2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa

Abstract

Objective. To provide evidence-based recommendations and expert guidance for the management of systemic polyarteritis nodosa (PAN).Methods. 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.Results. We present 16 recommendations and 1 ungraded position statement for PAN. 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.Conclusion. These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and imaging for patients with PAN.

Rapidly Progressive Coronary Aneurysm

“…These lesions result in microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarction. 2 However, the patient did not present any of the diagnostic criteria for polyarteritis nodosa according to American College of Rheumatology, 3 or for any other type of systemic vasculitis.…”

Colonic Perforation in Polyarteritis Nodosa With Concurrent Perforated Sigmoid Diverticulitis

“…Historically, PAN was associated with prior Hepatitis B Virus (HBV) infection. However, with an increase in the prevention of HBV infection, diagnosis of HBV-associated PAN is becoming increasingly rare with proportionally increased diagnosis of idiopathic PAN [1,3,4,9]. Idiopathic PAN is thought to be autoimmune as the clinical course is arrested with immunosuppression [3,10].…”

“…Idiopathic PAN is thought to be autoimmune as the clinical course is arrested with immunosuppression [3,10]. The American Society of Rheumatology has recommended diagnosing PAN based on clinical suspicion in tandem with tissue biopsy or angiography if tissue biopsy cannot be obtained [1]. PAN can involve all organs, including the brain, eyes, pancreas, testicles, ureters, breasts, and ovaries, but half of all cases can affect the gastrointestinal tract.…”

“…Polyarteritis nodosa (PAN) is systemic necrotizing arteritis involving mainly medium-sized vessels [1][2][3]. Polyarteritis often manifests with neuropathy or cutaneous changes [3].…”

Evolving Chest Pain in a Young Male Patient

“…Current guidelines recommend the use of azathioprine or methotrexate with glucocorticoids for the initial treatment of non-severe PAN. Individuals with non-severe PAN with symptoms that have evolved into severe disease despite initial therapy should be treated with CYC (79). An FFS of ≥1 indicates severe PAN, with 5-year mortality rates of 26% and 46% for individuals with an FFS of 1 and those with an FFS of >2, respectively (77).…”