Info

🌱 來自: adult-onset Still’s disease

Relapsing polychondritis

(Rheumatology 2018;57:1525)

• Inflammatory destruction of cartilaginous structures;
• typical onset age 40-60, ♂=♀, <1/million per y incidence
• Subacute onset of red, painful, and swollen cartilage;
• ultimately atrophic & deformed
• Multiple sites of cartilaginous inflammation:
  • bilateral auricular chondritis,
  • nonerosive inflammatory arthritis,
  • nasal chondritis,
  • laryngeal or tracheal chondritis,
  • valvulopathy. Ocular inflammation and cochlear/vestibular dysfxn also common.
• 40% of cases a/w
  • immunologic disorder (eg, RA, SLE, vasc., Sjögren’s),
  • cancer or MDS (including VEXAS; NEJM 2020;383:2628)
• Labs: ↑ ESR & CRP, leukocytosis, eosinophilia, anemia of chronic inflammation
• Bx (not req for dx): proteoglycan
  • depletion, perichondrial inflammation and replacement with granulation tissue and fibrosis;
  • immunofluorescence with Ig and C3 deposits
• Screen for pulm (PFTs, CXR/CT, ± bronch) and cardiac (ECG, TTE) involvement
• Rx guided by disease activity/severity:
  • steroids 1st line;
  • NSAIDs/dapsone for arthralgias, mild disease;
  • MTX, AZA, or biologics steroid-sparing;
  • cyclophosph if organ-threatening