Guideline sources The following summarized guidelines for the evaluation and management of polyarteritis nodosa (PAN) are prepared by our editorial team based on guidelines from the Vasculitis Foundation (VF/ACR 2021) and the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2019). 1 2 Calculator Calculator FFS score for polyarteritis nodos Revised Five Factor Score for pr Guidelines 1. Diagnostic investigations Diagnostic imaging: consider obtaining abdominal vascular imaging to aid in establishing the diagnosis and determining the extent of disease in patients with suspected PAN. C https://web.pathway.md/diseases/recB4PgbU7TPmFbzn 1/4 6/29/23, 11:16 PM Polyarteritis nodosa Pathway 2. Diagnostic procedures Biopsy: Consider performing deep-skin biopsy (reaching the medium-sized vessels of the dermis) over superficial skin punch biopsy to aid in establishing the diagnosis in patients with suspected PAN involving the skin. C Consider performing combined nerve and muscle biopsy over nerve biopsy alone to aid in establishing the diagnosis in patients with suspected PAN and peripheral neuropathy (motor and/or sensory). C 3. Medical management Induction of remission: consider initiating IV pulse corticosteroids over high-dose oral corticosteroids in patients with newly diagnosed active, severe PAN. C Show 6 more Maintenance of remission: Consider transitioning to another non-corticosteroid immunosuppressive agent over continuing cyclophosphamide in patients with newly diagnosed PAN achieved disease remission with cyclophosphamide. C Consider discontinuing non-corticosteroid immunosuppressive agents after 18 months over continuing treatment (indefinite) in patients with PAN in remission receiving non- corticosteroid immunosuppressive therapy. C Management of refractory disease: consider switching the non-corticosteroid immunosuppressive agent to cyclophosphamide over increasing corticosteroids alone in patients with severe PAN refractory to treatment with corticosteroids and non-corticosteroid immunosuppressive agents other than cyclophosphamide. C 4. Nonpharmacologic interventions Physical therapy: consider offering physical therapy in patients with PAN with nerve and/or muscle involvement. C 5. Therapeutic procedures Plasma exchange: avoid performing plasmapheresis combined with cyclophosphamide and corticosteroids over cyclophosphamide and corticosteroids alone in patients with newly diagnosed active, severe PAN. D 6. Specific circumstances Pediatric patients (diagnosis): Recognize that there are two different classification criteria for PAN: ACR criteria https://web.pathway.md/diseases/recB4PgbU7TPmFbzn 2/4 6/29/23, 11:16 PM Polyarteritis nodosa Pathway EULAR/PRINTO/PReS endorsed Ankara 2008 criteria. B Show 2 more Pediatric patients (laboratory tests): recognize that ANCA are typically negative in patients with PAN. B Pediatric patients (biopsy): perform targeted tissue biopsy in patients with suspected PAN. B Show 3 more Pediatric patients (diagnostic imaging): obtain catheter digital subtraction arteriography in centers with specific pediatric expertise for the diagnosis of PAN. B Show 6 more Pediatric patients (treatment): consider initiating NSAIDs and/or corticosteroids alone with careful monitoring of clinical and laboratory parameters in patients with cutaneous disease, in the absence of severe systemic inflammation and other major organ involvement. C Show 2 more 7. Follow-up and surveillance Follow-up: Consider obtaining follow-up abdominal vascular imaging in patients with a history of severe PAN with abdominal involvement becoming clinically asymptomatic. C Consider performing serial neurologic examinations instead of repeated EMG/nerve conduction studies (such as every 6 months) to monitor disease activity in patients with a history of peripheral motor neuropathy secondary to PAN. C Clinical findings Symptoms Past medical history Abdominal pain HBV infection Arthralgia Orchitis Headache Ocular exam Hematochezia Hematuria Cotton wool spots Hemoptysis Retinal detachment Malaise Retinal vasculitis Melena Integument exam Myalgia Nausea Livedo reticularis Testicular pain Palpable purpura Vomiting Petechiae Weight loss Skin nodules Skin ulceration Neurological exam Hematological findings Cranial nerve palsy https://web.pathway.md/diseases/recB4PgbU7TPmFbzn 3/4 6/29/23, 11:16 PM Polyarteritis nodosa Pathway Mononeuritis multiplex WBC count Peripheral neuropathy blood platelet count Polyneuropathy Musculoskeletal exam Arthritis Lab findings ESR serum CRP serum creatinine serum urea urine protein References 1. Nienke de Graeff, Noortje Groot, Paul Brogan et al. European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides - the SHARE initiative. Rheumatology Oxford). 2019 Apr 1;58 4 656 671. Open 2. Sharon A Chung, Mark Gorelik, Carol A Langford et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa. Arthritis Rheumatol. 2021 Aug;73 8 1384 1393. Open 3. Seza Ozen, Angela Pistorio, Silvia M Iusan et al. EULAR/PRINTO/PRES criteria for Henoch-Sch nlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II Final classification criteria. Ann Rheum Dis. 2010 May;69 5 798 806. Open https://web.pathway.md/diseases/recB4PgbU7TPmFbzn 4/4