Antiphospholipid syndrome–mediated acute cerebrovascular diseases and long-term outcomes

Abstract

Objectives The antiphospholipid syndrome (APS) is an autoimmune disease associated with thrombotic and non-thrombotic neurologic manifestations. APS is classified as primary (PAPS) or secondary (SAPS) when it co-exists with another autoimmune disease. We aim to describe the spectrum of acute cerebrovascular disease among patients with APS, their differences between stroke subtypes, and long-term functional outcomes. Methods Retrospective cohort study including adult (≥18 years) patients with APS followed in the stroke clinic of a tertiary-care reference center for autoimmune diseases in Mexico from 2009 to 2019. Results We studied 120 cases; 99 (82.5%) women; median age 43 years (interquartile range 35–52); 63.3% with SAPS. Demographics, comorbidities, and antiphospholipid antibodies (aPL) positivity were similar between APS type and stroke subtypes. Amongst index events, we observed 84 (70%) acute ischemic strokes (AIS), 19 (15.8%) cerebral venous thromboses (CVT), 11 (9.2%) intracerebral hemorrhages (ICH), and six (5%) subarachnoid hemorrhages (SAH). Sixty-seven (55.8%) were known patients with APS; the median time from APS diagnosis to index stroke was 46 months (interquartile range 12–96); 64.7% of intracranial hemorrhages (ICH or SAH) occurred ≥4 years after APS was diagnosed (23.5% anticoagulation-related); 63.2% of CVT cases developed before APS was diagnosed or simultaneously. Recurrences occurred in 26 (22.8%) patients, AIS, in 18 (69.2%); intracranial hemorrhage, in eight (30.8%). Long-term functional outcomes were good (modified Rankin Scale ≤2) in 63.2% of cases, during follow-up, the all-cause mortality rate was 19.2%. Conclusion We found no differences between stroke subtypes and APS types. aPL profiles were not associated with any of the acute cerebrovascular diseases described in this cohort. CVT may be an initial thrombotic manifestation of APS with low mortality and good long-term functional outcome.

Cerebral venous thrombosis in patients with autoimmune disease, hematonosis or coronavirus disease 2019: Many familiar faces and some strangers

“…Upon hospital discharge, 78% of patients had a complete resolution of the symptoms or mild symptoms (mRS = 0–1), 4% of patients recovered partially and maintained autonomy (mRS = 2), 10% of patients sustained mild to moderate impairments (mRS = 3–5), and 6% of patients died during hospitalization (mRS = 6) 15 . During 6 and 12 months of follow‐up, the mRS remained favorable, with the remaining symptoms disappearing 15,53 …”

Role of β2‐glycoprotein I in the pathogenesis of the antiphospholipid syndrome

“…This leads to endothelial cell dysfunction, thrombosis, acceleration of atherosclerosis, and an increased risk of myocardial infarction and stroke. 22,[62][63][64][65] Meanwhile, glomerular endothelium cell injury may be related to vasculitis, inflammation, and vascular sclerosis. 66 We found that HHcy induced the secretion of anti-β2GPI antibodies by B cells and the deposition of pathogenic antibodies within glomerular endothelial cells, ultimately reducing renal function.…”

“…The expression of these adhesion molecules promotes monocyte adhesion to endothelial cells and increases the secretion of the proinflammatory cytokines tumor necrosis factor and interleukin (IL)‐1β. This leads to endothelial cell dysfunction, thrombosis, acceleration of atherosclerosis, and an increased risk of myocardial infarction and stroke 22,62–65 . Meanwhile, glomerular endothelium cell injury may be related to vasculitis, inflammation, and vascular sclerosis 66 .…”

Relationship Between Gender Differences and Clinical Outcome in Patients With the Antiphospholipid Syndrome

“…In less than 1% of cases a catastrophic APS (CAPS), characterized by systemic microangiopathy is observed (9,10). It can be present as an isolated disorder ("primary" APS, PAPS) or can appear in association with SLE or other autoimmune diseases ("secondary" APS, SAPS) (11)(12)(13)(14). PAPS patients have a mean age at diagnosis of about 35-40 years and the disease is more common in women than in men, with a female to male ratio of about 3.5:1 (15).…”