Antiphospholipid Antibodies and Antiphospholipid Syndrome: Role in Portal Vein Thrombosis in Patients With and Without Liver Cirrhosis
The antiphospholipid syndrome (APS) has been associated with portal vein thrombosis (PVT). This study explored the contribution of antiphospholipid antibodies (aPL) to PVT in cirrhotic and noncirrhotic patients.
A total of 50 patients with liver cirrhosis and PVT, 50 patients with liver cirrhosis without PVT, 50 consecutive PVT without liver cirrhosis, and 50 controls. aPL tests: lupus anticoagulants (LAs), immunoglobulin G (IgG) anti-cardiolipin antibodies (aCL), IgG anti-beta-2-glycoprotein-I (β(2)GPI), and IgG β( 2)GPI-complexed with oxidized low-density lipoprotein antibodies (ox-LDL).
Lupus anticoagulants were negative in all patients. A titre of IgG aCL >40 IgG phospholipid units (GPL) was present in 2% of patients with liver cirrhosis and in none of the other groups. In all, 4% of patients with PVT without cirrhosis were positive for IgG β(2)GPI in the absence of any other positive aPL and labelled as primary APS.
aPL play no role in PVT associated with liver cirrhosis but can be tested in idiopathic PVT.
Available from: Massimo Primignani
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ABSTRACT: Decreased levels of most coagulation factors and thrombocytopenia are the main haemostatic abnormalities of cirrhosis. As a consequence, this condition was, until recently, considered as the prototype acquired coagulopathy responsible for bleeding. However, recent evidence suggests that it should, rather, be regarded as a condition associated with normal or even increased thrombin generation. The bleeding events that occur in these patients should, therefore, be explained by the superimposed conditions that frequently occur in this setting. Due to elevated levels of factor VIII (procoagulant driver) in combination with decreased protein C (anticoagulant driver), which are typically found in patients with cirrhosis, a procoagulant imbalance, defined as a partial resistance to the in vitro anticoagulant action of thrombomodulin, can be demonstrated. Whether this in vitro hypercoagulability is truly representative of what occurs in vivo remains to be established. However, the hypothesis that it may have clinical consequences is attractive and deserves attention. The possible consequences that we discuss herein include whether (i) cirrhosis is a condition associated with increased risk of venous thromboembolism or portal vein thrombosis; (ii) the hypercoagulability associated with cirrhosis has any other role outside coagulation (i.e. progression of liver fibrosis); and (iii) anticoagulation should be used in cirrhosis. Although apparently provocative, considering anticoagulation as a therapeutic option in patients with cirrhosis is now supported by a rationale of increasing strength. There may be subgroups of patients who benefit from anticoagulation to treat or prevent thrombosis and to slow hepatic fibrosis. Clinical studies are warranted to explore these therapeutic options.
Available from: Leendert A Trouw
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ABSTRACT: Rheumatoid arthritis (RA) is a common autoimmune disease affecting around 1% of the population. Although major advances have been made in the treatment of RA, still relatively little is known on disease pathogenesis and aetiology. From treatment studies it has become clear that treating patients early in their disease course will provide the best results. However, especially in the early phase of arthritis, in particular when the patients do not yet fulfil the criteria for RA, it is difficult to decide which patients would benefit most from an early and aggressive intervention. Good biomarkers are important to guide decisions in the clinical management of RA. Next to the well-known rheumatoid factor (RF) and the anti-citrullinated protein antibodies (ACPA), several new markers are now likely to become available with interesting potential. Besides antibody responses directed against citrullinated proteins, also antibodies against carbamylated proteins (anti-CarP) have recently been shown to be present in RA. Interestingly these anti-CarP antibodies are also present in around 20% of the ACPA-negative RA patients and are associated with more severe joint damage in this group. Apart from the antibodies that help in establishing the diagnosis and prognosis, also novel biomarkers that reflect clinical disease activity scores are being discovered. The development of biomarker-based disease activity scores might allow easy and frequent monitoring of patients to rapidly adjust treatment.
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ABSTRACT: Abdominal vein thrombosis is a rare, but potentially life-threatening form of venous thrombosis. It mainly involves the hepatic veins (Budd Chiari syndrome, BCS), portal veins (PVT) and mesenteric veins. In recent years several large-scale studies have been performed to study the underlying aetiological factors in these thrombotic disorders. Both inherited and acquired thrombophilia factors are frequently observed in these patients. Factor V Leiden mutation is frequently found in patients with BCS and prothrombin gene variant is seen more frequently in PVT. Myeloproliferative neoplasms (MPNs), including polycythemia vera and essential thrombocythemia, are underlying disorders in 30-40% of patients with abdominal vein thrombosis. Other aetiological factors are paroxysmal nocturnal haemoglobinuria (PNH), autoimmune disorders and hormonal factors. Recently, several new risk factors have been reported and are discussed in this review. BCS and PVT are multi-factorial disorders. In nearly 50% of patients two, and in 16% even three prothrombotic risk factors were found at presentation. Because patients with abdominal vein thrombosis have a high risk of recurrence immediate anticoagulant treatment is necessary. The duration of treatment is still a matter of debate because these patients also have a high risk of bleeding, especially those with portal hypertension. For BCS patients life-long anticoagulant treatment is advised. In patients with PVT it is recommended to tailor treatment to the individual patient based on the presence of an underlying prothrombotic disorder and the risk of bleeding.
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