Background Relapsing polychondritis (RP) is a rare systemic and inflammatory disease.
Objectives to assess clinical features, management and prognosis in RP.
Methods Retrospective medical records were collected from 16 centers for 37 patients with RP diagnosed by Michet criteria. Data included patients' demographics, clinical features, laboratory findings and outcome.
Results A total of 37 patients, (Male/female=19/18) with a mean disease onset of 45.7±11.8 years and median follow up period is 20 (1–360) months were enrolled. The most common initial complaints and organs involved were showed in table 1. Mean lead time to diagnose was 28.6±54.3 (0–228) months. Four patients had a known history of cartilage injury before the RP diagnosis (Three goiter operation and 1 resuscitation due to a drowning). Twelve of the patients (32.4%) had also various autoimmune or hematologic diseases. After the diagnosis, moderate or high dose prednisone (n=37, 100%), plus methotrexate (n=15, 40.5%) or azathioprine (AZA) (n=11, 29.7%) or hydroxychloroquine (HQ) (n=3, 8.1%) or cyclophosphamide (CYP) (n=2, 5.4%), or (AZA+ HQ n=3, 8.1%) treatments were initiated. In the case of resistant disease, CYP (n=6, %16.2), tocilizumab (n=2, 5.4%), etanercept (n=1, 2.7%), infliximab (n=1, 2.7%) and bevacizumab (n=1, 2.7%) treatments were given. Due to tracheal stenosis surgery applied to 2 patients, one patient died due to respiratory collapse, 21 patients on remission without any complications, 13 (35.1%) patients had developed tracheal stenosis (n=5, 13.5%), major airway cartilage loss (n=5, 13.5%), auricular collapse (n=2, 5.4%), nasal collapse (n=3, 8.1%), subglottic stenosis (n=2, 5.4%), hearing loss (n=5, 13.5%), vision loss (n=1, 2.7%) and deforming arthritis (n=1, 2.7%). In the univariate analysis, only major airway involvement had a relationship with complication development. (OR 12, 95% CI 1.97–72.8, p=0.007)
Conclusions RP is a rare disease which diagnosis is commonly delayed and lead to cartilage loss. RP developed after trauma in 4 patients, possibly trauma can be blamed as an etiologic factor. Awareness and early diagnosis results with better prognosis
Disclosure of Interest None declared