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Frequently relapsing anti-glomerular basement membrane antibody disease with changing clinical phenotype and antibody characteristics over time

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ABSTRACT

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.

No MeSH data available.


Serum creatinine, anti-GBM titers and disease presentations over time.
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SFW048F1: Serum creatinine, anti-GBM titers and disease presentations over time.

Mentions: A 41-year-old woman with normal baseline kidney function presented in December 2005 with pulmonary hemorrhage confirmed on CT scan and bronchoscopy, an elevated creatinine of 957 µmol/L (eGFR 5 mL/min/1.73 m2) requiring dialysis, >50 red blood cells (RBCs)/high power field (HPF) on urinalysis, negative anti-neutrophil cytoplasmic antibodies (ANCAs), and positive anti-GBM antibodies (see Figure 1). A renal biopsy showed crescentic glomerulonephritis in which 60% of the 24 glomeruli contained active cellular or fibrocellular crescents, with strong linear capillary glomerular staining on direct immunofluorescence (IF) that was IgG2 dominant (see Figure 2 and Table 1). None of the glomeruli had segmental or global sclerosis. She was treated with daily plasmapheresis, steroids and monthly intravenous (IV) cyclophosphamide for 6 months. Her hemoptysis resolved, and she recovered renal function but had residual chronic kidney disease with baseline creatinine of 170 µmol/L.Table 1.


Frequently relapsing anti-glomerular basement membrane antibody disease with changing clinical phenotype and antibody characteristics over time
Serum creatinine, anti-GBM titers and disease presentations over time.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5036897&req=5

SFW048F1: Serum creatinine, anti-GBM titers and disease presentations over time.
Mentions: A 41-year-old woman with normal baseline kidney function presented in December 2005 with pulmonary hemorrhage confirmed on CT scan and bronchoscopy, an elevated creatinine of 957 µmol/L (eGFR 5 mL/min/1.73 m2) requiring dialysis, >50 red blood cells (RBCs)/high power field (HPF) on urinalysis, negative anti-neutrophil cytoplasmic antibodies (ANCAs), and positive anti-GBM antibodies (see Figure 1). A renal biopsy showed crescentic glomerulonephritis in which 60% of the 24 glomeruli contained active cellular or fibrocellular crescents, with strong linear capillary glomerular staining on direct immunofluorescence (IF) that was IgG2 dominant (see Figure 2 and Table 1). None of the glomeruli had segmental or global sclerosis. She was treated with daily plasmapheresis, steroids and monthly intravenous (IV) cyclophosphamide for 6 months. Her hemoptysis resolved, and she recovered renal function but had residual chronic kidney disease with baseline creatinine of 170 µmol/L.Table 1.

View Article: PubMed Central - PubMed

ABSTRACT

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.

No MeSH data available.