From the Guidelines
Polyarteritis nodosa (PAN) stroke should be treated with aggressive immunosuppression, including high-dose corticosteroids and cyclophosphamide, along with standard stroke management and careful monitoring of disease activity and treatment response. The treatment approach for PAN-related stroke involves a combination of immunosuppressive therapy and management of stroke risk factors. According to the 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of polyarteritis nodosa 1, the diagnosis of PAN is generally confirmed by tissue biopsy of an affected organ or angiography, and treatment should focus on inducing remission and preventing long-term complications.
Key Components of Treatment
- High-dose corticosteroids, typically prednisone 1 mg/kg/day, combined with cyclophosphamide (2 mg/kg/day orally or 15 mg/kg IV pulse monthly) for aggressive immunosuppression
- Methylprednisolone pulse therapy (1000 mg daily for 3 days) may be initiated in severe cases with neurological involvement before transitioning to oral steroids
- Standard stroke management, including antiplatelet therapy (aspirin 81-325 mg daily), but anticoagulation must be used cautiously due to potential aneurysms in PAN
- Maintenance therapy with azathioprine (2 mg/kg/day) or methotrexate (15-25 mg weekly) for at least 18-24 months after achieving remission
Monitoring and Prophylaxis
- Regular monitoring of inflammatory markers (ESR, CRP), complete blood counts, and periodic vascular imaging to assess disease activity and treatment response
- Prophylaxis against steroid-induced complications, including calcium/vitamin D supplementation, PCP prophylaxis, and gastric protection, as recommended by the 2021 guideline for the prevention of stroke in patients with stroke and transient ischemic attack 1.
The choice of steroid-sparing agent should be made considering the profile of each agent and associated comorbidities, with options including mycofenolate mofetil, azathioprine, methotrexate, or rituximab 1.
From the Research
Polyarteritis Nodosa and Stroke
- Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that predominantly targets medium-sized arteries 2.
- Central nervous system involvement, including stroke, affects only 2% to 10% of PAN patients, often late during the disease course 3.
- The treatment of PAN relies on combining corticosteroids and an immunosuppressant, such as cyclophosphamide, in patients with poor prognoses 3, 4.
- In patients with hepatitis B virus-related PAN, plasma exchanges and antiviral drugs should be combined with corticosteroids 3.
- There is no direct evidence in the provided studies that specifically discusses the treatment of stroke in PAN patients.
- However, the treatment of PAN in general aims to control the disease and prevent further complications, including those affecting the central nervous system 2, 5, 6, 4.
Clinical Manifestations and Treatment
- Peripheral neuropathy is one of the most frequent and earliest symptoms of PAN, affecting 50% to 75% of patients 3.
- The treatment of PAN includes high-dose corticosteroids, which are combined with immunosuppressive agents when internal organs are involved and with life-threatening disease 5.
- Infliximab, a TNF inhibitor, has been suggested as a relatively safe and efficacious treatment option in refractory PAN 6.
- A prospective randomized trial of 71 patients found that the association of cyclophosphamide with corticosteroids and plasma exchanges reduced the incidence of relapses and improved the quality of the clinical response to therapy 4.