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PubMedDate not supplied

Noncardiogenic pulmonary edema and pulmonary fibrosis in falciparum malaria.

Noncardiogenic pulmonary edema is an uncommon but serious complication of falciparum malaria. A case of fatal noncardiogenic pulmonary edema complicating falciparum malaria is presented in which the unfavorable outcome resulted from rapidly developing pulmonary fibrosis, documented through open-lung biopsy. Possible mechanisms of lung injury in falciparum malaria include: impaired perfusion and tissue hypoxia in the pulmonary microcirculation caused by reduced effective circulating volume; abnormal autonomic effects on the lung resulting from reduced blood flow in the central nervous system; immunologic injury to alveolar-capillary structures; and morphologic changes in the surface membranes of infected erythrocytes leading to sequestration of parasitized erythrocytes in vascular beds and pulmonary capillary damage. That pulmonary involvement in falciparum malaria is usually associated with high-grade parasitemia, concurrent cerebral involvement, and delay in institution of antimalarial therapy emphasizes the importance of early diagnosis and treatment.

Biopsy↗

PubMed1989

An immunohistochemical study of architectural remodeling and connective tissue synthesis in pulmonary fibrosis.

Fibroblasts in healthy adult lung are quiescent, synthesizing little collagen. We studied lung biopsies from 30 patients with pulmonary fibrosis, using immunohistochemistry with monoclonal antibodies against the propeptides of type I collagen to localize fibroblasts actively synthesizing collagen. Adjacent sections were stained with antibodies to type III and IV collagen, fibrin, cytokeratin, plasma fibronectin, or EDIIIa-containing "cellular" fibronectin (cFN). In rapid pulmonary fibrosis, including the proliferative phase of diffuse alveolar damage, organizing pneumonia, and subacute idiopathic fibrosis, collagen-synthesizing cells were numerous in organizing exudate filling airspaces but were also seen in the interstitium of the alveolar walls, interlobular septa, and walls of blood vessels. The new matrix deposited in the airspaces also contained type III collagen and EDIIIa-containing fibronectin. In chronic pulmonary fibrosis, more than half of the biopsies showed foci of collagen synthesis and cFN deposition near the air-tissue interface. The foci were consistently localized outside remnants of basal lamina and therefore within airspaces. The results indicate that (1) fibrosis in chronic idiopathic pulmonary fibrosis results mainly from organization of exudate within airspaces, just as it does after acute lung injury, and (2) during this process, fibroblasts increase their synthesis of collagen and fibronectin coordinately. Foci of active matrix deposition provide evidence for the progressive nature of chronic pulmonary fibrosis.

Antibodies, Monoclonal↗

PubMed1991

Scanning electron microscopic analysis of mineral fiber content of lung tissue in the evaluation of diffuse pulmonary fibrosis.

The mineral fiber content of lung parenchyma in 24 cases of diffuse pulmonary fibrosis of unknown cause was determined by scanning electron microscopy and compared with that of 36 autopsy cases of histologically confirmed asbestosis and 20 autopsy cases of patients with normal lungs. Fibers were isolated from the lung using a hypochlorite digestion technique and collected on the surface of a polycarbonate filter. In addition, the types of fibers present (asbestos vs. other mineral fibers) were determined by energy dispersive x-ray analysis (EDXA). When the histologic grade of fibrosis in the cases of asbestosis was compared with the uncoated fiber content by means of linear regression analysis, it was determined that the fiber content of the 24 cases of diffuse pulmonary fibrosis of unknown cause was below the 95% confidence limit for asbestosis in every instance. Furthermore, the majority of fibers analyzed by EDXA were not asbestos in the cases with diffuse pulmonary fibrosis of unknown cause, whereas more than 90% of the fibers from the asbestosis cases were commercial amphiboles (amosite or crocidolite). It was concluded that most patients with advanced pulmonary fibrosis whose tissue samples do not meet histologic criteria for asbestosis do not have asbestos-induced fibrosis, even though there may be some history of exposure to asbestos. In such cases, scanning electron microscopic analysis of mineral fiber content and EDXA of the types of fibers present often provide useful information with regard to the correct classification of these cases.

Adult↗

PubMed2003

Transforming growth factor-beta1 gene polymorphisms are associated with disease progression in idiopathic pulmonary fibrosis.

Transforming growth factor-beta1 (TGF-beta1) is a cytokine that plays a key role in the development of idiopathic pulmonary fibrosis. There have been reports on the presence of two genetic polymorphisms in the DNA sequence encoding the leader sequence of the TGF-beta1 protein, located in codons 10 and 25. The objective of this study was to investigate the association between TGF-beta1 gene polymorphisms in codons 10 and 25 and the susceptibility to idiopathic pulmonary fibrosis and the progression of the disease. Compared with healthy control subjects (n = 140), patients with idiopathic pulmonary fibrosis (n = 128) showed no significant deviations in genotype or allele frequencies. One hundred and ten patients with idiopathic pulmonary fibrosis were followed up for 30.3 +/- 25 months. The presence of a proline allele at codon 10 was independently associated with a significant increase in alveolar arterial oxygen tension difference during follow-up, after controlling for the effect of treatment (coefficient = 0.59; 95% confidence intervals, 0.23 to 0.96; p = 0.002). These findings suggest that (1) TGF-beta1 gene polymorphisms in codons 10 and 25 do not predispose to the development of idiopathic pulmonary fibrosis; and (2) TGF-beta1 gene polymorphisms may affect disease progression in patients with idiopathic pulmonary fibrosis.

Adult↗

PubMed2006

Idiopathic pulmonary fibrosis: natural history and prognosis.

Idiopathic pulmonary fibrosis (IPF) is associated with a poor prognosis, although considerable interpatient heterogeneity may be observed. Baseline dyspnea, lung function abnormalities, and gas exchange impairments are predictive of outcome, but recent evidence suggests the change in these parameters over time is as predictive, and in some cases more predictive, of outcome. A change in forced vital capacity > or =10% may be an important surrogate marker for mortality risk in IPF patients. Histologic analysis shows the deposition of interstitial connective tissue and granulation tissue leading to formation of fibroblastic foci. Thus, ongoing epithelial damage and repair processes associated with persistent fibroblastic proliferation seem important in IPF natural history.

Humans↗

PubMed2003

[Outcomes and clinical characteristics of patients with pulmonary fibrosis and respiratory failure admitted to an intensive care unit. A study of 20 cases].

BACKGROUND AND OBJECTIVE: Previous reports on the outcome of patients with pulmonary fibrosis admitted to the intensive care unit (ICU) suggest a bad prognosis. The aim of our study was to evaluate the course and prognosis of patients with pulmonary fibrosis admitted to the ICU of our hospital because of acute respiratory failure. PATIENTS AND METHOD: Retrospective, case-series, observational study. We evaluated the clinical records of patients with pulmonary fibrosis referred to the intensive care unit in a tertiary university teaching hospital between January 1986 and June 2002. Complete information on the diagnosis and clinical course of the pulmonary disease, pulmonary function tests, current clinical status, ventilatory support and adjunctive therapies applied in the ICU, length of stay and mortality was collected. RESULTS: Twenty patients were included, 14 with idiopathic pulmonary fibrosis and 6 with pulmonary fibrosis and associated collagen vascular diseases. The mean (SD) interval between the diagnosis of fibrosis and the admission to ICU was 14 (20) months. All patients presented with severe acute respiratory failure (PaO2/FiO2 < 200). The cause of clinical worsening was identified in 8 (40%) cases (5 bacterial and 3 fungal infections). In the ICU, 17 patients required mecanical ventilation. All patients worsened progressively, with refractory hypoxemic respiratory failure (100%) and hemodynamic instability after endotraqueal intubation (70%). Mechanical ventilation was associated with a 100% mortality. CONCLUSION: An identifiable cause of acute respiratory failure could not be found in a significant proportion of patients despite a systematic work-up. Mechanical ventilation and aggressive life support measures do not seem to provide any further benefit in patients with pulmonary fibrosis and severe respiratory failure.

Aged↗

PubMed1977

Small airways in idiopathic pulmonary fibrosis. Comparison of morphologic and physiologic observations.

18 patients with idiopathic pulmonary fibrosis were studied to determine if they had morphologic evidence of small airways disease and if physiologic testing could predict morphologic findings. In the presence of normal airway function by standard physiologic studies (forced expiratory volume in 1 s/forced vital capacity and airway resistance by plethysmography), dynamic compliance, maximum expiratory flow-volume curves, and maximum flowstatic recoil curves were measured to detect physiologic alterations consistent with small airways abnormalities. These physiologic data were then compared with estimates of small airways diameter made in lung biopsy specimens.94% (17 of 18) of the patients had peribronchiolar fibrosis or peribronchiolar inflammation or bronchiolitis. 67% (12 of 18) had an overall estimate of small airways diameter of "narrowed," whereas 33% (6 of 18) had airways that overall were "not narrowed." 59% (10 of 17) had frequency-dependent dynamic compliance, 50% (9 of 18) had abnormal maximum expiratory flow-volume curves, and 39% (7 of 18) had abnormal maximum flow-static recoil curves. Comparisons between morphologic and physiologic data revealed a significant correlation between the results of dynamic compliance and the overall estimate of small airways diameter (P = 0.001), and the results of maximum flow-volume curves and the overall estimate of small airways diameter (P = 0.009); there was no significant correlation between the results of maximum flow-static recoil curves and the overall estimate of small airways diameter (P = 0.1). THE RESULTS OF THIS STUDY SUGGEST THAT: (a) idiopathic pulmonary fibrosis is a disease of small airways as well as alveoli; (b) dynamic compliance and the maximum expiratory flow-volume curve can predict the overall status of small airways diameter in idiopathic pulmonary fibrosis; and (c) whereas the maximum flowstatic recoil curve predicts the overall estimate of small airways diameter in most patients with this disease, it is the least sensitive of these three monitors of small airways.

Adult↗

PubMed1996

alpha 1-antitrypsin deficiency and idiopathic pulmonary fibrosis in a family.

Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease of unknown etiology with an estimated prevalence of 3 to 5 per 100,000. We describe a patient with a family history of both IPF and alpha 1-antitrypsin (AAT) deficiency. The patient had a 6-month history of worsening dyspnea when first seen, and he later died of multisystem organ failure and recalcitrant hypoxemia. He had IPF and AAT deficiency, with the phenotype PiSZ. His father and younger brother died of respiratory failure due to IPF. Other family members are heterozygous for AAT deficiency. A variety of inflammatory diseases have been associated with AAT deficiency, and an association between heterozygous AAT deficiency and IPF has been reported in the literature. Most current theories on the pathogenesis of IPF suggest an aberrant inflammatory response to some stimulus. This family cluster and another reported in the literature suggest that AAT deficiency may be a factor predisposing to inflammatory lung disease manifested by interstitial fibrosis.

Adult↗

PubMed1986

[Comparative profile of antinuclear antibodies in Gougerot-Sjögren syndrome with and without diffuse interstitial pulmonary fibrosis].

Eleven patients with an isolated Gougerot-Sjögren syndrome and a diffuse interstitial fibrosis were compared with twenty patients with an isolated Gougerot-Sjögen without pulmonary involvement. Patients with pulmonary fibrosis are younger and the evolution of their dry syndrome is shorter (p less than 0.05) than in patients without fibrosis. The frequency of extra-articular clinical manifestations (except for the lung) is identical in both groups. Antinuclear antibodies are present in 100 p. cent of patients with pulmonary fibrosis. Specific antibodies of soluble nuclear antigens are detected in 64 p. cent of them. This frequency is 55 p. cent in the group without pulmonary fibrosis. The specificities of these antibodies are anti-U1-RNP (3 cases), anti-SS-B (La) (3 cases), anti-SS-A (Ro) (2 cases), non identified (1 case). There was no serum containing antibodies Jo1 or anti-Sm. This immunological profile is identical to the profile found in isolated Gougerot-Sjögren syndromes without pulmonary fibrosis. The search for specific antibodies of soluble nuclear antigens permits to differentiate pulmonary fibrosis secondary to an isolated Gougerot-Sjögren syndrome, from primary diffuse interstitial fibrosis and fibrosis associated to a polymyositis.

Adult↗

PubMed1995

Approaches to the treatment of pulmonary fibrosis.

There was a general consensus at the workshop that pulmonary fibrosis is a highly lethal lung disorder and that current therapies for this disease have little effect on the natural history of the disease. There was also broad consensus that much has been learned about the pathogenesis of this disease in recent years and this information can be used to direct new therapies for this disease. As a complement to the findings from studies on pulmonary fibrosis itself, there has also been significant development of new therapies for other disorders that might be useful in treating patients with pulmonary fibrosis. The following is a list of some of the issues and recommendations that were generated from this workshop.

Anti-Inflammatory Agents↗

PubMed1990

Idiopathic pulmonary fibrosis. A rare cause of scintigraphic ventilation-perfusion mismatch.

A case of idiopathic pulmonary fibrosis with multiple areas of mismatch on ventilation-perfusion lung imaging in the absence of pulmonary embolism is presented. Idiopathic pulmonary fibrosis is one of the few nonembolic diseases producing a pulmonary ventilation-perfusion mismatch. In this condition, chest radiographs may not detect the full extent of disease, and xenon-133 ventilation imaging may be relatively insensitive to morbid changes in small airways. Thus, when examining patients with idiopathic pulmonary fibrosis, one should be aware that abnormal perfusion imaging patterns without matching ventilation abnormalities are not always due to embolism. In this setting, contrast pulmonary angiography is often needed for accurate differential diagnosis.

Diagnosis, Differential↗

PubMed2006

Evolving concepts in the early and accurate diagnosis of idiopathic pulmonary fibrosis.

The diagnosis of idiopathic pulmonary fibrosis (IPF) requires an integrated multidisciplinary approach involving pulmonologists, radiologists, and pathologists. Early recognition of IPF and of its differentiation from other interstitial lung diseases is important for directing patient management and predicting prognosis. Clinical suspicion of IPF should be raised when patients, particularly those over 50 years of age, present with dyspnea that is gradual in onset and progressive. A confident diagnosis of IPF can be made based on clinical assessment, pulmonary function test results, and radiographic and bronchoscopic evaluation. However, the definitive diagnosis of IPF requires a surgical lung biopsy to confirm the histopathologic pattern of usual interstitial pneumonia.

Diagnosis, Differential↗

PubMed2006

Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features.

Although acute exacerbations of idiopathic pulmonary fibrosis are well recognised, there are no studies documenting their prevalence or identifying pre-existing risk factors. This study analysed the clinical, radiological and pathological data of 11 patients who satisfied the criteria for acute exacerbation among 147 patients with biopsy-proven idiopathic pulmonary fibrosis. There were five additional patients who had similar demographics, radiology and surgical lung biopsy pathology, but had clinically less severe disease, and so were not included. The 2-yr frequency of acute exacerbation was 9.6% after the diagnosis. Most exacerbations were idiopathic, although two cases presented after surgical lung biopsy and one after bronchoalveolar lavage. No significant risk factor was found by univariate proportional hazard analysis. Imaging revealed diffuse bilateral ground-glass opacification superimposed on subpleural reticular and honeycombing densities. The biopsies of four patients taken during acute exacerbation exhibited diffuse alveolar damage superimposed upon usual interstitial pneumonia. The findings of this study demonstrate that acute exacerbation of idiopathic pulmonary fibrosis is rather common and this exacerbation is likely to have a spectrum of severity.

Acute Disease↗

PubMed1986

Early cellular events in pulmonary fibrosis.

In this review we have surveyed recent investigations of early cellular events in pulmonary fibrosis both in animal models and in human diseases. Analysis of the interactions of the numerous cell types in the lung following injury is an almost overwhelmingly complex enterprise. In the animal models experimental design has a profound effect on results, making it difficult to compare studies when species, fibrogenic agent, dose, route of exposure, schedule of administration, time course, and analytical methods may not be equivalent. In human diseases we are rarely able to obtain data at precisely the same time point in the course of the disease even among patients in the same study, and possible confounding variables present are legion. Transcending these difficulties for the moment, can we draw any conclusions from our current knowledge of early cellular interactions in pulmonary fibrosis? What is striking is not that there are so many agents that can potentially induce pulmonary fibrosis, but that the lung has such capabilities for recovery. Although the major effector cells may all initially participate in damaging the lung and initiating fibrosis, there is evidence that they may also have the capacity to participate in subsequent repair. Macrophages may initially recruit fibroblasts and stimulate them to proliferate, only to suppress them subsequently. Macrophage production of prostaglandins can lead to suppression of macrophage, neutrophil and lymphocyte responses, thus attenuating tissue injury and the development of fibrosis. Neutrophils may initially release toxic metabolites and enzymes that damage parenchyma. However, there is evidence that they may later play a role in attenuating fibrosis, perhaps through collagenase secretion, or through as yet unknown mechanisms. Lymphocytes may initially participate in a number of damaging ways by secreting chemoattractants for other cells and participating in destructive autoimmune processes. However, there is evidence that subpopulations of T cells may dramatically shift during the course of fibrosis, leading to attenuation of the process. It may thus be useful to consider irreversible pulmonary fibrosis as the end result of a process in which the balance of normal injury/repair mechanisms is disrupted. There is clearly no single "fibrogenic event." Rather, there seem to be a number of places where disruption of balance/repair processes may begin. In diseases of unknown etiology such as sarcoidosis or IPF, loss of control may occur at the genetic level, leading to the destructive alveolitis that is the apparent precursor of fibrosis.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗

PubMed2005

[Pneumology. Treatment of idiopathic pulmonary fibrosis: hopes and disappointment].

Idiopathic pulmonary fibrosis (IPF) is now recognized as a separate nosological entity. Despite the progresses in understanding the basic mechanisms of the disease, its prognosis remains poor. The classical treatment combines prednisone with a cytotoxic agent. Interferon gamma has the in vitro capacity of inhibiting fibroblasts proliferation. A pilot study showed positive results, but a more recent randomized double blind trial was unable to demonstrate a clear benefit to the patients. On the other hand there are many evidences for an oxydant-antioxydant imbalance in the pathogenesis of IPF. In a human controlled study N-acetylcysteine (NAC) at high doses (1800 mg per day orally) improved the pulmonary function tests when given on top of a combined therapy with prednisone and azathioprine.

Humans↗

PubMed2003

Idiopathic pulmonary fibrosis in a child.

Idiopathic pulmonary fibrosis (IPF) is a rare disease of unknown etiology and is usually associated with a poor prognosis. Up to the present, less than 50 cases of IPF in children have been reported in the English literature, and no case has ever been reported from Taiwan. Herein we report on a 2-year-old boy with IPF presenting with a rapid onset of dyspnea followed by respiratory failure. The diagnosis of IPF was verified with an open lung biopsy. Despite intravenous methylprednisolone pulse therapy and empiric nitric oxide treatment, he expired on the 35th day after admission due to profound hypoxemia. A diagnosis of IPF should be included in the differential diagnosis for patients presenting with unexplained shortness of breath and pulmonary interstitial infiltrations.

Adrenal Cortex Hormones↗

PubMed2006

Regulatory role of gammadelta T cells in the recruitment of CD4+ and CD8+ T cells to lung and subsequent pulmonary fibrosis.

The mechanisms by which T cells accumulate in the lungs of patients with pulmonary fibrosis are poorly understood. Because the lung is continually exposed to microbial agents from the environment, we repeatedly exposed C57BL/6 mice to the ubiquitous microorganism, Bacillus subtilis, to determine whether chronic exposure to an inhaled microorganism could lead to T cell accumulation in the lungs and subsequent pulmonary fibrosis. C57BL/6 mice repeatedly treated with B. subtilis for 4 consecutive weeks developed a 33-fold increase in the number of CD4+ T cells and a 354-fold increase in gammadelta T cells in the lung. The gammadelta T cells consisted almost entirely of Vgamma6/Vdelta1+ cells, a murine subset bearing an invariant TCR the function of which is still unknown. Treatment of C57BL/6 mice with heat-killed vs live B. subtilis resulted in a 2-fold increase in the number of CD4+ T cells in the lung but no expansion of gammadelta T cells indicating that gammadelta cells accumulate in response to live microorganisms. In addition, mice treated with heat-killed B. subtilis developed significantly increased pulmonary fibrosis compared with mice treated with the live microorganism. Mice deficient in Vgamma6/Vdelta1+ T cells when treated with B. subtilis had a 231-fold increase in lung CD4+ T cells and significantly increased collagen deposition compared with wild-type C57BL/6 mice, consistent with an immunoregulatory role for the Vgamma6/Vdelta1 T cell subset. These findings indicate that chronic inhalation of B. subtilis can result in T cell accumulation in the lung and fibrosis, constituting a new model of immune-mediated pulmonary fibrosis.

Animals↗

PubMed2000

Four cases of anti-myeloperoxidase antibody-related rapidly progressive glomerulonephritis during the course of idiopathic pulmonary fibrosis.

We report here 4 cases of rapidly progressive glomerulonephritis (RPGN) which developed during the management of idiopathic pulmonary fibrosis. In each patient, pulmonary disease preceded the onset of nephritis by 1 to 6 years. All patients had a high titer of serum autoantibodies against myeloperoxidase (MPO-ANCA) when the diagnosis of RPGN was made. Although the association of pulmonary fibrosis with ANCA-related glomerulonephritis has been occasionally described in the past literature, the sequence of pulmonary and renal injury has not been well defined. The present report demonstrates that idiopathic pulmonary fibrosis exists as a preceding condition in some patients with MPO-ANCA-related nephritis.

Aged↗