Ann Rheum Dis: first published as 10.1136/ard.48.10.853 on 1 October 1989. Downloaded from Annals of the Rheumatic Diseases 1989; 48: 853-855 Cerebrovascular accident and myocardial infarction associated with anticardiolipin antibodies in a young woman with systemic lupus erythematosus YORAM MAARAVI,' EYAL RAZ,1 DAN GILON,' AND ALAN RUBINOW2 From the 'Department ofMedicine, Mount Scopus, and the 2Rheumatology Unit, Department ofMedicine A, Hadassah University Hospital, Jerusalem, Israel SUMMARY A 26 year old woman with systemic lupus erythematosus, including malar rash, photosensitivity, and arthritis, developed a cerebrovascular accident and acute myocardial infarction. High titres to antinuclear factor, anti-DNA antibodies, positive Venereal Disease Research Laboratory (VDRL) test, and anticardiolipin antibodies were found in her serum. A possible association between the presence of anticardiolipin antibodies and the two major thrombotic events is discussed. Recent studies suggest that anticardiolipin anti- normal. The erythrocyte sedimentation rate was 97 bodies may be responsible for a variety of laboratory mm/h. Haemogram and coagulation tests were copyright. and clinical phenomena in up to 61% of patients normal. Urinary sediment, serum electrolytes, with systemic lupus erythematosus (SLE). These albumin, liver and kidney function tests, serum include lupus anticoagulant, biological false positive cholesterol, triglycerides, and high density lipopro- test for syphilis, thrombocytopenia, recurrent tein cholesterol were normal. Creatine kinase was abortions, intrauterine fetal death, and thrombo- initially 182 U/l (normal <100 U/l) and reached a embolism. 1-5 peak of 376 U/l on the second day, while aspartate We describe here a young woman with SLE, not aminotransferase was initially 30 U/l (normal receiving corticosteroid treatment, who developed a <40 U/1) and increased to 60 U/l. The electro- cerebrovascular accident and acute myocardial in- cardiogram on admission showed a non-Q wave http://ard.bmj.com/ farction in the presence of a high titre of anticardio- infarction of the inferior wall. Echocardiography lipin antibodies. disclosed neither valvular abnormalities nor vegetations. Antinuclear antibody titre was 3 on a Case report 1-4 scale. Venereal Disease Research Laboratory (VDRL) titre was 1/16. Anticardiolipin antibody A 26 year old woman was admitted in February 1986 concentration (measured by radioimmunoassay because of crushing anterior chest pain of a few using 125I protein A as a detecting developer) was on October 1, 2021 by guest. Protected hours' duration. From the age of 12 she was known 6090 cpm (normal <2000). Anti-DNA antibodies to have had a butterfly malar rash, photosensitivity, were 22 ig/ml (normal <1.5). Serum C3 was and recurrent bouts of arthritis. Five years before 530 mg/l and C4 was 340 mg/l (normal 600-1100 admission she developed a left hemispheric cerebro- and 250-500 respectively). A skin biopsy showed no vascular accident due to thrombotic obstruction of evidence of vasculitis. the left internal carotid artery, which was confirmed On admission to the intensive care unit she by carotid arteriography. She denied risk factors for responded remarkably to sublingual nitrates and ischaemic heart disease, taking oral contraceptives, calcium channel blockers. Treatment with nitrates or receiving corticosteroid treatment. On admission and aspirin was started during the following hours. she appeared acutely ill and her vital signs were The laboratory investigation indicated that she had SLE, and treatment with prednisone (60 mg/day) Accepted for publication 20 February 1989. Correspondence to Dr Yoram Maaravi, Department of Medicine, was started. In the remainder of her hospital course Hadassah University Hospital, Mount Scopus, PO Box 24035, we noticed severe emotional lability, which was Jerusalem 91240, Israel. attributed to her basic disease and to the steroid 853 Ann Rheum Dis: first published as 10.1136/ard.48.10.853 on 1 October 1989. Downloaded from 854 Maaravi, Raz, Gilon, Rubinow treatment. During the ensuing 30 months her tion and anticardiolipin antibodies inpatients with corticosteroid treatment was tapered slowly and, SLE is rare. Recently, Asherson et all and others25 presently (January 1989), she is receiving 10 mg described myocardial infarction associated with prednisone daily and aspirin, and functions normally. anticardiolipin antibodies in young patients suffer- ing from SLE, 'lupus-like' disease, and the 'primary' antiphospholipid syndrome. These data suggest that Discussion anticardiolipin antibodies may have had a patho- genetic role in the ischaemic events in our patient as The patient described here meets five of.the revised well as in others. criteria for SLE6: malar rash, photosensitivity, arthritis, positive antinuclear antibody test, and We gratefully acknowledge the help of Mrs Shirley Roth in the an immunological disorder (positive VDRL). Her preparation of this manuscript. clinical course is remarkable in that despite relatively mild disease that did not require intensive treatment she developed two life threatening thrombotic References events in the brain and heart. 1 Harris E N, Gharavi A E, Boey M L, et al. Anticardiolipin Cardiac involvement in SLE is well recognised antibodies detection by radioimmunoassay and association with thrombosis in SLE. Lancet 1983; ii: 1211-4. and most frequently manifests as pericarditis, myo- 2 Harris E N, Loizou S, Englert H, et al. Anticardiolipin carditis, and endocarditis.7 8 Myocardial infarction antibodies and lupus anticoagulant. Lancet 1984; iii: 1099. during the course of SLE is rare and only a few cases 3 Harris E N, Gharavi A E, Loizou S, et al. Cross-reactivity of have been reported.>18 The pathogenesis of antiphospholipid antibodies. J Clin Lab Immunol 1985; 16: 1-6. 4 Harris E N, Asherson R A, Gharavi A E, Morgan S H, Derue myocardial infarction in SLE is usually related to G J, Hughes G R V. Thrombocytopenia in SLE and related coronary atherosclerosis, possibly accelerated by autoimmune disorders: association with anticardiolipin anti- corticosteroid administration.11 Furthermore, bodies. Br J Haematol 1985; 59: 227-30. in coronary arteritis is a rare process, which may 5 Derue G J, Englert H J, Harris E N, et al. Fetal loss SLE: association with anticardiolipin antibodies. J Obstet Gynecolcopyright. culminate in myocardial infarction in patients with Neotal Nurs 1985; 5: 207-9. SLE.10 Our patient's youth, the lack of risk factors 6 Tan E M, Cohen A S, Fries J S, et al. The 1982 revised criteria for coronary heart disease, and the previous for the classification of SLE. Arthritis Rheum 1982; 25: 1271-7. 7 Libman E, Sacks B. A hitherto undescribed form of valvular thrombotic event in the presence of anticardiolipin and mural endocarditis. Arch Intern Med 1924; 33: 701-32. antibodies support the contention that the myo- 8 Dubois E L, Tuffanelli D L. Clinical manifestations of SLE: cardial infarction was due to coronary artery throm- computer analysis of 520 cases. JAMA 1964; 190: 104-11. bosis. 9 Homcy C J, Liberthson R R, Fallon J T, Gross S, Miller L M. report of six Anticardiolipin antibodies are detected in a spec- Ischemic heart disease in SLE in the young patient: cases. Am J Cardiol 1982; 49: 478-84. trum of autoimmune disorders, and high serum 10 Badui E, Garcia-Rubi D, Robles E, et al. Cardiovascularhttp://ard.bmj.com/ concentrations have been measured in up to 61% of manifestations of SLE. Prospective study of 100 patients. patients with SLE.1 Harris et al provided data Angiology 1985; 36: 431-41. anticardio- 11 Bulkley B H, Roberts W C. The heart in SLE and the changes suggesting that lupus anticoagulant and induced in it by corticosteroid therapy: a study of 36 necropsy lipin antibodies are identical and show closely patients. Am J Med 1975; 58: 243-64. related specificity. Their presence is associated with 12 Tsakraklides V G, Blieden L C, Edwards J E. Coronary recurrent fetal loss, thrombocytopenia, and recur- atherosclerosis and myocardial infarction associated with sys- temic lupus erythematosus. Am Heart J 1974; 87: 637-41. rent thromboembolic events, which is attributed to Kawai C. Acute 13 Takatsu Y, Hattori R, Sakaguohi K, Yui Y, on October 1, 2021 by guest. Protected their inhibition of the prothrombinase complex.45 myocardial infarction associated with systemic lupus erythema- Among the thromboembolic events, cerebral in- tosus documented by coronary arteriograms. Chest 1985; 88: farction was frequent. 19 20 Eight out of nine patients 147-9. of 14 Shalev Y, Green L, Pollack A, Bentwich Z. Myocardial with cerebral infarction had hi2gh concentrations infarction with central retinal artery occlusion in a patient with anticardiolipin antibodies.1 19 20 In addition, Petri antinuclear antibody-negative systemic lupus erythematosus. et al found that 50% of patients with SLE and Arthritis Rheum 1985; 28: 1185-7. cerebral infarction had raised anticardiolipin anti- 15 Vermylen J, Blockmans D, Spitz B, Deckmyn H. Thrombosis and immune disorders. Clin Haematol 1986; 15: 393-412. body concentrations.21 16 Asherson R A, Harris E N, Gharavi A, Hughes G R V. Furthermore, anticardiolipin antibodies may be a Myocardial infarction in systemic lupus erythematosus and useful marker in monitoring disease activity in 'lupus like' disease. Arthritis Rheum 1986; 29: 1292-3. infarction patients with SLE.22 23 It is noteworthy that recur- 17 Asherson R A, Mackay I R, Harris E N. Myocardial man vein women with in a young with systemic lupus erythematosus, deep rent fetal loss in anticardiolipin thrombosis and antibodies to phospholipid. Br HeartJ 1986; 56: antibodies/lupus anticoagulant can be reduced signi- 190-3. ficantly by the use of steroids and aspirin.24 18 Asherson R A, Kamashta M A, Baguley E, Oakley C H, The association between acute myocardial infarc- Rowell N R, Hughes G R V. Myocardial infarction and Ann Rheum Dis: first published as 10.1136/ard.48.10.853 on 1 October 1989. Downloaded from Major thrombotic events in a young woman with SLE 855 antiphospholipid antibodies in SLE, 'lupus-like' disease and the 22 Raz E, Michaeli J, Rosenmann E, Rubinow A, Popovtzer 'primary' antiphospholipid syndrome.