Neuromuscular

IMMUNE MYOPATHY WITH ANTIBODIES TO SIGNAL RECOGNITION PARTICLE

SRP pathology
  Early
  Intermediate
  Late
  Capillaries
  Complement deposition



EARLY PATHOLOGY


H&E stain

Acid phosphatase stain
Necrotic & Regenerating (Arrow) muscle fibers: Scattered


NADH stain

Coarse internal architecture
  in muscle fibers


Acid phosphatase stain

Scattered necrotic muscle fiber



Alkaline phosphatase stain

Alkaline phosphatase stains an increased number
  of endomysial vessels (Arrow)

INTERMEDIATE PATHOLOGY


H&E stain

Bimodal muscle fiber sizes


Acid phosphatase stain

Scattered necrotic muscle fiber


Acid phosphatase stain

Acid phosphatase positive cells in endomysium

LATE PATHOLOGY: Months


H&E stain

Increased endomysial connective tissue
Hypertrophic and small muscle fibers


Gomori trichrome stain

Many small rounded muscle fibers
Increased endomysial connective tissue



H&E stain

Marked increase in endomysial connective tissue
Disease course of only 6 months

CAPILLARY PATHOLOGY


Ulex europaeus agglutinin I stain

Normal muscle:
  Small endomysial capillaries


Ulex europaeus agglutinin I stain

SRP myopathy:
  Enlarged endomysial capillaries


Ulex europaeus agglutinin I stain

SRP myopathy: Endomysial capillaries
  Enlarged & Mildly reduced in number



MEMBRANE ATTACK COMPLEX: C5b-9 IN CAPILLARIES

Antibody vs C5b-9
C5b-9
Deposition in endomysial capillaries
  and necrotic muscle fiber cytoplasm (Arrow)

Antibody vs C5b-9
C5b-9
Irregular deposition in endomysial capillaries (Arrows)

Return to Inflammatory myopathies
Return to SRP myopathy

5/24/2011