Antiphospholipid antibodies and thrombosis

M Greaves1Greaves M Antiphosolipid antibodies and thrombosis.Lancet. 1999; 353: 1348-1353Summary Full Text Full Text PDF PubMed Scopus (286) Google Scholar reviews antiphospholipid antibodies and thrombosis. We noted a special pattern of antiphospholipid syndrome in a woman aged 42 years who was admitted to our intensive care unit because of sudden severe respiratory failure. Computed tomography scan on day 2 revealed ground-glass opacity, interstitial, reticular, and confluent infiltrates, peripheral pulmonary thromboembolism, pleural effusions; and basal partial atelactasis in both lungs. In the abdomen, perfusion defects in liver, kidneys, and spleen were suspected. The patient developed severe multiple organ dysfunction syndrome (MODS) that necessitated supportive therapy with invasive artificial ventilation, inotropic, and vasopressor therapy, continuous venovenous haemofiltration, and substitution of platelets and packed erythrocytes for severe thrombo-cytopenia and anaemia, respectively. Because of her medical history, including antinuclear antibody-negative lupus erythematosus with anticardiolipin antibodies, focal segmental glomerulo-nephritis, thrombosis of a central retinal artery, cerebral infarction in 1977, intrauterine fetal death in 1986, livedo reticularis, and moderate thrombocytopenia, we postulated haemorrhagic lupus pneumonitis or pulmonary manifestation of antiphospholipid syndrome with pulmonary capillaritis, alveolar haemorrhage, and repeated microvascular thrombosis as the cause of disease.2Asherson RA Cervera R Antiphosplipid antibodies and the lung.J Rheumatol. 1995; 22: 62-66PubMed Google Scholar Pulmonary thromboembolism can result in haemorrhage without infarction. On computed tomography haemorrhage appears as an area of ground-glass opacification or airspace consolidation that is indistinguishable from pneumonia or oedema.3Greaves SM Hart EM Brown K et al.Pulmonary thromboembolism: spectrum of findings on CT.AJR. 1995; 165: 1359-1363Crossref PubMed Scopus (41) Google Scholar Bronchoalveolar lavage was done, obtaining haemosiderin-laden alveolar macrophages, many erythrocytes, and a normal proportion of macrophages, granulocytes, and lymphocytes. High-dose cortisone therapy and anticoagulation with heparin was started. Lupus anticoagulant was positive. Immunological tests showed IgG anticardiolipin antibodies (initial 72·0 IgG phospholipid units [normal <15·0 units] and 36·0 units on day 17) and anti β2-glycoprotein I antibodies. IgM anticardiolipin antibodies, antinuclear antibody, antibodies against native DNA and extractable nuclear antigen, antibodies to neutrophil cytoplasma, and antibodies to basement membrane were negative. Circulating immuocomplexes, functional haemolytic complement, C3, and C4 were within normal limits. Echocardiography showed a grade II—III mitral regurgitation and vegetations on the mitral valve suggesting Libman-Sacks endocarditis, which is a known feature of antiphospholipid syndrome.4Nesher G Ilany J Rosenmann D et al.Valvular dysfunction in antiphospholipid syndrome: prevalence, clinical features, and treatment.Semin Arthritis Rheum. 1997; 27: 27-35Summary Full Text PDF PubMed Scopus (145) Google Scholar The dermatological manifestations were consistent with antiphospholipid syndrome. Because of progressive MODS we started serial plasma-pheresis and immunosuppressive therapy with cyclophosphamide on day 9, and her clinical condition improved. The patient was taken off the ventilator, cardiovascular drugs were tapered off, and haemofiltration was discontinued. She was transferred to another hospital after 19 days. Unfortunately, she died several days later from spontaneous severe intracranial haemorrhage. The major pathophysiological difference of this syndrome (known as catastrophic antiphospholipid syndrome) is the occurrence of mainly microvascular thrombosis affecting multiple organs such as lungs, heart, brain, kidney, liver, adrenal glands, or gastrointestinal tract.5Asherson RA Cervera R Piette JC et al.Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients.Medicine (Baltimore). 1998; 77: 195-207Crossref PubMed Scopus (496) Google Scholar