Study objective: To determine if elderly patients with Wegener's granu
lomatosis (WG) exhibit distinctive clinical features or outcomes compa
red, with patients whose conditions were diagnosed at younger ages. De
sign: Retrospective cohort study. Setting: University medical center.
Patients: Thirty-three patients with WG diagnosed when 60 years old or
older and 34 patients with WG diagnosed at age younger than 60 years,
identified by record review of all WG patients seen over an 11-year p
eriod. Results: The prevalence of specific clinical features, progress
ion to end-stage renal disease, mortality rate, and infectious and non
infectious complications of therapy were examined. The prevalence of u
pper respiratory tract involvement (rhinitis, sinusitis, otitis, epist
axis) and hemoptysis were significantly less common as initial manifes
tations in the elderly patients, although pulmonary infiltrates were s
een more commonly during the course of their disease. Renal insufficie
ncy was more common at the time of diagnosis in the elderly patients (
64% vs 35%; p<0.05). Most notably, CNS involvement was 4.5-fold more c
ommon in elderly patients (27% vs 6%; p=0.02). The overall incidence o
f infectious and noninfectious complications of therapy was similar be
tween the groups, although the mortality rate was markedly higher in t
he elderly patients (54% vs 19%; p<0.01). Almost all deaths were due t
o overwhelming infection. Conclusions: Elderly patients with WG presen
t with distinctive clinical features, particularly a relatively low in
cidence of upper respiratory tract complaints and a high incidence of
CNS involvement. The mortality risk from infectious complications of W
G is substantially higher in elderly patients, although this cannot be
attributed directly to adverse affects of therapy.