral administration of curcumin (300 mg kg(-1) 10 days before and daily thereafter throughout the experimental time period) inhibited BLM-induced increases in total cell counts and biomarkers of inflammatory responses in BALF. In addition, curcumin significantly reduced the total lung hydroxyproline in BLM rats. Furthermore, curcumin remarkably suppressed the BLM-induced alveolar macrophage production of TNF-alpha, superoxide and nitric oxide. These findings suggest curcumin as a potent anti-inflammatory and anti-fibrotic agent against BLM-induced pulmonary fibrosis in rats.

Animals↗
PubMed1983

The role of strain variation in murine bleomycin-induced pulmonary fibrosis.

In the present study, mice have been used to study the effect of strain variation on the development of pulmonary fibrosis induced by a single intratracheal administration of bleomycin. The fibrotic response was quantitated by biochemical measurement of collagen synthesis and deposition. Results indicate that variations in these parameters of fibrosis are evident depending on the strain being tested. The C57BL/6 were high responders. DBA/2 and Swiss mice were intermediate responders, and BALB/c mice were low responders. These findings indicate that genetic variation influences the development of bleomycin-induced pulmonary fibrosis. Also, because certain aspects of the fibrotic response in this model are similar to those found in other conditions, this model may be useful as a basis to study the role of genetics in general fibrotic mechanisms.

Animals↗
PubMed2004

Involvement of pulmonary endothelial cell injury in the pathogenesis of pulmonary fibrosis: clinical assessment by 123I-MIBG lung scintigraphy.

PURPOSE: Pulmonary microvascular endothelial injury may be involved in the pathogenesis of pulmonary fibrosis (PF). The aim of this study was to evaluate the pulmonary vascular status in patients with PF by lung scintigraphic assessment of 123I-metaiodobenzylguanidine (123I-MIBG), which reflects latent endothelial cell lesions. METHODS: We assessed lung 123I-MIBG kinetics and clinical indices in 23 PF patients and 16 controls. Mean uptake ratios of lung to mediastinum (L/M) were calculated in anterior planar images at 30 (early image) and 270 (delayed image) min after intravenous injection of 123I-MIBG. The pulmonary mean washout rate (WR) of 123I-MIBG was also calculated. RESULTS: The L/M ratio in early images, but not in delayed images, was significantly lower in the PF patients than in the controls (L/M(early) 1.41+/-0.14 vs 1.53+/-0.10, p<0.01; L/M(delayed) 1.28+/-0.10 vs 1.33+/-0.07, p=NS). WR was significantly reduced in the PF patients compared with the controls (28.6%+/-3.1% vs 34.2%+/-5.1%, p<0.001). In the study subjects (PF patients plus controls) there were significant relationships between lung WR of (123)I-MIBG and other diagnostic parameters for the severity of PF, such as vital capacity (r=0.625, p<0.0001), total lung capacity (r=0.691, p<0.0001), carbon monoxide diffusing capacity (r=0.622, p<0.0001), serum angiotensin-converting enzyme activity (r=0.422, p<0.01), carbohydrate antigen KL-6 levels (r=-0.495, p<0.01) and surfactant protein-D levels (r=-0.461, p<0.01). When control subjects were excluded, similar significant correlations were observed between WR and %TLC (r=0.508, p<0.05), DL(CO) (r=0.593, p<0.01) and serum ACE activity (r=0.515, p<0.05) in the PF patients. CONCLUSION: These results suggest that endothelial cell injury plays a significant role in the pathogenesis of PF, and that lung WR of 123I-MIBG, which is a specific marker of endothelial damage, can serve as a novel diagnostic tool to evaluate the functional severity of PF.

3-Iodobenzylguanidine↗
PubMed1998

Idiopathic pulmonary fibrosis: predicting response to therapy and survival.

Idiopathic pulmonary fibrosis (IPF) is associated with significant morbidity and mortality despite aggressive therapy. Thirty-eight patients with biopsy-proven IPF were studied to identify pretreatment features that could be used to predict short-term improvement in pulmonary function and improved longer term survival. In all patients, a pretreatment clinical (dyspnea), radiographic (chest radiograph), and physiologic (pulmonary function including exercise saturation) score was generated (CRP). A high-resolution CT scan (HRCT) was independently scored by four radiologists for ground glass (CT-alv) and linear opacity (CT-fib) on a scale of 0-4. Open lung biopsy samples were scored for cellular infiltration, interstitial fibrosis, desquamation, and granulation by an experienced pulmonary pathologist. All patients were treated with 3 mo of high-dose steroids and the CRP scoring repeated. Patients were divided into three groups: responders with a greater than 10-point drop in CRP (n = 10); stable with +/- 10 point change in CRP (n = 14); and nonresponders with > 10 point rise in CRP or death (n = 14). Those responding to steroids were treated for 18 mo in a tapering fashion. In all others, steroids were tapered quickly and oral cyclophosphamide prescribed. Responders (10 of 38) had a lower age (45.1+/-4.3 yr) than nonresponders (61.4+/-3.5 yr) or those remaining stable (53.1+/-3.3 yr) (p = 0.01). Pretreatment CRP was higher in responders (58.8+/-5.6) than nonresponders (40.5+/-4.7) or stable individuals (37.6+/-4.7) (p = 0.01). Cellular infiltration score of the open lung biopsies was higher in responders (7.6+/-0.6) than stable individuals (5.7+/-0.5) (p = 0.04). The CT-alv scores were higher and CT-fib scores were lower in responders than nonresponders. Receiver operating curve (ROC) analysis was employed to identify pretreatment features of longer term survival (follow-up of 29.1+/-2.3 mo). Only CT-fib (p = 0.009) and pathology fibrosis score (p = 0.03) were able to predict mortality. A pretreatment CT-fib score > or = 2.0 demonstrated 80% sensitivity and 85% specificity in predicting survival. Those patients who did not respond to initial steroid therapy demonstrated a worse long-term survival and greater likelihood of decreased pulmonary function. We demonstrate that pretherapy pulmonary function, pathologic and radiographic parameters are different in individuals who respond to initial prednisone therapy. Only HRCT imaging and pathologic fibrosis were able to reliably predict long-term survival in patients with biopsy-proven IPF.

Adult↗
PubMed2003

Mixed myelodysplastic syndrome and myeloproliferative disorder with bone marrow and pulmonary fibrosis: the role of megakaryocytes.

The case of an 80-year-old woman displaying myelodysplastic syndrome evolving into a myeloproliferative disorder with myelofibrosis and pulmonary fibrosis, is reported. This case is characterized by an initial presentation of a myelodysplastic syndrome with normal karyotype and moderate fibrosis, its evolution towards a myeloproliferative disorder with myelofibrosis and the worsening of pulmonary fibrosis in parallel to the acceleration of the myeloproliferative disorder and myelofibrosis. These features and the high concentration of plasma platelet factor-4 suggest a role of megakaryocyte/platelet degranulation in the development of fibrosis.

Aged↗
PubMed1990

[Pulmonary fibrosis and autoimmune manifestations. A diagnostic problem].

A case of 54-year-old male who, during the development of a pulmonary fibrosis, showed extrapulmonary autoimmune symptoms (polyarthritis and hemolytic anemia), is presented. The antinuclear antibodies and rheumatoid factor positives have been described in up to 30% of the idiopathic pulmonary fibrosis, complicating the differential diagnosis with lung fibrosis caused by alterations of connective tissue. We discuss the prognosis and treatment of this difficult diagnosis.

Antibodies, Antinuclear↗
PubMed2003

Radiographic evidence of pulmonary fibrosis and possible etiologic factors at a nickel refinery in Norway.

Animal studies have shown that nickel compounds may induce pulmonary fibrosis, but so far only limited documentation in humans has been available. Radiographs of 1046 workers in a nickel refinery in Norway were read blindly and independently by three NIOSH certified B-readers, according to the ILO standards. Pulmonary fibrosis (PF) was defined as a median reading of ILO score > or = 1/0 and following this criterion, 47 cases (4.5%) were identified. In logistic regression models, controlling for age and smoking, there was evidence of increased risk of PF with cumulative exposure to soluble nickel or sulfidic nickel (p = 0.04 for both). For metallic nickel a p-value of 0.07 was found. For soluble nickel there was a dose-response trend for 4 categories of cumulated exposure. In the group with the highest cumulative exposure to soluble nickel (low exposure as reference), the crude odds ratio for PF was 4.34 (95% CI 1.75-10.77). The risk adjusted for age, smoking, asbestos and sulfidic nickel was 2.24 (0.82-6.16), with a dose-response trend. The corresponding figures for sulfidic nickel were 5.06 (1.70-15.09, crude) and 2.04 (0.54-7.70, adjusted for age, smoking, asbestos and soluble nickel). However, the dose-response trend was less clear for sulfidic nickel. Controlling for estimated asbestos exposure at the refinery tended to increase the odds ratios of soluble and sulfidic nickel. This study indicates that in addition to age and smoking exposure to soluble and sulfidic nickel compounds are risk factors of PF in humans. Since the number of cases identified in this study is small and undetected confounders may have been present, further studies in other cohorts are appropriate.

Adult↗
PubMed1994

Clubbing of the fingers and smooth-muscle proliferation in fibrotic changes in the lung in patients with idiopathic pulmonary fibrosis.

In our study of 52 patients with idiopathic pulmonary fibrosis (IPF), we found that the incidence of clubbing of the fingers was significantly more frequent in male than in female patients and in patients who showed lesser grades of honeycombing and higher grades of smooth-muscle proliferation in the pulmonary fibrotic changes (p < 0.01). Smooth-muscle proliferation in fibrotic changes of open lung biopsy specimens correlated with the mode of detection of IPF and the presence of clubbing of the fingers, duration of symptoms of the lower respiratory tract, and a higher extent of pulmonary infiltrates on chest radiographs (p < 0.05). However, the presence of clubbing of the fingers or grades of smooth-muscle proliferation in the pulmonary fibrotic changes did not correlate with the 2-year survival after open lung biopsy.

Adult↗
PubMed2004

[Influence of compound biejia ruangan prescription on extracelluar matrix in bleomycin induced pulmonary fibrosis rats].

OBJECTIVE: To study the influence of compound Biejia Ruangan Prescription (CBRP) on extracelluar matrix in bleomycin induced pulmonary fibrosis rats. METHOD: 54 male Sprague-Dawley rats were randomly divided into 6 groups (9 rats in each group). Rats in the model control group, positive medicine group, and high, moderate and low CBRP groups were injected with bleomycin A5 by trachea, and rats in sham-model control group with same volume normal saline. 29 days after the injection, CBRP solution of different dosages (1.4 g x kg(-1), 0.7 g x kg(-1), 0.35 g x kg(-1)) was respectively given to rats in the high, moderate and low CBRP group by gavage, while equal volume of normal saline was given to those in the sham-model control group and model control group, and an equal volume of prednisone (0.56 mg x kg(-1)) was given to those in positive medicine control group. On the 80th day, the levels of III-collagen, IV-collagen, laminin and hyaluronic acid in the serum were determined, the determination of hydroxyproline in lung homogenates was analyzed, and the right lung was incised to make pathological sections which were stained with Hematoxylin-Eosin (HE) and Masson staining for pathological diagnosis. RESULT: CBRP could decrease the levels of III-collagen, IV-collagen, laminin and hyaluronic acid in the serum. CONCLUSION: CBRP may play its therapeutic role by leveling down the content of extracellular matrix in rats with pulmonary fibrosis induced by Bleomycin A5.

Animals↗
PubMedDate not supplied

Time course of matrix metalloproteases and tissue inhibitors in bleomycin-induced pulmonary fibrosis.

To investigate simultaneously localization and relative activity of MMPs during extracellular matrix (ECM) remodeling in bleomycin-induced pulmonary fibrosis in rat, we analyzed the time course of the expression, activity and/or concentration of gelatinases MMP-2 and MMP-9, collagenase MMP-1, matrylisin MMP-7, TIMP-1 and TIMP-2, both in alveolar space (cellular and extracellular compartments) and in lung tissue. MMP and TIMP expression was detected (immunohistochemistry) in lung tissue. MMP activity (zymography) and TIMP concentration (ELISA) were evaluated in lung tissue homogenate (LTH), BAL supernatant (BALs) and BAL cell pellet (BALp) 3, 7, 14, and 28 days after bleomycin intratracheal instillation. Immunohistochemistry showed an extensive MMP and TIMP expression from day 7 in a wide range of structural and inflammatory cells in treated rats. MMP-2 was present mainly in epithelia, MMP-9 in inflammatory cells. MMP-2 and MMP-9 activity was increased respectively in BAL fluid and BAL cells, with a peak at day 7. TIMP-1 and TIMP-2 concentration (ELISA) enhancement was delayed at day 14. In conclusion gelatinases and their inhibitors are significantly activated during bleomycin-induced pulmonary fibrosis. Marked changes in gelatinases activity are observed early in the alveolar compartment, with a prevailing extracellular activity of MMP-2 and a predominant intracellular distribution of MMP-9, while enzyme activity changes in lung parenchyma were less evident. In the repairing phase the reduction of gelatinases activity is synchronous with a peak of alveolar concentration of their inhibitors.

Animals↗
PubMed2000

[Idiopathic pulmonary fibrosis: a new histopathologic classification and clinical aspects].

Idiopathic pulmonary fibrosis (IPF) comprises different interstitial lung diseases of unknown origin. They are classified into four groups based on pathologic criteria: UIP, DIP, AIP and NSIP. This classification helps in terms of prognosis and treatment. In addition to history, physical examination and common investigations (chest X ray, pulmonary function testing), a bronchoalveolar lavage (BAL), a high-resolution CT scan and ideally a open-lung biopsy are needed to establish a precise diagnosis. The patients are usually treated by immunosuppressive agents, and mainly by corticoids. However, in UIP, lung transplantation is the only way to improve survival.

Anti-Inflammatory Agents↗
PubMed1995

Changes in pulmonary function test results after 1 year of therapy as predictors of survival in patients with idiopathic pulmonary fibrosis.

The study group consisted of 58 patients with idiopathic pulmonary fibrosis (IPF) recognized between 1970 and 1991 who were treated for their pulmonary disease, survived for at least 1 year from the time of initiation of treatment, and had forced vital capacity (FVC) measurements at the time of diagnosis and 9 to 15 months later. Forty-four of the patients also had a single-breath diffusing capacity (Dsb) measured initially and after 9 to 15 months of treatment and 33 patients had an arterial blood gas, breathing room air at the time of diagnosis and 9 to 15 months into therapy. Patients' conditions were classified as improved, unchanged, or worse after the year of treatment based on each of the three pulmonary function tests. A > or = 10% increase in FVC, > or = 20% increase in Dsb, and > or = 5 mm Hg decrease in alveolar-arterial difference in oxygen partial pressure [P(A-a)O2] defined improved function. A > or = 10% decrease in FVC, > or = 20% decrease in Dsb, and > or = 5 mm Hg increase in P(A-a)O2 defined worse function. Patients with < 10% change in FVC, < 20% change in Dsb, and < 5 mm Hg change in P(A-a)O2 were regarded as having unchanged conditions. Kaplan-Meier survival plots and the Cox proportional hazard regression model were used to analyze survival time in relation to change in pulmonary function after 1 year of therapy. Patients with an improved or unchanged FVC at 1 year had no difference in survival (p = 0.75), but both showed enhanced survival compared with patients with a > or = 10% reduction in FVC with 1 year of treatment (p < 0.001). Patients with an improved or unchanged Dsb at 1 year also had no difference in survival (p = 0.21) but again, both showed enhanced survival compared with patients with > or = 20% decrease in Dsb with 1 year of treatment (p < 0.001). Changes in gas exchange after 1 year of treatment did not correlate with survival in the three groups. There was a trend for longer survival in improved patients compared with those with worsening gas exchange, but the p value was not significant at 0.17. We conclude that changes in the FVC and Dsb after 1 year of treatment are strongly predictive of duration of survival in patients with IPF.

Adult↗
PubMed1996

Lung fibroblast alpha-smooth muscle actin expression and contractile phenotype in bleomycin-induced pulmonary fibrosis.

The emergence of the myofibroblast phenotype (characterized by alpha-smooth muscle actin expression) in wound healing and in tissues undergoing fibrosis is thought to be responsible for the increased contractility of the affected tissues. In bleomycin-induced pulmonary fibrosis, the myofibroblast is also responsible for the observed increase in collagen gene expression. To evaluate further these phenotypic changes in lung fibroblasts, contractile and other phenotypic properties of fibroblasts isolated from lungs of rats with bleomycin-induced fibrosis were compared with those of normal rats using in vitro models. Pulmonary fibrosis was induced in rats by endotracheal injection on day 0, and 7 and 14 days later the animals were sacrificed and lung fibroblasts isolated. Using immunofluorescence, < 10% of fibroblasts from control animals express alpha-smooth muscle actin when cultured as a monolayer. In contrast, 19% and 21% of cells from day 7 and day 14 bleomycin-treated animals, respectively, expressed this actin and with greater intensity than in control lung cells. This increase in actin expression was associated with enhanced contractility when evaluated using a three-dimensional cell culture model consisting of fibroblast-populated collagen gels. This enhanced contractility was abolished by treatment with antibody to transforming growth factor-beta (TGF-beta), whereas exogenous TGF-beta 1 and serum-stimulated contraction of control lung fibroblasts. TGF-beta 1 gene expression was greater in cells from bleomycin-treated animals than those from control lungs. These results show that cells with the myofibroblast phenotype are more abundant in fibrotic lung, and that these cells possess greater contractile capacity in vitro at least partly by virtue of their enhanced endogenous TGF-beta 1 gene expression.

Actins↗
PubMed2005

The effects of tamoxifen on radiation-induced pulmonary fibrosis in Wistar albino rats: results of an experimental study.

This study was performed to evaluate the effects of tamoxifen on pulmonary fibrosis, given concurrently with or after irradiation in Wistar albino rats. Twenty-one female Wistar albino rats were randomized into three groups. The first group (Group A) had tamoxifen, which was started after the completion of irradiation. The second group (Group B) had tamoxifen concomitant with irradiation. The third group (Group C) had only thoracic irradiation and did not receive tamoxifen. Whole lungs were irradiated to a total dose of 30Gy in ten fractions with Co60. Tamoxifen was continued until the animals were sacrificed 16 weeks after the start of irradiation. As an end point the percentage of lung with fibrosis for each rat was quantified with image analysis of histological sections of the lung. Groups were compared using the one-way ANOVA method and Bonferroni post hoc test. The mean percentage values of fibrosis were 10.03 for Group A, 36.81 for Group B, and 3.87 for group C (P<0.001). When the percentages of fibrosis were compared for each group, the difference was statistically significant between Group A and Group B (P<0.001) and between Group B and Group C (P<0.001). Concomitant use of tamoxifen appears to increase radiation-induced pulmonary fibrosis and it seems more convenient to delay tamoxifen until the completion of irradiation.

Animals↗
PubMed1998

Proteinases and proteinase inhibitors during the development of pulmonary fibrosis in rat.

Changes in the activities of several proteinases and their inhibitors were investigated during the development of bleomycin-induced pulmonary fibrosis in rat. Studies on the proteinase-anti-proteinase-ratio may contribute to the understanding of the mechanism of the development of pulmonary fibrosis and may help to develop therapeutic strategies to prevent tissue damage by proteolytic attack. In the acute inflammatory period the activity of metalloelastase in lung tissue increased by about 10-fold. The time course of changes in the activity of 72 kD gelatinase indicates that this gelatinase accounts at least partially for the elastolytic activity. Elastase inhibitory activity in lung tissue showed maxima at days 1 and 5 and high levels in the fibrotic phase. The increase of the elastase inhibitory activity at the beginning of the fibrotic period corresponds with elevated activity of alpha 2-macroglobulin. Alveolar fluid and alveolar macrophages did not contain elastase activity but contained high elastase inhibitory activity. During the period of chronic inflammation, the activities of the cathepsins L, B, H and S in lung tissue and in isolated alveolar macrophages were found to be strongly increased.

Animals↗