Treatment of PAN depends on the severity of the disease and the organs involved. For severe disease with neurologic, renal, cardiac, or gastrointestinal manifestations, cyclophosphamide and high-dose corticosteroids are used in concert to attain remission. Once symptoms have improved, glucocorticoids are slowly tapered. For less severe disease, corticosteroids can be used alone or in combination with methotrexate or azathioprine. In hepatitis B–associated PAN, management is aimed at both treatment of hepatitis and suppression of the immune system.
Prognosis is generally good, with a 5-year survival of greater than 80% in treated patients. Without treatment, 5-year survival is less than 15%. Renal, gastrointestinal, and neurologic involvement are associated with a poor prognosis.
The patient in this case received prednisone 1 mg/kg and was also given rituximab as steroid-sparing therapy. Rituximab was chosen because the patient was planning to travel out of the country, which would have made laboratory monitoring difficult. Rituximab has been shown to be effective for PAN in case reports. This patient's prednisone dose was tapered over a period of 6 months, and her digital ulcers improved.
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Cite this: Kelsey N. Rigby. Rheumatology Case Challenge: A 54-Year-Old Woman With Discolored Toes and Nonhealing Ulcers - Medscape - Apr 27, 2023.