Title
Management of Polyarteritis Nodosa
Authoring Organization
Publication Month/Year
July 7, 2021
Last Updated Month/Year
December 12, 2024
Document Type
Guideline
External Publication Status
Published
Country of Publication
US
Document Objectives
These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and imaging for patients with PAN.
Target Patient Population
Patients with POLYARTERITIS NODOSA (PAN)
PICO Questions
In patients with suspected PAN with and without gastrointestinal symptoms, what is the impact of non-invasive vascular imaging vs. conventional catheter-based imaging on diagnostic accuracy, disease-related outcomes, and diagnostic testing-related adverse events?
In patients with suspected cutaneous or systemic PAN involving the skin, what is the impact of a deep skin biopsy vs. skin punch biopsy on diagnostic accuracy, disease-related outcomes, and diagnostic testing-related adverse events?
In patients with suspected PAN and peripheral neuropathy (motor and/or sensory), what is the impact of nerve and muscle biopsy vs. nerve biopsy alone on diagnostic accuracy, disease-related outcomes, and diagnostic testing-related adverse events?
In patients with newly-diagnosed PAN with active and severe disease, what is the impact of pulse intravenous glucocorticoids compared to high dose oral glucocorticoids disease-related outcomes and treatment-related adverse events?
In patients with newly-diagnosed PAN with active and severe disease, what is the impact of cyclophosphamide with high dose glucocorticoids vs. high dose glucocorticoids alone on disease-related outcomes and treatment-related adverse events?
In patients with newly-diagnosed PAN with active and severe disease, what is the impact of cyclophosphamide vs. other non-glucocorticoid non-biologic immunosuppressive therapy on disease-related outcomes and treatment-related adverse events?
In patients with newly-diagnosed PAN with active and severe disease, what is the impact of plasmapheresis combined with cyclophosphamide and glucocorticoids vs. cyclophosphamide and glucocorticoids alone on disease-related outcomes and treatment-related adverse events
In patients with newly-diagnosed PAN with active and severe disease, what is the impact of using non-glucocorticoid non-biologic immunosuppressive therapy (excluding cyclophosphamide) with glucocorticoids vs. glucocorticoids alone on disease-related outcomes and treatment-related adverse events?
In patients with newly-diagnosed PAN who have achieved remission with cyclophosphamide, what is the impact of transitioning to another non-glucocorticoid non-biologic immunosuppressive agent vs. continuing with cyclophosphamide on disease-related outcomes and treatment-related adverse events?
In patients with newly-diagnosed PAN with active disease and severe manifestations, what is the impact of cyclophosphamide vs. rituximab on disease-related outcomes and treatment-related adverse events?
In patients with newly-diagnosed PAN in remission after remission induction therapy, what is the impact of a rapid taper of glucocorticoids (<6 months) vs. a slow taper (≥ 6 months) on disease-related outcomes and treatment-related adverse events?
In patients with newly diagnosed PAN with active and non-severe disease, what is the impact of adding of non-glucocorticoid non-biologic immunosuppressive therapy to glucocorticoids vs. using glucocorticoids alone on disease-related outcomes and treatment-related adverse events?
In patients with PAN in remission on non-glucocorticoid non-biologic immunosuppressive therapy, what is the impact of discontinuation of non-glucocorticoid non-biologic immunosuppressive therapy after 18 months vs. continued treatment on disease-related outcomes and treatment-related adverse events?
In patients with PAN who has nerve and/or muscle involvement, what is the impact of physical therapy vs. no physical therapy on disease-related outcomes?
In patients with PAN with refractory disease on glucocorticoids alone, what is the impact of adding of cyclophosphamide vs. increasing the glucocorticoid dose alone on disease-related outcomes and treatment-related adverse events?
In patients with PAN with refractory disease on glucocorticoids and cyclophosphamide, what is the impact of adding plasmapheresis vs. increasing immunosuppression on disease-related outcomes and treatment-related adverse events?
In patients with PAN with refractory disease on glucocorticoids and non-glucocorticoid nonbiologic immunosuppressive therapy (excluding cyclophosphamide), what is the impact of switching to cyclophosphamide vs. increasing glucocorticoid dose alone on disease-related outcomes and treatment-related adverse events?
In patients with PAN and Adenosine Deaminase 2 deficiency what is the impact of TNF-alpha inhibitors (e.g., infliximab, etanercept, adalimumab) versus glucocorticoids alone on disease-related outcomes and treatment-related adverse events?
In patients with a history of severe PAN who is clinically asymptomatic but has newly elevated inflammatory markers without a clear etiology, what is the impact of vascular imaging (both invasive and non-invasive) vs. clinical assessment alone on disease-related outcomes and diagnostic testing-related adverse events?
In patients with a history of severe PAN who is clinically asymptomatic, what is the impact of routine vascular imaging (both invasive and non-invasive) every 6 months vs. vascular imaging only prompted by clinical symptoms/signs on disease-related outcomes and diagnostic testing-related adverse events?
In patients with a history of peripheral motor neuropathy secondary to PAN, what is the effect of routine EMG/NCS every 6 months vs. routine neurologic exam alone on disease-related outcomes and treatment-related adverse events?
Inclusion Criteria
Male, Female, Adolescent, Adult, Child, Older adult
Health Care Settings
Ambulatory
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Diagnosis, Assessment and screening, Treatment, Management
Diseases/Conditions (MeSH)
D056653 - Rheumatoid Vasculitis, D014657 - Vasculitis, D010488 - Polyarteritis Nodosa
Keywords
vasculitis, Polyarteritis Nodosa, PAN, Periarteritis, Polyarteritis
Source Citation
Chung SA, Gorelik M, Langford CA, et al. 2021 American College of Rheumatology/ Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa. Arthritis & Rheumatology, Vol. 73, No. 8, August 2021, pp 1384–1393. doi 10.1002/art.41776
Supplemental Methodology Resources
Data Supplement, Data Supplement, Data Supplement, Data Supplement, Data Supplement