What is it about?
A 70-year-old man presented with one month of hematuria and mild right sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of anti-MPO antibodies, a kidney biopsy which demonstrated necrotizing 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-GBM and c-ANCA/anti-PR3 antibodies. However, this patient’s glomerulonephritis was unique because he presented negative for anti-GBM antibodies and positive for p-ANCA/anti-MPO antibodies.
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Why is it important?
This is now the second documented case of a patient with crescentic glomerulonephritis who is negative for anti-GBM antibody and positive for anti-MPO antibody.
Perspectives
This case was a true diagnostic mystery, especially since most clinicians are familiar with the already rare condition colloquially known as Goodpasture’s disease, but not the more unusual cases of glomerulonephritis such as in this patient. Insights from this case may assist other clinicians in diagnosing unique presentations of glomerulonephritis and formulating treatment plans for patients on immunosuppressive therapy.
Alexander Hanna
Read the Original
This page is a summary of: Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody, BMJ Case Reports, August 2019, BMJ,
DOI: 10.1136/bcr-2019-229256.
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