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IgG4-related dacryoadenitis and sialadenitis

ORPHA:79078 · Clinical subtype · Subtype of disorder

HPO 表現型（共 33 項）

Very frequent (99-80%)(4)

  • Abnormal salivary gland morphologyHP:0010286
  • Abnormality of the submandibular glandsHP:0010287
  • Enlarged lacrimal glandsHP:0007734
  • Increased circulating IgG4 concentrationHP:0032300

Frequent (79-30%)(1)

  • Enlargement of parotid glandHP:0011801

Occasional (29-5%)(19)

  • Abnormal optic nerve morphologyHP:0000587
  • Abnormality of the extraocular musclesHP:0008049
  • Abnormality of the orbital regionHP:0000315
  • Antinuclear antibody positivityHP:0003493
  • AutoimmunityHP:0002960
  • Cytoplasmic antineutrophil antibody positivityHP:0032230
  • Facial edemaHP:0000282
  • FatigueHP:0012378
  • FeverHP:0001945
  • Generalized muscle weaknessHP:0003324
  • Increased circulating IgA concentrationHP:0003261
  • Increased circulating IgM concentrationHP:0003496
  • Keratoconjunctivitis siccaHP:0001097
  • MyositisHP:0100614
  • Palpebral edemaHP:0100540
  • Periorbital fullnessHP:0000629
  • ProptosisHP:0000520
  • Weight lossHP:0001824
  • XerostomiaHP:0000217

Very rare (<4-1%)(9)

  • Abnormal lacrimal duct morphologyHP:0011481
  • BlindnessHP:0000618
  • LymphadenopathyHP:0002716
  • Nodular goiterHP:0005994
  • Optic nerve compressionHP:0007807
  • Retroperitoneal fibrosisHP:0005200
  • ThrombocytopeniaHP:0001873
  • ThyroiditisHP:0100646
  • Tubulointerstitial nephritisHP:0001970