Sessa A, Meroni M, Gattini G (eds): Systemic Lupus erythematosus: Renal Vasculitis. Contriti Nephrol. Basel, Karger, 1992, vol 99, pp 99-101

Two Cases of Juvenile Systemic Lupus erythematosus with Life-Threatening Central Nervous System Involvement: Striking Association with Antiphospholipid Antibodies Rolando Cimaz, Elisa Cohen, Fabrizia Corona, Carlo Bonfanti, Rosario Star tari, Maria Bardare

Antiphospholipid (aPi) antibodies are autoantibodies directed against different phospholipids that can be found in sera of patients affected with connective tissue diseases. They can be detected with different tests, including serologic tests for syphilis (VDRL, Wassermann), tests for the circulating lupus-like anticoagulant (kaolin clotting time ratio, dilute Russel viper venom time, activated partial thromboplastin time, etc.), and solid-phase radíoimmunoassags (RIA) or enzyme-linked immunosorbent assays (ELISA) using cardiolipin or other negatively charged phospholipids as antigens. However, there is a lack of concordance between the results of these tests, because they detect overlapping populations of antibodies. Moreover, aPl cross-react to a certain degree with anti-dsDNA, due to a biochemical similarity (common phosphodiester-linked phosphate groups separated by carbon atoms) [1]. Figure 1 shows the possible interrelationships between antibodies to phospholipids, anti-dsDNA, lupus anticoagulant and VDRL tests. Several studies have suggested an association between specific aPl and the presence of certain clinical features, particularly recurrent venous and/or arterial thromboses, thrombocytopenia, and recurrent fetal loss [2]. aPl antibodies are detected in different connective tissue diseases, but mainly in systemic lupus erythematosus (SLΕ), in which aPI positivity varies from 21 to 81 % in different series [ 3]. Although studies in the pediatric age are very few, there might be a greater frequency of aPl-positive SLΕ patients in children than in adults, although no correlation with clinical symptoms has yet been found [4, 5].

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1st Pediatric Clinic, Center of Rheumatology, University of Milan, Italy

Cimaz et al.

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Fig. 1. Overlapping between different autoantibodies.

Materials and Methods In our Center of Pediatric Rheumatology, we have been studying aPI prevalence in our SLE patients since 1990, and we found 2 positive cases (out of 8 tested). aPl antibodies were measured by an enzyme immunoassay (Asserachrom APA, Diagnostica Stago, France), using a mixture of cardiolipin and different types of phospholipids as antigens. The interpretation of test results was made with a calibration curve, using log-log graph paper to plot aPl concentration on the x-axes and corresponding optical density values on the ordinates. Results have been classified in the following range: normal 100 PL.

Case 2 D.L., female, born March 1979. Disease onset in 1988, with fever, spleen and liver enlargement, anemia, followed after several months by

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Case 1 S.L., female, born January 1985. Disease onset in 1989, with fever, skin rash, proteinuria and hematuría. Steroid treatment did not work as expected. March 1990: The girl complained of long-lasting right eye pain, followed after 3 weeks by generalized seizures, with prolonged apneas and subsequent coma; she was then transferred to the Intensive Care Unit, where artificial ventilation and antiepileptic treatment were started, with resolution of coma in 6 days. Amaurosis and subsequent visual hallucinations followed with complete resolution after a total of 21 days. EEG showed slow diffuse activity, with theta-delta waves and frontotemporal spikes; brain CT and MRI scans showed enlargement of the ventricular spaces and scissures. These results were compatible with diffuse cerebritis, possibly due to vaso-occlusive disorders. Serological tests gave positivity of anti-dsDNA and aPl (17.5). Low-dose aspirin was added to steroids, with resolution of neurological symptoms, and decrease of aPl levels. A 12-month follow-up shows a favorable course, with the patient in good clinical conditions and no disease flare-ups.

Juvenile SLΕ with Life-threatening Central Nervous System Involvement

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butterfly rash and knees arthritis. Steroid treatment was partially successful. When aged 10 years, the girl had lower limb paralysis and neurological bladder, with spontaneous recovery in a few days (psychogenic symptoms? vascular involvement with transverse myelitis?). Spine IRI was normal, and lupus anticoagulant was positive. August 1990: The patient started showing speech difficulties, diminished strength and motility of the right limbs, peripheral right facial nerve paralysis, and choreoatetotic movements of the right arm. EEG and brain IRI were normal; lupus anticoagulant was positive (KCTR = 1.4, normal

Two cases of juvenile systemic lupus erythematosus with life-threatening central nervous system involvement: striking association with antiphospholipid antibodies.

Sessa A, Meroni M, Gattini G (eds): Systemic Lupus erythematosus: Renal Vasculitis. Contriti Nephrol. Basel, Karger, 1992, vol 99, pp 99-101 Two Case...
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