82 year old female with rapidly progressive mononeuritis multiplex & Multi-organ failure
Rheumatoid factor = 616; ANCA-; NfL 777; C4<4; IgM M-protein; Cryoglobulins, borderline
Nerve: Occluded Artery; Focal infarction
Focal nerve infarction: Also see
Behçet
Epineurial Vessel (Artery): Chronic Pathology, Occlusion
Vessel Structure
Damaged wall: Elastin layer interrupted & irregular
Lumen: Absent or Contains large cells, possibly endothelium
Neovascularization
Abnormal staining for NCAM
Size: Large at some levels
Calcification: In some regions of vessels wall
Hemosiderin: Present in Epineurium
Epineurial connective tissue Surrounding vessel: Scattered histiocytes
See:
Epineurial vessels, control
![](../pics/biopsy/vasculitis/ravasc/nvvessgt2sm.jpg)
Gomori trichrome stain
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![](../pics/biopsy/vasculitis/ravasc/nvvesshe2sm.jpg)
H&E stain
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![](../pics/biopsy/vasculitis/ravasc/nvvessvvg2sm.jpg)
VvG stain
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![](../pics/biopsy/vasculitis/ravasc/nvvessalpsm.jpg)
Alkaline phosphatase stain
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![](../pics/biopsy/vasculitis/ravasc/nvvessvvgsm.jpg)
VvG stain
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![](../pics/biopsy/vasculitis/ravasc/nvvessnfgncamrsmvh.jpg)
Neurofilament (Green) + NCAM (Red)
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![](../pics/biopsy/vasculitis/ravasc/nvvessatp.jpg)
ATPase pH 4.3 stain
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![](../pics/biopsy/vasculitis/ravasc/nvvessnfil.jpg)
Neurofilament stain
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![](../pics/biopsy/vasculitis/ravasc/nvvessacp1sm.jpg)
Acid phosphatase stain
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![](../pics/biopsy/vasculitis/ravasc/nvvessacp2sm.jpg)
Acid phosphatase stain
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Cryoglobulin/Rheumatoid Vasculitis: Nerve Infarction
![](../pics/biopsy/vasculitis/ravasc/ravascnvhe.jpg)
H&E stain
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Perineurium: Pale in regions
Endoneurial vessels: Large size & lumen
Epineurium: Increased numbers of small vessels (Above)
Myelin sheaths: Many are abnormally pale-stained (Below)
![](../pics/biopsy/vasculitis/ravasc/ravascnvgt.jpg)
Gomori trichrome stain
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Endoneurial Vessels: Enlarged with large lumen
![](../pics/biopsy/vasculitis/ravasc/ravascendovesstb.jpg)
Toluidine blue stain
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Endoneurial vessels: Many are large (Also see below)
Histiocytes with Lipid droplets: Scattered in endoneurium
Myelin remnants (Ovoids): Scattered in endoneurium
Axon Loss
Myelinated Axons: Marked loss
Endoneurial Microvessels: Often large; Some dark stained
![](../pics/biopsy/vasculitis/ravasc/ravascnvvvg3.jpg)
VvG stain
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Axons, Large & Small: Nearly complete loss
Perineurium: Thick & Irregular structure
![](../pics/biopsy/vasculitis/ravasc/ravascnvnfil2.jpg)
Neurofilament stain
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Axon & Myelin Degeneration
Myelin sheaths: Abnormal, strong acid phosphatase stain
![](../pics/biopsy/vasculitis/ravasc/ravascacp.jpg)
Acid phosphatase stain
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Nerve Infarction: Schwann cell loss in focal regions
Schwann cells, Non-myelinating: Reduced in region of endoneurium
![](../pics/biopsy/vasculitis/ravasc/ravascnvncam.jpg)
NCAM stain
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![](../pics/biopsy/vasculitis/ravasc/nfgncamr.jpg)
Neurofilament (Green) + NCAM (Red)
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Schwann cells, Non-myelinating (Red): Reduced in regions of endoneurium
Axons: Complete loss in fascicles with loss of Schwann cells
![](../pics/biopsy/vasculitis/ravasc/nfgncamr2.jpg)
Neurofilament (Green) + NCAM (Red)
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Schwann cells (Green & Yellow)
Reduced numbers in region of endoneurium (Left)
"Denervated"" type: Most co-stain for P0 & NCAM (Yellow)
![](../pics/biopsy/vasculitis/ravasc/ncamgp0r.jpg)
NCAM (Green) + P0 (Red)
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Endoneurial Damage: C5b-9 staining of Endoneurium
C5b-9 stains
Endoneurium: Cells & Connective tissue
Perineurium: Irregular structure; Thick
![](../pics/biopsy/vasculitis/ravasc/ravascc5b9endon.jpg)
C5b-9 stain
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Endoneurial Damage: Hemorrhage
![](../pics/biopsy/vasculitis/ravasc/ravascendohemtb.jpg)
Toluidine blue stain
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Endoneurial Cells after Axon loss
![](../pics/biopsy/vasculitis/ravasc/emcells2.jpg)
From: R Schmidt
|
Histiocytes
Schwann cells: With myelin & lipid debris
Mast cell
Fibroblast processes
Schwann cells
With Myelin & Lipid debris
Büngner band components
![](../pics/biopsy/vasculitis/ravasc/emcells1.jpg)
From: RSchmidt
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Epineurial Damage
![](../pics/biopsy/vasculitis/ravasc/ravascalplp.jpg)
Alkaline phosphatase stain
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Alkaline Phosphatase
Stains: Connective tissue around nerve; Epineurium
![](../pics/biopsy/vasculitis/ravasc/ravascalpmp.jpg)
Alkaline phosphatase stain
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Cryoglobulinemia/Rheumatoid Disorder: Muscle
Vessel Structure: Damaged
See:
Perimysial vessels, control
![](../pics/biopsy/vasculitis/ravasc/ravascmuschelp.jpg)
H&E stain
|
Perimysial Vessels (Arteries)
Size: Very large
Wall Structure
Inner layer: Fibrinoid necrosis
Outer layer: Replaced by large, "palisaded" cells
Lumen: Large cells, possibly endothelium, adherent to inner wall
![](../pics/biopsy/vasculitis/ravasc/ravascmuschemp.jpg)
H&E stain
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![](../pics/biopsy/vasculitis/ravasc/ravascmuscgthp.jpg)
Gomori trichrome stain
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Fibrinoid Necrosis: In central region of artery
Large cells: Replace wall structure
![](../pics/biopsy/vasculitis/ravasc/ravascmuscr.jpg)
Congo red stain
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![](../pics/biopsy/vasculitis/ravasc/ravascmusampda.jpg)
AMPDA stain
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Fibrinoid Necrosis: Inside fibril layer
Fibril layer of vessel wall: Irregular
Outer layer: Replaced by large cells
![](../pics/biopsy/vasculitis/ravasc/ravascmuscvvghp.jpg)
VvG stain
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See:
Perimysial vessels, control
Fibrinoid Necrosis: C
5b-9 stain
![](../pics/biopsy/vasculitis/ravasc/ravascfibnecc5b9.jpg)
C5b-9 stain
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Histiocytic Arteritis
![](../pics/biopsy/vasculitis/ravasc/ravascmuscacpsm.jpg)
Acid phosphatase stain
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Histiocytes (Acid phosphatase stained cells)
Replace vessel wall
Nearly all cells are stained
![](../pics/biopsy/vasculitis/ravasc/ravascacphp.jpg)
Acid phosphatase stain
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Esterase stains
Clusters of cells in vessel wall
? Early granulomatous change
![](../pics/biopsy/vasculitis/ravasc/ravascmuscacphp.jpg)
Esterase stain
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Arterial Smooth Muscle Layer: Incomplete & Damaged
![](../pics/biopsy/vasculitis/ravasc/ravascmuscatp43hp.jpg)
ATPase pH 4.3 stain
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Perimysial Connective Tissue: Alkaline phosphatase stain around damaged artery
![](../pics/biopsy/vasculitis/ravasc/ravascmuscalphp.jpg)
Alkaline phosphatase stain
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Alkaline phosphatase stains
Perimysial connective around, & extending away from, damaged artery
![](../pics/biopsy/vasculitis/ravasc/ravascmuscalplp.jpg)
Alkaline phosphatase stain
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Vessel Endothelium: Strong stain by NADH
![](../pics/biopsy/vasculitis/ravasc/ravascnadh.jpg)
NADH stain
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2/8/2024