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Primary Sjögren disease

ORPHA:289390 · Disease · Disorder

HPO 表現型（共 82 項）

Very frequent (99-80%)(2)

  • Keratoconjunctivitis siccaHP:0001097
  • XerostomiaHP:0000217

Frequent (79-30%)(17)

  • Abnormality of the kidneyHP:0000077
  • Abnormality of the musculatureHP:0003011
  • Abnormality of the nervous systemHP:0000707
  • Abnormality of the skinHP:0000951
  • Anti-carbonic anhydrase VI antibody positivityHP:0034066
  • Anti-parotid secretory protein antibody positivityHP:0034069
  • Anti-Ro/SS-A antibody positivityHP:0033555
  • Anti-salivary protein antibody positivityHP:0034064
  • AnxietyHP:0000739
  • ArthralgiaHP:0002829
  • Chronic painHP:0012532
  • FatigueHP:0012378
  • ParotitisHP:0011850
  • Polyarticular arthropathyHP:0005195
  • Reduced circulating complement concentrationHP:0004431
  • Tubulointerstitial nephritisHP:0001970
  • Usual interstitial pneumoniaHP:0031950

Occasional (29-5%)(54)

  • Abnormal cerebellum morphologyHP:0001317
  • Abnormal pulmonary interstitial morphologyHP:0006530
  • Abnormal pulmonary thoracic imaging findingHP:0031983
  • Abnormality of blood and blood-forming tissuesHP:0001871
  • Abnormality of the peripheral nervous systemHP:0410008
  • Airway obstructionHP:0006536
  • Anti-dsDNA antibody positivityHP:0020151
  • Anti-ribosome Po antibody positivityHP:0034076
  • Anti-Ro52/TRIM21 antibody positivityHP:0034093
  • ArteritisHP:0012089
  • ArthritisHP:0001369
  • Atypical behaviorHP:0000708
  • Biliary cirrhosisHP:0002613
  • BronchitisHP:0012387
  • Chronic hepatitisHP:0200123
  • CryoglobulinemiaHP:0100778
  • Cutis marmorataHP:0000965
  • Decreased circulating complement C3 concentrationHP:0005421
  • Decreased circulating complement C4 concentrationHP:0045042
  • Decreased circulating immunoglobulin concentrationHP:0004313
  • Decreased total CD4+ T cell proportionHP:0032218
  • Decreased total leukocyte countHP:0001882
  • Decreased total lymphocyte countHP:0001888
  • DepressionHP:0000716
  • Dry skinHP:0000958
  • Erythema nodosumHP:0012219
  • Functional motor deficitHP:0004302
  • GlomerulonephritisHP:0000099
  • Increased circulating immunoglobulin concentrationHP:0010702
  • Lichenoid skin lesionHP:0031452
  • LymphadenopathyHP:0002716
  • Lymphocytic interstitial pneumoniaHP:0006527
  • LymphomaHP:0002665
  • Lymphoproliferative disorderHP:0005523
  • MeningitisHP:0001287
  • Morphological central nervous system abnormalityHP:0002011
  • Muscle weaknessHP:0001324
  • MyalgiaHP:0003326
  • MyositisHP:0100614
  • Nonproductive coughHP:0031246
  • Normochromic anemiaHP:0001895
  • Normocytic anemiaHP:0001897
  • Optic neuritisHP:0100653
  • PurpuraHP:0000979
  • Raynaud phenomenonHP:0030880
  • Renal insufficiencyHP:0000083
  • SeizureHP:0001250
  • Skin ulcerHP:0200042
  • Somatic sensory dysfunctionHP:0003474
  • ThrombocytopeniaHP:0001873
  • ThyroiditisHP:0100646
  • Vaginal drynessHP:0031088
  • VasculitisHP:0002633
  • VitiligoHP:0001045

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

  • Abnormal spinal cord morphologyHP:0002143
  • ChoreaHP:0002072
  • Chronic active hepatitisHP:0200120
  • Cognitive impairmentHP:0100543
  • Corneal perforationHP:0100583
  • DementiaHP:0000726
  • Multiple mononeuropathyHP:0032018
  • Peripheral neuropathyHP:0009830
  • Sensorimotor neuropathyHP:0007141