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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.

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  1. ^ a b c d e 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.

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