Differential Diagnosis

Any part of the venous circulation may undergo occlusion in APS. Deep and superficial veins of the lower limbs are most frequently involved, followed by PE and arm vessels. In these instances and in subjects who are relatively young (< 45 years), the differential diagnosis rests on laboratory tests aiming at the identification of congenital or other acquired thrombophilic states (Table 9.1). Some clinical features may point toward systemic disorders with a higher than average risk of venous...

Coronary Artery Disease

Accelerated atherosclerosis in SLE, besides classical risk factors, such as age, sex, smoking, hyperlipidemia, hypertension, diabetes, hyperhomocysteinemia, cronic renal insufficiency, obesity, is related to a permanent pro-inflammatory state, long-term steroid administration and aPL. The prevalence of aPL in patients with myocardial infarction seems to be between 5 and 15 . Elevated levels of aCL imply an increased risk for the development of myocardial infarction and recurrent cardiac events....

Systemic Hypertension

The prevalence of hypertension in APS has been described in 30-50 of the patients, depending on the cohort, and seems to be more prevalent in PAPS than in secondary APS. Etipathogenesis is summarized in Table 7.20. Livedo reticularis is a frequent accompanying sign, and has been found in up to 80 of the hypertensive APS patients. The severity of hypertension varies from mild labile to severe accelerated hypertension. In case of APS patients with poorly controlled hypertension with...

Antiphospholipid Syndrome Handbook

MD, PhD Lupus Research Unit The Rayne Institute King's College London School of Medicine St. Thomas' Hospital London UK ISBN 978-1-84628-522-6 e-ISBN 978-1-84628-735-0 Springer London Dordrecht Heidelberg New York British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library Library of Congress Control Number 2009932752 Springer-Verlag London Limited 2010 Apart from any fair dealing for the purposes of research or private study, or...

Catastrophic Antiphospholipid Syndrome

Catastrophic APS develops in a minority (< 1 ) of patients with aPL.It is characterized by acute, life-threatening, diffuse thrombotic microvasculopathy, multiple vascular occlusion, and high titer aPL, with a predilection for kidney, lung, brain, heart, skin, and gastrointestinal tract (Fig. 7.16). Classification criteria for CAPS are shown in Table 7.14. Fig. 7.16. Catastrophic antiphospholipid syndrome. Fig. 7.16. Catastrophic antiphospholipid syndrome. Table 7.14. Preliminary criteria for...

Thrombotic Microangiopathy TMA

TMA is the characteristic histologic lesion of the microvascu-lature in APS-related nephropathy, affecting glomerular capillaries, afferent arterioles, and interlobular arteries (Fig. 7.10). Of course, it is not pathognomonic of APS, as there is a wide range of conditions that present the same histological appearance (Table 7.6). Depending on the degree and extension of damage, patients could have isolated hypertension (mild to severe), proteinuria Fig. 7.9. Primary APS with giant skin ulcer on...

Headache

Mitral Valve Thrombosis

One of the most prominent features in patients with APS is headache. This symptom, a common complaint of APS patients in clinical practice, can vary from classic intermittent migraine to almost continuous incapacitating headache. The association of migraine and aPL is controversial with widely varying results from different series. One of the major problems is that headaches, often non-migrainous, have been loosely termed migraine, and these headaches may precede or accompany TIAs or CVAs. The...

Osteonecrosis Avascular Necrosis or Aseptic Necrosis

The prevalence of symptomatic osteonecrosis is 2-3 in APS patients, but up to 20 of PAPS may have asymptomatic osteonecrosis. Several factors have been associated with this disorder (Table 7.21), although the pathogenic mechanisms still remain partially elucidated. The most predominant hypotheses are included in Table 7.22. Table 7.21. Etiologic factors associated with osteonecrosis. Trauma Hematologic disorders Sickle cell disease, Thalassemias, DIC, Polycythemia, Hemophilia, Clotting...

Autoimmune Thrombocytopenia

Thrombocytopenia is found in 25-40 of patients with APS, and is one of the most common presenting manifestations. Among these patients, thrombocytopenia is more frequently observed in patients with SLE than in patients with PAPS. aPL-related thrombocytopenia is a chronic, usually mild and is seldom associated with hemorrhagic complications. Bleeding may be related to several causes other than thrombocytopenia Table 7.7. Hematologic disorders in APS. Autoimmune thrombocytopenia Autoimmune...