The role of heat shock proteins in Behçet's disease

H. Direskeneli¹, G. Saruhan-Direskeneli²

¹Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Marmara University, Istanbul and ²Department of Physiology, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey. 

ABSTRACT
Heat shock proteins (HSP) are highly conserved molecules with scavenger activity that are involved in the correct folding of newly synthesized proteins. Increased T and B cell activity against 60/65 kD HSP is observed in different ethnic populations in Behçet's disease (BD) with both ab and gd T cell responses. Although the specificity of these responses is not clear, animal models of uveitis treated with either subcutaneous and oral HSP-derived peptides suggest a significant role of HSPs in the immunopathogenesis of BD. Recent developments in the innate immune system with the description of toll-like receptors (TLR) and HSP60 as a ligand for TLR-2 and TLR-4 suggest also the role of HSP60 as an endogenous 'danger' signal to the immune system with rapid inflammatory cytokine release and the enhancement of adaptive Th1-type responses. Activation of both innate and adaptive responses with HSPs also fit well into the clinical spectrum of BD with both early, limited responses (recurrent ulcers, pathergy, etc.) and chronic lesions (posterior uveitis, thrombosis, neuro-BD, etc.).

Key words
Behçet's disease, heat-shock proteins, immune pathogenesis.

The studies by the authors and their collaborators are supported by grants from the Turkish Scientific and Technical Council (TUBITAK) and the Research Funds of Istanbul and Marmara Universities.
Please address correspondence to: H. Direskeneli, MD, Division of Rheumatology, Marmara University Hospital, Tophanelioglu Cad. 13/15, 81190, Altunizade, Istanbul, Turkey.
E-mail: direskeneli@superonline.com

Clin Exp Rheumatol 2003; 21: (Suppl. 30): S44-S48.
© Copyright Clinical and Experimental Rheumatology 2003.