Relapsing Polychondritis Follow-up

Updated: Sep 25, 2017
  • Author: Nicholas Compton, MD; Chief Editor: Herbert S Diamond, MD  more...
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Follow-up

Prognosis

In earlier studies, the 5-year survival rate associated with relapsing polychondritis was reported to be 66%-74% (45% if relapsing polychondritis occurs with systemic vasculitis), with a 10-year survival rate of 55%. More recently, Trentham and Le found a survival rate of 94% at 8 years. [19] However, those data may represent relapsing polychondritis in patients with less severe disease than patients studied in earlier reports.

The most common causes of relapsing polychondritis–related death include infection secondary to corticosteroid treatment or respiratory compromise (10-50% of deaths result from airway complications), systemic vasculitis, and malignancy unrelated to relapsing polychondritis.

Dion et al identified three factors associated with death in patients with relapsing polychondritis, as follows [27] :

  • Male sex
  • Cardiac abnormalities
  • Concomitant myelodysplastic syndrome or other hematologic malignancy

Complications of relapsing polychondritis such as saddle-nose deformity, systemic vasculitis, laryngotracheobronchial stricture, arthritis, and anemia in patients younger than 51 years portend a poorer prognosis than in age-matched patients with relapsing polychondritis without complications. Among patients older than 51 years, only anemia is associated with a poorer prognosis. Renal involvement is a poor prognostic factor at all ages.