What is the mortality and morbidity associated with vasculitis?

Updated: Dec 10, 2018
  • Author: Nadia Jennifer Chiara Luca, MD; Chief Editor: Lawrence K Jung, MD  more...
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Morbidity and mortality in systemic vasculitides has been reviewed by Phillip and Luqmani; however, it is mainly based on adult data. [20]

In Kawasaki disease, acute mortality is 0.12% (mainly cardiac-related deaths). With appropriate treatment, the rate of coronary aneurysm development is approximately 2-4%.

Henoch-Schönlein purpura is usually a self-limited condition. The long-term prognosis relies mainly on the severity of renal involvement. The overall risk of end-stage renal disease is 1-3%, but this risk can increase to 20% if the patient presents with significant nephritis or nephrotic syndrome. [21] In adults with Henoch-Schönlein purpura, a higher rate of renal impairment is noted, and the 5-year survival rate is only approximately 75%.

Takaysu arteritis is associated with a 10-year survival rate of approximately 87%.

Polyarteritis nodosa and Churg-Strauss syndrome are associated with a 5-year survival rate of 75-80%.

Microscopic polyangiitis is associated with a 5 year survival rate of 45-75%.

Granulomatosis with polyangiitis (formerly Wegener granulomatosis) is associated with significant morbidity and mortality. Approximately 11% of patients require mechanical ventilation and/or require dialysis. [19] Survival is approximately 75% at 5 years in adults; survival data are not reported in pediatric patients.

Long-term data in childhood PACNS are lacking, but early recognition and treatment has been associated with good recovery. [22]

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