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CASE REPORT
Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody
  1. Alexander Hanna1,
  2. Jenny Ross2 and
  3. Fernanda Heitor3
  1. 1 Medical School, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
  2. 2 Department of Pathology, Northwestern Memorial Hospital, Chicago, Illinois, USA
  3. 3 Department of Medicine, Northwestern Memorial Hospital, Chicago, Illinois, USA
  1. Correspondence to Alexander Hanna, AlexBHanna{at}gmail.com

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 cytoplasmic antibodies/antimyeloperoxidase antibodies (p-ANCA/anti-MPO).

  • hepatitis B
  • chronic renal failure
  • interstitial lung disease
  • nephrotic syndrome
  • malignant disease and immunosuppression

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

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Footnotes

  • Contributors AH wrote and revised the manuscript and created tables, figures and images. JR took immunofluorescence photos and contributed to discussion section. FH contributed to discussion section.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Obtained.

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