Lethal Manifestations of Systemic Lupus Erythematosus in a Forensic Context

Abstract

Systemic lupus erythematosus is an autoimmune connective tissue disorder that affects multiple organs. While the clinical manifestations may vary in intensity over time and be associated with chronic disease, occasional cases occur where sudden and unexpected death has occurred. Cardiovascular disease is common, with accelerated atherosclerosis, intravascular thrombosis associated with antiphospholipid syndrome, and hypertensive cardiomegaly. Vasculitis with superimposed thrombosis may result in critical reduction in blood to vital organs, such as the heart and brain with infarction. Mesenteric ischemia may be caused by vasculitis, thrombosis, and accelerated atherosclerosis and may result in lethal intestinal infarction. Other diverse causes of sudden death include myocarditis, epilepsy, pulmonary hypertension, pulmonary thromboembolism, and sepsis. The autopsy evaluation of such cases requires careful examination of all organs with extensive histological sampling to include blood vessels, and microbiological sampling for bacteria, viruses, and fungi.

Marantic endocarditis – A not so benign entity

“…9 Other smaller non-infective vegetations may occur in rheumatic fever and systemic lupus erythematosus. 10,11 Since vegetations in marantic endocarditis have little inflammatory reaction at the site of attachment, they can detach more readily than vegetations in infective endocarditis and thus cause multiple organ embolism. 3 Systemic embolisation is the main clinical consequence, occurring in 40% of patients.…”

Fatal sepsis by Klebsiella pneumoniae in a patient with systemic lupus erythematosus: the importance of postmortem microbiological examination for the ex post diagnosis of infection

“…Infection is the predominant cause of mortality because of impaired cellular and humoral immune responses, genetic immune abnormalities, and associated comorbidities [12][13][14]. Bacteraemia-related mortality is higher than that caused by other more common infections involving lungs, gastrointestinal, and genitourinary tract [15][16][17]. Bacteraemia in SLE patients is typically caused by common and opportunistic pathogens: gram-positive cocci (i.e., Staphylococcus aureus, Streptococcus pyogenes, Streptococcus pneumoniae) and gram-negative bacilli (i.e., Escherichia coli, Salmonella enteritidis, Salmonella typhimurium, and Pseudomonas aeruginosa) [12,16,18].…”

Acute mesenteric ischaemia and unexpected death

“…11 In addition to atherosclerosis, thrombosis may be provoked by hypercoagulable states, hypovolaemia or shock. 9 Mesenteric vasculitis with superimposed thrombosis has also been reported in systemic lupus erythematosis, 12 although infarcts related to autoimmune conditions tend to be small and superficial. 8 Other rare causes of arterial thrombosis include dissections and aneurysms.…”

“…Far less commonly there may be other causes of mesenteric arterial thombosis such as vasculitis associated with underlying systemic lupus erythematosus and so careful histologic sampling should be undertaken. 12 Thromboses tend to be associated with more extensive areas of intestinal infarction than those caused by embolism. 11 It should be recognized, however, that atherosclerotic narrowing of the mesenteric arteries is not uncommon, with 6e10% of the population having greater than 50% stenosis of at least one mesenteric artery identified at autopsy.…”