Management of Autoimmune Systemic Diseases in the Intensive Care Unit

The diagnosis of an autoimmune systemic disease (SD) and/or its management in the intensive care unit (ICU) is dependent on dialogue between the intensivist and specialists in these diseases. However, some clinical (syndromic associations) or biological signs should lead the intensivist to suspect these diseases. Several biological or histological investigations can be rapidly performed in the ICU to confirm the diagnosis. Both treatments of a potential flare-up of the suspected disease and a concurrent infectious complication need often to be started simultaneously. While waiting for the effects of these specific treatments, supportive treatment may include the initiation of non-invasive ventilation or, in severe specified cases, invasive mechanical ventilation with extra-corporeal supports like Renal Replacement Therapy (RRT) and extra-corporeal membrane oxygenation (ECMO). Referent centers should be asked for validation of the therapeutic options, especially when some drugs are used off-label for these severe patients. An integrative diagnostic and therapeutic approach is proposed to guide the intensivist in this complex management.