Recommendations/statements for the management of Polyarteritis Nodosa (PAN) | |
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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 6Â 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 18Â 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 |