GLOMERULONEPHRITIS-PAUCI IMMUNE GN

SUMMARY

1. Characterised by negative or few Ig deposits on immunofluorescence or immunohistochemistry.
2. Cellular, fibrocellular and fibrous crescents may be present depending on the stage of the disease process.
3. 80-90% of patients are sero-positive for ANCA, related to ANCA-associated vasculitis.
4. Assoc. vasculitis: Microscopic Polyangiitis (MPA), Granulomatosis with Polyangittis (Wegener’s/GPA), Eosinophilic Granulomatosis with Polyangiitis (eGPA).
5. Pathologic subtypes: sclerotic (worse survival), focal (better prognosis), crescentic, mixed^[1].

SUBTYPES
6. GPA: assoc. with PR3-ANCA; URTI & ocular symptoms; relapse rates increased.
7. Microscopic Polyangittis: assoc. with MPO-ANCA, ILD. Good outcome when treated with Retuximab^[2].

Reference(s)

↑ Berden et al (2010) Histopathologic classification of ANCA-associated glomerulonephritis. JASN
↑ Unizony et al (2016) Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type. Ann Rheum Dis

Wilkinson, I., Furmedge, D. and Sinharay, R. (2017). Oxford handbook of clinical medicine. Oxford: Oxford University Press. Get it on Amazon.
Feather, A., Randall, D. and Waterhouse, M. (2020). Kumar And Clark’s Clinical Medicine. 10th ed. S.L.: Elsevier Health Sciences. Get it on Amazon.
Hannaman, R. A., Bullock, L., Hatchell, C. A., & Yoffe, M. (2016). Internal medicine review core curriculum, 2017-2018. CO Springs, CO: MedStudy.
Therapeutic Guidelines. Melbourne: Therapeutic Guidelines Limited. https://www.tg.org.au [Accessed 2021].

[1] Berden et al (2010) Histopathologic classification of ANCA-associated glomerulonephritis. JASN

[2] Unizony et al (2016) Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type. Ann Rheum Dis

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