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IgG4-related kidney disease

ORPHA:449395 · Clinical subtype · Subtype of disorder

HPO 表現型（共 59 項）

Very frequent (99-80%)(3)

  • Increased circulating IgG concentrationHP:0003237
  • Increased circulating IgG4 concentrationHP:0032300
  • Tubulointerstitial nephritisHP:0001970

Frequent (79-30%)(13)

  • Acute kidney injuryHP:0001919
  • Antinuclear antibody positivityHP:0003493
  • Chronic kidney diseaseHP:0012622
  • Decreased circulating complement C3 concentrationHP:0005421
  • Decreased circulating complement C4 concentrationHP:0045042
  • Elevated circulating creatinine concentrationHP:0003259
  • HematuriaHP:0000790
  • Increased circulating IgG1 concentrationHP:0032298
  • ProteinuriaHP:0000093
  • Reduced circulating complement concentrationHP:0004431
  • Renal insufficiencyHP:0000083
  • Renal interstitial immunoglobulin depositsHP:0032616
  • Rheumatoid factor positiveHP:0002923

Occasional (29-5%)(39)

  • Abdominal painHP:0002027
  • Abnormal aortic morphologyHP:0001679
  • Abnormal breast morphologyHP:0031093
  • Abnormal lung morphologyHP:0002088
  • Abnormal mediastinum morphologyHP:0045026
  • Abnormal mesentery morphologyHP:0100016
  • Abnormal ureter morphologyHP:0025633
  • Abnormality of the anterior pituitaryHP:0011747
  • AlbuminuriaHP:0012592
  • ArteritisHP:0012089
  • CholecystitisHP:0001082
  • Chronic sinusitisHP:0011109
  • DacryocystitisHP:0000620
  • Decreased circulating retinol-binding protein concentrationHP:0031032
  • Decreased liver functionHP:0001410
  • Enlarged kidneyHP:0000105
  • FatigueHP:0012378
  • Increased circulating IgE concentrationHP:0003212
  • Increased total eosinophil countHP:0001880
  • Inflammatory abnormality of the skinHP:0011123
  • Interstitial pneumonitisHP:0006515
  • LymphadenitisHP:0002840
  • LymphadenopathyHP:0002716
  • Lymphocytoma cutisHP:0031549
  • Membranous nephropathyHP:0012578
  • MeningitisHP:0001287
  • Nephrotic range proteinuriaHP:0012593
  • PancreatitisHP:0001733
  • PericarditisHP:0001701
  • PleuritisHP:0002102
  • ProstatitisHP:0000024
  • Retroperitoneal fibrosisHP:0005200
  • Sclerosing cholangitisHP:0030991
  • SialadenitisHP:0031281
  • ThyroiditisHP:0100646
  • Ureteral obstructionHP:0006000
  • UrethritisHP:0500006
  • Urinary bladder inflammationHP:0100577
  • Weight lossHP:0001824

Very rare (<4-1%)(3)

  • Elevated circulating C-reactive protein concentrationHP:0011227
  • HydronephrosisHP:0000126
  • Pedal edemaHP:0010741

Excluded (0%)(1)

  • Sterile pyuriaHP:0100586