Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody

BMJ Case Reports
Alexander HannaFernanda Heitor

Abstract

A 70-year-old man presented with 1 month of haematuria and mild right-sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of antimyeloperoxidase antibodies, a kidney biopsy which demonstrated necrotising crescentic glomerulonephritis with linear immunofluorescence of the basement membrane, and electron microscopy which exhibited thickening of the glomerular basement membrane. Incidentally, the patient was discovered to have a latent hepatitis B infection, which complicated immunosuppressive therapy. He was treated with a course of plasmapheresis and methylprednisolone, followed by entecavir for hepatitis B prophylaxis, and finally by rituximab. This case of glomerulonephritis was notable for its resemblance to the better known Goodpasture's disease. Typically, Goodpasture's syndrome exists on a spectrum from seronegative disease to double-positive disease that presents with both anti-glomerular basement membrane (anti-GBM) and cytoplasmic-antineutrophil cytoplasmic antibodies/antiproteinase 3 antibodies (c-ANCA/anti-PR3). However, this patient's glomerulonephritis was unique because he presented negative for anti-GBM antibodies and positive for perinuclear-antineutrophil c...Continue Reading

References

Oct 2, 2004·Kidney International·Jeremy B LevyCharles D Pusey
Dec 21, 2010·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Zhao CuiMing-hui Zhao
Jun 16, 2011·Seminars in Respiratory and Critical Care Medicine·Hidehiro Yamada
Sep 5, 2013·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Chiun HsuUNKNOWN Taiwan Cooperative Oncology Group
Nov 6, 2013·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Sophie OhlssonMårten Segelmark
Feb 1, 2014·Clinical Kidney Journal·Masafumi SugiyamaMasanori Funauchi
May 9, 2015·Journal of Autoimmunity·Maxime TouzotDavid Saadoun
Oct 9, 2015·Clinical Medicine Insights. Circulatory, Respiratory and Pulmonary Medicine·Yasuhiro KatsumataHisashi Yamanaka

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Citations

Apr 3, 2021·BMJ Case Reports·James PoveyThalakunte Muniraju

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Methods Mentioned

BETA
electrophoresis
biopsy
light microscopy
PCR
electron microscopy
ELISA

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