Abstract

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that predominantly affects mediumsized arteries, and is often the consequence of viral infections, mainly HBV. We describe a case of Hepatitis C associated PAN in a patient who had eluded diagnosis for several years. A 55 year old Egyptian female with a history of migraine, B cell lymphoma (in remission), and depression was admitted with diffuse abdominal pain. She denied nausea, vomiting, hematochezia or fever. She had been seen in the GI clinic over many years with post prandial epigastric pain and nausea with negative imaging and endoscopic testing and had been diagnosed with functional dyspepsia. Physical examination revealed the patient looked pale and was in distress with mild tenderness to deep palpation in the left upper abdominal quadrant, with no guarding or rebound. Rectal exam was negative for masses and blood. Lab work was pertinent for a drop in Hgb from 12 g/dl to 9 g/dl. Other routine lab work was normal. After 2 visits to the ED she received a CT abdomen/pelvis, which revealed intra abdominal hemorrhage in the left upper quadrant around the body and tail of the pancreas, between stomach and spleen with extension to the pararenal space (figure 1). Subsequent angiogram disclosed splenic pseudoaneurysm treated by embolization and background changes to vasculature with ectatic and stenosed vessels consistent with PAN (figure 2). Hepatitis B serology was negative but she tested positive for hepatitis C, genotype 4 with viral load of 56,000. A serum cryoglobulin test was negative but perinuclear antineutrophilic cytoplasmic antibody test was positive. Serum complement levels were reduced. She was diagnosed as having classic PAN associated with hepatitis C. She was treated with pegylated interferon, and ribavirin but was a non responder at 4 weeks. She was then treated with sofosbuvir, pegylated interferon and ribavirin for 12 weeks with successful eradication of HCV with remarkable improvement in symptoms. HCV associated PAN is rare but accounts for 20 % of all HCV related vasculitis. It tends to occur more often in older females and up to 20 years from infection. Sustained remission is closely related to successful HCV clearance. It is also likely that the patients past history of B cell lymphoma was linked to HCV as well, with epidemiological studies revealing 2-3 times increased risk of B-cell lymphoma in HCV patients. Indolent disease will often respond to anti viral therapy, but aggressive disease requires traditional chemotherapy. Unfortunately our patient had never been tested for HCV prior to this presentation. In conclusion, we describe the first case of a patient with a prior history of B cell lymphoma, presenting with splenic artery pseudoaneurysm rupture from PAN from HCV successfully treated with new generation anti-viral therapy.Figure 1Figure 2

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