By M. A. Khamashta, Maria L. Bertolaccini, Oier Ateka-Barrutia (auth.)
Antiphospholipid Syndrome guide offers the key medical gains of antiphospholid syndrome, or Hughes’ Syndrome, discussing analysis, therapy and administration of the disease, in a convenient brief sensible publication.
Bringing jointly some of the parts of specialization that can stumble upon antiphospholipid syndrome, a bit on scientific gains specializes in a number of organs in flip, explaining how antiphospholipid syndrome could be manifested within the organ in query. additionally, suggestions on antiphospholipid syndrome in being pregnant and pediatric antiphospholipid syndrome is included.
This reader-friendly, quickly pocket-reference is an invaluable consultant to clinicians from basic and inner medication disciplines, and specifically to experts in rheumatology, hematology, cardiovascular medication, neurology, nephrology, dermatology, chest medication and obstetrics.
Munther A. Khamashta, MD, FRCP, PhD is Senior Lecturer, Guy’s, King’s & St Thomas’ college of medication and Honorary advisor healthcare professional, , Rayne Institute, St Thomas’ clinic, London, united kingdom. he's a global chief in antiphospholipid syndrome, having labored within the box for over 20 years.
Maria L. Bertolaccini, MD, PhD is Lecturer established at Lupus learn Unit, The Rayne Institute, St. Thomas' health facility, London, united kingdom and is the dealing with Editor of the Lupus magazine.
Oier Ateka-Barrutia, MD is predicated on the sanatorium de Navarra, Pamplona, Spain. He has additionally labored along Dr Khamashta and Dr Bertolaccini as an out of the country medical fellow on the Lupus study Unit, The Rayne Institute, St. Thomas’ clinic, London, UK.
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5–5% PAPS. 19. PE is assumed to be the leading cause of PH in APS and its frequency has been ranged in 17–33% of APS patients. The prevalence of aPL in patients with chronic thromboembolic PH has varied between 10 and 20%. The possible role of aPL in the pathophysiology of “unexplained” PH has been proposed, but remains unclear to date. 52 Chapter 7. 18. Conditions associated with pulmonary hypertension. 19. Pulmonary hypertension in APS. APS-related Pulmonary embolism (acute/chronic) Left-sided heart failure Heart valve dysfunction Myocardial infarction Myocardiopathy Portal hypertension Pulmonary veno-occlusive disease Not directly APS-related Chest disorder leading to chronic hypoxia Fibrosing alveolitis Coincidental Evaluation includes careful personal and familial history, complete physical examination, ECG, chest Rx, transesophageal echocardiogram, pulmonary function test with arterial blood gas tension, and additional sleep studies when sleep apnea may be suspected, routine blood tests, liver function tests, complete autoantibody screening including aPL, HIV serology, and either pulmonary angiogram or helicoidal chest CT scan that should be preferred to ventilation-perfusion isotopic scan.
10 Osteoarticular Manifestations 53 Treatment options are conditioned by the mechanism and cause of PH. However, chronic anticoagulation is needed in all cases, at least to prevent the development of superimposed thrombosis. When PH results from chronic thromboembolism, inferior vena cava filter may be recommended, and successful thromboendarterectomy has been performed in some patients with very severe disease. Transplantation, either double-lung, single lung, or heartlung, may cure the disease, but mortality remains high and donors scarce.
Am J Med 86(4):391–399 Asherson RA, Khamashta MA, Baguley E, Oakley CM, Rowell NR, Hughes GRV (1989b) Myocardial infarction and antiphospholipid antibodies in SLE and related disorders. Q J Med 73(272):1103–1115 Asherson RA, Higenbottam TW, Dinh Xuan AT, Khamashta MA, Hughes GRV (1990) Pulmonary hypertension in a lupus clinic: experience with twenty-four patients.