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Results for “FIBROSITIS”

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PubMed1990

Pulmonary gamma interferon production in patients with fibrosing alveolitis.

Patients with fibrosing alveolitis have active inflammation within their lung interstitium. Previous studies have focused on the humoral (immune complex) driven processes. In this study increased pulmonary gamma interferon production has been evaluated. Bronchoalveolar lavage cells were obtained from 40 patients with fibrosing alveolitis, 22 with cryptogenic fibrosing alveolitis, and 18 with connective tissue disease associated (CTD) fibrosing alveolitis. Increased gamma interferon production was seen in 12 (30%) patients and was similar in the two study groups. Up to 512 units/10(6) cells were released over 24 hours, showing that the amounts of gamma interferon released could be as large as those seen in other pulmonary diseases associated with active cellular immune processes, such as sarcoidosis. Spontaneous gamma interferon production was related to increased serum concentrations of IgG and IgM but not to serum IgA, antinuclear antibody, or rheumatoid factor titres. There was no relation between gamma interferon production and pulmonary uptake of gallium-67 citrate. The ratio of helper-inducer (Leu-3) to suppressor-cytotoxic (Leu-2) cells in bronchoalveolar lavage fluid was similar in the two study groups and was similar in patients whose cells produced gamma interferon and those whose cells did not. These data suggest that gamma interferon is released in the lungs of a proportion of individuals with cryptogenic fibrosing alveolitis and CTD-fibrosing alveolitis, suggesting a role for this cytokine in mediating these diseases.

Bronchoalveolar Lavage Fluid↗
PubMed2000

Lung cancer and cryptogenic fibrosing alveolitis. A population-based cohort study.

Cryptogenic fibrosing alveolitis has been reported to be associated with an increased risk of lung cancer. However, it has recently become apparent that cigarette smoking may be a risk factor for cryptogenic fibrosing alveolitis as well as for lung cancer, and so may confound the association between these conditions. We have therefore estimated the independent increase in lung cancer incidence in patients with cryptogenic fibrosing alveolitis compared with the general population in a population-based cohort study involving 890 subjects with cryptogenic fibrosing alveolitis and 5, 884 control subjects drawn from the United Kingdom General Practice Research Database. The incidence of lung cancer was markedly increased among patients with cryptogenic fibrosing alveolitis (rate ratio [RR] 7.31, 95% confidence interval [95% CI] 4.47 to 11.93, p < 0.001), and adjustment for previous smoking history had little effect on this odds ratio (adjusted RR: 8.25, 95% CI 4.70 to 11.48, p < 0.001). This increase in lung cancer incidence remained when the analysis was restricted to current smokers (RR 7.36, 95% CI 1.54 to 35.19, p = 0.012). This study provides clear evidence that the incidence of lung cancer is increased in patients with cryptogenic fibrosing alveolitis, and that this effect is independent of the effect of cigarette smoking.

Aged↗
PubMed1993

The predictive value of appearances on thin-section computed tomography in fibrosing alveolitis.

Fibrosing alveolitis, a condition characterized by a poor prognosis and a limited response to treatment, is readily identified by thin-section computed tomography (CT). Fibrotic and inflammatory histologic results, obtained at open lung biopsy, both have characteristic CT patterns. To evaluate whether CT could predict prognosis and response to therapy, we examined the CT appearances of 76 patients with lone cryptogenic fibrosing alveolitis and 66 patients with fibrosing alveolitis associated with systemic sclerosis. CT abnormalities were categorized as predominantly a ground-glass pattern (Grade 1), mixed (Grade 2), or predominantly a reticular pattern (Grade 3). In cryptogenic fibrosing alveolitis, 4-yr survival was highest in association with CT Grade 1 and higher with CT Grade 2 than with CT Grade 3, independent of the extent of abnormal lung on CT, duration of dyspnea, or severity of depression of DLCO or FVC, p < 0.001. A response to therapy in previously untreated cryptogenic fibrosing alveolitis was seen most frequently with CT Grade 1 and more frequently with CT Grade 2 than with CT Grade 3, p < 0.025. In systemic sclerosis, CT appearances were not predictive of 4-yr survival; a response to therapy was seen more frequently with CT Grade 2 (three of seven patients) than with CT Grade 3 (zero of six patients). These data have shown that CT appearances are of prognostic value in fibrosing alveolitis and that they are likely to play an increasing role in disease-staging in this condition.

Adult↗
PubMed2005

Cyclophosphamide and low-dose prednisolone in idiopathic pulmonary fibrosis and fibrosing nonspecific interstitial pneumonia.

The present study compared the efficacy of cyclophosphamide combined with low-dose prednisolone in the treatment of idiopathic pulmonary fibrosis (IPF) with efficacy in idiopathic fibrosing nonspecific interstitial pneumonia fibrosing (NSIP). A total of 27 patients with IPF and 12 patients with fibrosing NSIP were included in this study. All patients had undergone surgical lung biopsy. The diagnoses were made based on clinical, radiological and pathological findings. All patients were treated with intermittent pulse therapy with methylprednisolone for 4 weeks, followed by cyclophosphamide with low-dose prednisolone. According to pulmonary function tests, four of 27 patients with IPF had improved, 22 remained unchanged, and one had worsened at the completion of pulse therapy. After 1 yr of combination therapy, four of 27 patients had improved, 14 remained unchanged, and nine had worsened. After pulse therapy, four of 12 patients with fibrosing NSIP had improved, and eight remained unchanged. After 1 yr of combination therapy, eight of 12 patients had improved, four remained unchanged, and none had worsened. Median survival of IPF patients was 4.1 yrs, which is significantly worse than that of fibrosing NSIP patients. In conclusion, patients with fibrosing nonspecific interstitial pneumonia had a more favourable response to combination therapy and a better survival than those with idiopathic pulmonary fibrosis.

Anti-Inflammatory Agents↗
PubMed1983

Prevalence of primary and secondary fibrositis.

Of 1,473 consecutive new patients seen in an outpatient rheumatology clinic, 3.7% met criteria for "primary fibrositis." Secondary fibrositis was diagnosed in 12.2% of patients with rheumatoid arthritis (RA), 15.7% of patients with primary neck and back pain syndromes and 6.7% of patients with osteoarthritis (OA). When conditions presumed to be associated with secondary fibrositis were excluded, primary fibrositis was identified in 55 of 405 patients or 13.6%. Two hundred fifteen or 14.6% of all patients had either primary or secondary fibrositis. Fibrositis may be the most common disorder seen in rheumatic disease practice after OA and RA.

Arthritis, Rheumatoid↗
PubMed1985

Clinical characteristics of fibrositis. II. A "blinded," controlled study using standard psychological tests.

Twenty-two patients with fibrositis and 22 control patients selected from a general medical outpatient population were given 3 standardized psychological questionnaires: the Beck Depression Inventory, the Spielberger State and Trait Anxiety Inventory, and the SCL-90-R. There were no statistically significant differences between fibrositis patients and control patients on any of these tests, a finding at variance with a commonly held belief that patients with fibrositis have an underlying psychological disorder. While psychological factors may be important in some patients with fibrositis, these results indicate that the presence of a psychopathologic condition is not mandatory for the persistence of fibrositis.

Adult↗
PubMed1977

Fibrosing mediastinitis causing pulmonary arterial hypertension without pulmonary venous hypertension. Clinical and necropsy observations.

Clinical and morphologic observations are described in two patients with severe pulmonary arterial hypertension without pulmonary venous hypertension from fibrosing mediastinitis. In one patient, both main pulmonary arteries and one major pulmonary vein were severely narrowed by dense fibrous tissue; in the second patient, only the right main pulmonary artery was severely narrowed. Both patients had normal intrapulmonary arteries and normal pulmonary parenchyma. Of nine previously described necropsy patients with pulmonary hypertension due to fibrosing mediastinitis, seven had severe narrowing of multiple large pulmonary veins and in six of them the pulmonary hypertension was entirely due to pulmonary venous obstruction. In one other patient, the pulmonary hypertension was due to obstruction of one main pulmonary artery and several large pulmonary veins. Each of these seven previously described patients had severe changes in the small intrapulmonary arteries. Of the other two previously described patients with pulmonary hypertension from fibrosing mediastinitis, one had severe narrowing of only the main right pulmonary artery, and the other, of both main pulmonary arteries. Thus, although pulmonary arterial hypertension in patients with fibrosing mediastinitis is usually due to obstruction of multiple large pulmonary veins and to severe secondary changes in small intrapulmonary arteries, fibrosing mediastinitis can cause severe pulmonary hypertension by obstructing the right or both main pulmonary arteries.

Adult↗
PubMed1986

Psychologic studies in fibrositis.

During the past five years, there have been a number of controlled reports regarding the possible association of fibrositis with psychologic disorders. The results of these studies are quite different, which is not surprising in view of the differences in patient populations and study instruments used. We gave the Diagnostic Interview Schedule to 82 patients with fibrositis and to control subjects, and we found an association of depression with fibrositis. Depression was also more common in first-degree relatives of patients with fibrositis. However, in most instances, the depression antedated fibrositis by more than one year, indicating a possible psychobiologic association, rather than a casual one.

Arthritis, Rheumatoid↗
PubMed1986

Development of criteria for the diagnosis of fibrositis.

The essential symptoms of fibrositis--widespread aching and pain, disturbed sleep, morning stiffness, and fatigue--are common in both rheumatic and nonrheumatic patients. But the essential sign of fibrositis--widespread local tenderness over specific anatomic sites ("tender points")--is rare in any patients except those with fibrositis. Clinical criteria for the diagnosis of fibrositis rely heavily on a high tender point count in the presence of characteristic fibrositic symptoms. Multiple tender points are uncommon in normal subjects and in those with rheumatic and nonrheumatic disorders. The tender point count thus also serves to distinguish fibrositis from other musculoskeletal diseases.

Fibromyalgia↗
PubMed1981

An analysis of submaximal exercise responses in patients with sarcoidosis and fibrosing alveolitis.

An increase work rate exercise test was performed by 15 patients with sarcoidosis and by 20 patients with fibrosing alveolitis. The patients with sarcoidosis had a moderate reduction in total lung capacity (TLC) and transfer factor (DLCO), with chest radiographs showing widespread pulmonary infiltration but no evidence of fibrosis. The patients with fibrosing alveolitis had a significantly greater reduction in TLC and DLCO than those in the sarcoidosis group. Values for cardiac frequency (fH) and ventilation(V) were interpolated to the standard oxygen uptakes of 0.75, 1.0 and, where possible, 1.5 litres/min (33.5, 44.6 and 67 mmol/min respectively). The tidal volume at the ventilation of 20 and 30 litres/min was also determined. The exercise responses were compared to two groups of 20 normal men; each group being age matched to one group of patients. The fH at oxygen uptakes of 0.75, 1.0 and 1.5 litres/min were significantly higher in both patient groups than in the normal men. The submaximal indices for V were significantly greater in both patients groups than in the normal subjects at all three levels of oxygen uptake, and significantly greater in patients with fibrosing alveolitis than in those with sarcoidosis. The tidal volumes at 20 and 30 litres/min were smaller than normal in both patient groups but differences were removed by normalizing for differences in vital capacity. The maximum exercise ventilation measured in the patients with fibrosing alveolitis was significantly correlated with measurements of lung volume. Submaximal indices detect significant abnormalities during exercise in patients with pulmonary fibrosis and represent an alternative method for documenting abnormal exercise responses. Despite comparable radiological abnormalities the functional impairment in fibrosing alveolitis is much greater than in sarcoidosis. Thus the physiological abnormalities are not comparable quantitatively although they share a common qualitative difference.

Adult↗
PubMed2000

Exposure to antidepressants and the risk of cryptogenic fibrosing alveolitis: a case-control study.

The explanations for the emergence of cryptogenic fibrosing alveolitis as a new clinical entity during the second half of the 20th century are unclear. The authors have previously reported evidence of an increased risk of cryptogenic fibrosing alveolitis in relation to the use of antidepressant drugs. The authors have now tested this hypothesis a priori in an analysis of computerized general practice records for 890 cases of cryptogenic fibrosing alveolitis and 5,884 matched controls drawn from the UK General Practice Research Database. Exposure to antidepressants at the time of diagnosis was increased in cases compared to controls (odds ratio (OR) 1.52, 95% confidence interval (95% CI) 1.24-1.86), and this increase remained if the analysis was restricted to exposures 4 yrs prior to diagnosis (OR 1.50, 95% CI 0.98-2.30). However this increased prescribing was not specific to any particular class of antidepressant or individual drug, and there was no evidence of a dose-response relationship between exposure to amitriptyline (the most commonly prescribed antidepressant) and disease. The presented data do not allow any firm conclusion to be made as to whether there is a causal relationship between antidepressant exposure and cryptogenic fibrosing alveolitis, but it seems unlikely that exposure to tricyclic antidepressants shortly before diagnosis is a strong risk factor for cryptogenic fibrosing alveolitis.

Aged↗
PubMed1999

T-cell receptor gene usage in patients with fibrosing alveolitis and control subjects.

BACKGROUND: Fibrosing alveolitis is characterized by inflammation, fibrosis and increased numbers of activated CD4+ T-cells in the lower respiratory tract. The aims of this study were to compare the T-cell antigen receptor repertoire in the lungs of subjects with fibrosing alveolitis systemic sclerosis (FASSc) with cryptogenic fibrosing alveolitis (CFA) and normal control subjects, to determine whether FASSc is driven by a specific T-cell trigger and is determined by a T-cell driven immune response, and to assess the clonality of CD4+ and CD8+ TcR usage in subjects with FASSc. MATERIALS AND METHODS: We used reverse transcription polymerase chain reaction with specific V alpha- and V beta-chain primers to identify the TcR gene usage in biopsy material, bronchoalveolar lavage fluid or peripheral blood from our subjects. RESULTS: We found individual-specific restriction of V alpha- and V beta-chain usage in lung biopsies from patients and control subjects. To establish whether this was due to expression bias in the CD4+ or CD8+ T-cells and was restricted to the lung, the alpha beta-T-cell receptor chain usage was assessed in T-cell subsets separated from the lungs of patients with fibrosing alveolitis and was compared with that of the peripheral blood. There was no consistent difference in the expression of any variable family chain among the population studied, although there was a significant difference between lung and peripheral blood lymphocyte V beta-families in CD8+ T-cells (P = 0.0007). CONCLUSION: We conclude that there is individual TcR V alpha- and V beta-expression bias in subjects with fibrosing alveolitis.

Adult↗
PubMed1999

[Histomorphological differential diagnosis between occupational and "idiopathic" pulmonary fibroses].

Pulmonary fibroses are terminal stages of exposure to a whole series of very diverse noxae. Both occupational and nonoccupational causes must be distinguished. Finally, there is the large collective category "idiopathic" fibroses. In terms of their systematics, fibroses can be classified in five largegroups. The classification criteria are etiological when the causes are known, and morphological-descriptive in cases of idiopathic fibrosis. Occupational and "idiopathic" fibroses often cannot be distinguished in biopsy material because of their great histomorphological similarity. In such circumstances, the result of further analyses is crucial. For this reason, the possibility that an occupational pulmonary fibrosis is present must also be considered even in "idiopathic" pulmonary fibroses in order to arrange for further analyses such as BAL, ashing and energy-dispersive X-ray analysis to be performed.

Diagnosis, Differential↗
PubMed1992

In vivo levels and in vitro production of interferon-gamma in fibrosing interstitial lung diseases.

The in vivo role of interferons in the development of fibrosis is not fully understood but it is known that interferons can suppress fibroblast proliferation and collagen synthesis in vitro. We have recently demonstrated that in a group of patients with sarcoidosis having predominant pulmonary involvement, patients with the highest levels of circulating interferon-gamma (IFN-gamma) more frequently resolved on corticosteroids, suggesting that they had a less 'fibrotic' component to their disease. We now report that in two other diseases, where the tendency to develop pulmonary fibrosis is greater than in sarcoidosis, namely cryptogenic fibrosing alveolitis (CFA) and fibrosing alveolitis associated with the systemic connective tissue disease progressive systemic sclerosis (FA + PSS), very few patients have elevations in IFN-gamma in their serum. However, as in sarcoidosis, those with the highest levels responded to corticosteroids (P less than 0.05). Attempts to measure IFN-gamma levels in the lungs, using cell-free bronchoalveolar lavage (BAL) fluid supernatants, were negative in all the study groups, suggesting that these samples may be inadequate for such studies. To investigate whether there might be an intrinsic defect in T lymphocyte function associated with predisposition to fibrosing lung diseases, we then investigated the in vitro production of IFN-gamma by lymphocytes separated from the blood of 18 untreated patients (six with CFA, six with FA + PSS and six with sarcoidosis). IFN-gamma production was impaired in 10 (56%) (two with CFA, four with FA + PSS and four with sarcoidosis). A higher proportion of the fibrosing alveolitis patients (CFA or FA + PSS) with impaired IFN-gamma production have subsequently shown spontaneous lung functional deterioration. These findings suggest that impaired IFN-gamma release might be a potentiating factor in the pathogenesis of these fibrosing lung diseases.

Adult↗
PubMed1984

Carpal tunnel syndrome and subsequent rheumatoid arthritis in the 'fibrositis' syndrome.

Eight of 11 patients who satisfy Smythe's criteria for the 'fibrositis' syndrome have subsequently developed carpal tunnel syndrome. Six of the 8 patients have gone on to develop an inflammatory polyarthritis, 3 of whom are seropositive and satisfy the criteria for classical rheumatoid arthritis. The relationship between the 'fibrositis' syndrome and rheumatoid arthritis is not clear, but the additional complication of carpal tunnel syndrome in 'fibrositis' points to certain patients with 'fibrositis' developing an inflammatory polyarthritis of rheumatoid type. Acceptance on a definition of 'fibrositis' would enable further prospective studies to take place, but at present the term is used to describe many other nonarticular conditions that do not satisfy Smythe's criteria.

Adult↗
PubMed1988

Functional similarities of asbestosis and cryptogenic fibrosing alveolitis.

The pathological features in the lung in asbestosis and cryptogenic fibrosing alveolitis are similar. Patients with asbestosis, however, appear to have less severe impairment of transfer factor (TLCO) than those with fibrosing alveolitis for a given level of radiographic abnormality when assessed on the basis of the International Labour Organisation (ILO) profusion score. The impairment of lung function in the two disorders has been compared in more detail in 29 patients with asbestosis and 25 with fibrosing alveolitis, arterial oxygen desaturation during exercise being used to define the severity of the disorders. Arterial oxygen saturation (ear oximeter) and oxygen uptake were measured during incremental exercise on a cycle ergometer. TLCO (single breath technique) and total lung capacity (TLC, plethysmograph) were measured. Chest radiographs were graded for profusion according to the ILO international classification. Patients with asbestosis had significantly higher mean values for TLCO and TLC and lower mean profusion scores than those with fibrosing alveolitis. When stratified for the degree of arterial oxygen desaturation, however, no significant differences were found in TLCO, TLC, or profusion score between the two disorders. To the extent that arterial oxygen desaturation with exercise reflects the morphological severity of the disease, these results suggest that, for a given degree of interstitial lung disease, asbestosis and cryptogenic fibrosing alveolitis are functionally and radiologically similar.

Asbestosis↗
PubMed1989

Decreased pulmonary distensibility in fibrosing alveolitis and its relation to decreased lung volume.

The relation between pulmonary distensibility, lung volume, and elastic recoil pressure was examined in 45 patients (38 men) with cryptogenic fibrosing alveolitis (mean age 61 (SD 14) years). Exponential analysis of static pressure-volume data obtained during deflation of the lungs gave the exponent K, an index of the distensibility of the remaining inflatable lung tissue. Total lung capacity (TLC) was measured in a body plethysmograph or by nitrogen washout. The results were compared with values obtained in 147 healthy subjects (95 men), of mean age 39 (SD 16) years. Fibrosing alveolitis decreased K by 0.62 (SEM 0.04) kPa-1. This decrease was approximately equal to 2 SD of the regression of log K on age in healthy subjects. TLC was decreased to a mean of 70% (SD 14%) predicted in the patients with fibrosing alveolitis. Lung recoil pressure at maximum inspiration was about twice the expected value and regression analysis showed that most of this increase was related to the decreased K rather than to the decreased TLC. In the men with fibrosing alveolitis the regression of height standardised TLC (TLC/Ht3) on K was significant (p less than 0.02); the regression slope was similar to that for 95 healthy men, but was displaced to a smaller lung volume. The dependence of TLC/Ht3 on K is consistent with the close relation between K and peripheral airspace size found in normal lungs. In fibrosing alveolitis decreased pulmonary distensibility probably reflects a decrease in airspace size, whereas most of the decrease in lung volume reflects the loss of inflatable tissue in the fibrotic process.

Aged↗
PubMed1991

Clinical significance of serum levels of a carbohydrate antigen, sialyl SSEA-1, in patients with fibrosing lung disease.

Serum levels of sialyl SSEA-1 antigen, a carbohydrate antigen, were measured by radioimmunoassay in 142 patients with nonmalignant lung diseases. In 20 of 41 patients with fibrosing lung disease, either idiopathic or associated with collagen disease, the serum sialyl SSEA-1 levels were abnormally elevated. In patients with other lung diseases, the serum levels were almost within normal limits, less than 38.0 units/ml. In fibrosing lung disease the serum levels ranged from 13.8 to 147.0 units/ml and were largely concurrent with the degree of disease activity. The therapeutic effects of corticosteroid, which were evaluated with clinical-radiographic-physiologic scores and survivals in the patients with elevated serum levels, were significantly lower than those of the patients with the normal range of antigen levels. An immunohistochemical study performed on autopsied lungs from five patients with fibrosing lung disease indicated that the antigen was selectively expressed in the pulmonary epithelial cells that covered the remodeling alveolar septi in the lungs. No antigen was detectable by immunostaining in normal pulmonary epithelium among five normal lungs. From these findings, it is thought that the elevated levels of serum sialyl SSEA-1 may be derived from proliferating epithelial cells that were dominant in the late stage of fibrosing lung disorders. The measurements of serum sialyl SSEA-1 in patients with fibrosing lung disease may be clinically useful in establishing the degree of disease activity that has an influence on patient prognosis and therapeutic response.

Adrenal Cortex Hormones↗