AC-17 - Fibrillar Segmental
Descrição
Enhanced decoration of short segments, periodic dense bodies, along the stress fibers. e.g. anti-alpha-actinin, anti-vinculin.
        Associação Antigênica
alpha-actinin, vinculin
        Doenças Associadas
myasthenia gravis, Crohn`s disease, ulcerative colitis
        Relevâncias Clínicas - Primeiro nível
Found very infrequently in a routine serology diagnostic setting.  Antigens recognized include ?-Actinin and Vinculin; specific immunoassays for these autoantibodies are currently not commercially available
                            
                        Relevâncias Clínicas - Segundo nível
In SLE and lupus nephritis cross-reactive anti-?-actinin and anti-dsDNA autoantibodies have been reported as pathogenic
                            
                        Reported as part of the anti-cell membrane antibody spectrum that characterize patients with lupus nephritis; also reported as a biomarker to differentiate patients with lupus nephritis from those without renal involvement
                            
                        "Reported with relatively high prevalence (~40%) in patients with AIH type 1 and associated with more severe disease, clinical and histological disease activity, predictor of therapeutic response, and double positivity with anti-ssDNA antibodies Autoantibodies to vinculin: "
                            
                        Reported in 2 of 31 patients with chronic inflammatory demyelinating neuropathy
                            
                        Most reports describe autoantibodies directly binding to specific antigens (i.e. antigen-specific immunoassays) and none actually shows correlations with the AC-17 pattern as such; specific immunoassays for these autoantibodies are currently not commercially available.
                            
                        Referências
                                    Beppu M, Sawai S, Satoh M, et al. Autoantibodies against vinculin in patients with chronic inflammatory demyelinating polyneuropathy. J Neuroimmunol 2015;287:9-15.
                                    Gueguen P, Dalekos G, Nousbaum JB, et al. Double reactivity against actin and alpha-actinin defines a severe form of autoimmune hepatitis type 1. J Clin Immunol 2006;26:495-505.
                                    Renaudineau Y, Dalekos GN, Gueguen P, et al. Anti-alpha-actinin antibodies cross-react with anti-ssDNA antibodies in active autoimmune hepatitis. Clin Rev Allergy Immunol 2008);34:321-5.
                                    Seret G, Canas F, Pougnet-Di Costanzo L, et al. Anti-alpha-actinin antibodies are part of the anti-cell membrane antibody spectrum that characterize patients with lupus nephritis. J Autoimmun 2015;61:54-61.
                                    Zachou K, Oikonomou K, Renaudineau Y, et al. Anti-alpha actinin antibodies as new predictors of response to treatment in autoimmune hepatitis type 1. Aliment Pharmacol Ther 2012;35:116-25.
                                    Zhang WH, Pan HF, Zhao XF, et al. Anti-alpha-actinin antibodies in relation to new-onset systemic lupus erythematosus and lupus nephritis. Mol Biol Rep 2010;37:1341-5.
                                    Zhao Z, Weinstein E, Tuzova M, et al. Cross-reactivity of human lupus anti-DNA antibodies with alpha-actinin and nephritogenic potential. Arthritis Rheum 2005;52:522-30.
                
            
         
                     
                     
                     
                         
                         
                        