Antiphospholipid syndrome associated with infections: clinical and microbiological characteristics of 100 patients

Abstract

Objective: To describe and analyse the clinical characteristics of 100 patients with antiphospholipid syndrome (APS) associated with infections. Methods: Patients were identified by a computer assisted search (Medline) of published reports to locate all cases of APS published in English, Spanish, and French from 1983 to 2003. The bilateral Fisher exact test was used for statistics. Results: 59 female and 41 male patients were identified (mean (SD) age, 32 (18) years (range 1 to 78)): 68 had primary APS, 27 had systemic lupus erythematosus, two had ''lupus-like'' syndrome, two had inflammatory bowel disease, and one had rheumatoid arthritis. APS presented as a catastrophic syndrome in 40% of cases. The main clinical manifestations of APS included: pulmonary involvement (39%), skin involvement (36%), and renal involvement (35%; nine with renal thrombotic microangiopathy, RTMA). The main associated infections and agents included skin infection (18%), HIV (17%), pneumonia (14%), hepatitis C (13%), and urinary tract infection (10%). Anticoagulation was used in 74%, steroids in 53%, intravenous immunoglobulins in 20%, cyclophosphamide in 12%, plasma exchange in 12%, and dialysis in 9.6%. Twenty three patients died following infections and thrombotic episodes (16 with catastrophic APS). Patients given steroids had a better prognosis (p = 0.024). The presence of RTMA and requirement for dialysis carried a worse prognosis (p = 0.001 and p = 0.035, respectively). Conclusions: Various different infections can be associated with thrombotic events in patients with APS, including the potentially lethal subset termed catastrophic APS. Aggressive treatment with anticoagulation, steroids, and appropriate antibiotic cover is necessary to improve the prognosis.

The Prevalence of Positive Serum Anticardiolipin Antibodies and Asymptomatic Bacteriuria in Women with Recurrent Abortions

“…Recently, many infections have been emphasized and reported on several occasions to not only potentially trigger the production of these antibodies but also be accompanied by the clinical manifestation of APL itself [14]. Bayoumi et al [15] reported that antiphospholipid syndrome is characterized by the presence of pathogenic autoantibodies against β2 glycoprotein-1 (β2GP1).…”

“…McNeil et al [17] and Asherson et al [18] reported the presence of antiphospholipid antibodies in infectious diseases such as HIV, tuberculosis, measles, chickenpox, hepatitis, parvovirus and rubella. In a study on 100 patients complaining of antiphospholipid syndrome, Cervera R et al [14] found that a urinary tract infection was the associated infection in 10% of these patients. Asherson et al [18] reported that 35% of catastrophic APS cases were preceded by an infection, with urinary tract infections reported to occur in 6% of the cases.…”

“…Considerable reported evidence has suggested that β2GP1 is the main antigenic target for aPL. An S. aureus protein (Sbi) that binds β2GP1 had been reported to serve as a target molecule for IgG binding [14]. In addition to its presence in the plasma, β2GP1 is expressed on the surface membranes of different cell types involved in the pathogenesis of the syndrome, including endothelial cells, monocytes and trophoblasts [20].…”

A Case of Libman-Sacks Endocarditis that Developed after Infective Endocarditis

“…The association between LSE and APS was noted in 1985 and additional evidence has been reported that antiphospholipid antibodies are related to the pathogenesis of valvular heart disease [8][9][10]. On the other hand, several studies have suggested the association of APS/LSE and infection [2,11,12]. Cervera et al reported that various infections could be related to thrombotic events in patients with APS, and that catastrophic APS, which is the most severe form of APS, seemed to be triggered mainly by infections [2,13].…”

“…One of the triggering conditions of APS is infectious diseases such as skin infections, human immunodeficiency virus infection, pneumonia, hepatitis C virus infections, and urinary tract infections [2]. We describe here a case of a 38-year-old woman with Staphylococcus aureus infective endocarditis (IE) and multiple systemic embolisms.…”