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Home , Hemiparesis, Tetraplegia

Ann Rheum Dis: first published as 10.1136/ard.44.7.491 on 1 July 1985. Downloaded from

Annals of the Rheumatic Diseases, 1985, 44, 491-493

Case report as a presenting feature of systemic lupus erythematosus complicated by pulmonary

R J HARDIE1 AND D A ISENBERG2 From the 'Department of Medicine and 2Department of Rheumatology, University College Hospital, Gower St, London WCIE 6AU

SUMMARY A 28-year-old woman presenting with subacute onset of a tetraplegia is described who was shown to have active systemic lupus erythematosus in association with high circulating anticardiolipin binding and lupus anticoagulant activity. The patient later developed severe symptomatic systemic and pulmonary hypertension and required emergency resuscitation. This case provides further support for an association between antiphospholipid antibodies and the clinical features of central nervous system (CNS) involvement and pulmonary hypertension in SLE. copyright. Key words: CNS lupus, antiphospholipid antibodies, pulmonary hypertension.

Involvement of the central nervous system in ing week. The gradually improved, and systemic lupus erythematosus (SLE) may be a major she was discharged after a further two weeks. http://ard.bmj.com/ problem in terms of management and prognosis, In July 1980 she sought the opinion of a London though it is very seldom a presenting feature of the neurologist, who found a marked spastic left hemi- condition. An association has been described be- with involvement of the face on the left side tween the 'lupus anticoagulant', circulating anti- together with pathological right-sided bodies directed against phospholipid antigens, parti- and bilateral extensor plantar responses. There was cularly cardiolipin, and recurrent arterial and no other cranial nerve abnormality and no sensory venous thromboses including cerebral thromboses.' deficit. A computerised tomographic scan of the on September 24, 2021 by guest. Protected In view of the recent report of a further possible was normal, and a tentative diagnosis of association of lupus anticoagulant activity and pul- in 1978 was made. She returned to monary hypertension2 we describe a patient who Egypt walking unaided but with a spastic ; there presented with a tetraplegia and was found to have was no functional in her upper limbs. active SLE with high anticardiolipin binding and In August 1984 while visiting relatives in London lupus anticoagulant activity, who developed pul- she developed fever, generalised weakness, and monary hypertension. over a few days. Examination revealed an asymmetrical spastic tetraparesis, more Case report on the left than the right, with bilateral sustained ankle and extensor plantars. Cranial nerves A 28-year-old Egyptian female was admitted to were normal, and there was no sensory deficit. A hospital in Alexandria in August 1978 with a fever of fever of 38-4°C, sinus tachycardia of 120 per minute, 38°C and a left hemiparesis which developed over a blood pressure 150/120 mmHg, and a mid-systolic few hours and reached a maximum over the follow- murmur at the cardiac apex were noted. Patchy Accepted for publication 28 January 1985. discoloration of the skin over both calves was also Correspondence to Dr D A Isenberg. present. 491 Ann Rheum Dis: first published as 10.1136/ard.44.7.491 on 1 July 1985. Downloaded from

492 Hardie, Isenberg

Investigations on admission included: Hb 10-1 (mmHg): mean central venous pressure 1, right g/dl; leucocytes 6-0x 109/l; platelets 276x 109/l; ventricle 60/7, pulmonary artery 60/23, mean pul- erythrocyte sedimentation rate 130 mm/h; monary artery wedge pressure 6. Diagnoses were prothrombin ratio 14/11 s; partial thromboplastin- made of 'functional' tricuspid and mitral regurgita- kaolin time 50 s with a control time of 30 s, which tion secondary to increased vascular resistance. did not correct on mixing with normal plasma The hypertension was subsequently well control- (indicating that a lupus anticoagulant was present); led with nifedipine, though proteinuria had in- thrombin time 13/11-5 s; blood cultures negative; creased to 6-5 g/day, and azathioprine was added to urea 3-2 mmol/l; creatinine 58 Rmol/l; albumin 31 the steroid therapy. There has been no significant g/l; globulin 47 g/l with raised IgG 24.8 g/l (normal recovery of the neurological deficit three months 8-18 g/l), normal IgA and IgM, no monoclonal after its onset. band; normal liver enzymes and thyroxine; 24-hour urinary protein excretion was 0-22 g, 0-54 g, 1-80 g, Discussion 6-48 g on consecutive weekly collections. Positive antinuclear antibody (ANA) titre 1/2560, Neuropsychiatric manifestations of systemic lupus homogeneous pattern (rat liver substrate); positive are common, reported in 37%4 and 59%5 of two immunofluorescence on human epithelial cells large series. The commonest features are psychiatric (HEp2) > 1:640; positive immunofluorescent anti- illness and , but about 10% of patients may body to double-stranded deoxyribonucleic acid 1:40 have evidence of pyramidal tract (usually with Crithidia luciliae; anti-DNA assay 141 U/ml attributed to a 'cerebrovascular accident'), and rare (normal <25 U/ml: Amersham kit - Amersham cases of transverse myelopathy have been International,, Bucks.); negative direct Coombs' described.6 7 However, neurological presentation is test, rapid plasma reagin test and Treponema very rare.8 In one series5 3% of patients with SLE pallidum haemagglutination test; serum C-reactive presented with seizures, but all other neuropsychi-

protein 189 mg/l (normal <12 mg/l); polyethylene atric features developed later in the course of theircopyright. glycol precipitable immune complexes 50-80 disease. Fulford et al.9 reported five women with mg/l (normal <100 mg/l; anticardiolipin antibodies slowly progressive spastic resembling measured by ELISA assay:3 IgM 66% (normal , in whom weakly positive auto- <15), IgG 8.3% (normal <5). nuclear antibodies were detected. They proposed Cervical myelography was normal, with cerebro- the term 'lupoid sclerosis' and also reviewed the few spinal fluid (CSF) protein 1.0 g/l. Computerised other published accounts of SLE patients with brain scan tomographic showed dilation of the neurological disturbance at presentation. http://ard.bmj.com/ ventricular system. Echocardiography showed left In our patient the first manifestation of SLE may ventricular hypertrophy without evidence of valvu- have been the initial left hemiparesis in 1978, and by lar vegetations. 1980 bilateral pyramidal tract lesions were present The patient was treated with bolus methylpredni- without any evidence of haemorrhage or infarct on solone therapy, 1 g per day for two days, and then computerised tomography (CT) of the brain. A oral prednisolone 40 mgiday. The hypertension spastic tetraplegia of subacute onset later developed initially settled without specific treatment. Over the with unequivocal active SLE, as shown by the next three weeks the platelet count gradually accompanying positive ANA, strongly positive anti- on September 24, 2021 by guest. Protected dropped to 45 x 109/l and then recovered to the DNA antibodies, proteinuria, and thrombocy- initial level at presentation. Four weeks after topenia. The CSF protein concentration was also admission she became acutely unwell with clinical raised, though cervical myelography and repeat CT evidence of poor cardiac output, tricuspid regurgita- brain scan failed to show a structural . tion, systemic and pulmonary hypertension, but The pathogenesis of cerebral and neither pulmonary nor peripheral oedema. Aus- lesions is uncertain and probably multifactorial. cultation revealed a gallop rhythm, accentuated Vasculitis, non-inflammatory microvascular lesions, pulmonary second heart sound, and a loud systolic and brain-reactive antibodies have all been murmur radiating to the axilla. At one stage the invoked.10 More recently an association of central blood pressure rose to 190/150 mmHg and required nervous system involvement with multiple cerebral, treatment with intravenous diazoxide, when the deep venous, and other thromboses has been mean central venous pressure was 25 cm of water recognised in those patients who possess a circulat- (20 mmHg). Chest x-ray showed an enlarged heart ing antibody known as the 'lupus anticoagulant'.1' but clear lung fields. About an hour later when the The lupus anticoagulant, found in up to one blood pressure had fallen to 150/100 mmHg, a quarter of patients with SLE, prolongs the partial Swan-Ganz catheter gave the following readings thromboplastin-kaolin time in vitro, yet paradox- Ann Rheum Dis: first published as 10.1136/ard.44.7.491 on 1 July 1985. Downloaded from

Tetraplegia as a presenting feature of systemic lupus erythematosus 493 ically is associated with a hypercoagulable state and this case, and Dr B Colaco of the Middlesex Hospital for measuring with an increased risk of thrombocytopenia and anticardiolipin antibody levels. spontaneous abortion. Furthermore, pulmonary References hypertension has recently been described in six SLE 1 Hughes G R V. Thrombosis, abortion, cerebral disease, and the patients without parenchymal lung disease or overt lupus anticoagulant. Br Med J 1983; 287: 1088-9. pulmonary embolism, five of whom possessed lupus 2 Asherson R A, Mackworth-Young C G, Boey M L, et al. Pulmonary hypertension in systemic lupus erythematosus. Br anticoagulant activity, and intrapulmonary micro- Med J 1983; 287: 1024-5. thrombosis has been postulated. 3 Colaco C B, Male D. Anti-phospholipid antibodies in syphilis It has been known for many years that those and a thrombotic subset of SLE: distinct profiles of epitope patients with biological false positive Wassermann specificity. Clin Exp Immunol 1985; 59: 449-56. 4 Feinglass E J, Arnett F C, Dorsch C A, et al. Neuropsychiatric reactions had a high incidence of the lupus anti- manifestations of systemic lupus erythematosus: diagnosis, coagulant. It is now believed that the former clinical spectrum, and relationship to other features of the phenomenon is due to the presence of a circulating disease. Medicine (Baltimore) 1976; 55: 323-39. antibody directed against cardiolipin, a phospho- 5 Estes D, Christian C L. The natural history of systemic lupus erythematosus by prospective analysis. Medicine (Baltimore) lipid constituent of cell membranes, and that the 1971; 50: 85-95. lupus anticoagulant is also an antiphospholipid 6 Andrianakos A A, Duffy J, Suzuki M, Sharp J T. Transverse antibody. Harris and colleagues12 have shown a myelopathy in systemic lupus erythematosus. Ann Intern Med significant correlation between the degree of in- 1975; 83: 616-24. 7 Warren R W, Kredich D W. and acute crease of IgG anticardiolipin antibody level and the central nervous system manifestations of systemic lupus erythe- degree of positivity of the lupus anticoagulant. High matosus. Arthritis Rheum 1984; 27: 1058-60. titres of anticardiolipin antibodies are apparently 8 Silverstein A. Cerebrovascular accidents as the initial major also associated with venous and cerebral manifestation of lupus erythematosus. NY State J Med 1963; 63: 2942-8. thromboses. 13 9 Fulford K W M, Catterall R D, Delhanty J J, et al. A collagen The mechanism of CNS lesions in SLE may then disorder of the nervous system presenting as multiple sclerosis.

be linked with the presence of this family of Brain 1972; 95: 373-86. copyright. antibodies. In addition to causing disturbances of 10 Bernstein R M. Cerebral lupus: a peep into Pandora's box. J Rheumatol 1982; 9: 817-8. microcirculation it is possible that these antibodies 11 Boey M L, Colaco C B, Gharavi A E, et al. Thrombosis in may cross react with complex brain phospholipids systemic lupus erythematosus: striking association with the such as sphingomyelin. The present case provides presence of circulating lupus anticoagulant. Br Med J 1983; 287: further support for an association between these 1021-3. 12 Harris E N, Loizou S, Englert H, et al. Anticardiolipin antilipid antibodies and the clinical features of CNS antibodies and lupus anticoagulant. Lancet 1984; ii: 1099. involvement and pulmonary hypertension in SLE. 13 Harris E N, Gharavi A E, Boey M L, et al. Anticardiolipin http://ard.bmj.com/ antibodies: detection by radioimmunoassay and association with thrombosis in systemic lupus erythematosus. Lancet 1983; We would like to thank Dr D P Brenton for permission to report ii: 1211-4. on September 24, 2021 by guest. Protected