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: G S Hoffman, J D Filie, H R Schumacher Jr, E Ortiz-Bravo, M G Tsokos, J C Marini, G S Kerr, Q H Ling, D E Trentham
• Journal: Arthritis and rheumatism (Arthritis Rheum) Vol. 34 Issue 11 Pg. 1466-75 (Nov 1991) ISSN: 0004-3591 [Print] United States
• PMID: 1953824 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Collagen

Topics

• Autoimmunity (immunology)
• Collagen (immunology, ultrastructure)
• Ehlers-Danlos Syndrome (immunology, pathology)
• Female
• Humans
• Infant, Newborn
• Osteolysis (immunology, pathology)
• Synovial Membrane (pathology)
• T-Lymphocytes (immunology)
• Vasculitis, Leukocytoclastic, Cutaneous (immunology, pathology)