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词条 Pauci-immune
释义

  1. See also

  2. References

  3. External links

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}}Pauci-immune (pauci- Latin: few, little) vasculitis is a form of vasculitis that is associated with minimal evidence of hypersensitivity upon immunofluorescent staining for IgG.[1]

Normally a kidney sample will be subjected to immunofluorescence if rapidly progressive glomerulonephritis is a concern. In this case, the immunofluorescence can show three patterns: linear, granular and negative (pauci-immune).[1] The linear and granular patterns are examples of positive immunofluorescence and are associated with Goodpasture syndrome and post-streptococcal glomerulonephritis accordingly.[1] A negative pattern or pauci-immune pattern can be associated with systemic vasculitis such as microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (EGPA) or granulomatosis with polyangiitis (GPA).[1][2] In many cases however, it is limited to the kidney and it is thus called idiopathic.

Since it can be associated with the three systemic vasculitides mentioned above, a pauci-immune pattern finding can be associated with antineutrophil cytoplasmic antibodies (ANCA).[3] Therefore, an ANCA test should follow a negative immunofluorescence result in order to distinguish between the above-mentioned systemic vasculitis.[1]

Peak incidences in 50- to 60-year-olds symptoms include intermittent fever / weight loss / shortness of breath / joint pain.

See also

  • Systemic vasculitis#Pauci-immune
  • Goodpasture Syndrome and Poststrep Glomerulonephritis
  • Microscopic polyangiitis, Eosinophilic granulomatosis with polyangiitis or Granulomatosis with polyangiitis

References

1. ^{{Cite book|title = Fundamentals of Pathology|last = Sattar|first =|publisher = 2011|year =|isbn =|location =|pages = 130}}
2. ^{{cite web |url=http://path.upmc.edu/cases/case51/dx.html |title=Final Diagnosis -- Case 51 |work= |accessdate=2009-06-08}}
3. ^{{cite journal |vauthors=Bollée G, Noël LH, Suarez F, etal |title=Pauci-immune crescentic glomerulonephritis associated with ANCA of IgA class |journal=Am. J. Kidney Dis. |volume=53 |issue=6 |pages=1063–7 |date=June 2009 |pmid=19084310 |doi=10.1053/j.ajkd.2008.10.039 |url=http://linkinghub.elsevier.com/retrieve/pii/S0272-6386(08)01604-1}}

External links

  • paucity
{{Systemic vasculitis}}{{Cutaneous-condition-stub}}

1 : Vascular-related cutaneous conditions

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