What are the prognosis for relapsing polychondritis (RP)?

Updated: Dec 24, 2020
  • Author: Nicholas Compton, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

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.

A study analyzing mortality rates of relapsing polychondritis based on organ involvement at disease onset reported that the 5-year mortality was higher in patients with respiratory onset (15%) and lowest in patients with auricular onset (5.9%).  The mortality rate in patients with CNS, ocular, and inner ear involvment, among others, was also 15%. [29]

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.

Although the life expectancy in all patients with relapsing polychondritis is decreased compared with age- and sex-matched healthy individuals, patients with renal involvement have a significantly lower age-adjusted life expectancy. In those with renal disease, uremia is the third most frequent cause of death.

Dion et al identified three factors associated with death in patients with relapsing polychondritis, as follows [30] :

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

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