| Domain | Activity level | Description |
|---|---|---|
| PNS Rate as ‘No activity’ stable long-lasting features related to damage or PNS involvement not related to the disease | No=0 | Absence of currently active PNS involvement |
| Low=5 | Mild active PNS involvement, such as pure sensory axonal polyneuropathy shown by NCS or trigeminal (V) neuralgia *Proven small fibre neuropathy | |
| Moderate=10 | Moderately 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=15 | Highly 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.