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Review
. 2019 Dec;40(6):792-803.
doi: 10.1055/s-0039-1697975. Epub 2019 Oct 28.

Acute Pulmonary Exacerbations in Cystic Fibrosis

Christopher H Goss  1   2
Affiliations
Review

Acute Pulmonary Exacerbations in Cystic Fibrosis

Christopher H Goss. Semin Respir Crit Care Med. 2019 Dec.

Abstract

With the improving survival of cystic fibrosis (CF) patients and the advent of highly effective cystic fibrosis transmembrane conductance regulator therapy, the clinical spectrum of this complex multisystem disease continues to evolve. One of the most important clinical events for patients with CF in the course of this disease is an acute pulmonary exacerbation. Clinical and microbial epidemiology studies of CF pulmonary exacerbations continue to provide important insight into the disease course, prognosis, and complications. This work has now led to a number of large scale clinical trials with the goal of improving the treatment paradigm for CF pulmonary exacerbation. The primary goal of this review is to provide a summary of the pathophysiology, the clinical epidemiology, microbial epidemiology, outcome and the treatment of CF pulmonary exacerbation.

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Conflict of interest statement

Disclosure The authors report no conflicts of interest in this work.

Figures

Figure 1.
Figure 1.
FEV1 for nonresponders and responders at baseline, treatment initiation, and the best FEV1 in the 3 months after treatment (n = 4,391). Responders are in white, nonresponders are in gray. The boxes represent the middle 50% of patients; the whiskers include all patients in each group. The horizontal line within the box represents the median FEV1. The x axis represents three time points: the best FEV1 in the 6 months before admission (baseline); FEV1 at treatment initiation (exacerbation); and the best FEV1 in the 3 months after treatment (follow-up). Reprinted with permission from the AJRCCM. * represent P<0.05
Figure 2a and 2b.
Figure 2a and 2b.
2a: Patient’s mean the Chronic Respiratory Infection Symptom Score (CRISS) +/− SD by day of treatment with day 0 the beginning of IV treatment. The CRISS ranges from 0–100 with 100 being the worst possible respiratory symptoms. 2b: notes the FEV1 % predicted change from admission for IV antibiotics by days of treatment. The bar graph notes those with an absolute change of 5% predicted and 10% predicted by end of IV treatment and them by day 28.
Figure 2a and 2b.
Figure 2a and 2b.
2a: Patient’s mean the Chronic Respiratory Infection Symptom Score (CRISS) +/− SD by day of treatment with day 0 the beginning of IV treatment. The CRISS ranges from 0–100 with 100 being the worst possible respiratory symptoms. 2b: notes the FEV1 % predicted change from admission for IV antibiotics by days of treatment. The bar graph notes those with an absolute change of 5% predicted and 10% predicted by end of IV treatment and them by day 28.
Figure 3.
Figure 3.
Distribution of IV antibiotic treatment durations in the US CFFPR. Grey bars reflect the number of exacerbations where patients were admitted to the hospital. Black bars show numbers of patients of a given treatment duration who were never admitted to hospital as part of their exacerbation treatment. Reprinted with permission of the Journal of Cystic Fibrosis.
See this image and copyright information in PMC

References

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