Atopic myelitis, also described as idiopathic eosinophilic myelitis, is a rare disease that consists of localised and persistent inflammation of the spinal cord. It has been reported predominantly in Japan. This disease has specific features of elevated serum IgE level, active reaction to mite or cedar pollen specific antigen and stepwise progression of mostly the sensory symptoms. Atopic myelitis is often with coexistent atopic diseases, such as atopic dermatitis, bronchial asthma, allergic rhinitis and food allergies. Although the pathogenesis is still poorly understood, it has been reported that CCL11 and IL-9 were significantly increased in patients with atopic myelitis. Upregulation of CCL11 and IL-9 explain quite well the eosinophilic inflammation of the spinal cord. It has been suggested that immunotherapy, such as corticosteroids, intravenous immunoglobulin (IVIG), and plasma exchanges (PE), are effective in atopic myelitis, and that PE is the most beneficial immunotherapy.
Category
Immune system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
08 Diseases of the nervous system
Spinal cord disorders excluding trauma
8B41 Myelitis
H01863 Atopic myelitis
Comment
See also H00079 Asthma and H01358 Atopic dermatitis.
