Sudden death due to polyarteritis nodosa
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CASE REPORT
Sudden death due to polyarteritis nodosa Lisa B. E. Shields • Meredith Burge John C. Hunsaker III
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Accepted: 23 September 2011 / Published online: 8 October 2011 Springer Science+Business Media, LLC 2011
Abstract Classical polyarteritis nodosa (cPAN) refers to a rare, potentially fatal systemic transmural necrotizing vasculitis that usually affects medium-sized, and occasionally small, muscular arteries, primarily involves the kidneys, gastrointestinal tract, skin, nervous system, joints, and muscles, and is rarely, if ever, expressed in the lungs. The incidence of mortality has significantly decreased with recently developed treatment modalities, in particular antiviral medications. Sudden death due to previously undiagnosed cPAN is rarely encountered. We report a case of a young man who had been evaluated on three occasions by medical personnel in the 3 weeks prior to his death. He had complained of nonspecific symptoms of abdominal and perineal/suprapubic pain, nausea, vomiting, sensation of chilling, and constipation. The spectrum of diagnoses included ‘‘gastroenteritis,’’ enteric infection, and prostatitis. Found agonal at home and dying despite immediate cardiopulmonary resuscitation (CPR), he underwent a medicolegal autopsy, which revealed vasculitis of various organs, including heart (myocardium and epicardium) and extramural coronary arteries, liver, spleen, kidneys, adrenal glands, stomach and bowel, omentum, gallbladder, and pancreas. His sudden death was cardiac in nature due to PAN associated clinically with hepatitis B surface antigen
L. B. E. Shields Norton Neuroscience Institute, Louisville, KY, USA M. Burge J. C. Hunsaker III (&) Office of the Associate Chief Medical Examiner, 100 Sower Boulevard, Suite 202, Frankfort, KY 40601-8272, USA e-mail: [email protected] M. Burge J. C. Hunsaker III Department of Pathology and Laboratory Medicine, University of Kentucky College of Medicine, Lexington, KY, USA
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positivity (hepatitis B virus-associated PAN [HBV-PAN]). A complete autopsy with thorough histopathological examination is necessary to diagnose this uncommon yet potentially fatal vasculitis. Keywords Forensic pathology Sudden cardiac death Polyarteritis nodosa Non-infectious vasculitis
Introduction Initially described in 1866, polyarteritis nodosa (PAN) was the first vasculitis recognized [1]. The first reported case involved a patient with fever, muscle weakness, paresthesias, myalgias, anorexia, abdominal pain, and oliguria with inflammatory nodular lesions in arteries noted at autopsy [2]. The term ‘‘periarteritis nodosa’’ was originally coined, but has been updated to ‘‘polyarteritis nodosa’’ to reflect the transmural inflammation and to distinguish the inflammatory process from other vasculitides. Historically, the diagnosis of PAN has been poorly understood and elusive, often confused with and inaccurately labeled as other types of vasculitides, leading to improper treatment. Since Zeeks’ proposition of five distinct vasculitides in 1952, several approaches
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