Paraspinal and scapular myopathy associated with scleroderma.
OBJECTIVE
To describe a form of inflammatory myopathy with prominent involvement of the paraspinal and scapular muscles in patients with scleroderma.


METHODS
Review of clinical records, laboratory investigations, and muscle biopsies.


RESULTS
Patients presented with a "dropped head" resulting from weakness of the posterior cervical muscles (three cases) or camptocormia ("bent spine") resulting from weakness of the paraspinal muscles (two cases) and variable weakness and atrophy of shoulder girdle muscles with mild or absent pelvic girdle involvement. Biopsies from the deltoid or paraspinal muscles showed myositis of variable severity and scleroderma vasculopathy in all cases. The response to prednisolone and cytotoxic agents was poor, but there was a good response to intravenous immunoglobulin therapy in one case.


CONCLUSIONS
Patients with scleroderma may develop a restricted form of immune-mediated inflammatory myopathy with a predilection for the paraspinal and scapular muscles, which is poorly responsive to treatment with glucocorticoids and immunosuppressive agents and may require consideration of other treatment modalities.
Rojana-Udomsart
Arada
A
Centre for Neuromuscular and Neurological Disorders, University of Western Australia, Nedlands, Western Australia.
Fabian
Vicki
V
Hollingsworth
Peter N
PN
Walters
Susan E
SE
Zilko
Paul J
PJ
Mastaglia
Frank L
FL
D000328:Adult; D000974:Antibodies, Antinuclear / Q000378:metabolism; D015703:Antigens, CD / Q000378:metabolism; D004576:Electromyography / Q000379:methods; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology; D009135:Muscular Diseases / Q000150:complications* / Q000276:immunology / Q000473:pathology*; D012217:Rheumatoid Factor / Q000276:immunology; D012540:Scapula / Q000473:pathology*; D012594:Scleroderma, Localized / Q000150:complications* / Q000276:immunology; D013121:Spinal Curvatures / Q000473:pathology*
D000974:Antibodies, Antinuclear; D015703:Antigens, CD; D012217:Rheumatoid Factor