Symposium 11

Abstract

Autoimmune diseases are conditions of immune dysfunction, leading to inappropriate hyperactivation of the adaptive immunity and subsequent damage to various tissues of the host. Autoimmune conditions presenting in the rheumatology clinics (rheumatoid arthritis, systemic lupus erythematosus or immune mediated myositis) are often treated by immunosuppression, which are almost invariably associated with increased susceptibility to infections. However, immunosuppression can sometimes be used to conversely treat susceptibility to infections. An emerging group of autoimmune diseases which target the host’s own immune system (or associated cytokines) are becoming increasing recognized which phenotypically mimics various immunodeficiency syndromes – known as “phenocopies of PID”. One particular phenocopy of a PID known as Mendelian Susceptibility to Mycobacterial Disease (MSMD) has been increasingly recognized in our locality. This “acquired MSMD” is due to autoantibodies against anti-IFN[Formula: see text], leading to increased susceptibility to mycobacterial and other “IFN-[Formula: see text] dependent” infections. Alike other autoimmune diseases, appropriate and targeted immunosuppression can alleviate the effect of anti-IFN-[Formula: see text] antibodies and aid treatment of associated infections. During this talk, our experience in the diagnosis and management toward acquired MSMD in our locality will be discussed. We will also share findings from our novel diagnostic and functional tests, as well as outcomes of acquired MSMD patients treated with B-cell depleting therapies.