Pauci-immune

Pauci-immune (pauci- Latin: few, little), also known as Negative Immuno Fluorescence, is a general term used to refer to a form of vasculitis that is associated with minimal evidence of hypersensitivity upon immunofluorescence, that is, there will be limited to no immunofluorescence (dark) shown.[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 patters: linear, granular and negative (pauci).[1] The linear and granular patterns are examples of positive immunofluorescence and are associated with Goodpasture Syndrome and Poststrep Glomerulonephritis accordingly.[1] A negative pattern or Pauci-immune pattern can be associated with systemic vasculitis such as Microscopic polyangiitis, Churg–Strauss syndrome or Granulomatosis with polyangiitis.[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

References

  1. 1 2 3 4 5 Sattar. Fundamentals of Pathology. 2011. p. 130.
  2. ↑ "Final Diagnosis -- Case 51". Retrieved 2009-06-08.
  3. ↑ Bollée G, Noël LH, Suarez F, et al. (June 2009). "Pauci-immune crescentic glomerulonephritis associated with ANCA of IgA class". Am. J. Kidney Dis. 53 (6): 1063–7. doi:10.1053/j.ajkd.2008.10.039. PMID 19084310.

External links


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