How is Polyarteritis nodosa treated?
Decisions regarding the initial management of PAN without HBV infection depend on disease severity and organs involved (multiorgan versus single organ). For mild disease (constitutional symptoms, arthritis, anemia, skin lesions, normal glomerular filtration rate without cardiac, gastrointestinal, or neurologic involvement), initial treatment with prednisone 1 mg/kg per day for 4 weeks and then slow taper over 6 to 12 months if in remission. If they are intolerant to prednisone alone or unable to taper it, azathioprine (2 mg/kg) or methotrexate (20–25 mg weekly) can be added. For those with moderate to severe disease, treatment should include high doses of glucocorticoids and cyclophosphamide (CTX). If organ or life-threatening disease, intravenous pulse methylprednisolone (1 g/day × 3 days) followed by oral prednisone at a dose of 1 to 2 mg/kg per day is used and then slowly tapered. CTX (2 mg/kg daily oral or CTX 500–1000 mg/m 2 intravenous monthly with dose adjusted by white blood cell count) is added to the glucocorticoids for at least 4 months, but not longer than 12 months. Once the disease is in a stable remission, CTX can be switched to azathioprine or methotrexate for a total of 18 months of immunosuppressive therapy. Mycophenolate mofetil (2–3 g/day) can be used if unable to take azathioprine or methotrexate. Patients who fail to respond to CTX may benefit from rituximab. Hypertension control, pneumocystis pneumonia prophylaxis, and osteoporosis prevention are also important.
