A case of thrombotic microangiopathy and acute demyelinating central nervous system lesions as the first manifestation of systemic lupus erythematosus

Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder with a relapsing and remitting course and multi- organ involvement which can present with a wide range of neuropsychiatric manifestations. Acute disseminating encephalomyelitis (ADEM) is an autoimmune demyelinating disorder of the central nervous system (CNS). Thrombotic thrombocytopenic purpura (TTP) is a medical emergency which manifests with microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and neurological abnormalities and is known to be a rare association of SLE. We report a case of a 14 year old female who presented with fever and altered consciousness, subsequently diagnosed to have SLE with thrombotic microangiopathy and acute demyelinating CNS lesions in an ADEM-distribution

Keywords

• sle
• adem
• ttp
• thrombotic microangiopathy
• maha