ABSTRACT
Pulmonary infection and an excessive neutrophil-driven inflammatory response are responsible
for most of the morbidity and mortality associated with cystic fibrosis. Although
inflammation is first and foremost a protective response to injury or infection it
has the potential to cause considerable harm when it is excessive. Whereas most published
reports emphasize the damaging effects of the chronic inflammatory response in cystic
fibrosis, the beneficial effects are more difficult to quantify. Low levels of inflammation
may assist in clearing infection, particularly early in the disease process, and surges
of acute inflammation may be beneficial during exacerbations. Anti-inflammatory therapies
are used to modify the inflammatory response but there is clearly a need to preserve
the protective aspects of the inflammatory response because host defense and a fine
balance exist between benefit and harm. The underlying processes involved in the inflammatory
response are reviewed along with current and future anti-inflammatory therapies.
KEYWORDS
Cystic fibrosis - inflammation - host defense
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Duncan GeddesM.D. F.R.C.P.
Department of Respiratory Medicine, Royal Brompton Hospital
Sydney St., London SW3 6NP, UK
eMail: d.geddes@rbht.nhs.uk