Advanced lung disease (ALD) may be defined as a chronic, nonmalignant
lung disease that permanently impairs activities of daily living. Defi
ned in that fashion, the term encompasses the severe end of the spectr
um of many common and unusual lung diseases, including asthma, chronic
obstructive pulmonary disease (COPD), cystic fibrosis (CF), sarcoidos
is, the pneumoconioses, idiopathic pulmonary fibrosis, and pulmonary h
ypertension. Like ''end-stage kidney disease,'' the term advanced lung
disease is useful clinically because supportive treatment is similar
regardless of the original cause of organ injury. Many, but not all, p
atients with ALD are disabled. ALD is identified by a physician on the
basis of history, physical examination, and appropriate diagnostic st
udies, and is related to functional impairment. Disability is determin
ed by an insurance administrator or administrative law judge and, in a
dults, is related to ability to work.(42) Mortality statistics maintai
ned by the National Center for Health Statistics (NCHS) indicate that
COPD and allied conditions are the fifth leading cause of death in the
United States, after heart disease, cancer, accidents, and neurologic
disease(12) (Fig. 1). There was a 46.6% increase in the death rate fo
r chronic lung disease from 1979 to 1993, and a 7.5% increase from 199
2 to 1993.(12) The mortality statistics probably underestimate the mor
tality attributable to ALD because many individuals who are severely i
mpaired by smoking related chronic lung diseases actually die of other
causes, notably heart disease or cancer.(44) This review focuses on U
S data, although the burden of ALD worldwide obviously is more substan
tial. The NCHS is the principal source of information on morbidity and
mortality in the United States. It-obtains information through severa
l population-based surveys of representative samples of specific data
sources(45) (Table 1). Each of the surveys conducted by the NCHS provi
des data that are used to estimate some measure of disease incidence o
r prevalence, health care utilization rate, or death rate. Data are es
timated and reported principally for diseases with high incidences, su
ch as asthma and COED. Data on more rare conditions, such as CF, are g
enerally obtained from disease registries (when they exist), or from l
arge health care delivery databases, such as health maintenance organi
zations. Regardless of the source of information, it is not always pos
sible to determine the distribution of illness severity from databases
. As a result, many are less useful for assessing the extent of ALD, a
s previously defined.