Frequently relapsing anti-glomerular basement membrane antibody disease with changing clinical phenotype and antibody characteristics over time.
Anti-glomerular basement membrane (GBM) antibody disease is a typically monophasic autoimmune disease with severe pulmonary and renal involvement. We report an atypical case of frequently relapsing anti-GBM antibody disease with both anti-GBM antibody-positive flares with pulmonary and renal involvement, and anti-GBM antibody-negative flares that were pulmonary limited with no histologic renal disease. This is the first report of alternating disease phenotype and anti-GBM antibody status over time. Disease severity paralleled the detection of anti-GBM antibodies but was independent of IgG subtype staining along the GBM. This case suggests a role for changing subpopulations of pathogenic antibodies as an explanation for variation in disease phenotype and anti-GBM antibody results.
Faculty of Medicine , University of British Columbia , Vancouver, BC , Canada.
Bobby
B
Gu
Department of Pathology and Laboratory Medicine , St. Paul's Hospital and University of British Columbia , Vancouver, BC , Canada.
Alex B
AB
Magil
Division of Nephrology, University of British Columbia, 5th Floor Nephrology, 2775 Laurel Street, Vancouver, BC, Canada V5Z 1M9; BC Provincial Renal Agency, Vancouver, BC, Canada; Centre forHealth Evaluation and Outcome Sciences, St. Paul's Hospital, Vancouver, BC, Canada.
Sean J
SJ
Barbour
ELISA; Goodpasture's disease; anti-GBM antibody disease; glomerulonephritis; pulmonary hemorrhage
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