Table 3 2021 American college of rheumatology/vasculitis foundation guideline for the management of polyarteritis nodosa: summary of recommendations [7, 27]

Vascular imaging, tissue biopsy and diagnostic testing

For patients with suspected PAN, we conditionally recommend using abdominal vascular imaging to aid in establishing a diagnosis and determining the extent of disease

For patients with a history of severe PAN with abdominal involvement who become clinically asymptomatic, we conditionally recommend follow-up abdominal vascular imaging. Indefinite routine vascular imaging should be avoided if the abdominal vascular disease is shown to be quiescent

For patients with suspected PAN involving the skin, we conditionally recommend obtaining a deep-skin biopsy specimen (i.e., a biopsy reaching the medium-sized vessels of the dermis) over a superficial skin punch biopsy to aid in establishing a diagnosis

For patients with suspected PAN and peripheral neuropathy (motor and/or sensory), we conditionally recommend obtaining a combined nerve and muscle biopsy over a nerve biopsy alone to aid in establishing a diagnosis

For patients with a history of peripheral motor neuropathy secondary to PAN, we conditionally recommend serial neurologic examinations instead of repeated electromyography/ nerve conduction studies (e.g., every 6 months) to monitor disease activity

Treatment of active disease

For patients with newly diagnosed active, severe PAN, we conditionally recommend initiating treatment with cyclophosphamide and either pulse IV GCs or high-dose oral GCs over high-dose GCs alone

For patients with newly diagnosed active, severe PAN who are unable to tolerate cyclophosphamide, we conditionally recommend treating with other non-GC immunosuppressive agents and GCs over GCs alone

In patients with newly diagnosed active, severe PAN, we conditionally recommend against using plasmapheresis combined with cyclophosphamide and GCs over cyclophosphamide and GCs alone

For patients with newly diagnosed active, non-severe PAN, we conditionally recommend treating with non-GC immunosuppressive agents (MTX or AZA) and GCs over GCs alone

For patients with PAN in remission who are receiving non-GC immunosuppressive therapy, we conditionally recommend discontinuation of non-GC immunosuppressive agents after 18 months over continued (indefinite) treatment

Treatment of refractory disease

For patients with severe PAN that is refractory to treatment with GCs and non-GC immunosuppressive agents other than cyclophosphamide, we conditionally recommend switching the non-GC immunosuppressive agent to cyclophosphamide, over increasing GCs alone

Remission maintenance

For patients with newly diagnosed PAN who have achieved disease remission with cyclophosphamide, we conditionally recommend transitioning to another non-GC immunosuppressive agent over continuing cyclophosphamide

Other considerations

For patients with PAN with nerve and/or muscle involvement, we conditionally recommend physical therapy

For patients with clinical manifestations of DADA2, we strongly recommend treatment with tumor necrosis inhibitors over GCs alone