Histiocytic Arteritis: Rheumatoid Disorder/Cryoglobulinemia

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
  Epineurial: Artery Occlusion
  Also see
  Perimysial: Histiocytic Vasculitis
Nerve Fascicle: Focal Infarction

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
    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

Gomori trichrome stain

H&E stain

VvG stain

Alkaline phosphatase stain

VvG stain

Neurofilament (Green) + NCAM (Red)

ATPase pH 4.3 stain

Neurofilament stain

Acid phosphatase stain

Acid phosphatase stain

Cryoglobulin/Rheumatoid Vasculitis: Nerve Infarction

H&E stain
Perineurium: Pale in regions
Endoneurial vessels: Large size & lumen
Epineurium: Increased numbers of small vessels (Above)
Myelin sheaths: Many are abnormally pale-stained (Below)

Gomori trichrome stain

Endoneurial Vessels: Enlarged with large lumen

Toluidine blue stain
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

VvG stain

Axons, Large & Small: Nearly complete loss
Perineurium: Thick & Irregular structure

Neurofilament stain

Axon & Myelin Degeneration

Myelin sheaths: Abnormal, strong acid phosphatase stain

Acid phosphatase stain

Nerve Infarction: Schwann cell loss in focal regions

Schwann cells, Non-myelinating: Reduced in region of endoneurium

NCAM stain

Neurofilament (Green) + NCAM (Red)
Schwann cells, Non-myelinating (Red): Reduced in regions of endoneurium
Axons: Complete loss in fascicles with loss of Schwann cells

Neurofilament (Green) + NCAM (Red)

Schwann cells (Green & Yellow)
  Reduced numbers in region of endoneurium (Left)
  "Denervated"" type: Most co-stain for P0 & NCAM (Yellow)

NCAM (Green) + P0 (Red)

Endoneurial Damage: C5b-9 staining of Endoneurium

C5b-9 stains
  Endoneurium: Cells & Connective tissue
  Perineurium: Irregular structure; Thick

C5b-9 stain

Endoneurial Damage: Hemorrhage

Toluidine blue stain

Endoneurial Cells after Axon loss

From: R Schmidt
Schwann cells: With myelin & lipid debris
Mast cell
Fibroblast processes

Schwann cells
  With Myelin & Lipid debris
  Büngner band components

From: RSchmidt

Epineurial Damage

Alkaline phosphatase stain
Alkaline Phosphatase
  Stains: Connective tissue around nerve; Epineurium

Alkaline phosphatase stain

Cryoglobulinemia/Rheumatoid Disorder: Muscle

Vessel Structure: Damaged

See: Perimysial vessels, control

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

H&E stain

Gomori trichrome stain
Fibrinoid Necrosis: In central region of artery
Large cells: Replace wall structure

Congo red stain

AMPDA stain

Fibrinoid Necrosis: Inside fibril layer
Fibril layer of vessel wall: Irregular
Outer layer: Replaced by large cells

VvG stain
See: Perimysial vessels, control

Fibrinoid Necrosis: C5b-9 stain

C5b-9 stain

Histiocytic Arteritis

Acid phosphatase stain
Histiocytes (Acid phosphatase stained cells)
  Replace vessel wall
  Nearly all cells are stained

Acid phosphatase stain

Esterase stains
  Clusters of cells in vessel wall
  ? Early granulomatous change

Esterase stain

Arterial Smooth Muscle Layer: Incomplete & Damaged

ATPase pH 4.3 stain

Perimysial Connective Tissue: Alkaline phosphatase stain around damaged artery

Alkaline phosphatase stain
Alkaline phosphatase stains
  Perimysial connective around, & extending away from, damaged artery

Alkaline phosphatase stain

Vessel Endothelium: Strong stain by NADH

NADH stain

Return to Neuromuscular Home Page
Return to Pathology index