title: Intractable vasculitis, resorptive osteolysis, and immunity to type I collagen in type VIII Ehlers-Danlos syndrome.
abstract: 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.
authors.affiliation: Laboratory of Immunoregulation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892.
authors.forename: G S
authors.initials: GS
authors.lastname: Hoffman
authors.forename: J D
authors.initials: JD
authors.lastname: Filie
authors.forename: H R
authors.initials: HR
authors.lastname: Schumacher
authors.forename: E
authors.initials: E
authors.lastname: Ortiz-Bravo
authors.forename: M G
authors.initials: MG
authors.lastname: Tsokos
authors.forename: J C
authors.initials: JC
authors.lastname: Marini
authors.forename: G S
authors.initials: GS
authors.lastname: Kerr
authors.forename: Q H
authors.initials: QH
authors.lastname: Ling
authors.forename: D E
authors.initials: DE
authors.lastname: Trentham
mesh_terms: 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
chemical_list: D003094:Collagen