A case of simultaneous, biopsy-proven, classic, ANCA-positive Wegener's granulomatosis and anti-GBM disease, but without detectable circulating anti-GBM antibodies.
Wegener's granulomatosis (WG) is a systemic, necrotizing, granulomatous vasculitis of unknown etiology. Approximately 75% of cases present as classic WG with both pulmonary and renal involvement, while the remaining 25% of patients present with a limited form with either predominantly upper or lower respiratory tract symptoms. Ninety percent of WG patients have circulating anti-neutrophil cytoplasmic antibodies (ANCA), and approximately 10% have both circulating ANCA antibodies and concomitant anti-glomerular basement membrane (anti-GBM) disease on renal biopsy. Virtually all of these patients also have circulating anti-GBM antibodies. While it has been reported that some patients with ANCA vasculitis have circulating anti-GBM antibodies, and patients with anti-GBM disease may have positive ANCA, review of the literature does not demonstrate other cases of biopsy-proven, simultaneous, ANCA-associated vasculitis and anti-GBM disease. We report a case of simultaneous, biopsy-proven, classic, ANCA-positive WG and anti-GBM disease, but without detectible circulating anti-GBM antibodies. We present findings characteristic of both WG and linear IgG deposition along the GBM suggesting concurrent anti-GBM disease, in the absence of detectable circulating anti-GBM antibodies. Possible theories to explain the absence of these antibodies are discussed.
Gmurczyk
Aleksandra
A
Evanston Northwestern Healthcare, Northwestern University Feinberg School of Medicine, Chicago, USA. AGmurczy@nmff.org
Ahya
Shubhada N
SN
Goldschmidt
Robert
R
Kim
George
G
Ho
L Tammy
LT
Nash
Kevin
K
D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D001706:Biopsy; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D050533:Glomerular Basement Membrane / Q000276:immunology*; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D006801:Humans
D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies