Recurrent Goodpasture's disease due to a monoclonal IgA-kappa circulating antibody

• Published in: American journal of kidney diseases Vol. 34; no. 3; pp. 549 - 555
• Main Authors: Fervenza, Fernando C., Terreros, Daniel, Boutaud, Ariel, Hudson, Billy G., Williams, Roy A., Donadio, James V., Schwab, Thomas R.
• Format: Journal Article
• Language: English
• Published: Orlando, FL Elsevier Inc 01-09-1999; Elsevier
• Subjects: Anti-Glomerular Basement Membrane Disease - diagnosis; Anti-Glomerular Basement Membrane Disease - immunology; anti–glomerular basement membrane antibody disease; Basement Membrane - immunology; Basement Membrane - pathology; Biological and medical sciences; glomerulonephritis; Glomerulonephritis, IGA - diagnosis; Glomerulonephritis, IGA - immunology; Glomerulonephritis, IGA - pathology; Goodpasture's syndrome; Humans; IgA nephropathy; Immunoglobulin A (IgA) monoclonal gammopathy; Immunoglobulin A - blood; Immunoglobulin kappa-Chains - blood; Kidney Function Tests; Kidney Glomerulus - immunology; Kidney Glomerulus - pathology; Kidney Tubules - immunology; Kidney Tubules - pathology; Kidneys; Male; Medical sciences; Middle Aged; Monoclonal Gammopathy of Undetermined Significance - diagnosis; Monoclonal Gammopathy of Undetermined Significance - immunology; Nephrology. Urinary tract diseases; pulmonary hemorrhage; Recurrence; Urinary system involvement in other diseases. Miscellaneous; pulmonary hemorrhage; anti–glomerular basement membrane antibody disease; IgA nephropathy; glomerulonephritis; Goodpasture's syndrome; Immunoglobulin A (IgA) monoclonal gammopathy; Human; Kidney disease; Immunopathology; Kappa light peptide chain; IgA; Relapse; Urinary system disease; Respiratory disease; Autoimmune disease; Male; Monoclonal antibody; Hemorrhage; Basement membrane; Case study; Renal glomerulus; Goodpasture syndrome; Immunoglobulinopathy; Adult
• ISSN: 0272-6386; 1523-6838
• DOI: 10.1016/S0272-6386(99)70084-3

We describe the case of a 54-year-old man who first presented with a clinical syndrome manifested by recurrent pulmonary hemorrhage, hematuria, and mild renal insufficiency. Direct immunofluorescence of renal biopsy sections showed linear deposition of IgA-kappa in the glomerular (GBM) and tubular basement membranes. Serum protein immunoelectrophoresis was positive for a monoclonal immunoglobulin A (IgA)-kappa protein. Serum analysis showed circulating IgA anti-GBM antibodies. Treatment with high-dose steroids, cyclophosphamide, and plasma exchange resulted in resolution of the clinical picture. To the best of our knowledge, this is the first report of Goodpasture's disease associated with the presence of a circulating monoclonal IgA-kappa antibody.