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PubMed2000

Fibrosing colonopathy in an adult owing to over use of pancreatic enzyme supplements.

A woman, then in her late 20s, underwent a cholecystectomy in 1962 for gallstone disease and subsequent common bile duct stones were managed endoscopically. However, because of unrelenting pain, a pylorus preserving pancreaticoduodenectomy was done in 1990 and in the following years the patient took large amounts of pancreatic enzyme supplements. She developed large bowel obstruction in 1997 and a right hemicolectomy was undertaken. Histology confirmed fibrosing colonopathy of the ascending colon and caecum. Her pancreatic enzyme dose was reduced and her subsequent course has been uncomplicated.

Adult↗
PubMed2001

Successful treatment with adefovir dipivoxil in a patient with fibrosing cholestatic hepatitis and lamivudine resistant hepatitis B virus.

Fibrosing cholestatic hepatitis (FCH) is a severe clinical and histological variant of hepatitis B virus (HBV) infection seen most commonly in the HBV infected allograft after liver transplantation. Without treatment, FCH is fatal, rapidly and universally. Remission has been reported with lamivudine but is associated with evolving resistance to lamivudine. Adefovir dipivoxil has recently been reported to be a potent and highly effective inhibitor of HBV replication in both wild-type and lamivudine resistant HBV infection. We report a case of FCH 15 months after liver transplantation for HBV related cirrhosis despite therapy with lamivudine and hepatitis B immunoglobulin (HBIg). Within two weeks of commencing treatment with adefovir dipivoxil 10 mg once daily, the patient had made a remarkable recovery with resolution of jaundice and normalisation of liver biochemistry. HBV DNA and hepatitis B e antigen were lost from serum subsequently and liver histology had improved at four months. This case report suggests firstly, that advanced FCH can be reversed and secondly, that addition of adefovir dipivoxil to lamivudine and HBIg may be an effective antiviral strategy.

Adenine↗
PubMed1991

Effect of single lung transplantation on pulmonary hypertension in patients with end stage fibrosing lung disease.

OBJECTIVE: To investigate the effect of successful single lung transplantation on pulmonary haemodynamic variables and right ventricular function. DESIGN: Pulmonary haemodynamic variables and right ventricular function were measured at right heart catheterisation after single lung transplantation. The results were compared with the preoperative pulmonary haemodynamic variables measured at the time of assessment for transplantation. SETTING: A tertiary referral centre. PATIENTS: Five survivors of single lung transplantation performed for end stage lung disease. INTERVENTIONS: Cardiac catheterisation in all five patients at a mean of 18 months postoperatively. Preoperative catheter data were available for comparison in four. Right heart pressures and cardiac output were measured and right ventricular angiography was performed. Perfusion scans performed for clinical reasons were used to assess the percentage of cardiac output passing through each lung. MAIN OUTCOME MEASURES: Right heart pressures, cardiac output, right ventricular function, percentage perfusion to lungs. RESULTS: After operation mean peak right ventricular pressure fell from 53 mm Hg to 33 mm Hg, mean pulmonary artery pressure from 33 mm Hg to 18 mm Hg, total pulmonary resistance from 11.2 U x m2 to 5.8 U x m2, and pulmonary arteriolar resistance from 8.9 U x m2 to 3.6 U x m2. Pulmonary artery wedge pressure and cardiac index were unchanged. Right ventricular function improved in all patients. The transplanted lung received most of the cardiac output. CONCLUSION: In patients with moderate pulmonary hypertension and right ventricular dysfunction secondary to end stage fibrosing lung disease single lung transplantation was followed by an improvement in pulmonary haemodynamic variables and right ventricular function.

Blood Pressure↗
PubMed1972

Pathological findings after right lung transplantation in a patient with fibrosing alveolitis.

The pathological findings in a patient who died two months after right lung transplantation for fibrosing alveolitis are reported. The cause of death was haemoptysis, due to penetrating ulceration causing a fistula between the surface of the cartilagenous part of the main bronchus of the donor lung and the right upper lobe pulmonary artery. The opening in the donor bronchus was immediately distal to the line of the bronchial anastomosis and through an actively inflamed area. Other parts of the donor bronchus had microscopic changes suggesting ischaemia, emphasizing that inadequacy of blood supply to the donor extrapulmonary bronchus is one of the most serious hazards of lung transplantation. Within the lung, histological features of rejection were mild and there was minimal evidence of infection. Another question raised by this case is whether the donor lung was in the process of developing the original disease, evidence for which was sought electron microscopically but was not proven.

Adult↗
PubMed2000

Capsaicin induced cough in cryptogenic fibrosing alveolitis.

BACKGROUND: Cough is a common and troublesome symptom in cryptogenic fibrosing alveolitis (CFA) but the mechanisms responsible are not known. The cough threshold to inhaled capsaicin is increased in asthma and chronic obstructive pulmonary disease (COPD) where lung volumes are increased, but the relationship between cough response and symptom intensity has not been studied in CFA where lung volumes are reduced. METHODS: Capsaicin challenge tests were performed on 15 subjects with proven CFA and 96 healthy controls. Symptoms, as assessed by daily diary card cough score and by visual analogue scale (VAS), were related to the capsaicin sensitivity (C5) and compared with lung volumes. Volume restriction was produced in a group of 12 normal healthy subjects by a plastic shell tightly strapped to the chest wall. Capsaicin challenge tests were performed in these subjects, both strapped and unstrapped, to determine whether volume restriction altered the cough reflex. RESULTS: The median C5 response in normal subjects was more than 500 microM compared with 15.6 microM in those with CFA (p<0.001). The C5 response of the CFA patients was not related to symptoms of cough (whether measured by diary card or by VAS), nor was it related to percentage predicted total lung capacity (TLC) or forced vital capacity (FVC). Volume restriction of normal subjects with chest strapping successfully restricted lung volumes to levels similar to that of the CFA patients but did not change the sensitivity to capsaicin. CONCLUSIONS: The cough reflex measured using capsaicin is markedly increased in patients with CFA. This increase is not the result of alterations in the deposition of inhaled particles of capsaicin brought about by volume restriction. It could be related to reduced lung compliance leading to sensitisation of rapidly adapting receptors, other mechanical changes, or to destruction of pulmonary C fibres secondary to interstitial inflammation. The capsaicin test may be a useful method of objectively monitoring cough propensity in CFA.

Adult↗
PubMed2001

Fibrosing alveolitis in patients with rheumatoid arthritis as assessed by high resolution computed tomography, chest radiography, and pulmonary function tests.

BACKGROUND: Fibrosing alveolitis (FA) is a common and serious complication of rheumatoid arthritis (RA). Before the availability of high resolution computed tomographic (HRCT) scanning, it was difficult to diagnose accurately without recourse to biopsy. Prospective studies have reported a prevalence of interstitial lung disease (ILD) of 19-44%. The term ILD used by these authors encompasses a variety of appearances on HRCT scans. This prospective study used HRCT scanning to determine the true prevalence of FA in hospital outpatients with RA, and to study associated clinical characteristics. METHODS: One hundred and fifty consecutive patients with RA were selected from a hospital outpatient department, irrespective of the presence or absence of chest disease. All underwent a detailed clinical assessment, chest HRCT scanning, and conventional chest radiography within 4 weeks of full pulmonary function tests. RESULTS: Seventy percent of patients were current or reformed cigarette smokers. Twenty eight (19%) had FA, most frequently of reticular pattern, and 12 of this group (43%) also had emphysematous bullae. None of the previously suggested risk factors for developing FA were confirmed. Fifty four percent of patients with HRCT evidence of FA had bilateral basal chest crackles, 82% had a reduced carbon monoxide transfer factor (TLCO), 14% had restrictive pulmonary function tests, and 14% had bilateral chest radiographic signs of FA. CONCLUSIONS: HRCT evidence of FA was present in 19% of hospital outpatients with RA. Abnormalities on chest examination or on full pulmonary function tests, even without restrictive changes or chest radiographic abnormalities, should prompt physicians to request a chest HRCT scan when investigating dyspnoea in patients with RA.

Aged↗
PubMed1978

An outbreak of pleural mesothelioma and chronic fibrosing pleurisy in the village of Karain/Urgüp in Anatolia.

The 575 inhabitants of the remote Anatolian village of Karain suffered 11 deaths from pleural mesothelioma in 1975/76 and there were five cases of fibrosing pleurisy. In the previous five years there had been 25 cases of mesothelioma. Calcified pleural plaques were common on survey radiography. Asbestos does not occur in the local soil or rock, nor is it handled in the village, but a few fibres were found in the water. Fibres were also found in the pleural tissue of two of five cases examined. Inhabitants of the neighbouring villages are free of mesothelioma.

Adult↗
PubMed1980

Bronchoalveolar lavage fluid cell counts in cryptogenic fibrosing alveolitis and their relation to therapy.

Bronchoalveolar lavage was used to sample inflammatory cells from the lungs of 51 patients with cryptogenic fibrosing alveolitis (CFA) (24 smokers, 12 ex-smokers, and 15 non-smokers). The smokers with CFA have been compared with 15 smoking control subjects in whom there was no radiographic abnormality or clinical evidence of chronic bronchitis. Significantly lower volumes of lavage fluid were recovered from the smokers with CFA (p < 0.001) and the fluid contained lower percentages of macrophages (p < 0.01), reflecting increased percentages of eosinophils (p < 0.001) and neutrophils (p < 0.01). Similar changes were seen in the ex-smokers and non-smokers. There was also an increase in the percentages of lymphocytes when the whole group of CFA patients was compared with the control subjects (p less than or equal to 0.05). No significant differences were found when patients with "lone" CFA were compared with those having associated systemic disease. The only feature distinguishing smokers from non-smokers with CFA was the presence of pigmented cytoplasmic inclusions in the macrophages from the smokers (p < 0.001). However, there were lower numbers of pigmented macrophages in the smoking CFA patients by comparison with the control subjects suggesting either a change in phagocytic capacity or turnover rate in this disease. Profiles of differential cell counts in individual patients showed that increases of eosinophils over 3% or neutrophils over 4% or both with lymphocyte counts of less than 11% related to a poor clinical response to corticosteroids, but lymphocyte percentages greater than 11% related to improvement (p < 0.05).

Adult↗
PubMed1980

Cryptogenic fibrosing alveolitis and lung cancer.

Lung cancer was found in 20 (9.8%) of 205 patients with cryptogenic fibrosing alveolitis (CFA) or 12.9% of the 155 patients in this series followed to death. An excess relative risk of lung cancer of 14.1 was found in patients with CFA compared to the general population of comparable age and sex, allowing for the lengths of follow-up of the CFA patients. The relative risk for male smokers was (observed/expected) 15+1.06 = 14.2, and for female smokers (O/E) 2/0.3 = 6.7. Only one male and one female non-smoker had lung cancer. These data suggest that there is an excess risk of lung cancer not wholly accounted for by age, sex, or smoking habit. The distribution of histological types was not obviously different from that found in lung cancer without pulmonary fibrosis. Large opacities suggestive of lung cancer were present at the time of first hospital attendance for symptoms relating to CFA in four of the 20 patients. Finger clubbing was present in 19 (95%) compared with 116/185 (63%) of those so far not developing cancer. There were no other clinical differences at presentation. In particular, cancer was not found especially in those with longer survival from the onset of symptoms of CFA or with a greater initial radiographic change.

Adolescent↗
PubMed1981

Interrelationship between lung volume, expiratory flow, and lung transfer factor in fibrosing alveolitis.

Fifty patients with fibrosing alveolitis studied on 104 occasions exhibited significant direct correlations between vital capacity (VC), maximum mid-expiratory flow rate (MMFR), and transfer factor for carbon monoxide (TLCO). Forced expired volume in the first second (FEV1)/VC ratio bore a weak negative correlation with VC. Peak expiratory flow, MMFR, and maximum flow rates at 50% and 25% of VC were often reduced in patients with severe grades of pulmonary dysfunction. It appears that as the severity of the fibrotic process increases, the lung volumes shrink and the transfer factor for CO decreases. The total lung capacity decreases predominantly on account of reduction in VC. With a decrease in lung volume the MMFR also falls. Decrease in flow rates at low lung volumes is greater as compared to the fall in peak flow. The expiratory flow rates however were normal or even increased when related to absolute lung volume. Some patients exhibit disproportionate expiratory slowing as evidenced by a decrease in MMFR which is out of proportion to the reduction in VC. These patients also have a reduced FEV1/VC ratio. These changes are probably the consequence of associated peripheral airway narrowing.

Adult↗
PubMed1983

Prognosis of cryptogenic fibrosing alveolitis.

We have analysed retrospectively 100 consecutive patients with cryptogenic fibrosing alveolitis, who were treated with corticosteroids and followed for at least three years. At the time of diagnosis biopsy specimens were available in 64 cases. The clinical, radiographic, physiological, and histological features and the response to steroid treatment have been correlated with the prognosis. An early objective functional improvement was observed in 30%. Analysis of the survival data (life table and log rank test) showed a longer survival in younger patients and in patients with a shorter duration of symptoms before presentation, less radiographic abnormality, and less impairment of diffusing capacity, but not clearly in patients with more cellular histological appearances. The most favourable prognostic sign seemed to be an early response to steroid treatment.

Adolescent↗
PubMed1984

Cryptogenic fibrosing alveolitis: pattern of disease in the lung.

The distribution of disease has been studied in 10 patients with histologically confirmed cryptogenic fibrosing alveolitis by means of computed tomography of the lungs, gallium 67 uptake scintigraphy, and ventilation and perfusion scintigraphy. The findings of computed tomography and scintigraphy showed no correlation with the UICC/Cincinnati grades of the plain chest radiographs, even when these were supplemented with information from lateral and oblique chest films. Computed tomography showed subpleural shadowing in every patient except one who had responded well to corticosteroid treatment. Subpleural shadowing was commoner in the inferior and posterior parts of the lung lobes and was commonest in the posterior lower subpleural areas. The central lung showed three types of change--firstly, a dense shadowing, which was sometimes associated with much increased gallium uptake; secondly, dilated smaller airways with small bullae, suggesting airway disease; and, thirdly, gross bullous change.

Humans↗
PubMed1986

Computed tomography based estimates of regional gas and tissue volume of the lung in supine subjects with chronic airflow limitation or fibrosing alveolitis.

Twelve patients with chronic airflow limitation and 12 patients with a histological diagnosis of fibrosing alveolitis were studied. The calculated mean (SD) tissue volume of a single lung at total lung capacity was 467 (91) ml in the patients with alveolitis, which was 43% (14%) more than predicted for healthy people of the same age, sex, and height. The tissue volume of a single lung at total lung capacity was 436 (82) ml in the patients with chronic airflow limitation, which was 26% (21%) more than predicted. At residual volumes the tissue contents of the fibrotic and the obstructed lungs changed very little (to 407 (84) ml and 433 (84) ml respectively). This allowed tissue volume to be used as a marker of position within the lung, to match inspiratory and expiratory slices and to calculate regional ventilation. In both groups local ventilation was diminished and more variable than in healthy lungs--that is, in the mid 70% of lung volume the local residual volume to total gas volume ratios (RV/TGV) were 32% (10%) in the fibrotic group and 66% (14%) in the group with chronic airflow limitation, compared with 23% (5%) in healthy subjects. As expected, the fibrotic lungs were much denser (0.246 (0.036) g/ml) and the lungs with chronic airflow obstruction were less dense (0.114 (0.026) g/ml) than were healthy lungs (0.126 (0.017) g/ml).

Adult↗
PubMed1990

Vertical gradients of lung density in supine subjects with fibrosing alveolitis or pulmonary emphysema.

Computed tomography was used to determine the vertical gradient of density in the peripheral lung tissue of 12 patients with histologically proved fibrosing alveolitis and 12 patients with chronic bronchitis and evidence of pulmonary emphysema on the computed tomograms. Measurements were made at total lung capacity and at residual volume and compared with similar measurements from 12 normal subjects reported in a previous study. At residual volume the mean peripheral tissue density in the emphysematous lungs was 0.081 kg/l compared with 0.426 kg/l in the fibrotic lungs and 0.323 kg/l in the normal lungs. The observed densities in the three groups were compared with those in a theoretical model predicting the vertical changes of lung density caused by gravitational effects that would be found in lungs with differing compliance. The emphysematous lungs showed a much greater increase of density with descent down the lung than that predicted for normal lungs, and the results were explicable by an increase in compliance. The fibrotic lungs showed considerably less change in density than expected, implying loss of compliance. It is suggested that local changes of compliance are important determinants of vertical density gradients in diseased lungs.

Adult↗
PubMed1992

New perspectives on basic mechanisms in lung disease. 1. Lung injury, inflammatory mediators, and fibroblast activation in fibrosing alveolitis.

It is over 25 years since Scadding first defined the term fibrosing alveolitis. It has since been established that complex mechanisms underlie its pathogenesis, including epithelial and endothelial injury, vascular leakage, production of inflammatory cells and their mediators, and fibroblast activation. Only through a detailed knowledge of how these cellular and molecular events are interlinked will we learn how to combat this disease, which is notoriously resistant to present treatments. So far the only therapeutic advances have been refinements in immunosuppression, and even these treatments are frequently disappointing. We believe that future advances in treatment will come from the development of agents that protect endothelial and epithelial cells from further injury and agents that can inhibit release of inflammatory mediators. A better knowledge of the mechanisms of collagen gene activation and the biochemical pathways of collagen production may also allow the identification of vulnerable sites at which new treatments may be directed. A combined approach to modifying appropriate parts of both the inflammatory component and the fibroblast/collagen component should provide a new stimulus to research. Further epidemiological studies are also needed to identify the environmental causes of lung injury that initiate the cascade of events leading to interstitial fibrosis.

Collagen↗
PubMed1994

Localisation of a pulmonary autoantigen in cryptogenic fibrosing alveolitis.

BACKGROUND--Cryptogenic fibrosing alveolitis (CFA) is believed to have an immunological pathogenesis with a persisting inflammatory reaction to an as yet unidentified pulmonary antigen(s). A high frequency of IgG autoantibodies has previously been found in the plasma of patients with CFA to an extractable 70-90 kDa lung antigen by Western blotting. Preliminary immunohistochemical studies with patient IgG had indicated that the target protein(s) might be associated with alveolar epithelial lining cells which have previously been suggested as the site of immunological attack in CFA. METHODS--In order to confirm this finding immunohistochemical analysis and Western blotting were performed on a human type II alveolar cell line (A549) using CFA patient plasma. In order to study further the distribution of the antigen, antibodies were raised in a rabbit to the partially purified 70-90 kDa CFA lung protein. RESULTS--The results showed that the human CFA autoantibody recognised a 70-90 kDa protein with a cytoplasmic distribution present in the A549 cells, confirming previous observations. The immune rabbit IgG recognised a protein of similar molecular weight by Western blotting of protein derived from lung biopsy samples of patients with CFA and A549 cells. In addition it immunoprecipitated protein(s) of this molecular weight from lung biopsy protein extracts from patients with CFA. The precipitated protein(s) were found to cross-react with the autoantibody found in the plasma of patients with CFA. Immunohistochemical analysis with immunised rabbit antibody revealed positive staining of type I and II alveolar epithelial lining cells in CFA. A similar pattern of epithelial staining was also observed with the rabbit IgG on biopsy specimens of lung from patients with sarcoidosis and control lung tissue, although this was more focal and less intense. No positive staining was seen on sections from a number of non-pulmonary tissues (colon, liver, kidney, tonsil, lymph node, skin, cervix). Cytoplasmic staining of the A549 cell line was also detected. CONCLUSIONS--The 70-90 kDa protein recognised by autoantibodies in patients with CFA is associated with pulmonary epithelial lining cells. The immune rabbit IgG produced appears to recognise antigen by Western blotting and immunohistochemical staining of lung tissue in a similar pattern to the patient autoantibodies. Immunohistochemical data obtained with this antibody suggest that the putative autoantigen against which patients with CFA mount a humoral immune response may be endogenous and specific to the lung.

Aged↗
PubMed1996

Mortality rates from cryptogenic fibrosing alveolitis in seven countries.

BACKGROUND: Registered mortality from cryptogenic fibrosing alveolitis (CFA) in England and Wales has increased substantially since the specific International Classification of Diseases code for CFA was introduced in 1979. However, since a significant proportion of deaths from CFA are misclassified as post inflammatory fibrosis (PIF), it is possible that the observed rise in CFA mortality is due to diagnostic transfer from this code. To investigate this, and to assess mortality trends in other countries, annual CFA and PIF mortality data from England and Wales, USA, Australia, Scotland, Canada, New Zealand, and Germany were analysed. METHODS: Crude annual mortality rates were calculated and rates standardised by Poisson regression to allow assessment of changes over time and comparison between countries, sexes, and age groups. The relative trends in mortality from CFA and PIF were assessed by calculating the annual ratio of CFA to PIF deaths. RESULTS: Men were more likely than women to die from both CFA and PIF in all countries. The highest standardised CFA mortality rate occurred in England and Wales, and the lowest in Germany. Since 1979 mortality from CFA has increased in England and Wales, Australia, Scotland and Canada, but there was no trend in CFA mortality in New Zealand or Germany. In the USA mortality from CFA was low and has fallen. Mortality from PIF increased in all countries except New Zealand and Germany, and the highest PIF mortality, together with the greatest increase over time, was seen in the USA. Changes over time in the annual ratio of CFA to PIF deaths in all countries were small, implying that diagnostic transfer is not a major cause of the increasing CFA mortality. CONCLUSIONS: Mortality from CFA continues to increase in England and Wales and in many other countries. Diagnostic transfer from PIF does not appear to be a major cause of this.

Adolescent↗