Management of Polyarteritis Nodosa: ACR updated guidelines

With the aim to provide evidence-based recommendations and expert guidance for the management of systemic polyarteritis nodosa (PAN) the expert panel undertook the recent study.

Twenty-one clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for systemic, non–hepatitis B–related PAN. Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the quality of evidence and formulate recommendations. Each recommendation required ≥70% consensus among the Voting Panel.

In the recent guidelines,experts present 16 recommendations and 1 ungraded position statement for PAN.

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.Very low
• For patients with a history of severe PAN with abdominal involvement who become clinically asymptomatic, we conditionally recommend follow-up abdominal vascular imaging. Very low
• 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. Very low
• 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. Very low
• 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. Very low
• For patients with newly diagnosed active, severe PAN, we conditionally recommend initiating treatment with IV pulse GCs over high-dose oral GCs. Very low
• For patients with newly diagnosed active, severe PAN, we conditionally recommend initiating treatment with cyclophosphamide and high-dose GCs over high-dose GCs alone.Very low to low
• For patients with newly diagnosed active, severe PAN, we conditionally recommend initiating treatment with cyclophosphamide and GCs over rituximab and GCs.Very low to low
• 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. Very low
• For patients with newly diagnosed active, nonsevere PAN, we conditionally recommend treating with non-GC immunosuppressive agents and GCs over GCs alone. Very low
• In patients with newly diagnosed active, severe PAN, we conditionally recommend against using plasmapheresis combined with cyclophosphamide and GCs over cyclophosphamide and GCs alone.Low
• 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. Very low
• Ungraded position statement: The optimal duration of GC therapy for PAN (e.g., tapering off by 6 months or longer than 6 months) is not well established, and thus, the duration of therapy should be guided by the patient's clinical condition, values, and preferences. Very low

Treatment of refractory disease

• Recommendation: 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. Very low

Remission maintenance

• Recommendation: 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. Very low

Other considerations

• Recommendation: For patients with PAN with nerve and/or muscle involvement, we conditionally recommend physical therapy. Very low
• Recommendation: For patients with clinical manifestations of DADA2, we strongly recommend treatment with tumor necrosis inhibitors over GCs alone.

"Most recommendations were graded as conditional due to the paucity of evidence. These recommendations support early treatment of severe PAN with cyclophosphamide and glucocorticoids, limiting toxicity through minimizing long-term exposure to both treatments, and the use of imaging and tissue biopsy for disease diagnosis. These recommendations endorse minimizing risk to the patient by using established therapy at disease onset and identify new areas where adjunctive therapy may be warranted."the panel concluded.

For full article follow the link: https://doi.org/10.1002/art.41776

Source: Arthritis & Rheumatology