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Sudden death due to polyarteritis nodosa
Lisa B E Shields1, Meredith Burge, John C Hunsaker
1Norton Neuroscience Institute, Louisville, KY, USA.
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 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.
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