Autonomic nervous system and smooth muscle cell involvement in systemic sclerosis: ultrastructural study of 3 cases.
OBJECTIVE
To investigate morphological abnormalities in nerve and smooth muscle structures of the anorectal wall underlying gastrointestinal dysfunction in patients with systemic sclerosis (SSc).


METHODS
We performed deep rectal biopsy in 3 patients with limited scleroderma of relatively recent onset and intestinal symptoms.


RESULTS
We found ultrastructural signs of axonal degeneration and cytoskeletal abnormalities in the bundles of unmyelinated fibers. There was also focal degeneration of smooth muscle cells, often in association with the presence of partially degranulated mast cells. Many mast cells were also observed in close relation to nerve fibers and vessels. The enteric vessels often showed basal lamina reduplication and hypertrophied endothelial cells with obliterated lumen. No significant fibrosis was found.


CONCLUSIONS
Our findings indicate early involvement of the autonomic nervous system and to a lesser extent of smooth muscle cells. We confirmed the presence of early vascular lesions and involvement of mast cells in the pathological process.
Malandrini
A
A
Institute of Neurological Sciences, University of Siena, Italy. malandrini@unisi.it
Selvi
E
E
Villanova
M
M
Berti
G
G
Sabadini
L
L
Salvadori
C
C
Gambelli
S
S
De Stefano
R
R
Vernillo
R
R
Marcolongo
R
R
Guazzi
G
G
D000328:Adult; D001341:Autonomic Nervous System / Q000473:pathology / Q000648:ultrastructure*; D017675:CREST Syndrome / Q000473:pathology; D005260:Female; D006801:Humans; D008407:Mast Cells / Q000473:pathology; D008875:Middle Aged; D009130:Muscle, Smooth / Q000473:pathology / Q000648:ultrastructure*; D009412:Nerve Fibers / Q000473:pathology; D012007:Rectum / Q000648:ultrastructure; D012595:Scleroderma, Systemic / Q000473:pathology*