Rheumatology

IgG4-Related Disease

IgG4관련질환

Multi-organ swelling from IgG4+ plasma cell infiltration

How common
Common
Typical age
Variable age

What is it?

Core pathophysiology of IgG4-Related Disease: autoimmune/inflammatory tissue damage.

Commonly affected: Joints, connective tissue, vessels (variable)

How it develops

  1. TriggerGenetic susceptibility and environmental triggers for IgG4-Related Disease
  2. Immune activationAutoantibody production and immune cell activation
  3. Tissue inflammationChronic inflammation in target tissues
  4. ProgressionCourse depends on treatment

Symptoms

  • Joint/muscle painClassic IgG4-Related Disease pain pattern
  • Stiffness/dysfunctionMorning stiffness with reduced mobility
  • ConstitutionalFatigue, low-grade fever, weight loss

How it is examined

  • Joint/systemic examSwelling, tenderness, range of motion
  • Lab evaluationESR, CRP, autoantibodies, complement

Imaging

Joint/pelvic X-ray for structural assessment.

  • Joint changes
  • Soft tissue swelling

MRI or US for synovitis/enthesitis if needed.

  • Synovitis
  • Enthesitis

Non-surgical care

  • NSAIDsFirst-line pain/inflammation control
  • DMARD/immunosuppressantMethotrexate, hydroxychloroquine, etc.
  • RehabilitationMaintain joint mobility

Conservative options are generally tried first. Medications listed here can have side effects — discuss them with your prescriber.

When surgery is considered

Refractory disease or advanced organ damage

Procedures that may be discussed

  • Biologics (TNF/IL inhibitors)
  • JAK inhibitors
  • Joint replacement (end-stage)

Outlook

Good with early diagnosis and treatment.

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