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At least 127 records · Page 7Linked to original sources
PubMed2000

A novel rat model of chronic fibrosing cholangitis induced by local administration of a hapten reagent into the dilated bile duct is associated with increased TNF-alpha production and autoantibodies.

BACKGROUND/AIM: The cholangiopathies represent hepatobiliary diseases in which bile-duct epithelial cells are targets for destructive processes, including immune-mediated damage. We describe a novel rat model of chronic fibrosing cholangitis induced by administration of the hapten reagent 2,4,6-trinitrobenzenesulfonic acid (TNBS) into the dilated bile duct. METHODS: The common bile duct was dilated due to a mild stenosis in 8-week-old female Lewis rats. TNBS (50 mg/kg) was injected during a second laparotomy. RESULTS: TNBS-treatment reproducibly resulted in chronic fibrosing cholangitis. In retrograde cholangiography the bile ducts showed irregularities, beading and strictures. Alkaline phosphatase levels remained abnormal throughout the study period. Immunohistochemical staining showed an increased number of macrophages, CD3+ T-lympbocytes and MHC class II antigen upregulation. The spontaneous interferon-gamma, tumor necrosis factor-alpha and interleukin-10 production of liver-derived mononuclear cells was increased. Anti-neutrophil cytoplasmic antibodies with specificity against myeloperoxidase, catalase and actin were found between 1 and 12 weeks after TNBS injection. CONCLUSIONS: We established a novel rat model of chronic fibrosing cholangitis with histologic, cholangiographic, serologic and immunologic similarities to human primary sclerosing cholangitis. This model may be used to study pathomechanisms of chronic cholangitis without concomitant inflammatory bowel disease.

Animals↗
PubMed2005

Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis: report of a new case with literature review.

Nephrogenic fibrosing dermopathy (NFD) is a fibrosing condition of the skin that is being described increasingly in patients with renal diseases, many of whom are on dialysis therapy or have undergone renal transplantation. Its etiopathology is unknown, and no standard therapy currently exists. We describe a patient with NFD for whom histopathologic studies indicated that the fibrotic process affected subcutaneous tissue, striated muscles, diaphragm, pleura, pericardium, great vessels of the heart, left ventricle and septum, and tunica albuginea in addition to the dermis. Fibroblast-like cells positive for CD34 and CD45RO and scattered CD68-positive cells were found in affected tissues. The presentation of our case is unusual in that the disease process started in the lower abdomen and upper extremities and involved the upper extremities to a greater extent than the lower extremities. Our findings indicate that the fibrosis associated with NFD can extend beyond dermis and, as part of a systemic fibrosing disorder, can involve subcutaneous tissues, striated muscles, diaphragm, pleura, pericardium, and myocardium. We therefore suggest that "nephrogenic systemic fibrosis" would be a more appropriate term for this disease entity.

Adult↗
PubMed2003

Successful treatment of fibrosing cholestatic hepatitis using adefovir dipivoxil in a patient with cirrhosis and renal insufficiency.

Fibrosing cholestatic hepatitis is a deleterious manifestation of hepatitis B virus infection in immunocompromised patients. Without treatment, this condition is usually fatal within weeks of onset. Liver retransplantation has not been successfully performed to date, and treatment intervention was generally unsuccessful before the advent of adefovir dipivoxil. However, concerns have been expressed about the use of this agent in patients who are renally compromised. A 40-year-old liver transplant recipient with hepatitis B virus reinfection, resistance to lamivudine, and fibrosing cholestatic hepatitis complicated by terminal renal impairment and spontaneous bacterial peritonitis was treated with adefovir dipivoxil 10 mg after every dialysis. Since initiating treatment with adefovir dipivoxil 10 mg, a dramatic virologic and clinical improvement was observed in this patient. The patient returned to work full-time within 6 months of starting adefovir dipivoxil without the need for liver retransplantation. Serum HBV DNA (Amplicor HBV; Roche Diagnostics, Basle, Switzerland) decreased by 6 log(10) copies/mL and became negative (< 400 copies/mL) within 8 weeks of treatment and remains negative at the last available assessment. The patient continues to require renal dialysis, but is generally well. Creatinine clearance improved from 8 mL/min to 16 mL/min during the course of treatment. No adverse events related to adefovir dipivoxil were observed. Adefovir dipivoxil resulted in significant clinical improvement in this patient with hepatitis B virus-induced fibrosing cholestatic hepatitis, despite the presence of renal impairment and lamivudine resistance.

Adenine↗
PubMed2003

Nephrogenic fibrosing dermopathy (scleromyxedema-like illness of renal disease).

Nephrogenic fibrosing dermopathy or scleromyxedema-like illness of renal disease is a recently reported disorder. It manifests as scleromyxedema-like skin lesions without associated paraproteinemia, occurring in the setting of renal disease. In the majority of cases skin lesions of nephrogenic fibrosing dermopathy develop after hemodialysis or renal transplantation; however, the origin is still unknown. We report 4 new cases of nephrogenic fibrosing dermopathy and review the literature. The clinical and histopathologic features, differential diagnosis, possible etiology, and treatment options are reviewed.

Adult↗
PubMed2002

The impact of coexisting connective tissue disease on survival in patients with fibrosing alveolitis.

OBJECTIVES: Previous reports have suggested that patients who have fibrosing alveolitis in association with a connective tissue disease (FA-CTD) have a better prognosis than patients with 'lone' cryptogenic fibrosing alveolitis (LCFA). The present study was designed to compare the survival of a general population-based sample of patients with FA-CTD and LCFA both with each other and with the general population. METHODS: A survival analysis was performed using data for 107 patients with FA-CTD, 872 with LCFA and 5958 controls matched for age, sex and general practice, drawn from the General Practice Research Database. The data were analysed using Cox regression, adjusting for a number of potential confounders, including age, gender, smoking habit and use of oral corticosteroids. RESULTS: The median follow-up period was 2.1 yr and during this time 54 (50%) patients with FA-CFA, 386 (44%) patients with LCFA and 601 (10%) controls died. The mortality rates for patients with FA-CTD, LCFA and the controls were 284, 270 and 41 per 1000 person-yr respectively. After adjusting for age, gender, smoking habit and exposure to oral corticosteroids, patients with FA-CTD had a marginally worse survival than patients with LCFA (hazard ratio 1.20, 95% confidence interval 0.90-1.61). Compared with the general population controls, patients with either LCFA or FA-CTD had a considerably worse prognosis (hazard ratio 5.56, 95% confidence interval 4.77-6.49). CONCLUSIONS: The median survival in patients with fibrosing alveolitis is less then 3 yr. We found no evidence to support previous reports of a better prognosis amongst patients with FA-CTD.

Arthritis, Rheumatoid↗
PubMed1997

Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders.

Clinically distinct fibrosing processes affecting the skin, selected internal organs, or both in a characteristic pattern are a common cause of morbidity. In addition to systemic sclerosis, the prototype idiopathic fibrosing disorder, these conditions include the eosinophilia-myalgia syndrome, epidemic toxic oil syndrome, eosinophilic fasciitis, localized forms of scleroderma, keloid, and the newly described entity of fibrosing colonopathy. The pathogenesis of these disorders, although still incompletely understood, appears to share similarities with that of systemic sclerosis. Insights into these diseases have recently emerged from epidemiologic and toxicoepidemiologic investigations, in situ hybridization and polymerase chain reaction amplification of target genomes, and in vivo and in vitro experimental research. Minor contaminants in food supplements, activation and degranulation of eosinophils, altered expression of CD34 antigen on dendritic cells, disordered regulation of fibroblast apoptosis and proliferation, infection with Borrelia organisms, and cytokines such as transforming growth factor-beta, interleukin-4, and connective tissue growth factor are implicated in inducing an accentuated and persistent fibrogenic host response to injury, resulting in tissue fibrosis. In addition, humoral and cellular autoimmunity may also be implicated.

Colonic Diseases↗
PubMed2003

Nephrogenic fibrosing dermopathy: the first 6 years.

PURPOSE OF REVIEW: Nephrogenic fibrosing dermopathy (NFD) is a newly recognized scleroderma-like fibrosing skin condition. It develops in patients with renal insufficiency. This review summarizes recent case reports and examines theories of disease pathogenesis. Information from the Yale University NFD Registry Project, as well as published case reports, is included to provide a contextual framework upon which to base these theories. RECENT FINDINGS: Recent studies have contributed to a clearer definition of the clinical spectrum, epidemiology, and pathogenesis of NFD. The findings of yellow scleral plaques and circulating antiphospholipid antibodies have been proposed as markers of NFD in recent case reports. In addition, epidemiologic data have yielded several distinct clinical patterns of disease onset. Lastly, dual immunohistochemical staining for CD34 and procollagen in the spindle cells of NFD suggest that the dermal cells of NFD may represent circulating fibrocytes recruited to the dermis-a finding previously undescribed in normal skin. SUMMARY: Scenario classification of NFD is a means for simplifying the search for multifactorial disease triggers, and may be helpful in predicting prognosis and response to therapy. The technique of dual immunolabeling, although not yet fully characterized as a diagnostic test, may provide a sensitive and specific method of diagnosis, as well as a starting point in the investigation of other cutaneous fibrosing disorders. The postulate that NFD may represent a systemic disorder mediated by aberrantly functioning circulating fibroblast precursor fibrocytes is explored.

Adolescent↗
PubMed1996

CT evaluation of fibrosing alveolitis--applications and insights.

The clinical management of patients with fibrosing alveolitis has traditionally been empirical; without lung biopsy, a definitive diagnosis may be impossible and decisions about treatment are often arbitrary. With the advent of high-resolution computed tomography (CT), the proportion of patients needing lung biopsy for confirmation of the diagnosis of fibrosing alveolitis has declined. There is now much evidence that CT provides an accurate guide to disease activity, and more recent work has shown the important role of CT in providing new insights into the biologic behaviour and pathogenesis of fibrosing alveolitis.

Biopsy↗
PubMed1993

Ventilation perfusion radionuclide imaging in cryptogenic fibrosing alveolitis.

There is increasing interest in ventilation perfusion (V/Q) imaging in cryptogenic fibrosing alveolitis because of the data these scans provide on the dynamic V/Q relationships in such patients undergoing single lung transplantation. However, the full spectrum of V/Q abnormalities in this disease is poorly defined. We therefore analysed the V/Q scans of 45 consecutive patients with advanced cryptogenic fibrosing alveolitis being considered for single lung transplantation. Scans were classified according to the presence, severity and degree of matching of defects in ventilation and perfusion images and the results were compared with the data obtained from lung function tests. Ventilation images showed defects in 13 (29%) and 'washout delay' in 15 (33%) patients; 10 (22%) patients had asymmetric distribution of ventilation with one lung receiving > 60% of total ventilation. Perfusion images showed normal perfusion in 8 (18%), mild defects in 18 (40%) and major defects in 19 (42%) patients. The distribution of perfusion between lungs was significantly asymmetric in 20 (45%) patients. V/Q images were matched in 15 (33%), mildly mismatched in 15 (33%) and severely mismatched in 15 (33%) patients, but the degree of V/Q mismatch did not show a relationship to KCO, PaO2 or A-aO2 gradient. The appearances were atypical of pulmonary embolism in eight patients. V/Q images in cryptogenic fibrosing alveolitis show a diverse range of appearances and may mimic pulmonary embolism. V/Q imaging complements the data obtained from lung function tests and is particularly useful in defining the differential function of each lung which is particularly important in the assessment of patients for single lung transplantation.

Adult↗
PubMed2000

Idiopathic fibrosing pancreatitis and Crohn's disease: an interesting association.

Idiopathic fibrosing pancreatitis is an uncommon condition in children and adolescents of unknown aetiology. This syndrome has been reported in 36 cases so far. To our knowledge none of these cases was definitively associated with Crohn's disease. In this report we describe a young female patient who developed Crohn's disease of the colon 5 years after having been diagnosed with idiopathic fibrosing pancreatitis. The differential diagnosis between this syndrome associated with Crohn's disease and pancreatic Crohn's disease or fibrosing colonopathy, an entity related to pancreatic enzyme therapy, is discussed.

Adolescent↗
PubMed2004

Autoantibodies in systemic sclerosis and fibrosing syndromes: clinical indications and relevance.

PURPOSE OF REVIEW: Systemic sclerosis, or scleroderma, is associated with a variety of autoantibodies, each of them having their own clinical associations. The fibrosing disorders, other than systemic sclerosis, represent a diverse group of diseases with systemic or localized effect and with limited understanding of their pathogenesis. The purpose of this review is to analyze the literature on the clinical usefulness of examining serum autoantibodies in patients with known or suspected scleroderma and fibrosing disorders. RECENT FINDINGS: Studies on autoantibodies within the past year highlight their clinical utility in systemic sclerosis. Anticentromere antibodies are most often seen with limited cutaneous involvement and lower frequency of pulmonary fibrosis and lower mortality (despite an increased risk for pulmonary hypertension) compared with anti-Scl-70 and antinucleolar antibodies. Anti-Scl-70 antibodies are associated with diffuse cutaneous involvement, increased frequency of pulmonary fibrosis, and higher mortality. The anti-polymyositis-scleroderma autoantibody is associated with the polymyositis-scleroderma overlap syndrome. Anti-Th/To antibodies are associated with milder skin and systemic involvement but with more severe pulmonary fibrosis and overall worse prognosis. Anti-RNA-polymerase family antibodies and antifibrillarin antibodies are predictive of diffuse cutaneous and systemic involvement and greater mortality. Less specific autoantibodies for systemic sclerosis and limited data on some other autoantibodies limit their clinical utility in patients with systemic sclerosis. For the most part, the association between autoantibodies and fibrosing disorders other than systemic sclerosis remains inconclusive. SUMMARY: Autoantibodies in systemic sclerosis provide important and prognostic information and are useful in defining clinical subsets of the disease. When used appropriately, they can be a useful instrument in the management of scleroderma.

Autoantibodies↗
PubMed2004

Fibrosing colonopathy in an adult cystic fibrosis patient after discontinuing pancreatic enzyme therapy.

Fibrosing colonopathy, a complication of cystic fibrosis, has generally been reported in young children with exposure to high doses of pancreatic enzymes. The authors report the case of a 25-year-old male with cystic fibrosis who presented with gradually progressive symptoms of bowel obstruction. Pathologic examination of the right colon revealed findings consistent with fibrosing colonopathy. This case is distinctive because of the adult presentation, and because the patient's symptoms developed long after he had discontinued taking a high-strength enzyme preparation. This case suggests that multiple etiologic factors, and not simply exposure to pancreatic enzymes, may result in fibrosing colonopathy in adults with cystic fibrosis.

Adult↗
PubMed1993

Expression of tumour necrosis factor-alpha in cryptogenic fibrosing alveolitis.

We have studied 15 cases of cryptogenic fibrosing alveolitis stained with a monoclonal antibody reactive with human tumour necrosis factor-alpha (TNF alpha), a cytokine that has been implicated in inflammation and fibrosis. Seven were examples of lone cryptogenic fibrosing alveolitis and eight were examples of cryptogenic fibrosing alveolitis in patients with systemic sclerosis. There was widespread staining of epithelial cells, particularly hyperplastic type II pneumocytes. Macrophages stained only weakly. In a control group of 10 patients dying of unrelated conditions, staining for TNF alpha was weak and, in the alveolar epithelium, was confined to a very occasional type II pneumocyte. The strong expression of TNF alpha in hyperplastic type II pneumocytes suggests that TNF alpha produced during alveolar epithelial regeneration may play a part in the fibrosis seen in this disease.

Antibodies, Monoclonal↗
PubMed2006

Alopecia areata masquerading as frontal fibrosing alopecia.

SUMMARY Postmenopausal women with frontal recession may represent a diagnostic challenge, as frontal fibrosing alopecia and alopecia areata may be clinically difficult to distinguish. A 53-year-old postmenopausal woman presented with a progressive fronto-temporal marginal alopecia with sparing of her eyebrows. Scalp biopsy of the affected frontal hairline revealed peribulbar lymphocytic inflammation, but no evidence of lichenoid inflammation, perifollicular fibrosis or scarring. Whereas the pathology strongly favoured alopecia areata, the clinical features overlapped with frontal fibrosing alopecia, a variant of lichen planopilaris targeting the frontal scalp. This paper presents an atypical clinical presentation of alopecia areata, which may be mistaken for frontal fibrosing alopecia.

Administration, Topical↗
PubMed1979

Acute diffuse interstitial fibrosing pneumonitis and bilateral renal cortical necrosis.

An autopsy case of a 69-year-old male with acute diffuse interstitial fibrosing pneumonitis complicated by bilateral renal cortical necrosis was presented. Autopsy revealed acute diffuse interstitial fibrosing pneumonitis, bilateral renal cortical necrosis, non-bacterial thrombotic endocarditis, involving the aortic and mitral valves, and some interesting vascular lesions, dissemination of fibrinoid change of arterioles and fibrin thrombus of small vessels in various organs; accumulation of polymorphonuclear leukocytes in the lumen of the smaller interlobular arteries and arterioles of the kidney with cellular infiltration and disintegration of the wall; severe disorganization of the wall with intraluminar and intramural fibrinous exudation in smaller branches of the hepatic artery; diminution and disarrangment of muscle fibers and patchy hyalinization in the media of the renal and interlobar arteries. The inter-relationship between acute diffuse interstitial fibrosing pneumonitis, bilateral renal cortical necrosis which may be regarded as a 'hallmark' of the generalized Shwartzman reaction, and disseminated intravascular coagulation was discussed.

Acute Disease↗
PubMed1996

Classification of lung sounds in patients with asthma, emphysema, fibrosing alveolitis and healthy lungs by using self-organizing maps.

The performance of the self-organizing map (SOM), an artificial neural network, was evaluated in the classification of lung sounds. Patients with asthma (n = 8), emphysema (n = 8) and fibrosing alveolitis (n = 8), and patients with healthy lungs (n = 8) were selected for the study. Fast Fourier transform (FFT) spectra from midinspiratory breath sounds recorded at the right lower lobe area were used to construct feature vectors in the learning and classification process of SOM. The sound segments did not contain wheezing sounds. The lung sounds of 25/32 (78%) patients were classified correctly, with an overall kappa (kappa) value of 0.71. The agreement between the clinical and proposed diagnoses based on classification of lung sounds was good among patients with emphysema (kappa = 0.92) and those with healthy lungs (kappa = 0.83), but only moderate among patients with asthma (kappa = 0.52) and fibrosing alveolitis (kappa = 0.54). This is due to the limitations in distinguishing breath sounds of asthmatics without wheezing sounds from those with crackles in fibrosing alveolitis by the spectral pattern alone. The results indicate that SOM based on FFT spectra is potentially useful in the classification of lung sounds, e.g. in health screening or in differential diagnosis of pulmonary disorders. To enhance the performance of SOM, other features of lung sounds should be combined with FFT spectra.

Adult↗
PubMedDate not supplied

Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy: clinical aspects.

Nephrogenic systemic fibrosis, previously known as nephrogenic fibrosing dermopathy, is a novel fibrosing disorder characterized by prominent cutaneous and systemic fibrosis in patients with renal failure. Although initially descriptions of this disorder was described to be purely cutaneous, numerous subsequent reports have shown remarkable systemic involvement with fibrosis of numerous internal organs, including striated muscles, myocardium, microvasculature, lungs, and others, accompanied by elevation of systemic inflammation markers, such as erythrocyte sedimentation rate and C-reactive protein. The cutaneous manifestations are characterized by erythematous to browny skin induration with a peau d'orange appearance, plaques, nodules, and papules. Histopathologically, there is dermal and subdermal thickening with abundant collagen fibers and mucin deposits and a striking increase in the number of spindle-shaped fibroblastic cells. The disorder is clinically distinct from scleromyxedema, systemic sclerosis, and other cutaneous fibrosing syndromes. The cause is unknown, and currently there is no uniformly effective treatment.

Female↗
PubMed1977

Fibrosing alveolitis in infancy and childhood.

Fibrosing alveolitis is a rare, diffuse lung disease characterized by varying combinations of two histological features: thickening of alveolar walls and the presence of large mononuclear cells in the alveolar spaces. Clinical details of 10 children with fibrosing alveolitis are reported. The main symptoms in children are tachypnoea or dyspnoea, cough, poor weight gain, and cyanosis. The condition is similar to that in adults, but it is usually a more acute illness, and if untreated, more predictably fatal. Respiratory failure, pulmonary hypertension, and cardiac failure are the major complications. Less commonly, superimposed bacterial infection and pneumothorax occur. Chest x-rays often show a sequence of changes with a ground-glass appearance and fine mottling in the early stage of the disease, progressing to a picture of mainly hilar linear markings in those children who recover. The histological features at lung biopsy or necropsy are described; these correlated poorly with the radiological features, steroid responsiveness, and clinical course. Lung function tests in 3 older children showed evidence of markedly reduced lung volumes in 2. Static lung compliance in 4 children in the acute stage of the illness was normal in 3 and diminished in one. The response to steroid therapy was analysed in cases from the literature and the 10 reported cases. No spontaneous remissions occurred, all the survivors having been treated with corticosteroids. In children fibrosing alveolitis is almost always a corticosteroid-responsive disease. An appropriate course of prednisolone would be of at least 4 week's, but preferably of 8 weeks' duration, at a minimum daily dose of 2 mg/kg. After improvement the steroid withdrawal should be cautious and protracted, comprising at least a year's continuous treatment.

Adrenal Cortex Hormones↗