Version 2.74


The water channel aquaporin 4 (AQP4) receptor regulates the water homeostasis in the central nervous system (CNS). Autoantibodies to the AQP4 receptor bind to the receptor and cause neuromyelitis optica (NMO) which is an idiopathic, severe, demyelinating disease of the CNS that primarily affects the optic nerve and spinal cord. Before the discovery of the AQP4 receptor antibody NMO was often misdiagnosed as a variant of multiple sclerosis (MS). With improved clinical laboratory tests and distinct immunological and pathological attributes the two diseases are less often confused. Distinguishing MS and NMO is especially important because they have different treatments. Treatment for NMO includes corticosteroids and plasmapheresis; typical medications used to treat MS, such as beta-interferon and glatiramer acetate are not effective against NMO. During the disease process, the aquaporin 4 receptor antibody binds to the aquaporin 4 receptor initiating the activation of complement, inflammatory demyelination and necrosis. Eighty to ninety percent of patients with NMO experience recurrent episodes of optic neuritis and myelitis. Many patients with NMO also have other autoimmune diseases including thyroiditis, systemic lupus erythematosus
and Sjogren's syndrome.[PMID: 17706564] Detection of APQ4-IgG is indicative of a relapsing disease. Immediate treatment with immunosuppressive drugs reduces the frequency and disability of future attacks. [] Source: Regenstrief LOINC

Basic Part Properties

Aquaporin 4 water channel Ab.IgG
Part Type
Component (Describes the core component or analyte measured)
Created On

LOINC Terminology Service (API) using HL7® FHIR®

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Language Variants

Tag Language Translation
zh-CN Chinese (China) 视神经脊髓炎抗体.IgG
Synonyms: Devic 氏综合征 IgG;Devic 综合征 IgG;Devic氏综合征 Ab.IgG;Devic氏综合征抗体.IgG;Devic综合征 Ab.IgG;Devic综合征抗体.IgG;NMO Ab.IgG;NMO 抗体.IgG;视神经脊髓炎 Ab.IgG;视神经脊髓炎 IgG