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Therapeutic algorithm of central nervous system involvement developed by EULAR-Sjögren Syndrome Task Force Group

The EULAR-Sjögren Syndrome Task Force Group therapeutic algorithm for central nervous system involvement is based on the type of neurologic manifestation. The authors suggested that corticosteroids plus cyclophosphamide could be the first- and second-line therapeutic approach in patients with CNS vasculitis and neuromyelitis optica spectrum disorder but that corticosteroids should be rapidly tapered with the aim of decreasing the risk of severe infection. Rituximab or/plus plasma exchanges may also be recommended for the treatment of this manifestation, especially in patients with severe or refractory disease, but its use is currently limited by the lack of US Food and Drug Administration or European Medicines Agency approval for these indications. When lymphocytic meningitis does not respond to symptomatic treatment, the same therapeutic algorithm should be followed. (Ramos-Casals M, Brito-Zerón P, Bombardieri S, et al. EULAR recommendations for the management of Sjogren's syndrome with topical and systemic therapies. Ann Rheum Dis 2020;79(1):3-18.)

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Associated Disorders

  • Angiitis
  • Aquaporin autoantibody
  • B12 deficiency
  • Brown-Sequard syndrome
  • Carpal tunnel syndrome
  • Chronic progressive myelitis
  • Devic syndrome
  • Entrapment neuropathies
  • HTLV-1 infection
  • Lower motor neuron disease
  • Lupus
  • Mononeuritis multiplex
  • Multiple cranial neuropathy: diplopia, trigeminal, facial (Bell palsy), cochlear
  • Multiple-sclerosis like syndromes
  • Myopathy
  • Neurogenic bladder
  • Neuromyelitis optica
  • Painful sensory neuropathy
  • Pure sensory neuropathy
  • Radiculoneuropathy (demyelinating polyradiculoneuropathy)
  • Sensorimotor (axonal) polyneuropathy
  • Sensorineural hearing loss
  • Sensory ataxic ganglionopathy with antiganglion neuron antibodies
  • Sicca syndrome
  • Subacute combined degeneration
  • Transverse myelitis
  • Vasculitic neuropathy