0521779407-C02 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 20:53
Churg Strauss Disease 359follow-up
During Treatment
■Regularly assess disease activity by history and physical examination
including bilateral blood pressures and heart exam
➣GCA
After one month, taper corticosteroid dose by about 5 mg every
two weeks to 10–15 mg/day, and more slowly thereafter. Most
relapses occur in the first 18 months of treatment. Average
duration of therapy is about 2–3 years.
➣TATherapy may be protracted; (^1) / 4 of patients will require years of
immunosuppression
Subclavian involvement is common (90%), and blood pressure
readings may not reflect aortic root pressure. Angiography and
echocardiography may help
Follow-up vascular imaging required.
Surgical intervention for critical stenosis or aneurysms.
➣WG,CS
Monitor urinalysis, chest radiograph, clinical status
Long-term immunosuppression is usually required
c-ANCA roughly correlates with disease activity
complications and prognosis
■GCA
➣Thoracic aortic aneurysms 17 times, abdominal aneurysms 2.5
times, more likely than in age-matched controls
➣Visual loss (6–10%)
➣Stroke
➣Patients with GCA have a normal life expectancy
■TA
➣Aortic root involvement with valvular insufficiency and conges-
tive heart failure in 20%
➣Stroke
➣Progressive arterial and aorta lesions may require surgery
➣5 year survival 83%; 10 year survival 58% in patients with severe
disease
■WS, CS
➣Renal failure, stroke, mononeuritis, vision loss; tracheal stenosis,
nasal deformity, infection
➣5 year survival >80%