Session Title: Antiphospholipid Syndrome
Session Type: Abstract Submissions (ACR)
The occurrence of antiphospholipid syndrome (APS) following an infection has been increasingly reported in the literature. We conducted a systematic review of case reports documenting the development of primary APS, catastrophic APS (CAPS), or APS related features following infection to identify potentially putative agents, and to summarize the patients’ clinical and immunologic characters, treatment administered, and patient outcomes.
We searched the Medline, EMBASE, Web of Science, and Cochrane CENTRAL databases with no language restriction from inception through October 2013 for case reports of patients with APS or CAPS related features after an infection. Study selection, data extraction, and quality appraisal were performed by 2 independent researchers. We extracted data on the related infection, clinical presentation, antiphospholipid (aPL) antibody profiles, treatment, and patient outcomes.
From 2,257 unique citations, 247 publications met our inclusion criteria, reporting on 281 cases. Three different groups of patients were identified according to the clinical presentation reported: 1) patients whose symptoms met the classification criteria for definitive APS or CAPS (24.2%), 2) patients who did not meet the criteria for APS or CAPS but developed transient thromboembolic phenomena with elevated aPL antibodies during an infection and recovered completely (44.8%), and 3) patients who developed transiently elevated aPL antibodies after an infection but no clinical consequences related to APS or CAPS (31%).
The most common preceding infections across all groups was viral (55.5% of cases), including human immunodeficiency virus, hepatitis C virus, and parvovirus B19. Bacterial infections were reported in 36.7% of cases with streptococci, mycoplasma pneumonia, and coxiella burnetii infections being the most common. Parasitic infections were reported in 3.9%, fungal infections in 2.1%, and multiple types of infection in 8.9%. Infection was the sole precipitating factor in 79.7% of reported cases.
Thromboembolic manifestations were the most common presenting feature among patients in groups 1 and 2. Patients across all groups had at least 1 positive aPL antibody with persistent positive antibodies observed mainly among patients in group 1. Anticardiolipin (aCL) antibodies were the most frequently reported, often with coexisting IgG and IgM antibodies. Anticoagulation therapy was the mainstay of treatment in the majority of cases in groups 1 and 2. Clinical outcomes varied considerably and included persistent manifestations of APS or CAPS and death in Group 1. Resolution of clinical events and disappearance of aPL antibodies were most frequently reported in groups 2 and 3.
Development of aPL antibodies, APS, and CAPS can occur after infection with various virus, bacteria, fungus and parasites. Further longitudinal studies should be conducted to better quantify the risk and outcomes of APS or CAPS after infection.
M. A. Lopez-Olivo,
M. E. Suarez-Almazor,
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/antiphospholipid-syndrome-following-infection-a-systematic-review-of-case-reports/