Intractable vasculitis, resorptive osteolysis, and immunity to type I collagen in type VIII Ehlers-Danlos syndrome.
A unique patient with type VIII Ehlers-Danlos syndrome and cutaneous vasculitis, resorptive osteolysis, and cardiac valvular disease is described. Collagen analyses identified morphologic and physical abnormalities of type I collagen. The patient's T lymphocytes could be propagated in vitro with type I collagen and produced a 60-kd lymphokine that bound this protein. Cellular autoimmunity to type I collagen may be responsible for this patient's intractable clinical condition.
Hoffman
G S
GS
Laboratory of Immunoregulation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892.
Filie
J D
JD
Schumacher
H R
HR
Ortiz-Bravo
E
E
Tsokos
M G
MG
Marini
J C
JC
Kerr
G S
GS
Ling
Q H
QH
Trentham
D E
DE
D015551:Autoimmunity / Q000276:immunology; D003094:Collagen / Q000276:immunology* / Q000648:ultrastructure; D004535:Ehlers-Danlos Syndrome / Q000276:immunology* / Q000473:pathology; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D010014:Osteolysis / Q000276:immunology* / Q000473:pathology; D013583:Synovial Membrane / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000276:immunology* / Q000473:pathology
D003094:Collagen