Relapsing Polycondritis

Relapsing polycondritis (RP) is a rare disease described for the first time a century ago under the term “polycondropathy” by Wartenhorst [1] and which owes its current name to Pearson et al. [2]. It is a systemic inflammatory immune-mediated disease that affects the cartilage tissues of ears, nose, and tracheobronchial tree but also the joints, eyes, inner ear, and cardiovascular system among several other systemic manifestations [3, 4]. It can present with different phenotypes. Some are life threatening such as the hematological phenotype associated with myelodysplasia and the respiratory one with predominant tracheobronchial involvement; other phenotypes are more benign, such as those characterized by isolated intermittent involvement of the cartilage of the nose and ears [5]. It is a very rare disease, with an estimated incidence of 0.7–3.7 per million person years [6–8]. A recent estimated prevalence of RP was in the United States 4.5 per million [9], while in the Hungarian population a prevalence of 20 per million has been calculated [8]. It appears to be ubiquitous across all ethnic groups although the majority of patients reported are of Caucasian origin and the disease is rare among sub-Saharan Africans [10]. It can appear at any age even although appears more frequently between the age of 40 and 50 years. For some authors there is a slight female predominance [11], while for others the disease affects both sexes similarly [10]. Predisposing genetic factors have been hypothesized and the occurrence of the disease in the same family has been reported [12].