Sudden death due to polyarteritis nodosa

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.

Single-organ gallbladder vasculitis: Characterization and distinction from systemic vasculitis involving the gallbladder. An analysis of 57 patients

“…Lithiasic cholecystitis and/or chronic cholelithiasis was the clinical presentation in 44% of patients, 3,4,11,14,17,19,31,34,38,40,46,48,50,51,61 45.7% presented with acalculous cholecystitis, 1,3,5,12,16,18,20,22,23,28,32,36 -38, 42-48,51,58-60 3.4% with bile duct obstruction, 10,15 and 6.7% did not exhibit abdominal symptoms; GV was discovered because of abnormal GB findings in imaging studies, 9,38 exploratory laparotomy (current study Patient 5), or necropsy. 56 GB-related manifestations were the only expression of GV in 20 (33%) patients. Among patients with GV, abdominal symptoms were followed by systemic features in 5 (13.5%), systemic and GB manifestations were concomitantly present in 14 (37.8%), and systemic symptoms were the initial manifestation in 18 (48.6%) patients.…”

“…At the time of abdominal surgery, other regions with vasculitis involvement were found, including the small 11 In the patient in whom GV was diagnosed postmortem, autopsy revealed vasculitis in multiple organs. 56 In 3 patients autopsy was performed after the initial cholecystectomy, and findings included vasculitis of the superior mesenteric artery with arterial rupture (Patient 5) and vasculitis affecting multiple intraabdominal organs. 4 Abdominal angiography was performed in 7 patients and vascular abnormalities were detected in 3 who had GB-SV (Table 4).…”

“…9,10,12,37,46,54 GB-SOV was found in 20 (33%) patients and GB-SV in 41 (67%). Forms of SV that affected the GB were PAN (n ¼ 10), 4,12,22,32,38,45,46,51,54 HBV-associated vasculitis (n ¼ 8), 10,11,17,38,46,56,60 cryoglobulinemic (essential or HCVassociated) vasculitis (n ¼ 6), 9,14,19,36,50 MPA (n ¼ 4), 5,31,38,40 EGPA (Churg-Strauss) (n ¼ 4), 20,43,48,61 IgA vasculitis (Henoch-Schönlein) (n ¼ 2), 23,28 giant cell arteritis (n ¼ 1), 47 and vasculitis associated with autoimmune diseases (n ¼ 6) 11,16,18,42,58 (see Table 4). Systemic diseases with associated vasculitis included RA (n ¼ 3), 11,16,18 SLE (n ¼ 2), 42,58 and systemic sclerosis (n ¼ 1).…”

“…10,12,16,20,23,38,40,43,46,47,58,61 Two (10%) GB-SOV patients died years later from unrelated conditions, 3,11 and 11/30 (37%) GB-SV patients died from complications derived from disease activity or infections. 4,11,16,18,38,46,51,56 …”

Reply to the letter by Dr. Hejna

“…We thank Dr. Hejna both for his generous comment on our recent case report, Sudden death due to polyarteritis nodosa [1] (PAN), and for his gentle historical ''correction'', namely, that the genial Bohemian pathologist and Professor of Pathology at the University of Vienna, Karel Rokitanský (Carl Freiherr von Rokitansky), published in a disquisition on aneurysms, the first ''macroscopic'' description of necrotizing arteritis in 1852 [2]. History confirms a well-documented evolution eventuating in the current nosology and diagnostic criteria for PAN, which were midwifed by many nineteenth century continental giants of medical science and pathology [3].…”