Info

🌱 來自: rheumatology

IgG4-related disease

• Definition & etiology (NEJM 2012;366:539; Nat Rev Rheumatol 2020;16:702)

  • Characterized by tumor-like inflammatory lesions that can affect nearly any organ

• Etiology: ? autoimmune; unclear role of IgG4; may have h/o atopy

  • ♂ >♀, mean age ~ 60.
  • Incidence ~1/100,000 per y in Japan, but elsewhere unknown.

• Clinical manifestations of IgG4-related disease 󰒗 (Arthritis Rheumatol 2015;67:2466 & 2020;72:7)

  • Insidious progression;
  • multiple lesions may be present synchronously or metachronously

Diagnosis and management (Lancet Rheumatol 2019;1:e55)

• Biopsy w/ specific findings:
  • lymphoplasmacytic infiltrate
  • w/ significant IgG4+ plasma cell infiltrate,
  • storiform fibrosis,
  • obliterative phlebitis
• ↑ serum IgG4 (Se 90%, Sp 60%); may have low C3, C4
• Highly responsive to steroids but relapse common.
  • Efficacy of DMARDs in maintenance remains unclear but B-cell depleting agents appear promising (Eur J Intern Med 2020;74:92).