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IgG4-related retroperitoneal fibrosis

ORPHA:49041 · Clinical subtype · Subtype of disorder

HPO 表現型（共 43 項）

Very frequent (99-80%)(1)

  • PainHP:0012531

Frequent (79-30%)(18)

  • Abdominal painHP:0002027
  • AnorexiaHP:0002039
  • Anti-thyroid peroxidase antibody positivityHP:0025379
  • Deep dermal perivascular inflammatory infiltrateHP:0031191
  • Elevated circulating C-reactive protein concentrationHP:0011227
  • Elevated circulating creatinine concentrationHP:0003259
  • Elevated erythrocyte sedimentation rateHP:0003565
  • FatigueHP:0012378
  • Flank painHP:0030157
  • HydronephrosisHP:0000126
  • HypertensionHP:0000822
  • Increased blood urea nitrogenHP:0003138
  • Large vessel vasculitisHP:0005310
  • Low back painHP:0003419
  • Normocytic anemiaHP:0001897
  • Renal insufficiencyHP:0000083
  • Unilateral renal hypoplasiaHP:0012583
  • Weight lossHP:0001824

Occasional (29-5%)(14)

  • Acute kidney injuryHP:0001919
  • Anti-smooth muscle antibody positivityHP:0003262
  • Antineutrophil antibody positivityHP:0003453
  • ConstipationHP:0002019
  • FeverHP:0001945
  • Hashimoto thyroiditisHP:0000872
  • Membranous nephropathyHP:0012578
  • Nausea and vomitingHP:0002017
  • Pedal edemaHP:0010741
  • Psoriasiform dermatitisHP:0003765
  • Rheumatoid arthritisHP:0001370
  • Rheumatoid factor positiveHP:0002923
  • Systemic lupus erythematosusHP:0002725
  • Ureteropelvic junction obstructionHP:0000074

Very rare (<4-1%)(10)

  • Budd-Chiari syndromeHP:0002639
  • DysuriaHP:0100518
  • HematuriaHP:0000790
  • Hydrocele testisHP:0000034
  • ImpotenceHP:0000802
  • Nephrotic syndromeHP:0000100
  • Renal tubular epithelial necrosisHP:0008682
  • Renovascular hypertensionHP:0100817
  • Retrograde ejaculationHP:0012877
  • VaricoceleHP:0012871