Relapsing polychondritis

Background

• Rare autoimmune/inflammatory disorder

Clinical Manifestations

• Acute pain, swelling, and erythema of cartilage
  • Episodes remit over weeks or months, and recur over years
• Most commonly of pinna (spares the other soft tissues), followed by nasal cartilage and arthritis
  • Arthritis may be symmetric or asymmetric, large or small joint, but is typically non-deforming
• Other tissues may include:
  • Eye, including conjunctivitis, scleritis, iritis, keratitis, and chorioretinitis
  • Cartilage of the larynx, trachea, or bronchi
  • Internal ear
  • Cardiovascular system, including aortic regurgitation, mitral regurgitation, pericarditis, myocarditis, aortic aneurysm, and aortitis
  • Kidney
  • Skin
• May eventually lead to:
  • Floppy ears
  • Saddle nose deformity
  • Pectus excavatum
  • Visual, auditory, and vestibular abnormalities
  • Tracheal narrowing and tracheal collapse
• Can occur in the context of systemic vasculitis (such as leukocytoclastic vasculitis or polyarteritis nodosa), myelodysplastic syndrome, or cancer is possible

Differential Diagnosis

• Syphilis
• Granulomatosis with polyangiitis

Diagnostic Criteria

• Michet criteria require 2 major, or 1 major with 2 minor criteria1
• Major criteria:
  • Auricular cartilage inflammation
  • Nasal cartilage inflammation
  • Laryngotracheal cartilage inflammation
• Minor criteria:
  • Seronegative arthritis
  • Ocular inflammation
  • Hearing loss
  • Vestibular dysfunction