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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.

Background: Chronic cough is associated with an increased sensitivity to inhaled capsaicin in a number of conditions but there are no data for patients with more severe asthma or chronic obstructive pulmonary disease (COPD). Moreover, the relationships between the capsaicin response (expressed as the concentration of capsaicin provoking five coughs, C5), self-reported cough, and routine medication is not known., Methods: The cough response to capsaicin in 53 subjects with asthma, 56 subjects with COPD, and 96 healthy individuals was recorded and compared with a number of subjective measures of self-reported cough, measures of airway obstruction, and prescribed medication. In asthmatic subjects the relationships between the cough response to capsaicin and mean daily peak flow variability and non-specific bronchial hyperresponsiveness to histamine were also examined., Results: Subjects with asthma (median C5 = 62 mM) and COPD (median C5 = 31 mM) were similarly sensitive to capsaicin and both were more reactive than normal subjects (median C5 >500 mM). Capsaicin sensitivity was related to symptomatic cough as measured by the diary card score in both asthma and COPD (r = -0.38 and r = -0.44, respectively), but only in asthma and not COPD when measured using a visual analogue score (r = -0.32 and r = -0.05, respectively). Capsaicin sensitivity was independent of the degree of airway obstruction and in asthmatics was not related to PEF variability or PC(20) for histamine. The response to capsaicin was not related to treatment with inhaled corticosteroids but was increased in those using anticholinergic agents in both conditions., Conclusions: These data suggest that an increased cough reflex, as measured by capsaicin responsiveness, is an important contributor to the presence of cough in asthma and COPD, rather than cough being simply secondary to excessive airway secretions. The lack of any relationship between capsaicin responsiveness and airflow limitation as measured by the FEV(1) suggests that the mechanisms producing cough are likely to be different from those causing airways obstruction, at least in patients with COPD.

Background: Non-invasive positive pressure ventilation (NIPPV) has been shown to be beneficial in the treatment of acute exacerbations of chronic obstructive pulmonary disease (COPD) complicated by respiratory failure. A survey was undertaken to assess the availability of NIPPV for the treatment of acute exacerbations of COPD and to determine how NIPPV is delivered in hospitals in the UK., Methods: A questionnaire was sent to consultants with an interest in respiratory medicine from 268 of the hospitals found in the BTS directory. The questionnaire enquired about the hospital as well as the availability of NIPPV in the hospital. If NIPPV was available in the hospital, details of implementation, staffing and funding were determined., Results: Replies to the questionnaire were received from 98.5% of consultants. NIPPV was available in 48% of hospitals, these hospitals tending to serve larger populations and to have more respiratory physicians than the hospitals where NIPPV was not available. There was considerable regional variation in the availability of NIPPV. In hospitals where NIPPV was not available the reason(s) were lack of consultant training in 53%, lack of other staff training in 63%, financial in 63%, and doubt about the benefit of NIPPV in 15% of cases. In those hospitals where NIPPV was available, clinical practice varied greatly: 68% of centres treated fewer than 20 patients a year with this form of treatment and 9% treated more than 60 patients a year. Although NIPPV was paid for completely from the trust equipment budget in 46 hospitals (41%), other money such as research or charitable funds were used at least partially in the other hospitals and NIPPV was financed solely from research or charitable funds in 41 hospitals (37%)., Conclusions: Equipment for NIPPV is available in less than half of the acute hospitals in the UK. In those in which it is available it is generally underused. Lack of training and problems with funding are generally given for the failure to introduce NIPPV.

A case of relapsing polychondritis presenting as tracheomalacia is reported in which an unusual low pitched sound was heard at the mouth and over the chest wall during expiration. The sound was associated with expiratory airflow limitation and oscillation on the flow trace of approximately 50 Hz. Spectral analysis of the sound showed it to have the characteristics of sounds produced by flutter in flow limited flexible tubes. These observations suggest that the sound was produced by airflow induced flutter in the trachea and main airways and is further evidence in support of the dynamic flutter theory of wheeze production.

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.

Background: Although mortality from tuberculosis has continued to fall in recent years, there has been little change in the case fatality rate for tuberculosis over the same period. This has previously been shown to be due to the increasing proportion of cases of tuberculosis occurring in the elderly. Tuberculosis mortality and case fatality were therefore analysed to determine if this disappointing trend in case fatality rate has occurred from disease in all or only certain sites., Methods: A retrospective analysis of the tuberculosis mortality and case fatality rates in England and Wales for the period 1972-92 was carried out. The average annual percentage change in tuberculosis was calculated for each disease site and by age group and the results were compared., Results: The analysis showed that, although the mortality rate fell steadily by 5.6% per annum, the case fatality rate decreased by only 0.9% (95% CI -1.7 to -0.1) per annum. The case fatality rate for respiratory and central nervous system disease declined, but no decline in tuberculosis at "other" sites was observed (1.01% (+2.2 to -0.2) for all age groups combined). In the group aged 75 and over, however, the proportion of deaths due to disease at other sites increased by 3.2% (2.2 to 4.3) per annum whilst in the other age groups the mortality rate declined., Conclusions: This analysis suggests that clinicians may be becoming less able to recognise non-respiratory presentations of tuberculosis, particularly in the elderly, and underlines the need to consider tuberculosis as a diagnosis to avoid delay in treatment.

A case of obstructive sleep apnoea associated with the Arnold-Chiari malformation is described, in which the loss of pharyngeal sensation seems to have played an important part in the aetiology of the obstruction of the upper airway.