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At least 469 records · Page 26Linked to original sources
PubMed1997

Cryptogenic fibrosing alveolitis with preserved lung volumes.

BACKGROUND: Cryptogenic fibrosing alveolitis (CFA) is an uncommon disorder of unknown aetiology characterised by interstitial fibrosis which typically shows a restrictive pattern on pulmonary function testing. Some patients with CFA and relative preservation of lung volumes have been described and it has been suggested that their volume preservation may be due to concomitant emphysema. In a retrospective study the relative frequency of preserved lung volumes in CFA, its relationship to emphysema determined by CT scanning, its clinical features, and its subsequent natural history were investigated. METHODS: Using predefined characteristics 48 patients with CFA were identified from pulmonary function records over a three year period. Volume preservation was defined as a forced vital capacity (FVC) of > 80% predicted at presentation. Patients with relative volume preservation were compared with those with more typical pulmonary restriction and clinical data at presentation, and details of their subsequent prognosis, treatment and loss of lung function with time were obtained. Where available, computed tomographic (CT) scans for the two groups were compared in a blinded fashion to score the extent of fibrosis and the presence of concomitant emphysema. RESULTS: Twenty one (44%) of the patients with CFA had a FVC of > 80% predicted. They were more likely to be male (76% versus 48%) and to be current smokers (57% versus 22%) with a heavier life time cigarette consumption than the restricted patients (mean (SE) 38 (4.6) versus 25 (4.5) pack years). There were no significant differences in prognosis and subsequent treatment between the groups. Comparable HRCT scans were available in 23 subjects (seven preserved, 16 restricted). They showed no difference in extent of the pulmonary fibrosis but patients with volume preservation were more likely to show concomitant emphysema (86% versus 19%). Patients with emphysema on HRCT scans were heavier smokers (41(10) versus 21(17) pack years) than those without emphysema but there was no difference in the extent of CFA score between the two groups. CONCLUSIONS: In this area of high smoking prevalence a significant number of patients with CFA presented with relative preservation of lung volumes and FEV1/FVC ratio. In many of these subjects this appears to reflect coincidental emphysema. This may make interpretation of gas transfer factor used to monitor progression in CFA difficult. However, there was no evidence that lung volumes at presentation were of prognostic significance.

Age of Onset↗
PubMed1998

Detection of anti-cytokeratin 8 antibody in the serum of patients with cryptogenic fibrosing alveolitis and pulmonary fibrosis associated with collagen vascular disorders.

BACKGROUND: It has been suggested that the humoral immune system plays a role in the pathogenesis of cryptogenic fibrosing alveolitis (CFA). Although circulating autoantibodies to lung protein(s) have been suggested, none of the lung proteins have been characterised. The purpose of this study was to determine the antigen to which the serum from patients with pulmonary fibrosis reacted. METHODS: The anti-A549 cell antibody was characterised in a patient with CFA using Western immunoblotting and immunohistochemical staining of A549 cells. As we identified that one of the antibodies against A549 cells was anti-cytokeratin 8, the expression of mRNA of cytokeratin 8 in A549 cells was evaluated. In addition, we attempted to establish an enzyme linked immunosorbent assay to measure the levels of anti-cytokeratin 8 antibody in the serum of patients with CFA and pulmonary fibrosis associated with collagen vascular disorders (PF-CVD). RESULTS: Initially two anti-A549 cell antibodies were detected in the serum of patients with pulmonary fibrosis, one of which was characterised as anticytokeratin 8 antibody by Western immunoblotting. We were able to establish an ELISA to measure anti-cytokeratin 8 antibody and found significantly higher levels in patients with CFA and PF-CVD than in normal volunteers, patients with sarcoidosis, pneumonia, and pulmonary emphysema. CONCLUSIONS: One of the anti-A549 cell antibodies in the serum of patients with CFA was against cytokeratin 8. The serum levels of anti-cytokeratin 8 antibody were increased in patients with CFA and PF-CVD. These results suggest that anticytokeratin 8 antibody may be involved in the process of lung injury in pulmonary fibrosis.

Adult↗
PubMed1992

Fibrosing alveolitis: chest radiography and CT as predictors of clinical and functional impairment at follow-up in 26 patients.

Findings on the original and follow-up chest radiographs and computed tomographic (CT) scans were correlated with clinical and functional parameters in 26 patients with fibrosing alveolitis. Assessment of chest radiographs included determination of a standard profusion score and an average profusion score. The CT assessment included pattern, extent, and distribution of disease. The standard profusion score showed no significant correlation with clinical or functional parameters (P greater than .05). However, the average profusion score of the six lung zones correlated with severity of dyspnea and with static lung volumes (P less than .01). Extent of irregular linear opacities on CT scans correlated with severity of dyspnea and impairment in gas transfer (carbon monoxide-diffusing capacity) (P less than .01). The profusion of ground-glass opacities on the radiograph showed no significant correlations (P greater than .05). The profusion and extent of ground-glass opacities on CT scans correlated with severity of dyspnea, impairment in gas transfer, and reduction in static lung volumes (P less than .01). Ground-glass opacities on CT scans preceded and predicted the development of irregular linear opacities on follow-up CT scans and correlated with an increase in the average profusion score of the chest radiograph (P less than .01).

Adrenal Cortex Hormones↗
PubMed1994

Structure and function in fibrosing alveolitis.

Lung volumes, maximal expiratory volume and flows, elastic recoil pressure, and impedances at various levels of the bronchial tree, as well as tissue impedance, were measured in 13 human lungs that demonstrated generalized fibrosis at autopsy. After fixation, mean internal chord of air spaces (Lma), transsection length of the alveolar walls (Lmw), and internal diameter (d) and density of membraneous bronchioles (n/cm2) were determined in the same lungs by use of morphometric techniques. The results of functional and structural measurements were correlated and compared with measurements obtained similarly in 10 normal lungs. This study shows that the functional alterations typical for fibrosing alveolitis can be attributed to the replacement of part of the parenchyma by fibrous tissue and to modifications of the mechanical properties of still-functional units. The lungs were characterized structurally by an increase of Lmw and d. In addition, in three lungs, an increase of Lma and a decrease of n/cm2 were observed. Functionally, these lungs demonstrated a pattern compatible with a mixture of fibrosis and emphysema.

Adult↗
PubMedDate not supplied

Increased vitronectin and endothelin-1 in the breath condensate of patients with fibrosing lung disease.

BACKGROUND: Non-specific interstitial pneumonia (NSIP) and fibrosing alveolitis associated with systemic sclerosis (FASSc) are diseases of unknown aetiology that are characterised by the accumulation of mononuclear cells, followed by the progressive deposition of collagen within the interstitium and subsequent destruction of lung airspace. Better understanding of mediators involved in fibrosis may be useful for early diagnosis and in clinical monitoring of disease progression. OBJECTIVE: The aim of this study was to investigate the presence of two profibrotic markers, the vitronectin and the endothelin-1 (ET-1) in the airways of NSIP and FASSc patients. METHODS: Ten NSIP (6 males, age 57 +/- 2 years) and 15 FASSc (8 males, age 55 +/- 4 years) patients were recruited along with 10 normal subjects (4 male, age 52 +/- 2 years). Vitronectin and ET-1 concentrations were measured in their breath condensate, using a specific enzyme immunoassay. RESULTS: Higher levels of vitronectin and ET-1 were observed in NSIP and FASSc patients [median 92.8 (91.7-93.9) microg/ml; median 8.3 (7.9-9.3) pg/ml] than in control subjects [median 80.3 (89.3-91.4) microg/ml; p < 0.01; median 5.3 (4.9-5.9) pg/ml, p < 0.0001]. We also found increased concentrations of vitronectin in patients with clinical deterioration compared to those remaining stable and in ex-smokers compared to non-smokers and, increased vitronectin and ET-1 in patients treated with steroids compared to untreated patients. CONCLUSION: These findings justify further studies of vitronectin and ET-1 levels in exhaled breath condensate, as a means of monitoring activity and predicting progression of pulmonary fibrosis.

Biomarkers↗
PubMedDate not supplied

Potential limitations of clinical criteria for the diagnosis of idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis.

BACKGROUND: The need to perform surgical lung biopsy (SLB) in all cases of suspected idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis (IPF/CFA) is controversial. The American Thoracic Society (ATS) and the European Respiratory Society (ERS) recently endorsed explicit clinical criteria for the diagnosis of IPF/CFA in the absence of SLB. Prior studies evaluating clinical criteria for the diagnosis of IPF/CFA have been limited in that either they were performed by clinicians with expertise in the diagnosis of IPF/CFA or they did not utilize explicit diagnostic criteria. We investigated the accuracy of the ATS/ERS criteria when applied in a general pulmonary medicine setting. OBJECTIVES: To determine the interobserver variability of clinical criteria for the diagnosis of IPF/CFA. METHODS: This was a retrospective, blinded evaluation by three board certified pulmonary physicians without extensive experience in the evaluation of IPF/CFA performed at a United States Army tertiary care academic medical center. Patients referred for surgical lung biopsy as part of a diagnostic evaluation of interstitial lung disease (ILD) were evaluated. The physicians reviewed high-resolution computed tomography scans of the chest (HRCT) and clinical data for each patient. The physicians were blinded to all other data and to the opinions of other study participants. Employing the histologic presence of usual interstitial pneumonia (UIP) coupled with appropriate clinical findings as the gold standard for a diagnosis of IPF/CFA we determined the accuracy and interobserver variability for a diagnosis of IPF/CFA based on HRCT alone and based on the ATS/ERS clinical criteria. RESULTS: The sensitivity and positive predictive value for a HRCT diagnosis of IPF/CFA were 71% each while specificity and negative predictive value were 67% each. For the ATS/ERS criteria sensitivity, specificity, positive predictive value and negative predictive value were 71, 75, 77 and 69%, respectively. The interobserver variability, expressed as a kappa coefficient, for HRCT and the ATS/ERS criteria were 0.59 and 0.53, respectively. CONCLUSIONS: Both HRCT and the ATS/ERS clinical criteria may lead to misdiagnosis of patients with ILD. Further studies are needed to fully characterize the accuracy of these tests when applied in a routine pulmonary medicine practice setting.

Adult↗
PubMed2004

Nephrogenic fibrosing dermopathy in a renal transplant recipient with tubulointerstitial nephritis and uveitis.

We report a patient with nephrogenic fibrosing dermopathy. He had chronic renal failure with arthritis, uveitis and histologically severe tubulointerstitial nephritis for which he received a renal transplant from a family relative. After an episode of acute renal failure with the transplant he developed painful, erythematous, firm papules and plaques with geographic borders on the legs, anterior thorax and elbow. A skin biopsy revealed increased fibroblast and collagen fiber content of the dermis and subcutaneous septae. Mucin deposition, sparse smooth-muscle-actin-positive cells and an increased number of CD34-positive cells in the deep dermis were found. After several weeks of hemodialysis, the lesions changed from an inflammatory to a purely sclerotic phase. The fibrocyte, a recently described circulating cell type, that is deposited in scar tissue may be involved in the pathogenesis of this novel pseudosclerodermatous skin disorder.

Diagnosis, Differential↗
PubMed2004

Myofascial pain, fibromyalgia or fibrositis?

The terms myofascial pain, fibromyalgia and fibrositis are critically examined. They constitute diagnostic labels for non-specific musculoskeletal aches and pains. Analysis of the evidence shows that none of these labels is substantiated by hard physical signs or by laboratory evidence of consistent pathological or biochemical abnormality. What is the objective evidence for disorder(s) of muscle, fascia or fibrous tissues, so clearly indicated by these diagnostic names? Alternative terms such as 'regional pain syndrome' or 'chronic pain syndrome' merely redefine the clinical problem without providing a mechanism or basis for diagnosis. Despite different diagnostic criteria, these conditions, along with chronic fatigue syndrome, have many demographic and clinical similarities, most notably tender trigger points. Indeed, the terms are often used interchangeably. There are few differences in the symptoms, physical findings, laboratory tests, functional status, psychosocial features and psychiatric disorders. This paper seeks not to deny the existence of aches and pains, but to critically examine the utility of these terms. The only claimed physical sign is the presence of tender trigger points over muscles or muscle attachments. Research suggests that tender points are a measure of general distress related to pain complaints but separately associated with fatigue and depression. They are present in some normal subjects and are variable in occurrence in time in the same individual. They reflect no demonstrable pathology. It is therefore argued that none of these commonly used diagnoses represent distinct disease entities. A possible but unproven alternative hypothesis is that such symptoms relate to neural pain with both peripheral and central components, and in some instances psychological or wilful embellishment.

Facial Pain↗
PubMed1976

Comparison between pathological and biochemical investigations on an experimental model of fibrosing alveolitis.

Subcutaneously injected Freund's adjuvant caused a fibrosing alveolitis in guinea pigs. This model served to compare biochemical and morphological parameters. The phospholipids were quantitatively extracted from lung tissue, separated and estimated. The fatty acid composition of lecithin was analysed. We observed a correlation between the histologic alterations and the content of phospholipids. A constantly higher phospholipid content was measured in the affected lungs in comparison to the normal ones; however, the composition of the phospholipids changed. The close correlation between the course of the inflammation and the biochemical pattern seems to indicate a function-correlated control of lung metabolism.

Animals↗
PubMed1981

Different therapies and factors influencing response to therapy in idiopathic diffuse fibrosing alveolitis.

The success of 3 different treatment regimens was compared in 15 patients with diffuse fibrosing alveolitis. Improvement or deterioration of lung function (vital capacity (VC) and PaO2 changes during exercise) were used as a control of therapy. The combination of prednisolone and azathioprine proved to be most effective: in 4 of 6 patients both VC and PaO2 improved. Combinations with D-penicillamine resulted in a high number of unwanted side effects: in 5 of 13 treated patients it had to be withdrawn. 6 patients were treated with prednisolone alone; only 1 of them improved. Patients with a short duration of symptoms prior to therapy and initially mild impairment of lung function showed a higher degree of improvement during therapy. The consequence for the management of lung fibrosis is to establish diagnosis and treatment as early as possible.

Adult↗
PubMed1986

High-dose intravenous methylprednisolone pulse therapy as initial treatment in cryptogenic fibrosing alveolitis. A pilot study.

Ten patients with fibrosing alveolitis were treated in a simple random design initially with either a high dose of methylprednisolone (5 patients) or a conventional dose of prednisolone (5 patients) followed by a maintenance dose of 30 mg prednisolone daily. The patients were followed for 6 weeks. No significant (p greater than 0.05) differences were observed between patients with high and low initial dose of glucocorticosteroids, as regards forced vital capacity, transfer factor for carbon monoxide or symptom scores after 3 and 6 weeks of treatment. After 6 weeks the dyspnoea score and the 67Ga uptake decreased, on average, by 74 and 85%, respectively, while forced vital capacity and transfer factor increased by 15 and 29%.

Adult↗
PubMed1990

Cryptogenic fibrosing alveolitis. A study of an indigenous African population.

Cryptogenic fibrosing alveolitis (CFA) has not been described previously in any large group of indigenous African patients. This was a retrospective study of 46 such patients diagnosed as having CFA during a 73-month period. The clinical spectrum of illness was similar to that of other groups studied worldwide. Cigarette smoking was associated with a poorer outcome and is a potentially preventable cause of deterioration.

Adrenal Cortex Hormones↗
PubMed1988

[Fibrosing alveolitis after combined radio- and chemotherapy. A case report].

A nineteen year old female patient with Hodgkin's disease of the mixed type was treated with sequential radio- and chemotherapy according to the ABVD-regimen. Following this treatment, symptoms of fibrosing alveolitis developed which were resistant to treatment and led to death. The analysis of clinical and pathological findings revealed a causal relation between the combination of polychemotherapy and irradiation of the thorax. This underscores the necessity of strong selection and supervision of patients in the case of combined radiotherapy of the thorax and chemotherapy, especially with adriamycin and bleomycin.

Adult↗
PubMedDate not supplied

Correlation of cytologic and histologic findings in fibrosing thyroiditis. A case report.

BACKGROUND: Fibrous thyroiditis is the rarest form of thyroiditis. Consequently, needle aspiration biopsy experience is limited. Aspirates generally yield paucicellular smears with, at the most, only occasional, small fragments of connective tissue. CASE: A 35-year-old man presented with a firm right thyroid mass. Aspiration yielded cellular smears with round and irregularly shaped, cellular aggregates. Coarse cytoplasmic granules and intranuclear inclusions were also noted. The lesion was surgically excised, and pathologic examination was consistent with fibrous thyroiditis. CONCLUSION: As confirmed by histopathologic examination, the aspirated atypical cellular aggregates represented thyroid follicular units that were distorted by entrapment within fibrous tissue. Fibrosing thyroiditis may yield an atypical smear pattern.

Adult↗
PubMed1997

Antioxidative and clinical effects of high-dose N-acetylcysteine in fibrosing alveolitis. Adjunctive therapy to maintenance immunosuppression.

In fibrosing alveolitis (FA), activated phagocytes cause excessive oxidative stress in the lower respiratory tract. Additionally, levels of glutathione, a major antioxidant of the human lung, are markedly reduced. Since N-acetylcysteine (NAC) is a known precursor for glutathione synthesis, we investigated the effect of NAC on redox balance and lung function in FA. Eighteen patients with an established diagnosis of FA were treated with 600 mg NAC three times daily for 12 wk in addition to their latest immunosuppressive therapy. Before and after NAC therapy, pulmonary function tests (PFTs) and bronchoalveolar lavage (BAL) were performed. BAL fluid was analyzed with regard to cell differential, glutathione status, and methionine sulfoxide content of BAL proteins (Met(O)), as an indicator of oxidative stress at the alveolar surface. There was an increase of total glutathione (GSHt = GSH +/- 2 x GSSG: 3.43 +/- 0.30 microM versus 4.20 +/- 0.66 microM, p < 0.05) and of reduced glutathione (GSH: 2.58 +/- 0.24 microM versus 3.42 +/- 0.54 microM, p < 0.005) in native BAL fluid and in the epithelial lining fluid (GSHt: 267.3 +/- 26.0 microM versus 367.1 +/- 36.0 microM, p < 0.005; GSH: 204.5 +/- 20.7 microM versus 302.9 +/- 32.2 microM, p < 0.005). The increase of GSH was accompanied by a decrease of Met(O) (6.83 +/- 0.71% versus 4.60 +/- 0.40%, p < 0.005). PFTs significantly improved during NAC treatment. We conclude that high-dose NAC significantly improved the antioxidant screen of the lungs by elevating GSH levels. Moreover, the decrease of Met(O) levels indicated an antioxidant effect at the alveolar surface. These biochemical changes were accompanied by an improvement of PFTs in patients under maintenance immunosuppression. NAC supplementation should, therefore, be considered as an adjunct therapy for FA.

Acetylcysteine↗
PubMed1999

A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis.

This study aimed to investigate whether there was a difference in outcome related to histologic pattern in cryptogenic fibrosing alveolitis (CFA) and to see whether there were correlations between clinical and radiologic findings and histology. One hundred thirteen lung biopsies from consecutive patients taken for the diagnosis of diffuse lung disease were reviewed and reclassified using the Katzenstein and Myers criteria for interstitial pneumonias. Patients lacking full investigational data at presentation and those with conditions predisposing to lung fibrosis were excluded, leaving 15 patients diagnosed with nonspecific interstitial pneumonia (NSIP) and 15 with usual interstitial pneumonia (UIP). Clinical and radiologic findings at presentation and serial lung function information and survival status in November 1998 were compared for the two groups. Survival was found to be significantly greater in the NSIP group compared with the UIP group (p < 0.001). This could not be explained by differences in treatment. Patients with UIP showed a progressive deterioration in lung function whereas those with NSIP remained stable. CT scans of patients with UIP showed more fibrosis than those of patients with NSIP (p < 0.011). A histologic diagnosis of NSIP is associated with a better prognosis than UIP. This subclassification of CFA is clinically useful.

Adult↗
PubMed2000

Increased risk of fibrosing alveolitis associated with interleukin-1 receptor antagonist and tumor necrosis factor-alpha gene polymorphisms.

Fibrosing alveolitis (FA) is characterized by persistent inflammation and elevated production of tumor necrosis factor-alpha (TNF-alpha), interleukin-1 beta (IL-1beta), and interleukin-1 receptor antagonist (IL-1ra) in the lung. Single base variations at position +2018 in the IL-1ra gene (IL-1RN) and position -308 in the TNF-alpha gene (TNF-A) are overrepresented in other chronic inflammatory disease populations. We have tested the hypothesis that predisposition to FA may also be influenced by these polymorphisms by genotyping 88 cases and matched controls from England and 61 cases and 103 unmatched controls from Italy. The rarer allele for IL-1RN and TNF-A was designated allele 2 in each case. For IL-1RN allele 2, in the English group, the relative odds of FA were increased in homozygous subjects by an odds ratio (OR) of 10.2 (95% confidence intervals [CI], 1.26 to 81.4; p = 0.03) and for carriers by an OR of 1.85 (95% CI, 0.94 to 3.63; p = 0.075). In the Italian population, the risk of FA was increased, in IL-1RN allele 2 homozygotes (OR, 2.54; 95% CI, 0.68 to 9.50; p = 0.2) and in carriers (OR 2.40; 95% CI, 1.26 to 4.60; p = 0.008). Carriage of TNF-A allele 2 was also associated with increased risk of FA in the English (OR, 1.85; 95% CI, 0.94 to 3.63; p = 0.075) and Italian (OR, 2.50; 95% CI, 1.14 to 5.47; p = 0.022) populations. These data suggest IL-1RN (+2018) allele 2 and TNF-A (-308) allele 2 confer increased risk of developing FA and, therefore, that unopposed IL-1beta and/or excessive TNF-alpha may play a pathophysiologic role in this condition.

Aged↗
PubMed2000

The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis.

Lone cryptogenic fibrosing alveolitis (CFA) is a progressive interstitial lung disease, with a median survival of 3 to 6 yr from the onset of dyspnea. CFA can be subdivided into prognostically significant histopathologic patterns, including nonspecific interstitial pneumonia (NSIP). We reviewed 78 patients with a clinicopathologic diagnosis of CFA, biopsied between 1978 and 1989, to evaluate the prevalence and prognostic significance of these histopathologic patterns, in particular NSIP. Biopsy appearances were reclassified by two pulmonary histopathologists as usual interstitial pneumonia (UIP) (47%), NSIP (36%), or desquamative interstitial pneumonia (DIP)/respiratory bronchiolitis-associated interstitial lung disease (RBILD) (17%). The kappa coefficient of agreement between pathologists was 0.49. In 67 cases, follow-up was complete to death or 10 yr after biopsy, with 50 deaths during a median follow-up of 42 mo (UIP, 89%; NSIP, 61%, DIP/RBILD, 0%). Survival was highest in DIP/RBILD and higher in NSIP than UIP, p < 0.0005. When analysis was confined to patients with UIP or NSIP, the mortality of UIP remained higher, p < 0.01, but the 5-yr survival in patients with fibrotic NSIP was only 45%, indicating that this histologic appearance is often associated with a poor outcome. A response to treatment was more frequent in DIP/RBILD than in NSIP (p < 0.01) or UIP (p < 0.0005). This study confirms the prognostic value of subclassifying patients with CFA according to histopathologic pattern. However, in patients with clinically typical CFA, a histologic diagnosis of fibrotic NSIP needs to be interpreted with caution and does not necessarily denote a good outcome.

Biopsy↗