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FRI0373 The Clinical and Demographic Features of Relapsing Polychondritis: A Nationwide Study
  1. H. Emmungil1,
  2. A. Erden2,
  3. O. Küçükşahin3,
  4. O. Zengin4,
  5. S. Ermurat5,
  6. K. Gök6,
  7. A. Doğru7,
  8. M. Kalfa8,
  9. L. Akyol9,
  10. F. Erbasan10,
  11. M. Çınar11,
  12. M.A. Balcı12,
  13. A. Yazıcı13,
  14. S. Yavuz14,
  15. B. Göker15,
  16. A. Sarı2,
  17. M. Turgay16,
  18. A.M. Onat4,
  19. B. Kısacık4,
  20. Y. Pehlivan5,
  21. S. Şenel6,
  22. M. Şahin7,
  23. M. Sayarlıoğlu9,
  24. Ö.N. Pamuk12,
  25. A. Çefle13,
  26. S.Z. Aydın1,
  27. U. Kalyoncu2
  1. 1Rheumatology, Koç University Hospital, Istanbul
  2. 2Hacettepe University
  3. 3Yıldırım Beyazit University, Ankara
  4. 4Gaziantep University, Gaziantep
  5. 5Uludağ University, Bursa
  6. 6Erciyes University, Kayseri
  7. 7Süleyman Demirel University, Isparta
  8. 8Acıbadem University, Istanbul
  9. 9Ondokuz Mayıs University, Samsun
  10. 10Antalya Education and Research Hospital, Antalya
  11. 11Gulhane Military Medical Academy, Ankara
  12. 12Trakya University, Edirne
  13. 13Kocaeli University, Kocaeli
  14. 14Bilim University, Istanbul
  15. 15Gazi University
  16. 16Ankara University, Ankara, Turkey

Abstract

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)

Table 1.

Clinical features at diagnosis

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

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