Mitochondrial carrier homolog 1 (Mtch1) antibodies in neuro-Behçet's disease

•Mtch1-antibody is a reliable and sensitive marker for Behcet’s disease.•Mtch1-antibody related apoptotic cell death might be involved in autoinflammation.•Mtch1 adds to the list of dementia related autoantigens in inflammatory disorders.

Efforts for the identification of diagnostic autoantibodies for neuro-Behcet's disease (NBD) have failed. Screening of NBD patients' sera with protein macroarray identified mitochondrial carrier homolog 1 (Mtch1), an apoptosis-related protein, as a potential autoantigen. ELISA studies showed serum Mtch1 antibodies in 68 of 144 BD patients with or without neurological involvement and in 4 of 168 controls corresponding to a sensitivity of 47.2% and specificity of 97.6%. Mtch1 antibody positive NBD patients had more attacks, increased disability and lower serum nucleosome levels. Mtch1 antibody might be involved in pathogenic mechanisms of NBD rather than being a coincidental byproduct of autoinflammation.