IgG4 Related Disease

Etiology

  • A systemic fibro-inflammatory d/o from infiltration of IgG4 producing plasma cells, can affect most organ systems
  • Sx caused by interfering w/fxn and/or local enlargement

Risk Factors

  • Age > 50
  • M>f

Symptoms

  • Sub-acute
  • Afebrile
  • Not typically acutely ill, not always progressive
  • One or multiple organs affected simultaneously
  • May have asthma, eczema, rhino-sinusitis or other allergic features
  • Organ specific sx include:
  • Generalized/local adenopathy
  • Autoimmune pancreatitis→ abdomen pain, wt loss, jaundice
  • Peri-orbital infiltration→ swelling, pain
  • Nose/sinus→ nasal obstruction, sinus dysfxn, d/c
  • Sub-mandibular/parotid→ enlargement or dysfxn
  • Hypothalamus/pituitary→ fatigue, ha, wt loss, visual changes
  • Thyroid→ increased size, hypothyroidism related sx
  • Aorta→aortitis w/aneurysm or dissection
  • Retroperitoneum→fibrosis w/ureteral obstruction

Physical Exam Findings

  • Organ specific enlargement, though this can be otherwise asx
  • Findings that are sequelae of organ specific dysfxn→ e.g. epigastric pain w/pancreatitis
  • Adenopathy, 1-2cm, non-tender; other based on affected organs