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Pulmonary Fibrosis (pulmonary + fibrosis)

Distribution by Scientific Domains

Medical Sciences	95%
Life Sciences	4%

Distribution within Medical Sciences

Respiratory Medicine	15%
Pathology	10%
Immunology	7%
Pharmacology & Pharmaceutical Medicine	5%
Allergy & Clinical Immunology	4%
Dermatology	3%
13 Other Subdomains	56%

Kinds of Pulmonary Fibrosis

bleomycin-induced pulmonary fibrosis

idiopathic pulmonary fibrosis

Selected Abstracts

Gastroesophageal Reflux Disease and Idiopathic Pulmonary Fibrosis

MOUNT SINAI JOURNAL OF MEDICINE: A JOURNAL OF PERSONALIZED AND TRANSLATIONAL MEDICINE, Issue 1 2009
Yevgenia Y. Pashinsky MD
Abstract Gastroesophageal reflux disease occurs with a higher prevalence in patients with idiopathic pulmonary fibrosis than in matched controls. Silent reflux occurs in about a third of patients with significant gastroesophageal reflux disease; thus, objective measurements are required to evaluate gastroesophageal reflux disease in patients with advanced lung diseases. We provide here a detailed description of acid and non-acid reflux and the diagnostic evaluation for pulmonologists and lung transplant surgeons suspecting reflux as a contributing factor in advanced lung diseases. We review the evidence for gastroesophageal reflux disease causing idiopathic pulmonary fibrosis and other select pulmonary diseases and the potential role of antireflux surgery in the management of advanced lung disease and transplant patients. Mt Sinai J Med 76:24,29, © 2009 Mount Sinai School of Medicine [source]

"End-stage" Pulmonary Fibrosis in Sarcoidosis

MOUNT SINAI JOURNAL OF MEDICINE: A JOURNAL OF PERSONALIZED AND TRANSLATIONAL MEDICINE, Issue 1 2009
Alvin T. Teirstein MD
Abstract Pulmonary fibrosis is an unusual "end stage" in patients with sarcoidosis. Fibrosis occurs in a minority of patients, and presents with a unique physiologic combination of airways dysfunction (obstruction) superimposed on the more common restrictive dysfunction. Imagin techniques are essential to the diagnosis, assessment and treatment of pulmonary fibrosis. Standard chest radiographs and CT scans may reveal streaks, bullae, cephalad retraction of the hilar areas, deviation of the trachea and tented diaphragm. Positive gallium and PET scans indicate residual reversible granulomatous disease and are important guides to therapy decisions. Treatment, usually with corticosteroids, is effective in those patients with positive scans, but fibrosis does not improve with any treatment. With severe functional impariment and patient disability, pulmonary hypertension and right heart failure may supervene for which the patient will require treatment. Oxygen, careful diuresis, sildenafil and bosentan may be salutary. These patients are candidates for lung transplantation. Mt Sinai J Med 76:30,36, © 2009 Mount Sinai School of Medicine [source]

An Ill Wind-Iatrogenic Air Embolus Around Pacing Leads During Defibrillator Implant with Coexisting Pulmonary Fibrosis

PACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 2 2006
DANIEL M. NINIO
We present a case of air embolism during biventricular defibrillator implantation in a 58-year-old man with pulmonary fibrosis. To prevent air entry after the dilator was removed, the subclavian venous sheath was covered before the lead was introduced. Air embolism occurred during inspiration around the lead body in the appropriate sized sheath but without a hemostatic valve. Extraordinary swings in intrathoracic pressure due to noncompliant lungs may have contributed to this unusual complication. [source]

Pulmonary response to airway instillation of autologous blood in horses

EQUINE VETERINARY JOURNAL, Issue 4 2007
F. J. DERKSEN
Summary Reasons for performing study: Exercise-induced pulmonary haemorrhage (EIPH) occurs in the majority of horses performing strenuous exercise. Associated pulmonary lesions include alveolar and airway wall fibrosis, which may enhance the severity of EIPH. Further work is required to understand the pulmonary response to blood in the equine airways. Objectives: To confirm that a single instillation of autologous blood into horse airways is associated with alveolar wall fibrosis, and to determine if blood in the airways is also associated with peribronchiolar fibrosis. Methods: Paired regions of each lung were inoculated with blood or saline at 14 and 7 days, and 48, 24 and 6 h before euthanasia. Resulting lesions were described histologically and alveolar and airway wall collagen was quantified. Results: The main lesion observed on histology was hypertrophy and hyperplasia of type II pneumocytes at 7 days after blood instillation. This lesion was no longer present at 14 days. There were no significant effects of lung region, treatment (saline or autologous blood instillation), nor significant treatment-time interactions in the amount of collagen in the interstitium or in the peribronchial regions. Conclusion: A single instillation of autologous blood in lung regions is not associated with pulmonary fibrosis. Potential relevance: Pulmonary fibrosis and lung remodelling, characteristic of EIPH, are important because these lesions may enhance the severity of bleeding during exercise. A single instillation of autologous blood in the airspaces of the lung is not associated with pulmonary fibrosis. Therefore the pulmonary fibrosis described in EIPH must have other causes, such as repetitive bleeds, or the presence of blood in the pulmonary interstitium in addition to the airspaces. Prevention of pulmonary fibrosis through therapeutic intervention requires a better understanding of these mechanisms. [source]

"End-stage" Pulmonary Fibrosis in Sarcoidosis

Pulmonary fibrosis: Cellular and molecular events

PATHOLOGY INTERNATIONAL, Issue 3 2003
Mohammed S. Razzaque
Connective tissue remodeling of the interstitium is an important feature of chronic lung diseases encompassing interstitial inflammatory changes and subsequent pulmonary fibrosis. The early inflammatory phase is usually associated with the release of several cytokines and chemokines by activated resident cells and infiltrating cells which, in turn, help further recruit inflammatory mononuclear cells. Cytokines and growth factors secreted by inflammatory cells and by interstitial cells (fibroblasts and myofibroblasts) play an important role in the fibrogenic phase of pulmonary fibrosis by inducing matrix synthesis. In addition, matrix-degrading enzymes and their inhibitors also contribute to extracellular matrix (ECM) remodeling in pulmonary fibrosis. This review addresses the pathophysiology of wound healing and different phases of pulmonary fibrosis. [source]

Preventive effect of hochu-ekki-to, a Japanese herbal medicine, on bleomycin-induced lung injury in mice

RESPIROLOGY, Issue 6 2007
Shunji TAJIMA
Objective: Pulmonary fibrosis is thought to be closely associated with the T-helper type-2 (Th2) immune response. Recent studies have shown that hochu-ekki-to (TJ-41), a Japanese herbal medicine, may correct the Th1/Th2 imbalance skewed to Th2. The present study was designed to investigate the preventive effect of TJ-41 on the development of bleomycin (BLM)-induced lung injury in mice. Methods: Female C57BL/6 mice were divided into a group given ordinary feed and another group given the same feed plus TJ-41 mixed in at a dose of 1 g/kg/day. Both groups were maintained on this diet for 8 weeks before and 5 weeks after administration of 2 mg/kg BLM intratracheally. Results: Mortality after BLM-induced lung injury was significantly lower in the TJ-41-treated mice. The hydroxyproline content and fluid content in the lung on day 35 was significantly lower in the TJ-41-treated mice. Histologically, TJ-41 reduced the number of infiltrating cells, thus ameliorating the destruction of the lung architecture, and attenuated the lung fibrosis score. Furthermore, TJ-41 inhibited the expression of the interleukin-5/interferon-, mRNA ratio in the lung on day 7. Conclusions: Treatment with TJ-41 partially prevented experimental lung fibrosis through the correction of the Th1/Th2 imbalance skewed to Th2. [source]

Pulmonary fibrosis in myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitides

RESPIROLOGY, Issue 2 2004
Sakae HOMMA
Objective: The association of pulmonary fibrosis (PF) with myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitides has not been well documented. The aim of this study was to assess the clinicopathological characteristics of PF in patients who tested positive for MPO-ANCA. Methodology: In this study, 31 patients (17 males and 14 females; mean age, 69 years) diagnosed as having PF with positive MPO-ANCA levels ranging from 10 to 840 EU with a mean of 112.5 EU, were evaluated clinicopathologically. Results: Among 31 patients with PF, 22 had underlying systemic diseases such as collagen vascular diseases, while nine had unknown aetiology. Evidence of glomerulonephritis was demonstrated in 14 patients. The clinical features were a history of dry cough and/or fine crackles in all 31 patients. Chest CT scans showed honeycombing in the lung bases in 26 patients. The histopathological features of the diseased lung tissues in all 11 autopsied cases were compatible with the usual interstitial pneumonia (UIP) pattern. Vasculitis was confirmed in bronchial arteries and/or pulmonary arterioles in five patients. The mortality was as high as 13 of the 31 patients. The causes of death were: deterioration of PF in five (two of whom were associated with pulmonary haemorrhage), lung cancer in two, pneumonia in four, and digestive tract bleeding in two. The survival rates in PF with MPO-ANCA-negative collagen vascular diseases, cryptogenic fibrosing alveolitis (CFA), and PF with positive MPO-ANCA, were compared. The 5-year survival rate in PF with positive MPO-ANCA was worse than in PF with MPO-ANCA-negative collagen vascular diseases and was the same for CFA. Conclusion: Although there was no correlation between MPO-ANCA titres and the activity of PF, this study demonstrated that the presence of positive MPO-ANCA was an unfavorable prognostic factor in patients with PF. [source]

C-C chemokine receptor 2 (CCR2) deficiency improves bleomycin-induced pulmonary fibrosis by attenuation of both macrophage infiltration and production of macrophage-derived matrix metalloproteinases

THE JOURNAL OF PATHOLOGY, Issue 5 2004
Toshiyuki Okuma
Abstract Macrophage infiltration is implicated in various types of pulmonary fibrosis. One important pathogenetic process associated with pulmonary fibrosis is injury to basement membranes by matrix metalloproteinases (MMPs) that are produced mainly by macrophages. In this study, C-C chemokine receptor 2-deficient (CCR2,/,) mice were used to explore the relationship between macrophage infiltration and MMP activity in the pathogenesis of pulmonary fibrosis, using the bleomycin-induced model of this disease process. CCR2 is the main (if not only) receptor for monocyte chemoattractant protein-1/C-C chemokine ligand 2 (MCP-1/CCL2), which is a critical mediator of macrophage trafficking, and CCR2 ,/, mice demonstrate defective macrophage migration. Pulmonary fibrosis was induced in CCR2,/, and wild-type (CCR2+/+) mice by intratracheal instillation of bleomycin. No significant differences in the total protein concentration in bronchoalveolar lavage (BAL) fluid, or in the degree of histological lung inflammation, were observed in the two groups until day 7. Between days 3 and 21, however, BAL fluid from CCR2,/, mice contained fewer macrophages than BAL fluid from CCR2+/+ mice. Gelatin zymography of BAL fluid and in situ zymography revealed reduced gelatinolytic activity in CCR2,/, mice. Immunocytochemical staining showed weaker expression of MMP-2 and MMP-9 in macrophages in BAL fluid from CCR2,/, mice at day 3. Gelatin zymography of protein extracted from alveolar macrophages showed reduced gelatinolytic activity of MMP-2 and MMP-9 in CCR2,/, mice. At days 14 and 21, lung remodelling and the hydroxyproline content of lung tissues were significantly reduced in CCR2,/, mice. These results suggest that the CCL2/CCR2 functional pathway is involved in the pathogenesis of bleomycin-induced pulmonary fibrosis and that CCR2 deficiency may improve the outcome of this disease by regulating macrophage infiltration and macrophage-derived MMP-2 and MMP-9 production. Copyright © 2004 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. [source]

BAL in the diagnosis of smoking-related interstitial lung diseases: Review of literature and analysis of our experience

DIAGNOSTIC CYTOPATHOLOGY, Issue 12 2008
Joanna Domaga, Ph.D., a-Kulawik M.D.
Abstract The group of interstitial lung diseases (ILDs) is formed by respiratory tract disorders, whose aetiology is unknown in the majority of cases, the clinical course differs and the prognosis is generally serious. Some of the ILDs have a potential relation to tobacco smoking and are known as smoking-related ILDs (sr-ILD). Bronchoalveolar lavage fluid (BALF) examination is one of the initial procedures in the diagnosis of ILD. Despite the fact that histological confirmation is the gold standard in ILD diagnosis in many studies, the number of reported biopsies was low. In this review we present the results of BALF examinations of patients with sr-ILD and discuss their value in the differential diagnosis with other types of ILD. An extremely high total cell count (about 50 × 106 cells) with significant predominance of pigmented alveolar macrophages is a characteristic pattern of BALF in sr-ILD. The greatest challenge in BALF cytology interpretation is to distinguish sr-ILD and idiopathic pulmonary fibrosis (IPF). IPF is characterised by an elevated proportion and absolute count of lymphocytes and neutrophils; in addition, BALF lymphocytosis is higher in non-specific interstitial pneumonia than in usual interstitial pneumonia (UIP). The population of alveolar macrophage of patients with sr-ILD differs markedly from the foamy and vacuolated cells that predominate in IPF/UIP. Thus, the absence of pigmented cells rather excludes sr-ILD and indicates other types of ILD. To summarise, the place of BALF in the diagnosis of sr-ILD seems to be established. Diagn. Cytopathol. 2008. © 2008 Wiley-Liss, Inc. [source]

Pulmonary response to airway instillation of autologous blood in horses

Molecular mechanisms underlying inflammatory lung diseases in the elderly: Development of a novel therapeutic strategy for acute lung injury and pulmonary fibrosis,

GERIATRICS & GERONTOLOGY INTERNATIONAL, Issue 3 2005
Takahide Nagase
In the elderly, inflammatory lung diseases, including acute lung injury and pulmonary fibrosis, are significant in terms of both mortality and difficulty in management. Acute respiratory distress syndrome (ARDS) is an acute lung injury and the mortality rate for ARDS ranges from 40 to 70% despite intensive care. Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal disorder of the lung parenchyma. No useful drugs are currently available to treat IPF. However, molecular mechanisms underlying these lung diseases are little understood and the development of a novel therapeutic strategy is urgently needed. Platelet-activating factor (PAF) and metabolites of arachidonic acid, i.e. eicosanoids, are lipid mediators that have various biological effects. A key enzyme for the production of these inflammatory mediators, including eicosanoids and PAF, is phospholipase A2. In particular, cytosolic PLA2 (cPLA2) is especially important. The purpose of this article is to report novel findings regarding the role of PAF and cPLA2 in lung inflammatory diseases, especially, acute lung injury and pulmonary fibrosis. To address this question, we used mutant mice, i.e. PAFR transgenic mice, PAFR gene-disrupted mice and cPLA2 gene-disrupted mice. We have shown that PAF and eicosanoids, downstream mediators of cPLA2, may be involved in the pathogenesis of ARDS and IPF, which are important diseases in the elderly. Although there exist extreme differences in clinical features between ARDS and IPF, both diseases are fatal disorders for which no useful drugs are currently available. On the basis of recent reports using mutant mice, cPLA2 might be a potential target to intervene in the development of pulmonary fibrosis and acute lung injury in the elderly. [source]

Impact of comorbidity on lung cancer survival

INTERNATIONAL JOURNAL OF CANCER, Issue 6 2003
C. Martin Tammemagi
Abstract Lung cancer is associated with smoking and age, both of which are associated with comorbidity. We evaluated the impact of comorbidity on lung cancer survival. Data on 56 comorbidities were abstracted from the records of a cohort of 1,155 patients. Survival effects were evaluated with Cox regression (outcome crude death). The adjusted R2 statistic was used to compare the survival variation explained by predictive variables. No comorbidity was observed in 11.7% of patients, while 54.3% had 3 or more (mean 2.97) comorbidities. In multivariate analysis, 19 comorbidities were associated with survival: HIV/AIDS, tuberculosis, previous metastatic cancer, thyroid/glandular diseases, electrolyte imbalance, anemia, other blood diseases, dementia, neurologic disease, congestive heart failure, COPD, asthma, pulmonary fibrosis, liver disease, gastrointestinal bleeding, renal disease, connective tissue disease, osteoporosis and peripheral vascular disease. Only the latter was protective. Some of the hazards of comorbidities were explained by more directly acting comorbidities and/or receipt of treatment. Stage explained 25.4% of the survival variation. In addition to stage, the 19 comorbidities explained 6.1%, treatments 9.2%, age 3.7% and histology 1.3%. Thirteen uncommon comorbidities (prevalence <6%) affected 21.2% of patients and explained 3.5% of the survival variation. Comorbidity count and the Charlson index were significant predictors but explained only 2.5% and 2.0% of the survival variation, respectively. Comorbidity has a major impact on survival in early- and late-stage disease, and even infrequent deleterious comorbidities are important collectively. Comorbidity count and the Charlson index failed to capture much information. Clinical practice and trials need to consider the effect of comorbidity in lung cancer patients. © 2002 Wiley-Liss, Inc. [source]

Smad3 as a mediator of the fibrotic response

INTERNATIONAL JOURNAL OF EXPERIMENTAL PATHOLOGY, Issue 2 2004
Kathleen C. Flanders
Summary Transforming growth factor-, (TGF-,) plays a central role in fibrosis, contributing to the influx and activation of inflammatory cells, the epithelial to mesenchymal transdifferentiation (EMT) of cells and the influx of fibroblasts and their subsequent elaboration of extracellular matrix. TGF-, signals through transmembrane receptor serine/threonine kinases to activate novel signalling intermediates called Smad proteins, which modulate the transcription of target genes. The use of mice with a targeted deletion of Smad3, one of the two homologous proteins which signals from TGF-,/activin, shows that most of the pro-fibrotic activities of TGF-, are mediated by Smad3. Smad3 null inflammatory cells and fibroblasts do not respond to the chemotactic effects of TGF-, and do not autoinduce TGF-,. The loss of Smad3 also interferes with TGF-,-mediated induction of EMT and genes for collagens, plasminogen activator inhibitor-1 and the tissue inhibitor of metalloprotease-1. Smad3 null mice are resistant to radiation-induced cutaneous fibrosis, bleomycin-induced pulmonary fibrosis, carbon tetrachloride-induced hepatic fibrosis as well as glomerular fibrosis induced by induction of type 1 diabetes with streptozotocin. In fibrotic conditions that are induced by EMT, such as proliferative vitreoretinopathy, ocular capsule injury and glomerulosclerosis resulting from unilateral ureteral obstruction, Smad3 null mice also show an abrogated fibrotic response. Animal models of scleroderma, cystic fibrosis and cirrhosis implicate involvement of Smad3 in the observed fibrosis. Additionally, inhibition of Smad3 by overexpression of the inhibitory Smad7 protein or by treatment with the small molecule, halofuginone, dramatically reduces responses in animal models of kidney, lung, liver and radiation-induced fibrosis. Small moleucule inhibitors of Smad3 may have tremendous clinical potential in the treatment of pathological fibrotic diseases. [source]

The central role of Fas-ligand cell signaling in inflammatory lung diseases

JOURNAL OF CELLULAR AND MOLECULAR MEDICINE, Issue 3 2004
G. A. DosReis
Abstract Following inflammation and injury in the lung, loss of epithelial cell precursors could determine the balance between tissue regeneration and fibrosis. This review discusses evidence that proapoptotic Fas-Fas ligand (FasL) signaling plays a central role in pulmonary inflammation, injury and fibrosis. FasL signaling induces inflammatory apoptosis in epithelial cells and alveolar macrophages, with concomitant IL-1, and chemokine release, leading to neutrophil infiltration. FasL signaling plays a critical role in models of acute lung injury, idiopathic pulmonary fibrosis and silicosis; blockade of Fas-FasL interactions either prevents or attenuates pulmonary inflammation and fibrosis. Serologic and immunohistochemical studies in patients support a major pathogenic role of Fas and FasL molecules in inflammatory lung diseases. Identification of the pathogenic role of FasL could facilitate the discovery of more effective treatments for currently untreatable inflammatory lung diseases. [source]

Increased serum levels of endostatin in patients with idiopathic pulmonary fibrosis

JOURNAL OF CLINICAL LABORATORY ANALYSIS, Issue 4 2005
Masaaki Sumi
Abstract Endostatin is an angiogenesis inhibitor that is an endogenously produced proteolytic fragment of type XVIII collagen. Serum levels of endostatin have been studied extensively in patients with malignant diseases. Recently, elevated serum endostatin levels were observed in patients with systemic sclerosis accompanying pulmonary fibrosis. To determine whether elevated serum endostatin can be observed in patients with idiopathic pulmonary fibrosis (IPF), we measured serum levels of endostatin in 69 patients with benign respiratory disease using an ELISA kit. The median of the serum endostatin levels in these patients was 50.8 pg/mL. Seven of 11 patients (63.6%) with collagen disease-associated pulmonary fibrosis (CDPF), and 19 of 24 patients (79.2%) with IPF had higher serum endostatin levels than the median level of the 69 patients. There was no statistical difference in serum endostatin levels between the patients with IPF and those with CDPF (P=0.7898). Serum endostatin levels in 24 patients with IPF were significantly higher than those in 34 patients with respiratory diseases other than IPF and CDPF (P=0.0001). Elevated serum levels of endostatin were observed in patients with IPF. Although the mechanisms are unclear, elevated serum levels of endostatin may be related to the fibrosing process in the lung. J. Clin. Lab. Anal. 19:146,149, 2005. © 2005 Wiley-Liss, Inc. [source]

Reactions to Penicillamine: A Case of Cutis Laxa, Elastosis Perforans Serpiginosa and "Pseudo" Pseudoxanthoma

JOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005
S. Frankel
This patient was a 61-year-old white female who received several years of penicillamine therapy for the treatment of cystinuria. She subsequently developed penicillamine induced cutis laxa, elastosis perforans serpiginosa, and pseudoxanthoma elasticum like skin lesions. In addition, she suffered from numerous chronic bilateral lower extremity skin ulcerations. Her past medical history was also significant for end stage renal disease requiring hemodialysis and pulmonary fibrosis. She presented to the University of Miami Wound Care Center in 1/04 for treatment of her chronic ulcerations. On physical examination, the patient had multiple large hyperpigmented plaques with central ulcerations on her lower extremities. Some of the ulcers had overlying crust and others were covered with yellow fibrinous tissue. She also had generalized thickened, lax skin with multiple folds. On her neck, thighs, back and arms were violaceous, atrophic, serpiginous plaques with peripheral crusted erosions. A biopsy taken from the patients left thigh revealed dermal elastosis and the features of pseudo-pseudoxanthoma. Two additional biopsies taken from the left thigh demonstrated elastosis perforans serpiginosa. This case highlights multiple skin manifestations of penicillamine therapy. [source]

The spectrum of cutaneous lesions in rheumatoid arthritis: a clinical and pathological study of 43 patients

JOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2003
C. M. Magro
Introduction:, Rheumatoid arthritis (RA) is an idiopathic arthropathy syndrome that has a propensity to affect the small joints of the hands and feet with extra-articular manifestations comprising skin lesions, neuropathy, pericarditis, pleuritis, interstitial pulmonary fibrosis and a systemic polyarteritis nodosa (PAN)-like vasculitic syndrome. The most widely recognized skin lesion is the rheumatoid nodule. Other skin manifestations are poorly defined. Materials and methods:, Using a natural language search of the authors' outpatient dermatopathology databases, skin biopsies from 43 patients with RA were selected for retrospective analysis in an attempt to define the dermatopathological spectrum of RA and its clinical correlates. Results:, The biopsies were categorized by the dominant histologic pattern, recognizing that in most cases there were additional minor reaction patterns. Palisading and/or diffuse interstitial granulomatous inflammation was the dominant pattern seen in 21 patients; the lesions included nodules, plaques and papules with a predilection to involve skin over joints. Besides interstitial histiocytic infiltrates and variable collagen necrobiosis, these cases also showed interstitial neutrophilia, vasculitis and pauci-inflammatory vascular thrombosis. The dominant morphology in 11 other patients was vasculopathic in nature: pauci-inflammatory vascular thrombosis, glomeruloid neovascularization, a neutrophilic vasculitis of pustular, folliculocentric, leukocytoclastic or benign cutaneous PAN types, granulomatous vasculitis, and lymphocytic vasculitis and finally occlusive intravascular histiocytic foci for which the designation of ,RA-associated intravascular histiocytopathy' is proposed. Rheumatoid factor (RF) positivity and active arthritis were common in this group, with anti-Ro and anticardiolipin antibodies being co-factors contributing to vascular injury in some cases. Immunofluorescent testing in three patients revealed dominant vascular IgA deposition. In nine patients, the main pattern was one of neutrophilic dermal and/or subcuticular infiltrates manifested clinically as urticarial plaques, pyoderma gangrenosum and panniculitis. Conclusions:, The cutaneous manifestations of RA are varied and encompass a number of entities, some of which define the dominant clinical features, such as the rheumatoid papule or subcutaneous cords, while others allude to the histopathology, i.e. rheumatoid neutrophilic dermatosis. We propose a more simplified classification scheme using the adjectival modifiers of ,rheumatoid-associated' and then further categorizing the lesion according to the dominant reaction pattern. Three principal reaction patterns are recognized, namely extravascular palisading granulomatous inflammation, interstitial and/or subcuticular neutrophilia and active vasculopathy encompassing lymphocyte-dominant, neutrophil-rich and granulomatous vasculitis. In most cases, an overlap of the three reaction patterns is seen. Co-factors for the vascular injury that we believe are integral to the skin lesions of RA include RF, anti-endothelial antibodies of IgA class, anti-Ro and anticardiolipin antibodies. [source]

IPF: new insight on pathogenesis and treatment

ALLERGY, Issue 5 2010
S. Harari
To cite this article: Harari S, Caminati A. IPF: new insight on pathogenesis and treatment. Allergy 2010; 65: 537,553. Abstract Recent years have seen a robust influx of exciting new observations regarding the mechanisms that regulate the initiation and progression of pulmonary fibrosis but the pathogenesis remains poorly understood. The search for an alternative hypothesis to unremitting, chronic inflammation as the primary explanation for the pathophysiology of idiopathic pulmonary fibrosis (IPF) derives, in part, from the lack of therapeutic efficacy of high-dose immunosuppressive therapy in patients with IPF. The inflammatory hypothesis of IPF has since been challenged by the epithelial injury hypothesis, in which fibrosis is believed to result from epithelial injury, activation, and/or apoptosis with abnormal wound healing. This hypothesis suggests that recurrent unknown injury to distal pulmonary parenchyma causes repeated epithelial injury and apoptosis. The resultant loss of alveolar epithelium exposes the underlying basement membrane to oxidative damage and degradation. Emerging concepts suggest that IPF is the result of epithelial,mesenchymal interaction. The initiation of this fibrotic response may depend upon genetic factors and environmental triggers; the role of Th1 or Th2 cell-derived cytokines may also be important. This process appears to be unique to usual interstitial pneumonia/IPF. It is clear that IPF is a heterogeneous disease with variations in pathology, high-resolution computed tomography findings, and patterns of progression. Idiopathic pulmonary fibrosis is a complex disorder, and no unifying hypothesis has been identified at present that explains all the abnormalities. [source]

Gastroesophageal Reflux Disease and Idiopathic Pulmonary Fibrosis

"End-stage" Pulmonary Fibrosis in Sarcoidosis

Upper and lower respiratory diseases after occupational and environmental disasters

MOUNT SINAI JOURNAL OF MEDICINE: A JOURNAL OF PERSONALIZED AND TRANSLATIONAL MEDICINE, Issue 2 2008
David J. Prezant MD
Abstract Respiratory consequences from occupational and environmental disasters are the result of inhalation exposures to chemicals, particulate matter (dusts and fibers) and/or the incomplete products of combusion that are often liberated during disasters such as fires, building collapses, explosions and volcanoes. Unfortunately, experience has shown that environmental controls and effective respiratory protection are often unavailable during the first days to week after a large-scale disaster. The English literature was reviewed using the key words,disaster and any of the following: respiratory disease, pulmonary, asthma, bronchitis, sinusitis, pulmonary fibrosis, or sarcoidosis. Respiratory health consequences after aerosolized exposures to high-concentrations of particulates and chemicals can be grouped into 4 major caterogies: 1) upper respiratory disease (chronic rhinosinusitis and reactive upper airways dysfunction syndrome), 2) lower respiratory diseases (reactive [lower] airways dysfunction syndrome, irritant-induced asthma, and chronic obstructive airways diseases), 3) parenchymal or interstitial lung diseases (sarcoidosis, pulmonary fibrosis, and bronchiolitis obliterans, and 4) cancers of the lung and pleura. This review describes several respiratory consequences of occupational and environmental disasters and uses the World Trade Center disaster to illustrate in detail the consequences of chronic upper and lower respiratory inflammation. Mt Sinai J Med 75:89,100, 2008. © 2008 Mount Sinai School of Medicine [source]

An Ill Wind-Iatrogenic Air Embolus Around Pacing Leads During Defibrillator Implant with Coexisting Pulmonary Fibrosis

Pulmonary fibrosis: Cellular and molecular events

Acute inorganic mercury vapor inhalation poisoning

PATHOLOGY INTERNATIONAL, Issue 3 2000
Sigeyuki Asano
Abstract Mercury contamination is a serious environmental problem worldwide. Two primary sources of contamination are dumping of large quantities of inorganic mercury and exposure in the mining industry. Although the actual fatal level of mercury vapor is not known, exposure to more than 1,2 mg/m3 of elemental mercury vapor (Hg0) for a few hours causes acute chemical bronchiolitis and pneumonitis. Two hours after exposure, lung injury appears as hyaline membrane formation, and finally, extensive pulmonary fibrosis occurs. Clinical findings correlate with the duration of exposure, the concentration of mercury, and the survival time after exposure. There is no correlation between pathological findings and the concentration of mercury in the tissues. Necrosis of proximal convoluted tubules may be attributed to the disruption of the enzyme systems of Hg2+ -sulfhydryl compounds. Metallothionein protein (MT), induced by the accumulation of Hg2+ in the kidneys, may play an important role in detoxication after it forms a non-toxic Hg2+ -MT compound. Despite the deposition of mercury in the brain, compared with organic mercury, inorganic mercury did not seem to damage the neurons. Drugs such as chelating agents and corticosteroids appear to effectively decrease the inflammation and delay pulmonary fibrosis. [source]

Pediatric lung disease: From proteinases to pulmonary fibrosis

PEDIATRIC PULMONOLOGY, Issue 5 2005
Felix Chua MRCP
Abstract One distinctive outcome of interstitial lung diseases in childhood is the abnormal accumulation of pulmonary extracellular matrix. The clinical consequence of such excessive connective tissue accumulation is known as pulmonary fibrosis. While numerous aspects of its pathogenesis have become familiar, many key events involved in its inception and progression still remain unclear. There is now compelling evidence that lung damage due to uncontrolled proteolysis may help drive critical processes that regulate fibrotic matrix remodeling. In this regard, a number of proteinases have been implicated in promoting both the initial lung injury and the fibroproliferative repair that follows. This review summarizes the knowledge of how different matrix-targeting enzymes may act to influence the development of pediatric pulmonary fibrosis. Understanding the scientific basis of this complex process may highlight opportunities to limit unwanted proteolysis and the intensity of its fibrotic sequelae. © 2005 Wiley-Liss, Inc. [source]

Original Article: Detection of p16 promoter methylation in premature rats with chronic lung disease induced by hyperoxia

PEDIATRICS INTERNATIONAL, Issue 4 2010
Xiaohong Yue
Abstract Background:, The aim of the present study was to investigate p16 promoter methylation in premature rats with chronic lung disease (CLD) induced by hyperoxia. Methods:, Eighty Wistar rats were randomized into the hyperoxia group (fraction of inspired oxygen [FiO2]= 900 mL/L) or the control group (FiO2= 210 mL/L), 40 for each group. Semi-nested methylation-specific polymerase chain reaction (sn-MSP) was applied to detect p16 promoter hypermethylation in lung tissues. Additionally, p16 mRNA and protein expression was detected on reverse transcription,polymerase chain reaction (RT-PCR), western blot and the strept actividin,biotin complex method. Results:, Extended exposure to hyperoxia led to increased methylation, and the methylation level reached a peak in the period of maximum pulmonary fibrosis in the hyperoxia group, while the methylation did not occur in the control group. The methylation rates on semi-nested PCR (sn-PCR) and nested-MSP were, respectively, 52.5% and 42.5% in the hyperoxia group. There was no statistically significant difference between the two methods. The p16 mRNA and protein expression was significantly higher in those with p16 promoter hypermethylation than those without. Conclusion:, Exposure to hyperoxia may induce p16 promoter hypermethylation in lung tissues in premature rats, and methylation risk increases as exposure extends. p16 promoter methylation induced by hyperoxia may be one of the mechanisms for low p16 mRNA and protein expression. [source]

Clinicopathological features of pure mica pneumoconiosis associated with Sjögren syndrome

AMERICAN JOURNAL OF INDUSTRIAL MEDICINE, Issue 3 2004
FCCP, Hideo Kobayashi MD
Abstract Background There are few reports on the clinical, radiologic, and pathologic features of pure mica pneumoconiosis. Methods A case of definite pure mica pneumoconiosis in a rubber factory worker is reported with the clinicopathological findings. Results Chest HRCT demonstrated subpleural and peribronchovascular interstitial thickening. The characteristic histologic features were pulmonary fibrosis accompanied by prominent histiocytic granulomas containing giant cells. Conclusions Pure mica pneumoconiosis can be identified by specific radiologic and pathologic findings. Am. J. Ind. Med. 45:246,250, 2004. © 2004 Wiley-Liss, Inc. [source]

Interstitial pulmonary fibrosis after severe exposure to welding fumes

AMERICAN JOURNAL OF INDUSTRIAL MEDICINE, Issue 4 2002
U. Buerke MD
Abstract Background Interstitial pulmonary fibrosis (IPF) is reported after long term, severe exposure to welding fumes in poorly ventilated workplaces. Methods Fifteen welders with IPF were examined,13 in our outpatient clinic,from 1990 to 1997. Occupational histories and examinations, lung function analyses, symptoms and clinical findings, histological analyses in 13 patients partly including SEM/EDX-analyses, chest X-rays, chest computed tomographies were conducted. Results Duration of work as welders was 28 years and the cumulative dose of welding fumes 221 mg/m3,×,years (median). Lung function studies found pattern of restriction or combined restriction-obstruction, lower diffusion capacity, and reduced blood oxygen tension at exercise. Histologicallly, patchy interstitial fibrosis was noted. Accumulations of particulate matter typically for welding fume were detected. EDX showed increase of iron load and close topographical relationship to welding fume particles embedded in areas of scattered fibrosis. Conclusion While epidemiological data are limited, it is reasonable to conclude that a causal relationship exists between IPF in welders with long term exposure to high concentrations of welding fumes. Am. J. Ind. Med. 41:259,268, 2002. © 2002 Wiley-Liss, Inc. [source]

Diffuse alveolar damage in idiopathic pulmonary fibrosis: Does aetiology matter?

RESPIROLOGY, Issue 6 2010
Tamera J. Corte
No abstract is available for this article. [source]