Defining an exacerbation of pulmonary disease in cystic fibrosis*

Abstract

Despite the importance of pulmonary exacerbations in CF in both clinical and research settings, both published evidence and consensus are lacking concerning the criteria used to define an exacerbation. The use of hospitalization as a surrogate measure presupposes uniformity among clinicians in diagnosis and treatment of exacerbations. Our aims were to evaluate consensus among clinicians about the variables considered helpful in diagnosing an exacerbation requiring treatment. A comprehensive list of symptoms, signs, and investigations used to define exacerbations was compiled from published trials. A written self-administered questionnaire included the list in age-appropriate groups to survey opinion about the helpfulness of each item, and the estimated proportion of patients admitted within a month of diagnosis of an exacerbation. This was sent to all clinicians managing CF patients in Australia. There were replies from 59/91 clinicians (65%), 41/60 (68%) from those managing children and 18/31 (58%) from those managing adults. Responses of those managing children and adults differed for 7/32 variables (Mann-Whitney test, P < 0.05). Clinic grouping did not show greater consensus among responses of pediatricians (Kruskal-Wallis test, P = 0.362). Consensus, >74% or <26% of respondents rating a variable helpful/very helpful, was found in only 50% of variables listed. Estimated admission rate within a month of diagnosis was 61% (30-100%) for those managing adults and 48% %5-100%) for pediatricians. A lack of consensus was found among clinicians managing CF about the variables considered in diagnosing an exacerbation. The estimated proportion admitted within a month of diagnosis was very variable. This demonstrated inhomogeneity in approach to diagnosis and management of an exacerbation suggests a significant heterogeneity of clinical care.

Effect of Treatment of Cystic Fibrosis Pulmonary Exacerbations on Systemic Inflammation

“…We did not observe any relationships between plasma inflammatory proteins and exacerbation severity, and this may reflect the heterogeneity of exacerbations in CF. In fact, there is ongoing debate on how CF pulmonary exacerbations should be defined (28,29). In practical terms, an exacerbation represents an acute deterioration in symptoms beyond the patient's usual day-to-day variation.…”

Exacerbations in cystic fibrosis {middle dot} 1: Epidemiology and pathogenesis

“…[32][33][34] Rosenfeld and colleagues, using data from a clinical trial, used a multivariate modelling approach to create an algorithm to identify participants with a pulmonary exacerbation. 33 Symptoms rather than physical examination and laboratory values were found to be more predictive of a pulmonary exacerbation.…”

Orta ve ağır akciğer tutulumu olan kistik fibrozlu hastalarda ölüme etki eden diğer risk etmenleri

“…The diagnosis of acute exacerbation of cystic fibrosis was made, when at least two of the following conditions were present: fever, newly-onset or increased cough or sputum production, hemoptysis, dyspnea, chest pain, fatigue, loss of appetite, a decrease of more than 10% in FEV1 or FVC and deterioration of pulmonary sounds (26). Patients with a diagnosis of acute exacerbation who had stable clinical findings were treated with oral antibiotics.…”