An unusual case of pulmonary-renal syndrome associated with defects in type IV collagen composition and anti-glomerular basement membrane autoantibodies

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Presentation transcript:

An unusual case of pulmonary-renal syndrome associated with defects in type IV collagen composition and anti-glomerular basement membrane autoantibodies David Charytan, MD, Brian MacDonald, BS, Hikaru Sugimoto, MD, PhD, Stephen Pastan, MD, Gerald Staton, MD, Randy Hennigar, MD, Raghu Kalluri, PhD American Journal of Kidney Diseases Volume 45, Issue 4, Pages 743-748 (April 2005) DOI: 10.1053/j.ajkd.2004.12.022 Copyright © 2005 National Kidney Foundation, Inc. Terms and Conditions

Fig 1 (A) Chest radiograph with bilateral alveolar infiltrates. (B) GBMs and renal tubules without significant abnormalities. (Periodic acid-Schiff; original magnification ×200.) (C) GBM shows occasional thinning (arrows) and thickening with patchy foot-process effacement (stars). (Electron microscopy; original magnification ×12,575.) (D) GBM of normal thickness with minimal foot-process effacement. American Journal of Kidney Diseases 2005 45, 743-748DOI: (10.1053/j.ajkd.2004.12.022) Copyright © 2005 National Kidney Foundation, Inc. Terms and Conditions

Fig 2 Patient serum (PT) binds exclusively with the α2NC1 domain of type IV collagen, whereas reference Goodpasture serum (GP) binds exclusively with α3NC1. Healthy control (HC) serum lacks antibodies to any type IV collagen domains. (A) Western blot using purified recombinant α1 to α3NC1 domains of human type IV collagen and serum diluted at 1:25. (B) ELISA using patient, Goodpasture’s, and healthy control sera with recombinant human α3NC1. Dilutions: solid bar =1:2.5 (PT, HC) or 1:75 (GP); hatched bar =1:5 (PT, HC) or 1:125 (GP); dotted bar =1:25 (PT, HC) or 1:250 (GP). American Journal of Kidney Diseases 2005 45, 743-748DOI: (10.1053/j.ajkd.2004.12.022) Copyright © 2005 National Kidney Foundation, Inc. Terms and Conditions

Fig 3 GBM deposition of IgG is (A) absent in normal human kidney and prominent in (B) glomeruli of the patient. α3NC1 has (C) prominent linear expression in the GBM from normal kidney, but (D) is significantly diminished and segmentally absent in the patient’s GBM. α5NC1 shows (E) global linear expression in normal GBM, but (F) is diminished and segmentally absent in the patient’s GBM. American Journal of Kidney Diseases 2005 45, 743-748DOI: (10.1053/j.ajkd.2004.12.022) Copyright © 2005 National Kidney Foundation, Inc. Terms and Conditions