**Table 3.**

*Five-factor score in AAV.*

every six months in combination with corticosteroids could be also used as induction therapy [57, 58]. Rituximab is often used in less severe cases with insufficient data in more severe neurological manifestations, however, some case studies are promising [7]. The five factor score (FFS) (**Table 3**) could be used to assess the prognosis and mortality of vasculitis in the next few years and then to guide when a more aggressive therapy is required, usually when FFS > 1 [59].

After the induction therapy, a maintenance therapy follows with oral immunosuppressants drugs such as azathioprine (1 mg/kg/day to 2 mg/kg/day), methotrexate (7.5 mg to 25 mg weekly), mycophenolate mofetil (1 g to 1.5 g, 2 times per day) or IV Rituximab pulsations every six months [7, 60]. Oral cyclophosphamide is not recommended because of the risk of serious complications [60, 61] such as hemorrhagic cystitis, alopecia, leukopenia, myelodysplasia, neoplasm, etc.

The symptomatic management of neuropathic pain consist of tricyclic antidepressants (i.e., amitriptyline, imipramine, nortriptyline, etc.), serotoninnorepinephrine reuptake inhibitors (i.e., duloxetine, venlafaxine) or antiepileptic drugs such as gabapentin and pregabalin, which are preferred because their better bioavailability [58]. Kinesitherapy should be included in the management of motor disability. PN in AAV requires regular medical visits due to the relapse risk.
