Neuromuscular

Sarcoidosis: Muscle

History
  Early images
General patterns
Granulomas
  Typical
  Small (Early)
  Resolving
Immune features
Muscle fibers
Perimysial pathology

Sarcoidosis: Early images of nodular sarcoid involving muscle (Sundelin 1925)


Sundelin 1


Sundelin

Resolving Granuloma


Sarcoidosis: Patterns of sarcoid involving muscle


Immune myopathies with Perimysial connective tissue pathology (IMPP) & Granulomas

H&E stain
Granuloma in Perimysium
Muscle fibers: Scattered small fibers in areas remote from granuloma

Perimysium: Fragmented structure
Myopathy: Multiple focal regions with
  Small muscle Fibers
  Increased endomysial connective tissue
Inflammation: Small cluster of cells, possibly replacing a muscle fiber

H&E stain

Histiocytic inflammation
  Clusters of histiocytes
    In granulomas
    Associated with necrotic muscle fibers
  Scattered histiocytes: in perimysial connective tissue

Acid phosphatase stain


Congo red stain
Chronic Sarcoid myopathy
  Perimysium & Endomysium: Replaced by Fat
  Granulomas with giant cells in endomysium or perimysium

Congo red stain

Sarcoid: Granulomas


H&E stain
Granuloma in Perimysium


H&E stain
Granulomas in Endomysium
  Large cells & multinucleated cells in central regions
  Smaller cells (Lymphocytes) in peripheral regions
  Small muscle fibers often neighbor granuloma in some areas

Congo red stain


VvG stain
Granuloma
  Small muscle fibers in periphery of granuloma


Acid phosphatase stain
Granuloma
  Acid phosphatase (Above) stains
    Around nuclei of individual histiocytes
    Diffusely in Cytoplasm of giant cell
  Esterase (Below) stains
    Cells within granuloma in confluent pattern

Esterase stain

Granuloma
  Cells in granuloma have abundant mitochondria

SDH stain

Granuloma
  Cells in granuloma stain strongly for histiocyte marker CD68
  Activated histiocytes (CD68+) are also scattered in endomysium, possibly near capillaries

CD68 stain

Sarcoid: Small Granulomas


H&E stain
Granulomas (Early)
  Histiocytes replace muscle fibers & remain in place

Congo red stain

VvG stain

Sarcoidosis, Chronic: Muscle fiber pathology


VvG stain
Sarcoidosis: Muscle fiber pathology
  Immune cells surround muscle fibers (Above)
  Muscle fiber damage: Muscle fibers focally invade & progressively replace muscle fibers (Below; Arrow)

Gomori trichrome stain

H&E stain
Sarcoidosis: Muscle fiber pathology
  Muscle fiber damage: Muscle fibers focally invade & replace muscle fibers

H&E stain
Many Cells replacing muscle fibers are histiocytes (CD68+)

CD68 stain

Sarcoidosis: Muscle fiber pathology
  Muscle fiber damage: Fragments of muscle fibers (Arrow) remain in some areas of a granuloma

H&E stain

Sarcoidosis: Resolving granulomas


H&E stain
Granulomas, Resolving
  Granulomas may resolve leaving focal regions (Arrow) with
    Increased, or damaged, connective tissue
    Scattered
      Cells, Some multinucleated
      Small muscle fibers

H&E stain


Congo red stain
Resolved Granulomas with focal regions of
  Cellular connective tissue
  Small muscle fibers

VvG stain

H&E stain


H&E stain
Multinucleated, Large cells: In regions of resolved granulomas

H&E stain

Sarcoidosis: Perimysial pathology


VvG stain
Perimysial Pathology: Perimysial connective tissue
  Structure: Fragmented
  Contents: Scattered histiocytes

VvG stain


H&E stain
Perimysial Pathology: Perimysial connective tissue
  Structure: Fragmented
  Contents: Scattered histiocytes

H&E stain


Acid phosphatase stain
Perimysial Connective Tissue
  Contains scattered histiocytes (Acid phosphatase + (Red)) in areas with normal & fragmented structure

Acid phosphatase stain

Esterase stain
Perimysial Connective Tissue
  Contains scattered histiocytes (Esterase + (Red)) in areas with normal & fragmented structure

Esterase stain

Perimysial Connective Tissue
  Regions in some patients have abnormal alkaline phosphatase staining

Alkaline phosphatase stain

Sarcoid muscle: Immune features

C5b-9 deposition: Endomysial & Perimysial connective tissue

C5b-9 stain

MHC Class I upregulation by muscle fibers

MHC Class I stain

MHC Class I stain

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12/16/2021