What is the role of ANCA antibody testing in the workup of Goodpasture syndrome (anti–glomerular basement membrane disease) (anti-GBM)?

Updated: Dec 16, 2020
  • Author: Pranay Kathuria, MD, FACP, FASN, FNKF; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Answer

At some time during the course of illness, as many as one third of patients with Goodpasture syndrome have circulating antineutrophilic cytoplasmic antibodies (ANCAs) in addition to anti-GBM antibody. [21] In most cases, the ANCA antibodies precede the development of anti-GBM antibodies by months to years. [27] It is postulated that the renal involvement in ANCA vasculitis leads to the exposure of antigens from the basement membrane and the formation of antibodies. These patients are referred to as double-positive.

Cytoplasmic ANCA (c-ANCA) and perinuclear ANCA (p-ANCA) are seen in the images below.

Cytoplasmic antineutrophilic cytoplasmic antibodies (c-ANCA), which can appear in Goodpasture syndrome, are also commonly observed in Wegener granulomatosis and other vasculitides.

Cytoplasmic antineutrophilic cytoplasmic antibodie Cytoplasmic antineutrophilic cytoplasmic antibodies (c-ANCA), which can appear in Goodpasture syndrome, are also commonly observed in Wegener granulomatosis and other vasculitides.

Perinuclear antineutrophilic cytoplasmic antibodies (p-ANCA), which can appear in Goodpasture syndrome, are also observed in Churg-Strauss vasculitis and occasionally in Wegener granulomatosis.

Perinuclear antineutrophilic cytoplasmic antibodie Perinuclear antineutrophilic cytoplasmic antibodies (p-ANCA), which can appear in Goodpasture syndrome, are also observed in Churg-Strauss vasculitis and occasionally in Wegener granulomatosis.

In the majority of double-positive patients, the ANCAs have specificity for myeloperoxidase (MPO-ANCA). [28, 29] In patients with both anti-GBM antibodies and MPO-ANCAs, histological findings differ from those of patients with anti-GBM antibodies only. The renal survival in these patients is similar to anti-GBM–positive patients and is worse compared with patients with MPO-ANCAs only.

In an analysis of the diagnostic performance of two rapid ANCA and anti-GBM test methods in 260 patients with suspected ANCA-associated small vessel vasculitis, de Joode and colleagues found that both the Dotblot and Phadia ELiA on anti-GBM, anti-PR3(s) and anti-MPO(s) performed well. Results with these tests were almost identical to those achieved with routine ELISA. [30]


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