Original ArticlePediatric-Onset Relapsing Polychondritis: Case Series and Systematic Review
Section snippets
Methods
We retrospectively investigated the databases of 3 French hospitals (Hôpital de la Pitié Salpetrière, Paris; Hôpital Saint Vincent de Paul, Paris; Hôpital Femme Mère Enfant, Lyon) to find cases of pediatric-onset RP diagnosed from 1961 to 2007. In all patients, we used the diagnostic criteria of Michet et al.3 To be included, a patient must have proven inflammatory episodes involving at least 2 of 3 sites—auricular, nasal, or laryngotracheal cartilage—or 1 of these sites and 2 other
Current French Pediatric Series
Ten patients were included, with the first episode of chondritis between 2 and 17 years of age. The characteristics of the patients are reported in Table I.
Familial autoimmunity was represented by inflammatory arthritis, type 1 diabetes mellitus, Hashimoto thyroiditis, and RP. Patient 9 and patient 10 are relatives (brother and sister).
The mean duration of follow-up was 14.4 years (SD, 13.4 years). Initial symptoms included chondritis (mainly auricular), arthritis, and conjunctivitis, and the
Discussion
RP is a rare disease that mainly affects adults during the fourth or fifth decade. We estimate that pediatric-onset RP represents <5% of the reported cases. Most pediatric-onset RP has been published as single case reports, and no published pediatric series previously has been available.
In our series, the age at onset varied from 1.7 months to 17 years, and young children and adolescent can be affected; no case has previously been reported before the age of 2 years.26, 35, 36 In the 1970s, a
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2016, Autoimmunity ReviewsCitation Excerpt :In fact, anti-cartilage antibodies are detected in at least 33% of RP patients, and their titers appear to correlate with disease severity. [15,16] It is considered an immune-mediated disease, as there is an overlap between well documented RP and other rheumatic and autoimmune diseases [3–5,7–10,17–19]. Although a large number of cases have been reported recently, and the knowledge of the clinical features, pathogenesis, and management of the RP has increased considerably, the literature data available are very limited [5,20].
Relapsing polychondritis: A 2016 update on clinical features, diagnostic tools, treatment and biological drug use
2016, Best Practice and Research: Clinical RheumatologyCitation Excerpt :Paediatric cases do not have specific features except a high prevalence of a personal or family history of other autoimmune diseases. The cartilaginous involvement does not delay growth [27]. The disease affects men and women equally [23,26], although some series show a slight female predominance [15,28].
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The authors declare no conflicts of interest.