Survival of patients with severe alpha 1-antitrypsin deficiency with special reference to non-index cases.
BACKGROUND
Previous estimates of the survival times of patients with alpha 1-antitrypsin deficiency have been based on selected patients.


METHODS
The survival times of 397 patients with severe alpha 1-antitrypsin deficiency identified by pulmonary impairment (index cases) or through family studies (non-index cases) were compared.


RESULTS
The overall median survival time was 54.5 years with no significant difference between men and women. Survival for index cases was less than for the non-index cases regardless of smoking history (49.4 years and 69.3 years respectively). When index and non-index cases were analysed separately there was no difference between the survival of smokers and never smokers in the index group. In the non-index group smokers had a shorter survival time than never smokers. The survival time of never smokers was similar to that of the normal Danish population.


CONCLUSIONS
The prognosis of severe alpha 1-antitrypsin deficiency is better than previously assumed and, although smoking is a major risk factor, the development of emphysema in patients with severe alpha 1-antitrypsin deficiency is multifactorial.
Seersholm
N
N
Department of Pulmonology, Bispebjerg Hospital, Copenhagen, Denmark.
Kok-Jensen
A
A
Dirksen
A
A
D000328:Adult; D017677:Age Distribution; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011656:Pulmonary Emphysema / Q000209:etiology* / Q000401:mortality; D012307:Risk Factors; D012907:Smoking / Q000009:adverse effects / Q000401:mortality; D015996:Survival Rate; D000515:alpha 1-Antitrypsin
D000515:alpha 1-Antitrypsin