Glomerulonephritis

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ANCA - Vasculitis (pauci-immune or minimal staining)

Disease

Gran

Renal

Pulm

Asthma

ANCA Type*

ANCA®

Wegener's granulomatosis

© 80%

(anu-PtU)

90%

Microscopic polyangiitis

90%

(anci-MPO)

70%

Churg-Strauss syndrome

®

45%

70%

©

p-ANCA (anti-MPO)

50%

•Predominant ANCA type; either p- or c-ANCA can be seen mi all three diseases (NEJM 1997;337:1512)

Anti-GBM Disease (linear staining)

Disease

Glomerulonephritis

Pulm hemorrhage

Anti-GBM

Goodpasture's

©

©

©

Anti-GBM disease

©

©

Immune Complex (IC) Disease (granular staining)

Renal-limited diseases Systemic diseases

Poststreptococcal GN

(PSGN. • ASIO.I C3)

(© ANA anti-dsDNA, I C3)

Membranoproliferative GN

(fever. '5 BCx. valvular disease. 1 C3)

Fibrillary glomerulonephritis

(® cryocrit. 1 C3.1 C4. HCV Ab)

IgA nephropathy

(normal C3)

Henoch-Schonlein purpura

(IgA nephropathy • systemic vasculitis, normal C3)

Workup (Archives 2001:161:25)

• AGN RPGN • lung hemorrhage is an emergency - • requires early Dx and Rx

• ANCA (Lancet 2006:368:404), anti-GBM, complement levels (C3, C4)

• Depending on clinical hx: ANA.ASLO. blood cultures, cryocrit. hepatitis serologies

• Renal biopsy with immunofluorescence (IF) i electron microscopy (EM)

• Consider GN mimics: thrombotic microangiopathy, cholesterol emboli.AIN. myeloma Figure 4-7 Approach to glomerulonephritis

Treatment

• ANCA ©, anti-GBM: immediate steroids ♦ cyclophosphamide; • plasmapheresis

• SLE nephritis: IV cyclophosphamide • steroids — azathioprine or MMF (jama 2005:293.3053);

induction with MMF (no cyclophosphamide) may be as effective (nejm 2005.353 2219)

• Other IC disease: ? steroids • alkylating agents; treat underlying systemic disease

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