The course of cryptogenic fibrosing alveolitis (CFA) in 96 patients is described. The mean survival from the onset of the first symptom until death was 47-4 months in all the 59 fatal cases, 49-6 months in those dying from CFA only, and 48-4 months in those dying from CFA in whom the course of the illness was not influenced by corticosteroids. There was a wide variation in the natural course of the disorder, varying from death within a year of the onset of symptoms in five patients to prolonged survival with slow or arrested progress in four patients not treated with corticosteroids. Most patients died from CFA but five developed bronchial carcinoma and two died after lung biopsy.The main factor influencing the prognosis was the effectiveness of corticosteroid drugs. Only 16% of the treated patients had a worthwhile (grade 2) response to these drugs and the five-year survival of this group was 67 %. In those patients in whom the course of the inness was uninfluenced by corticosteroids the five-year survival rate was only 20%. The histological appearances on lung biopsy gave a rough guide to the likely effect of corticosteroid therapy.Other factors influencing the prognosis were the degree of dyspnoea on presentation, the vital capacity, and the presence of arterial hypoxaemia. Patients who had had symptoms for more than four years before the first hospital attendance had a relatively good prognosis.By contrast, neither the extent of radiographic abnormality nor the presence of circulating rheumatoid and antinuclear factors influenced subsequent survival.

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Background-A previous retrospective study suggested that a policy of regular anti-pseudomonal antibiotic treatment improved pulmonary function and increased survival in patients with cystic fibrosis chronically infected with Pseudomonas species. The results of a prospective multicentre study to compare the eVects on pulmonary function and mortality of three monthly elective anti-pseudomonal antibiotic treatment with conventional symptomatic treatment are reported. Methods-Sixty patients with cystic fibrosis, chronically infected with P aeruginosa, were randomised to the two treatment arms (elective or symptomatic) and followed clinically at yearly reviews. The major end points were changes in forced expiratory volume in one second (FEV 1 ) and forced vital capacity (FVC). Survival was a secondary end point. Results-Patients in the symptomatic group received a mean of three antibiotic treatments each year and those in the elective group received four antibiotic treatments during each year of the study. No significant diVerences in FEV 1 and FVC were found between the two groups after three years. There was a statistically non-significant higher rate of deaths in the elective group (n = 4), three of which were associated with B cepacia infection, compared with the symptomatic group (n = 0). Conclusions-This study did not demonstrate an advantage of a policy of elective antibiotic treatment over symptomatic treatment in patients with cystic fibrosis chronically infected with Pseudomonas species. (Thorax 2000;55:355-358)

This study was designed to identify a core set of items that may characterize impaired health in chronic respiratory failure (CRF) since none of the questionnaires commonly used to measure impaired health in patients with CRF were developed for use in such patients.Questionnaire items, identified from several sources, were administered to 92 patients with stable severe chronic hypoxia or hypercapnia, together with physiological measures of disease severity, the Sickness Impact Profile (SIP) and the St George's Respiratory Questionnaire (SGRQ).Of the 152 items identified, only 28 correlated with patients' perceptions of overall health and were also independent of age, sex, disease or type of treatment and did not show floor or ceiling effects. Principal components analysis identified three specific factors: "daily activities", "cognitive function", and "invalidity". The total score for the whole 28-item set correlated with other measures of disease activity, including SGRQ (r=0.86) and SIP (r=0.70), but not spirometry. The interquartile range of the 28-item set was wider than that of the SGRQ or the SIP.This study characterized two areas of health impairment in chronic respiratory failure that had not previously been identified: effects of impaired cognitive function on daily life, and a sense of invalidity. The measurements properties of this 28-item set suggest that it may be a more discriminative instrument for patients with chronic respiratory failure than existing questionnaires.

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