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). The linear and granular patterns are examples of positive immunofluorescence and are associated with Goodpasture syndrome and post-streptococcal glomerulonephritis accordingly. 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). 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). Therefore, an ANCA test should follow a negative immunofluorescence result in order to distinguish between the above-mentioned systemic vasculitis.
- Systemic vasculitis#Pauci-immune
- Goodpasture Syndrome and Poststrep Glomerulonephritis
- Microscopic polyangiitis, Eosinophilic granulomatosis with polyangiitis or Granulomatosis with polyangiitis
- Sattar. Fundamentals of Pathology. 2011. p. 130.
- "Final Diagnosis -- Case 51". Retrieved 2009-06-08.
- 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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