234 Concurrent Immune Complex Glomerulonephritis and Anti-Neutrophil Cytoplasmic Antibodies-Associated Crescentic Glomerulonephritis

Abstract

Objective
Alteration in the immune system may be associated with immune complex mediated glomerulonephritis (GN) and/or other kidney diseases.

Methods and Materials
Here we report a small unusual series of immune complex diseases membranoproliferative glomerulonephritis (MPGN) and post-infectious glomerulonephritis (post-IGN) coexisting with possible anti-neutrophil cytoplasmic antibodies (ANCAs)-associated glomerulonephritis (GN).

Results
The first case is a 66-year-old man (M), positive ANCA at 1:80, serum creatinine (sCr) 1.72 mg/dL with 19% crescents in glomeruli and type 3 MPGN. The second case is a 55-year-old woman (F), positive ANCA at 1:40, sCr 4.87 mg/dL with 28% of crescents in glomeruli and type 1 MPGN. The third case is a 75-year-old F, positive ANCA at 1:160, sCr 3.86 mg/dL with 25% crescents in glomeruli and type 1 MPGN. The fourth case is a 76-year-old F, positive ANCA at 1:160, sCr 6.27 mg/dL with 66% crescents in glomeruli and post-IGN. The fifth case is a 63-year-old M, ANCA 1:640, sCr 4.4 mg/dL with 78% crescents in glomeruli and post-IGN. The sixth case is a 62year-old M, positive ANCA at 1:640 and sCr 6.71 mg/dL with 53% crescents in glomeruli and MPGN type 1.

Conclusion
The high percent of crescentic glomeruli cannot be explained by the relatively minor immune complex diseases but by positive ANCA; we therefore consider that the GNs most likely result from positive ANCA rather than being secondary to the immune complex-mediated GN.

Full text

Introduction
Membranoproliferative glomerulonephritis (MPGN) represents a pattern
of glomerular injury, which includes the thickening of the glomerular
basement membrane (and hence the name membrano-), and the increase
in the number of cells inside the glomerular tufts (and hence the name
proliferative).
It has other histopathologic findings as well; like the accentuation of
the glomerular lobules and the doubling of the glomerular basement
membrane. Crescents formation, however, is not characteristic, and if it
was present, it is usually small and focal, in less than 10% of the
patients (1).
Electron microscopy usually reveals the presence of immunoglobulins
and/ or C3 deposits in the glomerular basement membrane, and it is the
bases for the new MPGN classification (2).
Cases Presentation
We present 6 cases of MPGN with concurrent crescentic
glomerulonephritis (figure 1), in which the immune complex deposits
were minor and couldn’t explain the marked crescent formation.
However, Anti Neutrophil Cytoplasmic Antibodies (ANCA) were present
(Table 1).
Conclusion
The high percent of crescentic glomeruli cannot be explained by the
relatively minor immune complex deposits but by positive ANCA, we
therefore consider that the CGNs most likely result from positive ANCA
rather than being secondary to the immune complex mediated GN.
References
1. Zhou XJ. Membranoproliferative glomerulonephritis. In: Jennette JC, Olson
JL, Schwartz MM, Silva FG, editors. Heptinstall’s Pathology of the Kidney.
Philadelphia: Lippincott, Williams & Wilkins, 2007: 253–319.
2. Sethi S, Fervenza FC. Membranoproliferative glomerulonephritis: pathogenetic
heterogeneity and proposal for a new classification. Semin Nephrol 2011;31:341–
8.
Concurrent Immune Complex Glomerulonephritis
and ANCA-Associated Crescentic Glomerulonephritis
Mazen Toushan, Ping L. Zhang, Wei Li and Hassan D. Kanaan
Department of Anatomic Pathology ■ Beaumont Hospital, Royal Oak, MI
P18265_0917
Figure 1. Images from patient #6. A: affected glomeruli with increased
mesangial cellularity and a crescent at the upper right side of the
glomerulus, PAS stain x 50. B: near complete destruction of the glomerulus
by the extensive epithelial proliferation and the crescent formation, PAS
stain x 50. C: Electron microscopy image showing crescent formation
in the left part of the picture. D: Electron microscopy showing scattered
subendothelial deposits..
MPO – myeloperoxidase; PR3 – proteinase 3; Pro/cr – urine protein/creatinine, MPGN – membranoproliferative glomerulonephritis;
sCr – serum creatinine; CGN – crescentic glomerulonephritis; GN – glomerulonephritis
Table 1. Primary crescentic glomerulonephritis with concurrent immune complex mediated glomerulopathy
Cases History Diagnosis 1 (% of
glomeruli involved) Concurrent Follow-up for
Specific ANCA
ANCA:
(reference value:
< 1:20)
#1 66 year old man, negative anti-GBM antibody,
sCr: 1.72 mg/dl, Pro/Cr ratio 1.4. CGN (19%) MPGN, type
3
MPO +
PR3 - positive ANCA at 1:80
#2
55 year old woman, no anti-GBM antibody
testing result available, sCr: 4.87 mg/dl, Pro/
Cr ratio 5.9.
CGN (28%) MPGN, type
1
MPO +
PR3 - positive ANCA at 1:40
#3 75 year old woman, negative anti-GBM
antibody, sCr: 3.86 mg/dl, Pro/Cr ratio 6.3 CGN (25%) MPGN, type
1NA positive ANCA at 1:160
#4 76 year old woman, negative anti-GBM
antibody, sCr: 6.27 mg/dl, Pro/Cr 4.6. CGN (66%)
Post-
infectious
GN
MPO +
PR3 + positive ANCA at 1:160
#5 63 year old man, negative anti-GBM antibody,
sCr: 4.4 mg/dl, Pro/Cr ratio 7. CGN (78%)
Post-
infectious
GN
MPO –
PR3 - positive ANCA at 1:640
#6 62 year old man, negative anti-GBM antibody,
sCr: 6.71 mg/dl, urine protein: 3+ CGN (53%) MPGN type 1 MPO +
PR3 - positive ANCA at 1:640