Pauci-immune
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 patters: 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, 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
- Systemic vasculitis#Pauci-immune
- Goodpasture Syndrome and Poststrep Glomerulonephritis
- Microscopic polyangiitis, Churg–Strauss syndrome or Granulomatosis with polyangiitis
References
External links
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