DomainActivity levelDescription
PNS
Rate as ‘No activity’ stable long-lasting features related to damage or PNS involvement not related to the disease
No=0Absence of currently active PNS involvement
Low=5Mild active PNS involvement, such as pure sensory axonal polyneuropathy shown by NCS or trigeminal (V) neuralgia
*Proven small fibre neuropathy
Moderate=10Moderately active PNS involvement shown by NCS, such as axonal sensory–motor neuropathy with maximal motor deficit of 4/5, pure sensory neuropathy with presence of cryoglobulinemic vasculitis, ganglionopathy with symptoms restricted to mild/moderate ataxia, inflammatory demyelinating polyneuropathy (CIDP) with mild functional impairment (maximal motor deficit of 4/5 or mild ataxia) Or cranial nerve involvement of peripheral origin (except trigeminal (V) neuralgia)
High=15Highly active PNS involvement shown by NCS, such as axonal sensory–motor neuropathy with motor deficit ≤3/5, peripheral nerve involvement due to vasculitis (mononeuritis multiplex, etc), severe ataxia due to ganglionopathy, inflammatory demyelinating polyneuropathy (CIDP) with severe functional impairment: motor deficit ≤3/5 or severe ataxia
  • *We decided to add this item not included in the initial version since the link between this entity and SS was not clear until recently.

  • CIPD, chronic inflammatory demyelinating polyneuropathy; NCS, nerve conduction study.