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PubMed1989

[M. kansasii lung infection occurring in a compromised host with idiopathic pulmonary fibrosis].

A 61-year-old man was admitted to our hospital because of persisting cough, sputum and shortness of breath for four months. Brushing specimens and BALF bronchoscopically obtained revealed acid-fast bacilli and TBLB showed pathological findings consistent with interstitial pneumonia. Based on these results, clinical symptoms, chest roentgenograms on admission and identification of M. kansasii, a diagnosis of M. kansasii lung infection occurred in idiopathic pulmonary fibrosis was made. The patient's symptoms consistent with M. kansasii lung infection and his sputum became negative 6 weeks after antituberculosis chemotherapy with INH, SM and RFP. Because of an increasing dyspnea due to pulmonary fibrosis, however, the patient received oxygen therapy. This case suggested an increasing tendency of compromised hosts associated with M. kansasii lung infection.

Humans↗

PubMed1982

Pulmonary fibrosis following long-term nitrofurantoin therapy.

Three patients who developed pulmonary fibrosis following prolonged treatment with nitrofurantoin for chronic urinary tract infections are presented. They had received the drug for 3--4 years; all gave histories of 2--3 years of progressive shortness of breath and an unproductive cough. On examination, all 3 patients had bilateral crackles and 1 had finger clubbing. Chest radiographs showed bilateral shadowing and lung function tests a restrictive defect with reduced gas transfer. Lung biopsies showed extensive fibrosis in 2 patients and advanced honeycomb formation in 1. The response to steroid therapy in 2 patients was excellent, with resolution of symptoms, clearing of the chest radiographs and improvement in lung function. It is proposed that nitrofurantoin has no place in the long-term treatment of chronic urinary tract infections and that its use in acute infections should be questioned.

Adult↗

PubMed1998

Idiopathic pulmonary fibrosis and cyclosporine: a lesson from single-lung transplantation.

Idiopathic pulmonary fibrosis (IPF) has a poor prognosis and therapeutic options are limited with a 5-year survival of less than 50%. This report includes a case of histologically confirmed IPF in a patient whose native lung showed objective improvement as measured by high-resolution CT while he was receiving cyclosporine-based immunosuppressive therapy after single-lung transplantation.

Cyclosporine↗

PubMed2001

Polymyositis, pulmonary fibrosis and malignant lymphoma associated with hepatitis C virus infection.

Polymyositis has been associated with various viral infections, and a spectrum of immune-related diseases may occur with hepatitis C (HCV) infection. Both polymyositis and HCV infection may be accompanied by pulmonary fibrosis. An association between polymyositis and malignancy has also been reported. We report a 55-year-old woman accompanied cryoglobulinemia with HCV infection and manifesting polymyositis, pulmonary fibrosis and malignant lymphoma. Steroid therapy was effective to improve interstitial pneumonia, polymyositis, and liver function.

Cryoglobulinemia↗

PubMed1985

Idiopathic pulmonary fibrosis in adult respiratory distress syndrome. Diagnosis and treatment.

Ten consecutive patients who presented initially with adult respiratory distress syndrome, and who did not respond to conventional treatment, underwent open lung biopsy early in the course of their illness. Idiopathic pulmonary fibrosis was diagnosed and aggressive treatment with methylprednisolone sodium succinate and nutritional support was initiated. Eight patients survived and have not required long-term corticosteroid therapy. Previous reports have documented a mortality of greater than 85% with this particular syndrome of idiopathic pulmonary fibrosis in adult respiratory distress syndrome.

Adolescent↗

PubMed1986

A clinical, radiographic, and physiologic scoring system for the longitudinal assessment of patients with idiopathic pulmonary fibrosis.

In order to develop a reproducible, quantifiable means of assessment of the clinical status of patients with idiopathic pulmonary fibrosis (IPF), a composite clinical-radiographic-physiologic (CRP) scoring system was devised, using 7 variables: dyspnea, chest radiograph, spirometry, lung volume, diffusion capacity, resting alveolar-arterial PO2, and exercise O2 saturation. To assess this scoring system, we examined the relationships between CRP scores and histopathologic findings, including a cellular pathology score composed of abnormalities deemed to be potentially reversible, a fibrotic pathology score based on abnormalities felt to be essentially irreversible, and an index of overall pathologic derangement (total pathology score), derived from the sum of the cellular and fibrotic scores. The initial CRP determination at the time of open lung biopsy correlated significantly with the total pathology score (r = 0.61, p less than 0.001). The CRP score determined after 6 months of corticosteroid therapy showed a significant correlation with the fibrotic pathology score present on open lung biopsy (r = 0.76, p less than 0.001). The change in CRP after 6 months of corticosteroid therapy tended to reflect the cellular histopathologic component of the open lung biopsy (r = -0.43, p less than 0.10). Moreover, in none of these relationships did any individual component of the CRP score correlate better with the respective histopathologic index than did the CRP score itself. These data suggest that this CRP score is useful for the estimation of the severity of underlying pathologic derangement and for the longitudinal quantitative assessment of clinical impairment in patients with IPF.

Adult↗

PubMed2003

Cancer in interstitial pulmonary fibrosis and sarcoidosis.

The occurrence of lung cancer in patients with diffuse interstitial pulmonary disease is well-known. The incidence, however, varies and ranges from 9.8 to 38%. The pathogenesis of lung cancer in pulmonary fibrosis is unclear, but genetic as well as environmental factors seem to be involved.

Humans↗

PubMed1985

Effect of low hemoglobin levels on the diffusing capacity of the lungs for CO. Investigations in persons with healthy lungs and in patients with interstitial pulmonary fibrosis.

The influence of the hemoglobin concentration in the peripheral venous blood on the diffusing capacity of the lungs for CO (DLCO) was tested with the single-breath method. 9 patients with histologically verified interstitial pulmonary fibrosis of varying etiology and 26 persons with healthy lungs with anemia requiring transfusions were investigated before and after compensation of the anemia. It was found that the true maximum diffusing capacity of the lungs measured in anemia patients was too low. In the test subjects with healthy lungs, there was a positive linear correlation between the hemoglobin content of the blood and DLCO. A correction factor to calculate the true DLCO in patients with healthy lungs but with anemia is specified. In the patients with interstitial pulmonary fibrosis, the influence of the reduced diffusing capacity of the alveolocapillary membrane on the extent of the diffusion disorder was in each case greater than the effects due to the anemia.

Adult↗

PubMed1990

Isolated lung transplantation for pulmonary fibrosis.

The peri-operative anaesthetic management of 11 patients with pulmonary fibrosis undergoing single-lung transplantation is presented. Intra-operative problems, the early postoperative phase of recovery and intensive care, and other incidents in which general anaesthesia was required for the management of complications, are featured. Results, both short- and long-term, are mentioned. Major intra-operative events that cause concern appear to be related to the severity of the presenting illness and the development of respiratory failure. Others have reported the development of intra-operative cardiac failure. All cases were successfully managed operatively using conventional one-lung anaesthesia, although resort to partial cardiopulmonary bypass may have been indicated in some. The indications and attitudes to utilising cardiopulmonary bypass in the evolution of techniques for facilitating single-lung transplantation are reviewed.

Adult↗

PubMed2001

[Clinical presentation of own idiopathic pulmonary fibrosis patients according to international consensus statement].

Clinical presentation of idiopathic pulmonary fibrosis (IPF) restricted, according to current definition to usual interstitial pneumonia (UIP) was presented. 62 patients (39 males and 23 females) were assessed. The diagnosis of IPF/UIP has been based upon a combination of clinical, radiographic and physiologic features in majority of patients. Histologic confirmation from lung biopsy has been obtained in 16% of cases. Mean age of the patients was 64.4 +/- 8.0 years. Mean duration of symptoms was 20.1 +/- 14.1 months. The main symptom was exertional dyspnea. Crepitations were found in 98% of patients. Lung volumes were in normal range in substantial number of patients; TLC in 15 (24%) and FVC in 33 (53%) out of 62 patients. Disturbances of lung function concerned mainly gas exchange (DLCO diminished in 92% of cases) and lung compliance (diminished in all patients). Presentation of clinical, radiographic and physiologic features of IPF/UIP in a homogenous group of patients may be helpful in diagnosis of this common interstitial lung disease.

Aged↗

PubMed2006

A novel IkappaB kinase-beta inhibitor ameliorates bleomycin-induced pulmonary fibrosis in mice.

RATIONALE: IkappaB kinase-beta is a critical regulator in the activation of nuclear factor-kappaB (NF-kappaB), a transcription factor related to the expression and regulation of proinflammatory cytokines. OBJECTIVE: To evaluate if inhibition of IkappaB kinase-beta ameliorates pneumonitis and pulmonary fibrosis. METHODS: We examined whether a novel IkappaB kinase-beta inhibitor, IMD-0354, attenuates bleomycin-induced pulmonary fibrosis in mice. MEASUREMENTS AND MAIN RESULTS: Administration of IMD-0354 significantly improved the loss of body weight and survival of mice treated with bleomycin, whereas IMD-0354 alone did not cause any morphologic change in the lung. When mice were evaluated 28 d after bleomycin administration, IMD-0354 dose-dependently reduced the collagen content and fibrotic scores as shown by histologic examination. The findings in the bronchoalveolar lavage demonstrated that the proportions of neutrophils and lymphocytes were decreased in mice treated with IMD-0354 on Day 7 and 14, respectively. IMD-0354 treatment was confirmed to inhibit the activation of NF-kappaB, but not activator protein-1, in the lungs treated with bleomycin. The production of inflammatory cytokines tumor necrosis factor-alpha and interleukin-1beta was reduced in the lungs of mice treated with IMD-0354. CONCLUSIONS: These results suggest that IMD-0354 might be useful to ameliorate the inflammation in the lungs induced by fibrotic injury and the subsequent fibrogenesis via inhibiting the expression of profibrotic cytokines related to the activation of NF-kappaB.

Animals↗

PubMed1995

[Morphology of mast cells in experimental pulmonary fibrosis induced with bleomysin].

Bleomycin induces local inflammatory process with subsequent pulmonary fibrosis. An unknown chemoattractant induces the mast cell (MC) migration to the injured lung tissue. Aim of this study was evaluation of the number, topography and ultrastructure (TEM) of the MC in rat lungs with bleomycin-induced fibrosis. The bleomycin was administered once intratracheally (3.6 mg/kg). The animals were sacrificed on 7th. 14th and 21st day of experiment. MC were stained with Csaba's method. An evident increase in MC number related to the phase of experiment and stage of lung fibrosis was observed: 520, 1200, 4745 per cm2 section on the 7th, 14th and 21st day respectively. In control the MC number was 163 per cm2 section (p < 0.001). On the 7th day the MC were rich in red granules (mature granules with preponderance of heparin). They were located mainly in pleura and around the blood vessels, as in control. On the 14th and 21st day the majority of MC was situated in places of active fibrosing. They contained exclusively blue granules (the young granules with preponderance of biogenic amines), mixture of increased secretory function of the MC in the fibrotic lungs. Some of the MC granules showed fusion and altered matrix contents, other were emptied in piecemeal manner. A net of microtubules connected the granules was observed. Degranulation of MC may release heparin and cytokines able to stimulate synthesis of extracellular matrix. It has been suggested that heparin contributes to the fibrotic process and angiogenesis, stimulating directly or indirectly collagen synthesis by binding, stabilization and activation of fibroblast growth factor (FGF) and cell adhesion glycoproteins.

Animals↗

PubMed2026

Multi-omics approaches in idiopathic pulmonary fibrosis: from molecular mechanisms to therapeutic targets and precision medicine.

Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease with limited therapeutic options and marked molecular heterogeneity. Despite available antifibrotic therapies, disease progression remains poorly predictable, highlighting the need for improved mechanistic understanding and therapeutic targeting. This review summarizes recent advances in multi-omics research to elucidate the molecular mechanisms underlying IPF and to identify potential biomarkers and pharmacological targets. Multi-omics studies, including genomics, epigenomics, transcriptomics, proteomics, metabolomics, microbiome profiling, and single-cell sequencing, have revealed key pathogenic mechanisms in IPF. Genetic susceptibility factors such as MUC5B promoter variants and telomere-related genes contribute to disease risk. Epigenetic regulation, including DNA methylation, histone modifications, and non-coding RNAs, plays a central role in fibrotic remodeling. Transcriptomic and proteomic analyses have identified dysregulated signaling pathways, including TGF-β, mTOR, cellular senescence, and extracellular matrix remodeling. Metabolomic alterations indicate disrupted lipid and amino acid metabolism. Importantly, integration of multi-omics datasets enables the identification of molecular endotypes, candidate biomarkers, and potential therapeutic targets. However, challenges including data integration, tissue heterogeneity, limited cohort size, and the need for functional validation remain important barriers to clinical translation. Continued development of multi-omics approaches may facilitate more accurate disease classification and support the development of personalized therapeutic strategies for IPF.

biomarkers↗

PubMed2003

A study of the cough reflex in idiopathic pulmonary fibrosis.

Little is known about the pathogenesis of cough in idiopathic pulmonary fibrosis (IPF). We hypothesized that abnormalities of respiratory tract tachykinin-containing sensory nerves may be implicated. We studied cough response to capsaicin, substance P (SP), and bradykinin in 10 healthy control subjects and 10 patients with IPF. Six patients were tested before and after steroid therapy. Induced sputum cell counts and neurotrophic factor levels were also measured in 13 patients and 13 control subjects. The results show that cough sensitivity to capsaicin was greater in patients (p < 0.01). Neither SP nor bradykinin induced cough in normal subjects. SP and bradykinin induced cough in 7/10 patients (p < 0.002) and 2/10 patients (not significant) with IPF, respectively. Prednisolone caused a reduction in cough sensitivity to capsaicin (p < 0.05) and SP (p < 0.05) in all six patients treated. There were significantly more neutrophils (p = 0.001) and higher levels of nerve growth factor (p < 0.01) and brain-derived neurotrophic factor (p < 0.01) in patient's sputa. These findings suggest functional upregulation of lung sensory neurones in IPF. The cough response to inhaled SP in most patients may reflect disrupted respiratory epithelium. The response to corticosteroids demonstrates that the cough is amenable to therapy.

Aged↗

PubMed2006

[Pulmonary fibrosis--a therapeutic dilemma?].

The idiopathic interstitial pneumonias, especially the idiopathic pulmonary fibrosis (IPF), are life-threatening lung disorders, for which no effective treatment option exists. In view of IPF, the American Thoracic Society (ATS)/European Respiratory Society (ERS) consensus statement recommends a combined therapy with corticosteroids and azathioprine or cyclophosphamide, although data from conclusive clinical trials are yet missing and the recurrent clinical experience is that these drugs do not really help in IPF. Up to now, lung transplantation represents the last and only therapeutic option for IPF subjects. Based on new pathophysiological concepts of IPF, there are meanwhile a couple of different agents under preclinical and clinical assessment, and the increasing number of clinical trials ongoing in IPF raise the hope that an effective treatment comes into reach. The agents investigated and their targets are: acetylcysteine (reactive oxygen species [ROS] scavenging), interferon-gamma 1b (modulation of Th1/Th2 balance, direct antifibrotic effects), pirfenidone and GC 1008 (blockade of transforming growth factor-beta), FG 3019 (blockade of connective tissue growth factor), imatinib mesylate (blockade of platelet-derived growth factor), bosentan (blockade of endothelin), zileutin (blockade of leukotrienes), etanercept (blockade of tumor necrosis factor-alpha), heparin (alveolar anticoagulation). Hopefully, these new therapeutic strategies may help to improve prognosis of IPF in the future.

Acetylcysteine↗

PubMed1989

Respiratory muscle strength and gas exchange in neuromuscular diseases: comparison with chronic pulmonary emphysema and idiopathic pulmonary fibrosis.

To examine whether or not the respiratory muscle weakness is correlated with decrease in arterial oxygen tension (PaO2), respiratory muscle and pulmonary functions in 14 patients with neuromuscular diseases (NMD) were studied and compared with those of 12 patients with chronic pulmonary emphysema (CPE) and 15 patients with idiopathic pulmonary fibrosis (IPF). Respiratory muscle strength was assessed by maximal static inspiratory and expiratory mouth pressure at three lung volumes (RV, FRC and TLC). Although mean pulmonary functions in NMD showed virtually normal function, respiratory muscle strength was significantly less than the corresponding values in CPE and IPF. In NMD, maximal inspiratory mouth pressure at RV level (PImax) correlated positively with %TLC and %VC (r = 0.652 and r = 0.536, respectively). Moreover, PImax was significantly correlated with PaO2 (r = 0.561), but not with PaCO2. Maximal expiratory mouth pressure at TLC (PEmax) correlated positively with %TLC and %VC. In CPE and IPF, respiratory muscle strength had no correlation with PaO2 and PaCO2. These findings suggest that inspiratory muscle dysfunction in NMD may be one of the factors responsible for determination of the level of hypoxemia and lung volume.

Adult↗

PubMed2001

Evidence of type II pneumocyte apoptosis in the pathogenesis of idiopathic pulmonary fibrosis (IFP)/usual interstitial pneumonia (UIP).

BACKGROUND/AIMS: The pathogenesis of idiopathic pulmonary fibrosis (IPF)/usual interstitial pneumonia (UIP), a chronic and incurable human respiratory disease, is not well established. This study was designed to investigate whether the apoptosis of type II pneumocytes could be the precipitating factor in the pathogenesis of IPF. METHODS: Nineteen specimens obtained by retrospective review of the medical and pathological records of 55 patients with IPF, four normal subjects, and 10 disease control lungs were analysed. The selected specimens had normal alveoli with intervening patchy scarring of the lung parenchyma, fulfilling the pathological criteria for UIP. To identify individual cells undergoing apoptosis in the normal alveoli, electron microscopy and in situ end labelling of fragmented DNA were performed on paraffin was embedded sections using digoxigenin-11-dUTP and the enzyme terminal deoxynucleotidyl transferase. RESULTS: Apoptosis was detected in the normal alveoli of 17 of the 19 patients with IPF/UIP and was absent in the controls. Electron microscopy demonstrated apoptotic changes in type II pneumocytes. These results indicate that apoptotic type II pneumocyte death occurs in normal alveoli of IPF/UIP and could be the principal cause of several events that account for the histological, clinical, and functional alterations seen in IPF/UIP. CONCLUSIONS: In conclusion, numerous type II pneumocytes from the normal alveoli of most patients with IPF/UIP actively undergo programmed cell death. This finding may shed new light on the pathogenesis of this disease, with implications mainly for the treatment of affected patients.

Aged↗

PubMed2005

Idiopathic pulmonary fibrosis: how often is it really idiopathic?

The cause of idiopathic pulmonary fibrosis (IPF) is unknown. The pathology suggests that IPF results from serial lung injury. It has been suggested that gastroesophageal reflux disease (GERD) may relate to the cause or the progression of the disease. The aims of this study were to determine the prevalence of GERD, the clinical presentation of GERD, and the manometric and reflux profiles in patients with end-stage IPF. Between July 2003 and October 2004, 18 patients with IPF on the lung transplant waiting list were referred for evaluation to the Swallowing Center of the University of California San Francisco. On the basis of the results of the pH monitoring test (5 and 20 cm above the lower esophageal sphincter), the patients were divided into two groups: group A, 12 patients (66%), GERD+; group B, 6 patients (34%), GERD-. The incidence of heartburn and regurgitation was similar between GERD+ and GERD- patients; reflux was clinically silent in one third of GERD+ patients. Reflux was associated with a hypotensive lower esophageal sphincter and abnormal esophageal peristalsis, and it was present in the upright and supine position. The reflux often extended into the proximal esophagus. These results show the following: (1) Two thirds of patients with IPF had GERD; (2) symptoms could not distinguish between those with and without GERD; (3) reflux occurred in the presence of a hypotensive lower esophageal sphincter and abnormal esophageal peristalsis; and (4) reflux occurred in the upright and supine positions, and often extended into the proximal esophagus. We conclude that patients with IPF should be screened for GERD, and if GERD is present, a fundoplication should be performed before or shortly after lung transplantation.

Chi-Square Distribution↗