Antiphospholipid syndrome is an autoimmune disease that is characterized by the presence of several antiphospholipid autoantibody types. The clinical presentation involves thromboembolic events affecting vessels of all sizes, ultimately predisposing the patient to malignant hypertension and life-threatening vascular insults in the kidneys, the brain, the lungs, and several other organs. In severe cases, multiorgan failure is seen, with mortality rates of 50%. Thus, an early diagnosis is mandatory. The criteria consist of pregnancy-related events, laboratory confirmation of autoantibodies, and signs of blood vessel abnormalities.
Antiphospholipid syndrome is a still incompletely understood autoimmune disorder that possesses a highly variable clinical presentation, as virtually any organ or part of the body may be affected by the vascular pathological changes that occur . The symptoms stem from two events - formation of venous (or sometimes arterial) thrombosis and thrombotic microangiopathy (TMA)   . Venous thrombosis of the lower extremities is regarded as a frequent finding in patients with antiphospholipid syndrome, whereas pulmonary embolism (developing either from distal thrombi or cardiac valve vegetations) can appear in up to 50% of cases . In addition to the lower extremities, thrombotic changes often affect the subclavian, retinal, and renal arteries . In fact, the kidneys are the primary site where both thrombotic and TMA-related effects of antiphospholipid syndrome are exerted  . Occlusion of both larger and smaller renal vessels leads to profound hypertension, proteinuria, and renal insufficiency that presents with anuria . Apart from the kidneys, other organs that are prone to damage are the brain, the heart, the lungs, and the skin  . The term "catastrophic antiphospholipid syndrome" is used to describe patients who suffer from life-threatening forms of this disease  . Hypertension, hemolytic anemia, disseminated intravascular coagulation (DIC), microthrombotic events involving the heart and the brain, as well as adult respiratory distress syndrome (ARDS), are hallmarks of catastrophic antiphospholipid syndrome, which carries a mortality rate of 50%   .
Entire Body System
Jaw & Teeth
Skin ulcerations correlated with positive titres aCL IgM and ß2 GPI IgM (p 0.03 and 0.04, respectively), while pseudovasculitis correlated with positive titres ß2 GPI IgM (p 0.02). [ncbi.nlm.nih.gov]
We report a case of APS manifesting as cutaneous ulceration and necrosis in a patient with severe RA. [mdedge.com]
KEYWORDS: antiphospholipid antibody syndrome; cerebral venous thrombosis; headache; tadalafil [ncbi.nlm.nih.gov]
Three weeks after admission, the patient complained of headache and double vision. Neurologic examination revealed new horizontal diplopia, partial right sixth nerve palsy, and bilateral ptosis. [ajnr.org]
For those patients with less serious problems such as headaches, a daily low-dose of aspirin 75mg is usually advised. [lupusuk.org.uk]
The recognition of antiphospholipid syndrome rests on a thorough clinical and laboratory assessment and the diagnostic criteria are comprised of the following:
- Clinical criteria - Pregnancy-related events that raise suspicion toward antiphospholipid syndrome are death of previously healthy fetuses after the 10th week of gestation, premature births due to eclampsia, pre-eclampsia, or insufficiency of the placenta, or unexplained spontaneous abortions before the 10th week of gestation    . For this reason, it is imperative for physicians to obtain a detailed history that will reveal these findings. Confirmation of vascular thrombosis in any organ, regardless of vessel size, is the second clinical criteria    .
- Laboratory criteria - Detection of either lupus anticoagulant (LA), immunoglobulin (Ig) M or IgG anticardiolipin antibodies, or IgM or IgG anti-β2-glycoprotein-1 antibodies on two separate tests that are done 12 weeks apart using standard methods is crucial for identifying antiphospholipid syndrome    . Values of anticardiolipin and anti-β2-glycoprotein-1 antibodies should be greater than the 99th percentile  . Values of LA may not be accurate in the presence of antithrombic drugs, which implies that their use should be temporarily stopped for proper testing .
Although nephropathy, thrombocytopenia, and valvular heart disease are notable findings in these patients, at least one clinical and one laboratory criteria are needed in order to make the diagnosis of the antiphospholipid syndrome   .
Nevertheless, this tool is a late indicator to suggest any additional treatments. [ncbi.nlm.nih.gov]
The aim of this review article is to update on the medical care of pregnancy in these women to ensure the best maternal and fetal prognosis. Copyright 2017 Elsevier Ltd. All rights reserved. [ncbi.nlm.nih.gov]
Prognosis [ 15 ] The severity of the problem varies considerably and prognosis is therefore very variable. It can cause catastrophic and potentially lethal problems like massive pulmonary embolism, stroke and myocardial infarction. [patient.info]
KEYWORDS: Antiphospholipid syndrome/complications; disease progression; endocarditis/etiology/pathology/surgery; heart valve prosthesis; lupus erythematosus, systemic/complications; treatment outcome; tricuspid valve insufficiency/etiology/surgery [ncbi.nlm.nih.gov]
This study aimed to characterize the epidemiology of definite APS based on the 2006 updated international consensus (Sydney) classification criteria. [acrabstracts.org]
Links: aetiology epidemiology clinical features detection of lupus coagulant detection of anticardiolipin antibodies diagnostic criteria definitive diagnosis of antiphospholipid syndrome treatment prognosis antiphospholipid antibodies catastrophic antiphospholipid [gpnotebook.co.uk]
Name Handbook of Systemic Autoimmune Diseases Volume 12 ISSN (Print) 15715078 Fingerprint Antiphospholipid antibodies epidemiology thrombosis Immunology and Allergy Endocrinology, Diabetes and Metabolism Cite this APA Standard Harvard Vancouver Author [jhu.pure.elsevier.com]
Thrombosis in the young: epidemiology and risk factors: a focus on venous thrombosis. Thromb Haemost 1997 ; 78: 1 – 6. Google Scholar Medline ISI 9. Roubey, RA. [doi.org]
Université René Descartes Paris V, Centre de référence maladies auto-immunes et systémiques rares, Service de médecine interne Pôle médecine, Hôpital Cochin, AP-HP, 27 rue du Faubourg Saint Jacques, 75679 Paris cedex 14, France; INSERM U 1153, Center for Epidemiology [ncbi.nlm.nih.gov]
The goal of this review is to update clinicians and scientists on mechanistic and clinically relevant studies from the past 18 months, which have especially focused on inflammatory aspects of APS pathophysiology. [ncbi.nlm.nih.gov]
The complement block, which interrupts this amplification cycle, could prevent CAPS in patients with early TMA who face precipitating events. [ncbi.nlm.nih.gov]
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- Pengo V, Tripodi A, Reber G, et al. Update of the guidelines for lupus anticoagulant detection: Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibody of the Scientific and Standardisation Committee of the International Society on Thrombosis and Haemostasis. J Thromb Haemost. 2009;7(10):1737–1740.