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Pauci-immune glomerulonephritis

ORPHA:93126 · Disease · Disorder

HPO 表現型（共 37 項）

Obligate (100%)(1)

  • GlomerulonephritisHP:0000099

Very frequent (99-80%)(7)

  • Cytoplasmic antineutrophil antibody positivityHP:0032230
  • Decreased glomerular filtration rateHP:0012213
  • Elevated circulating creatinine concentrationHP:0003259
  • Microscopic hematuriaHP:0002907
  • ProteinuriaHP:0000093
  • Renal insufficiencyHP:0000083
  • Small vessel vasculitisHP:0011944

Frequent (79-30%)(5)

  • Abnormality of the respiratory systemHP:0002086
  • Abnormality of the skinHP:0000951
  • Crescentic glomerulonephritisHP:0008653
  • FeverHP:0001945
  • Glomerular sclerosisHP:0000096

Occasional (29-5%)(16)

  • Abdominal painHP:0002027
  • Abnormality of the eyeHP:0000478
  • Abnormality of the pulmonary vasculatureHP:0004930
  • Abnormality of the upper respiratory tractHP:0002087
  • Acute kidney injuryHP:0001919
  • Antinuclear antibody positivityHP:0003493
  • ArteritisHP:0012089
  • ArthralgiaHP:0002829
  • CoughHP:0012735
  • DyspneaHP:0002094
  • Hoarse voiceHP:0001609
  • Macroscopic hematuriaHP:0012587
  • Nephrotic range proteinuriaHP:0012593
  • Pulmonary hemorrhageHP:0040223
  • PurpuraHP:0000979
  • Tubulointerstitial nephritisHP:0001970

Very rare (<4-1%)(6)

  • Abnormality of the cardiovascular systemHP:0001626
  • Abnormality of the gastrointestinal tractHP:0011024
  • Multiple mononeuropathyHP:0032018
  • Oral ulcerHP:0000155
  • PancreatitisHP:0001733
  • ScleritisHP:0100532

Excluded (0%)(2)

  • GranulomatosisHP:0002955
  • Renal interstitial immunoglobulin depositsHP:0032616