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
Doença Associada
myasthenia gravis, Crohn`s disease, ulcerative colitis
Relevância Clínica (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
Relevância Clínica (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.