Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “FIBROSITIS”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 145 records · Page 8Linked to original sources
PubMed1998

Management of fibrosing pancreatitis in children presenting with obstructive jaundice.

BACKGROUND: Children with fibrosing pancreatitis are conventionally treated surgically to relieve common bile duct (CBD) obstruction caused by pancreatic compression. Residual pancreatic function has not been formally tested in these patients. AIMS: To evaluate the usefulness of non-surgical temporary drainage in children with fibrosing pancreatitis and to assess pancreatic function after resolution of their CBD obstruction. PATIENTS: Four children (1.5-13 years; three girls). METHODS AND RESULTS: Abdominal sonography and computed tomography revealed diffuse enlargement of the pancreas, predominantly the head. The CBD was dilated due to compression by the head of the pancreas. Pancreatic biopsy specimens obtained in three patients showed notable acinar cell atrophy and extensive fibrosis. Cystic fibrosis was excluded. No other cause of pancreatitis was identified. Pancreatic tissue from one patient contained viral DNA sequences for parvovirus B19 detected by polymerase chain reaction; serum IgM to parvovirus was positive. Three patients had temporary drainage of the CBD and one patient underwent a choledochojejunostomy. Serial imaging studies revealed resolution of the CBD obstruction with reduction in pancreatic size. Exocrine pancreatic function deteriorated. Three patients developed pancreatic insufficiency within two to four months of presentation. The fourth patient has notably diminished pancreatic function, but remains pancreatic sufficient. None has diabetes mellitus. CONCLUSIONS: Temporary drainage of the CBD obstruction is recommended in fibrosing pancreatitis in children along with close monitoring of the clinical course, before considering surgery.

Adolescent↗
PubMed2001

A survey of nocturnal hypoxaemia and health related quality of life in patients with cryptogenic fibrosing alveolitis.

BACKGROUND: A survey of overnight oximetry was conducted to estimate the prevalence of nocturnal hypoxaemia in patients with cryptogenic fibrosing alveolitis and to establish whether nocturnal hypoxaemia is related to quality of life. METHODS: All patients with cryptogenic fibrosing alveolitis attending Nottingham City Hospital were invited to enter the study. Spirometric measurements and capillary blood gas tensions were obtained and overnight oxygen saturation was recorded at home. Quality of life was assessed using the Short Form-36, Chronic Respiratory Questionnaire, Hospital Anxiety Depression Scale, and Epworth Sleepiness Score questionnaires. RESULTS: Sixty seven eligible patients were identified and 50 agreed to enter the study, although two were subsequently excluded because they already used oxygen overnight. In the remaining 48 the mean (SD) overnight oxygen saturation (SaO(2)) was 92.5 (4.3)% and the median number of dips greater than 4% per hour was 2.3 (interquartile range 1.5-5.3). Daytime oxygen level predicted mean overnight SaO(2) (1.94%/kPa, 95% CI 1.22 to 2.66, p<0.001) but percentage predicted forced vital capacity (FVC) did not (0.018%/% predicted FVC, 95% CI -0.04 to 0.08, p=0.5). Nocturnal hypoxaemia was associated with decreased energy levels and impaired daytime social and physical functioning, and these effects were independent of FVC. CONCLUSIONS: Nocturnal hypoxaemia is common in patients with cryptogenic fibrosing alveolitis and may have an impact on health related quality of life.

Aged↗
PubMed1977

Fibrosing alveolitis with autoimmune haemolytic anaemia: two case reports.

Two patients with fibrosing alveolitis and autoimmune haemolytic anaemia are described. One patient also had neurofibromatosis. The haematological associations of fibrosing alveolitis are discussed, and a possible relationship between autoimmune haemolysis and fibrosing alveolitis is suggested.

Aged↗
PubMed1983

Thoracoabdominal mechanics during tidal breathing in normal subjects and in emphysema and fibrosing alveolitis.

Thoracoabdominal motion and intrathoracic (oesophageal), intra-abdominal (gastric), and transdiaphragmatic pressures were measured during tidal breathing in 20 erect subjects--six normal, seven, with emphysema, and seven with fibrosing alveolitis. In normal subjects all diameters increased synchronously during inspiration and were accompanied by rises in abdominal and transdiaphragmatic pressures. Five patients with fibrosing alveolitis showed a reduction in upper ribcage motion, but normal lower ribcage and abdominal motion; stiff fibrotic lungs may sometimes impair the ability of the diaphragm to expand the upper ribcage and result in proportionally more expansion of the bases. Four emphysematous patients showed increased anteroposterior motion of the ribcage and four showed paradoxical inward motion of the lower ribcage during inspiration. These changes apparently result from increased activity of intercostal and accessory muscles and altered configuration of the diaphragm. The muscles of patients with large, overinflated lungs are at a greater disadvantage than those with small fibrosed lungs.

Abdomen↗
PubMed1988

Cardiovascular function at rest and on exercise in patients with cryptogenic fibrosing alveolitis.

Cardiovascular complications are common in fibrosing alveolitis, but there have been few physiological studies of the pulmonary circulation in this condition, and those that have been carried out have usually depended on right heart catheterisation. This paper reports non-invasive measurements of effective pulmonary blood flow, oxygen uptake, pulmonary arteriovenous oxygen content differences, and estimates of mixed venous oxygen saturation in 20 patients with histologically proved cryptogenic fibrosing alveolitis at rest and while exercising on a motorized treadmill. Results were compared with those of 20 age and sex matched normal subjects, at rest and at an arbitrarily chosen oxygen uptake of 0.75 l/min. The latter results were obtained by linear interpolation. Effective pulmonary blood flow was normal at rest, but oxygen dispatch to the tissues (blood flow x blood oxygen content) was significantly reduced at rest (mean reduction 190 (SD 68) ml/l/min; p less than 0.01) and at an oxygen uptake of 0.75 l/min (mean reduction 128 (50) ml/l/min; p less than 0.02), reflecting the presence of systemic arterial hypoxaemia. Pulmonary arteriovenous oxygen content differences were similar in patients and normal subjects, but mixed venous saturation was lower in the patients at rest (mean % reduction 6.8 (2.6); p less than 0.02) and at an oxygen uptake of 0.75 l/min (mean % reduction 9.6 (2.9); p less than 0.002). It is concluded that the supply of oxygen potentially available to the tissues is reduced at rest and during exercise in patients with fibrosing alveolitis and hence, by analogy with normal people exercising under hypoxic conditions, that pulmonary blood flow is inappropriately low in this condition. The low mixed venous oxygen saturation may contribute to the development of pulmonary hypertension in some patients. The rebreathing technique used in this study may be of use in monitoring treatment; it could be applied many times to the same patient, and might be a suitable way of following the response to pulmonary vasodilators.

Adult↗
PubMedDate not supplied

Fibrosing mediastinitis.

Fibrosing mediastinitis is a rare benign disorder caused by proliferation of acellular collagen and fibrous tissue within the mediastinum. Although many cases are idiopathic, many (and perhaps most) cases in the United States are thought to be caused by an abnormal immunologic response to Histoplasma capsulatum infection. Affected patients are typically young and present with signs and symptoms of obstruction or compression of the superior vena cava, pulmonary veins or arteries, central airways, or esophagus. There may be two types of fibrosing mediastinitis: focal and diffuse. The focal type usually manifests on computed tomographic (CT) or magnetic resonance (MR) images as a localized, calcified mass in the paratracheal or subcarinal regions of the mediastinum or in the pulmonary hila. The diffuse type manifests on CT or MR images as a diffusely infiltrating, often noncalcified mass that affects multiple mediastinal compartments. CT and MR imaging play a vital role in the diagnosis and management of fibrosing mediastinitis.

Fibrosis↗
PubMed1985

Spontaneous pneumothorax in cryptogenic fibrosing alveolitis.

The prevalence of spontaneous pneumothorax was studied in 82 patients with cryptogenic fibrosing alveolitis. In 46 patients the disease affected only the lung ('lone' fibrosing alveolitis); in the remaining 36 patients the interstitial lung disease was associated with several systemic diseases ('associated' fibrosing alveolitis). 3 patients with the 'lone' form had a spontaneous pneumothorax. This figure represents a prevalence of 3.6%. We did not observe this complication in any patient with the associated form. Suction drainage was ineffective in the 3 patients. One of them died as a consequence of pleural infection. In the other 2 subjects thoracotomy, resection of blebs and pleurectomy were required.

Adult↗
PubMed1994

Fibrosing alveolitis in systemic sclerosis. Bronchoalveolar lavage findings in relation to computed tomographic appearance.

Fibrosing alveolitis in systemic sclerosis is histologically identical to lone cryptogenic fibrosing alveolitis (CFA) (idiopathic pulmonary fibrosis). The inflammatory cell content of bronchoalveolar lavage (BAL) samples has been used as a guide to prognosis and treatment in CFA. In this study, the relationship was explored between BAL findings and the extent and pattern of disease within the lavaged lobe, as judged by thin-section computed tomography (CT), in systemic sclerosis. Thirty-eight nonsmoking patients were studied; none had been treated with corticosteroids or immunosuppressive agents, and 11 had no evidence of fibrosing alveolitis on CT. BAL neutrophils were markedly increased in association with extensive disease on CT compared with less extensive disease (p < 0.001) and normal appearances (p < 0.001); the extent of a reticular pattern on CT (denoting fibrosis) correlated with the neutrophil percentage count (p < 0.005) and total neutrophil count/ml (p < 0.01). BAL eosinophils were increased in less extensive as well as in extensive disease when compared with lobes with a normal CT appearance (p < 0.01); eosinophil percentage counts but not total eosinophil counts/ml correlated with the extent of a ground-glass pattern on CT (through to denote inflammation) (p < 0.05). These findings indicate that in systemic sclerosis a BAL neutrophilia is generally associated with extensive fibrotic disease, whereas a BAL eosinophilia is often seen in less advanced disease, particularly when CT appearances suggest lung inflammation.

Bronchoalveolar Lavage Fluid↗
PubMed1997

Lone cryptogenic fibrosing alveolitis: a functional-morphologic correlation based on extent of disease on thin-section computed tomography.

In most clinical series of patients with cryptogenic fibrosing alveolitis (CFA), disease severity is staged using lung function indices. However, many physiologic indices are measured in routine clinical practice; the choice of variable to evaluate functional severity is contentious. Computed tomography (CT) provides a reproducible means of quantifying the morphologic extent of disease. The aim of this study was to evaluate the functional consequences of smoking-related lung damage in CFA and to identify functional measures best reflecting the extent of fibrosing alveolitis on CT. Sixty-eight patients with CFA were studied. Fourteen patients with emphysema on CT were characterized by relative preservation of FVC and TLC (p < 0.005) and relative depression of DLCO (p < 0.05) and KCO (p < 0.00005). On multivariate analysis, the extent of fibrosing alveolitis and the presence of emphysema were independent determinants of functional impairment; there was no independent relationship between smoking history and functional abnormalities. In patients without emphysema on CT, percent predicted DLCO (r = -0.68), oxygen desaturation on exercise (r = 0.64), and the physiologic component of the clinical-radiographic-physiologic (CRP) score (r = 0.62) correlated much better with the extent of disease on CT than spirometric and plethysmographic volumes. A composite functional index was generated against the extent of disease on CT, using multivariate analysis; comparison with the CRP score suggested that the relationship between morphologic disease extent and the CRP score would be improved by the inclusion of DLCO and by the use of negative weighting for depression of FEV1. These findings indicate that in CFA, the presence of concurrent emphysema on CT has a more profound influence upon functional measures than the smoking history, and underline the importance of both the measurements of DLCO and exercise testing in the assessment of the severity of CFA.

Case-Control Studies↗
PubMed1993

Mast cells' contribution to the fibrosing alveolitis of the scleroderma lung.

Fibrosing alveolitis is frequently seen in scleroderma. Although usually causing progressive symptomatology, it may be found in patients reporting no respiratory symptoms. Mast cells may play a role in the pathogenesis of skin fibrosis in scleroderma. We determined the mast cells and other cellular content, as well as measuring the inflammatory mediators in the bronchoalveolar lavage fluid (BALF) in 17 scleroderma patients and nine control subjects to correlate BALF features with fibrosing alveolitis as determined by lung function testing and high-resolution computed tomographic scans. Bronchoalveolar lavage cells were enumerated after May-Giemsa-Grünwald (MGG), alcian blue, and safranine blue staining. Histamine, tryptase, eosinophil cationic protein, hyaluronic acid, and neutrophil-specific myeloperoxidase were measured by radioimmunoassay. In comparison with normal subjects the BALF of scleroderma patients showed an increased percentage of mast cells (p < 0.002, Mann-Whitney U test), and increased levels of histamine (p < 0.005), tryptase (p < 0.02), and hyaluronic acid (p < 0.004). The BALF of the eight scleroderma patients with an abnormal chest X-ray had a significantly greater number of mast cells (p < 0.04, Mann-Whitney U test), and significantly higher levels of histamine (p < 0.03, Mann-Whitney U test) and tryptase (p = 0.02, Mann-Whitney U test) than the nine scleroderma patients with a normal chest X-ray. This study demonstrates the importance of mast cells and mast-cell activation in the pathogenesis of the fibrosing alveolitis of scleroderma.

Adult↗
PubMed1995

Fibrosing alveolitis in systemic sclerosis: increase in memory T-cells in lung interstitium.

Despite the large numbers of T-cells present in the lungs in fibrosing alveolitis, their pathogenetic role is poorly understood. If these cells are involved in pathogenesis, they are more likely to express the CD45RO+ memory phenotype. To test this hypothesis, open lung biopsies from patients with fibrosing alveolitis associated with systemic sclerosis (FASSc) were compared with grossly normal lung taken from the periphery of lobes resected for lung cancer. Biopsies from eight patients with FASSc were compared with tissue from seven cancer controls. Paraffin sections were stained with a polyclonal anti-CD3 antibody for T-lymphocytes, monoclonal anti-CD45 antibody for leucocyte common antigen, and monoclonal anti-CD45RO antibody for primed T-lymphocytes. Staining was assessed quantitatively by computerized image analysis: in each case, the number of immunopositive cells was related to alveolar wall area and alveolar wall length. Mean alveolar wall thickness was increased in patients with FASSc (60.7 +/- 24.0 microns) compared with cancer controls (15.7 +/- 5.3 microns). Patients with FASSc had greater numbers of CD45+, CD3+ and CD45RO+ cells.mm-1 alveolar wall length compared with the controls. CD45RO+ cells made up 77% (median) of the CD3+ cells in FASSc, and their numbers per unit alveolar wall length were positively associated with alveolar wall thickness (r = 0.61). In conclusion, in fibrosing alveolitis of systemic sclerosis, most interstitial T-lymphocytes express the phenotype of memory cells; these cells are likely to be involved in the persistent inflammatory process.

Adult↗
PubMed1996

Averaged and time-gated spectral analysis of respiratory sounds. Repeatability of spectral parameters in healthy men and in patients with fibrosing alveolitis.

STUDY OBJECTIVE: To obtain a basis for assessment of changes in breath sound spectra in patients with pulmonary diseases, short-term and day-to-day repeatability of spectral parameters was studied. DESIGN: Breath sounds were recorded simultaneously from the trachea and from the chest twice at an interval of 15 min (short-term repeatability) and of 1 to 3 days (day-to-day repeatability). During recordings, air flow at the mouth was controlled, the target inspiratory and expiratory peak flow being 1.25 L/s. Inspiratory and expiratory breath sound spectra were averaged over 7 to 10 successive respiratory cycles. The repeatability of sound intensity (RMS), frequency of maximum intensity (Fmax), and median frequency (F50) was analyzed with analysis of variance. PARTICIPANTS: Short-term repeatability was studied in 10 healthy nonsmoking men (age 25 to 44 years), and day-to-day repeatability was studied in 10 healthy nonsmoking men (age 23 to 41 years) and in 12 patients with clinically stable fibrosing alveolitis (age 35 to 82 years). RESULTS: Short-term coefficient of variation (CoV) of Fmax and F50 was 2.6 to 6.7% when recorded from the chest, and 6.2 to 8.7% when recorded from the trachea. Day-to-day CoV of Fmax and F50 in healthy subjects was 4.7 to 8.5% and 5.0 to 8.7% recorded from the chest or from the trachea, respectively. Inspiratory day-to-day variation in those parameters was higher in patients with fibrosing alveolitis. CoV of RMS was high, ranging from 18 to 47% in different subject groups and sampling situations. CONCLUSIONS: Repeatability of F50 of averaged flow-controlled lung sound spectra is good both in healthy subjects and in patients with fibrosing alveolitis. Thus, F50 of respiratory sound spectra may be useful in monitoring of changes induced by respiratory diseases and interventions. These results emphasize the importance of standardization of recording conditions and of analyzing techniques.

Adult↗
PubMed1979

Mediastinal granuloma and fibrosing mediastinitis.

Thirty-one patients with mediastinal granuloma and fibrosing mediastinitis were seen at the Mayo Clinic from 1975 through 1977. Review of this series reveals that surgery is necessary to establish a diagnosis if the lesions are noncalcified and indeterminate. Fibrosing mediastinitis most likely develops after rupture of the fibrocaseous material from mediastinal lymph nodes into the mediastinum. Thoracotomy, with evacuation of the granulomas, is recommended, especially when the lesions are large, in order to prevent subsequent fibrosing mediastinitis with involvement of the contiguous structures, such as the superior vena cava, azygos vein, trachea, esophagus, and left atrium. In most patients, obstruction of the superior vena cava develops slowly, and efficient collateral venous circulation occurs, allowing long-term survival and minimal disability.

Adolescent↗
PubMed1982

Arterial PCO2 as an index of activity in fibrosing alveolitis.

Alveolar hyperventilation is a characteristic feature of the interstitial lung diseases, yet its pathogenesis remains unknown. We examined the relationship between inflammatory alveolar acinar cell counts and the steady state, resting arterial PCO2 in patients with fibrosing alveolitis. To eliminate the influence of overwhelming mechanical lung restriction or resting hypoxemia, we studied 20 patients who, despite having clinicopathologically confirmed fibrosing alveolitis, had vital capacities exceeding 50 percent of predicted, and arterial O2 saturations above 90 percent. There was a significant inverse relationship between the proportion of polymorphonuclear leukocytes (PMNs) in the recovered BAL fluid and the arterial PCO2 (r = -0.67; p less than 0.01). When PCO2 was above 35 mm Hg, the BAL PMN count was 8 percent or less (mean = 3.4; SD = 2.5), while the mean BAL PMN count among those patients whose PCO2 was less than 35 mm Hg was significantly higher (mean = 11.7; SD = 3.7; p less than 0.01). PCO2 levels were unrelated to arterial O2 saturation or PaO2. No relationship was found between the PCO2 and BAL lymphocyte counts. The findings suggest that in fibrosing alveolitis, the arterial PCO2 may be used as an indicator of the state of the inflammatory component of the alveolitis.

Carbon Dioxide↗
PubMed1986

Genetic studies in familial fibrosing alveolitis. Possible linkage with immunoglobulin allotypes (Gm).

A family containing 12 subjects spanning three generations and including six cases with clinical evidence of definite or probable fibrosing alveolitis has been investigated. Histologic confirmation was available for three cases. The subjects were between 15 and 54 years of age at diagnosis. Although the size of the sample is small, the mode of inheritance of fibrosing alveolitis within this family appeared to be dominant with incomplete penetrance. HLA typing showed that at least one of the affected siblings did not share any HLA haplotypes with other affected siblings in the third generation. This makes it unlikely that a disease gene would be in association with HLA genes on chromosome 6. In contrast, all affected siblings, as well as two as yet unaffected siblings, carried the immunoglobulin haplotype Gm 1. These studies indicate that familial fibrosing alveolitis in this family may be inherited by a dominantly inherited gene located on chromosome 14 close to the loci encoding for Gm.

Adolescent↗
PubMed1986

Pulmonary vascular occlusion and fibrosing mediastinitis.

Patients with fibrosing mediastinitis causing obstruction of pulmonary veins and arteries may present with many of the historic, physical, and laboratory findings of patients with pulmonary hypertension due to chronic thrombotic obstruction of major pulmonary arteries. Because the latter is subject to surgical correction, and the former is not, it is important to differentiate between the two and to be aware of the similarities in presentation. Three patients with pulmonary hypertension due to compression of pulmonary veins and the right pulmonary artery by fibrosing mediastinitis are presented who illustrate these points. A review of the literature documents other instances in which vascular involvement due to fibrosing mediastinitis has mimicked other types of pulmonary hypertension.

Adult↗
PubMed2000

Combined Riedel's Disease and Fibrosing Hashimoto's Thyroiditis: A Report of Three Cases with Two Showing Coexisting Papillary Carcinoma.

Extensive sclerosis of the thyroid gland can be seen in both benign and malignant conditions. The benign sclerosing lesions of the thyroid include Riedel's disease and fibrosing Hashimoto's thyroiditis. Although these conditions usually occur separately, rarely can they occur simultaneously. In malignant lesions, papillary thyroid carcinoma and anaplastic carcinoma of the thyroid can be associated with extensive sclerosis leading to partial or total effacement of the tumor. We report on three cases that showed simultaneous occurrence of Riedel's disease and fibrosing Hashimoto's thyroiditis. Two of these cases also showed papillary carcinoma (one case of Warthin's-like papillary carcinoma and one case of classic type). All patients were females (age range 32-67 yr) and presented with elevated antithyroglobulin antibodies. Two patients presented with a solitary thyroid mass, and from these one had multiple bilateral neck nodes and a paravertebral mass. The third patient presented with a multinodular gland adherent to neck structures. All patients underwent total thyroidectomy. Histologic sections showed extensive replacement of the thyroid parenchyma with dense keloidal fibrosis, intermixed well-developed lymphoid follicles, and scattered lymphocytes and plasma cells. In all cases the fibrotic process extended beyond thyroid capsule with involvement of the perithyroidal soft tissues and skeletal muscle consistent with Riedel's disease. One case showed a classic papillary carcinoma with bilateral lymph node metastases, and the other showed a Warthin's-like papillary carcinoma. In both cases the papillary cancers were surrounded by dense sclerosis. Immunohistochemical stains for B- and T-markers and immunoglobulin light chains showed a polyclonal population of the lymphoid cells. The simultaneous occurrence of Riedel's disease and fibrosing Hashimoto's thyroiditis is rare and most likely represents a coincidental phenomenon, because both of these conditions are distinct clinicopathologic entities.

Journal Article↗
PubMed1985

Magnetic resonance imaging of fibrosing mediastinitis.

Magnetic resonance imaging (MRI) was performed in seven patients with fibrosing mediastinitis. Comparison was made in each case to standard chest radiography and computed tomography (CT). Angiography was performed in three cases. Although MRI and CT were found to be equivalent in defining the extent of adenopathy, CT was superior at demonstrating calcifications, often important in making the diagnosis of fibrosing mediastinitis. MRI, however, offered complementary information, particularly in assessing vascular patency without the need for intravenous contrast media. On T2-weighted images, the adenopathy associated with fibrosing mediastinitis was noted to be of relatively low signal intensity, possibly indicating its benign nature.

Adult↗