Relapsing Hepatitis B-associated Vasculitis with Features of Polyarteritis Nodosa and c-Antineutrophilic Cytoplasmic Antibodies-associated Vasculitis.

Hepatitis B virus (HBV)-associated vasculitis manifests as vasculitis of the medium vessel and polyarteritis nodosa (PAN). HBV-associated PAN (HBV-PAN) is negative for antineutrophilic cytoplasmic antibodies (ANCA), without glomerulonephritis (GN) or pulmonary involvement; relapse does not occur after successful HBV seroconversion. We describe a case of HBV-associated vasculitis characterized by small and medium overlapping vasculitis and an atypical long-term course in a 60-year-old male with hypertension and HBV who initially presented with abdominal pain and scrotal swelling. The abdominal angiogram revealed a beaded morphology and aneurysms involving the mesenteric, hepatic, and intraparenchymal renal arteries. Tests for the HBV core antibody, cANCA, PR3, and rheumatoid factor were positive. He received pulse steroids and cytoxan for PAN and entecavir for HBV, which resolved the symptoms. Serum creatinine (SCr) rose and then remained stable. Multiple urinalysis measurements were negative. Two years later, the patient developed abdominal pain, orchitis, hemoptysis, and acute kidney injury (SCr = 11 mg/dL). The viral load of HBV was negative. Urinalysis showed 3+ blood and 3+ protein. The renal biopsy displayed pauci-immune crescentic GN with active crescents involving ~50% of the glomeruli. Two arteries had evidence of past arteritis with elastic lamina disruption. Acute necrotizing arteritis was absent. The patient recovered renal function after receiving pulse steroids, plasma exchange, rituximab, and cytoxan. Although the initial trigger may have been HBV, the vasculitis became self-perpetuating, as it recurred without HBV viremia. Patients with HBV-PAN who are ANCA+ should be closely followed because they could be at a higher risk of relapsing vasculitis.