Relapsing Polychondritis


Hx

  • Autoimmune-like episodic or progressive inflammation of cartilage and other connective tissue:
    • Ear, Eye, Nose, Large airway, Joint, Heart, Kidney & skin are all involved
  • Often comorbid disease
  • M=F, 50yo ± 10 years, Caucasians

Diagnosis

  • At least three of McAdam's diagnostic criteria
  • ≥ 1 the above clinical findings with positive histolgy Dx
  • Chondritis at two or more separate anatomic locations with response to steroids and/or dapsone

McAdams criteria

  1. Bilateral auricular chondritis
  2. Nonerosive, seronegative polyarthritis
  3. Nasal chondritis
  4. Ocular inflammation: conjunctivitis, keratitis, scleritis/episcleritis, uveitis
  5. Respiratory tract chondritis: laryngeal and/or tracheal cartilages
  6. Cochlear and/or vestibular dysfunction: neurosensory hearing loss, tinnitus and/or vertigo

Management

  • Dapsone- 50- 200mg OD
  • Steroids
  • Cyclophosphamide
  • Azathioprine
  • Cyclosporine

References

Kent PD, Michet CJ Jr, Luthra HS. Relapsing polychondritis. Curr Opin Rheumatol 2004; 16:56.


Content by Dr Íomhar O' Sullivan. Last review Dr ÍOS 28/05/21.