LSUHSC Renal Pathology Consultative Services - Case Study #2

Case Study #: 2007 0226

Clinical History:

A 86-year old male presented with decrease urine output and dark urine. He was afebrile, mildly hypertensive at 145/95. No systemic symptoms were present and he had no pertinent past renal-related medical history. No medicines had recently been introduced. Laboratory values were as follows: Urinalysis:  3+ hematuria                 3+ proteinuria                 Numerous rbc casts Creatinine: 7.2 mg/dl C3/C4 – normal Hepatitis B and C – negative ANA negative ANCA and AGBM serologies pending

Click for larger Figure 1: Although difficult to recognize 2 glomeruli are present both show global necrosis with abundant fibrin deposition and cellular crescent formation. Click for larger Figure 2: Silver stain of one glomerulus shows that only a few remnants of capillary loop basement membranes remain. Click for larger Figure 3: Direct immunofluorescence is required to resolve the differential of a crescentic glomerulonephritis (linear vs. granular vs. negative immunofluorescence) and serologies are necessary to confirm the IF findings. However, this glomerulus contains a finding that has an extremely high correlation with the specific diagnosis. You may be surprised by the correlation.     Question: What is the diagnostic finding? What diagnosis does it strongly suggest? Answer:   Diagnosis: Multinucleated giant cell Anti-glomerular basement membrane antibody-associated crescentic glomerulonephritis Discussion: The presence of a well form multinucleated giant cell in a crescentic glomerulonephritis, in my experience, is invariably associated with a linear IF and serologically confirmed AGBM antibody. This may seem surprising since a giant cell reaction is what you might expect for a granulomatous vasculitis, like Wegener’s granulomatosis, involving the kidney. However, the granulomatous component in Wegener’s is usually limited to ENT and lung lesions. The presence of giant cells in AGBM has been reported (Nephron 16: 415, 1976) and is illustrated in the 6th edition of Heptinstall’s Pathology of the Kidney (figure 13-14). Although it has never been emphasized as a useful diagnostic feature (probably because with IF and serology the diagnosis is never in doubt), I believe its specificity and sensitivity is over 90%.  In fact, I can’t recall a case of AGBM disease in the last several years that lacked giant cells, or a case with giant cells that did not turn out to be AGBM-mediated disease (sounds like a notion that merits investigation). References: General references on AGBM disease and Goodpasture’s syndrome:          Kidney Int 64:1535-1550, 2003.          J Am Soc Nephrol 10:244-2453, 1999.

Stephen M. Bonsib, MD  (Dr Bonsib's Bio)

Chairman Pathology

Director, Renal Pathology Consultative Services

Albert G. and Harriet G. Smith Professor of Pathology

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