Abstract: SA-PO247
Relapsing Hepatitis B (HBV)-Associated Vasculitis with Features of Polyarteritis Nodosa (PAN) and cANCA-Associated Vasculitis
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Khan, Usman A., OUHSC, Oklahoma City, Oklahoma, United States
- Ijaz, Sardar H., University of Oklahoma Health Sciences Center, Oklahoma city, Oklahoma, United States
- Khakwani, Aemen Shams, OUHSC, Oklahoma City, Oklahoma, United States
- Ghata, Joe, OUHSC, Oklahoma City, Oklahoma, United States
Introduction
HBV-associated vasculitis typically manifests as the medium vessel vasculitis, PAN. HBV-associated PAN (HBV-PAN) is ANCA(-), without glomerulonephritis (GN) or pulmonary involvement; relapse does not occur after successful HBV seroconversion. We describe a unique case of HBV-associated vasculitis with features of a small and medium sized overlap vasculitis and atypical long term course.
Case Description
The patient is a 60-year-old male with hypertension and HBV who initially presented with abdominal pain and scrotal swelling. Abdominal angiogram revealed beaded morphology and aneurysms involving mesenteric, hepatic, and intraparenchymal renal arteries. Hepatitis B core antibody, cANCA, PR3, and rheumatoid factor were (+). He received pulse steroids and Cytoxan for treatment of PAN and Entecavir for HBV, resulting in resolution of symptoms. Serum Cr rose from 0.8 mg/dL to 2.0 mg/dL during the first 2 months of treatment, and then remained stable. Multiple urinalysis (UA) measurements were negative. Two years later, the patient developed abdominal pain, orchitis, hemoptysis, and acute kidney injury (serum Cr of 11 mg/dL). HBV viral load was negative. UA showed 3+ blood and 3+ protein. Renal biopsy displayed pauci-immune crescentic GN with active crescents involving ~ 50% of the glomeruli (Figure A, Jones stain). Two arteries had evidence of past arteritis with elastic lamina disruption (Figure B, EVG stain); acute necrotizing arteritis was not present. The patient recovered renal function (most recent serum Cr=3.2 mg/dL) after therapy with pulse steroids, plasma exchange, Rituxan, and Cytoxan.
Discussion
Our patient with HBV-associated vasculitis initially presented with manifestations of medium vessel involvement, but with an unusual feature of cANCA/PR3 seropositivity. Relapse occurred 2 years later with PAN symptoms (orchitis, severe abdominal pain) and evidence of ANCA-associated small vessel vasculitis (GN, hemoptysis). While the initial trigger may have been HBV, the vasculitis later became self-perpetuating as it recurred during a time when there was no HBV viremia. Patients with HBV-PAN who are ANCA+, should be closely followed because they could be at higher risk of vasculitis relapse.