Hepatitis B associated fulminant polyarteritis nodosa: successful treatment with pulse cyclophosphamide, prednisolone and lamivudine following emergency surgery

For hepatitis B virus associated polyarteritis nodosa, alpha interferon and plasma exchanges have been proposed to be the first-line treatment. We report a case of hepatitis B surface antigen (HBsAg)-positive fulminant polyarteritis nodosa with predominant gastrointestinal involvement who showed good response to pulse cyclophosphamide, prednisolone, and lamivudine therapy. The patient, a 22-year-old man, presented with a short history of epigastric pain. Initial upper gastrointestinal endoscopy revealed gastritis and duodenal erosions. His pain did not respond to H2-receptor antagonists. He had slightly impaired liver function tests, and was HBsAg and hepatitis B e antigen (HBeAg) positive. Around 3 weeks after initial presentation, he developed massive gastrointestinal haemorrhage requiring resuscitation and emergency laparotomy. Microscopic examination of the resection specimens revealed necrotizing vasculitis of small and medium-sized arteries in the submucosa compatible with polyarteritis nodosa. The patient was treated with pulse cyclophosphamide and prednisolone, with lamivudine being added when he showed an acute rise in liver enzymes. He subsequently developed HBeAg seroconversion, and remained well 18 months after cessation of all immunosuppressives. We believe that the efficacy of pulse cyclophosphamide, prednisolone, and lamivudine in the treatment of hepatitis B virus associated polyarteritis nodosa, especially in comparison with interferon and plasma exchanges, deserves further evaluation.