Complications
Complications due to collapse of cartilage
can include facial disfigurement and obstruction of airways. Structures in the
ear and eye can collapse with devastating results. Vasculitis and unrelated
malignancy is also common.
Prognosis
The disease follows a relapsing and
remitting course with steady progression of the disease. The prognosis for any
individual will vary considerably depending on which systems are involved and
their response to therapy. With earlier recognition of the disease and optimal
treatment, the prognosis has improved, and in one study a 94% survival rate was
reported with an average 8-year follow-up.10 Up to 50% of deaths will be due to
pneumonia following involvement of cartilage in the respiratory system, with
cardiovascular involvement being the next most common cause of death. Another
group found that respiratory disease caused only 10% of deaths.3 The most
frequent causes of death were infection, systemic vasculitis, and malignancy.
