Systemic lupus erythematosus.
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Biomedical subjects
Publications and source records attributed to David P D'Cruz.
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Autoimmune connective tissue diseases are common conditions that often present non-specifically. Clinicians in emergency departments and acute medical assessment units may face these patients. This article provides an overview for the non-specialist clinician on the acute manifestations that may occur in these patients.
Antiphospholipid antibodies (aPL) and antiphospholipid syndrome (APS) are increasingly being recognized in children. Transient non-pathogenic aPL are often seen after childhood infections, while thrombotic events seem rare in those with true aPL. We discuss the main scenarios faced when dealing with children with aPL--asymtomatic aPL, primary APS and secondary APS. Children with thrombotic events present difficult management problems, as there is little evidence-based medicine in this area. We discuss the manifestations and management of childhood aPL--asymptomatic aPL, primary and secondary APS elucidated with case histories. Insufficient safety data on anticoagulation and limited information on the effects of warfarin, use of aspirin, duration and intensity of anticoagulation are some of the unresolved issues in managing aPL and APS in children. Multicenter randomized controlled trials may provide answers to some of these issues.
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OBJECTIVE: To describe the prevalence and clinical associations of abnormalities on electroencephalography (EEG) in patients with antiphospholipid syndrome (APS) and/or systemic lupus erythematosus (SLE) who have neuropsychiatric symptoms. METHODS: The study group comprised 57 patients (age </=50 years) with manifestations of neuropsychiatric illness. Patients with stroke, epilepsy, or other encephalopathies were excluded. Fourteen patients had APS, 24 patients were positive for antiphospholipid antibodies (aPL), and 19 patients had SLE without aPL. All patients underwent EEG and brain magnetic resonance imaging (MRI) while they were awake. RESULTS: Abnormal EEG findings (showing mainly bitemporal slow activity) were recorded in 37 of 57 patients (65%). Abnormal EEG findings were observed in all patients with APS and in 17 of 24 aPL-positive patients (71%), compared with 6 of 19 patients with SLE (32%) (P < 0.001 and P < 0.05, respectively). There was an association between abnormal EEG findings and the frequency of aPL positivity (at least 2 positive results) (P = 0.002). Three EEG recordings showed potentially epileptiform activity. Results of brain MRI were abnormal in 18 (31.6%) of 57 patients: 8 in the APS group (57.1%), 7 in the aPL-positive group (29.2%), and 3 in the SLE group (15.8%). None of the patients with normal EEG findings had abnormal MRI results. Thus, brain MRI findings were normal in the majority of patients. Patients with abnormal EEG results were more likely to report memory problems (P < 0.001). CONCLUSION: Our findings suggest that EEG abnormalities are common and correlate with the presence of aPL even in the absence of brain abnormalities on MRI. EEG should be considered in aPL-positive patients with neuropsychiatric symptoms, because use of antiaggregants or anticoagulation may need to be considered.
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OBJECTIVE: To assess the indications, efficacy, and tolerability of mycophenolate mofetil (MMF) in patients with systemic lupus erythematosus (SLE) resistant to other immunosuppressive therapy. METHODS: Records of 93 patients with SLE were retrospectively reviewed. Seven patients were excluded. The remaining 86 patients received other immunosuppressive drugs before MMF. Efficacy was measured by changes in daily oral prednisolone dose, European Consensus Lupus Activity Measurement Index (ECLAM), erythrocyte sedimentation rate (ESR), C-reactive protein, and dsDNA antibody titer. In renal patients, changes in serum creatinine, creatinine clearance, chromium-51 EDTA glomerular filtration rate (EDTA-GFR), and 24 hour urine protein excretion were also evaluated. RESULTS: Indications for MMF were mainly renal involvement (59% of patients), uncontrolled disease activity (14%), and other SLE related manifestations (13%). Overall, we found a significant reduction in the steroid dosage, ECLAM, ESR, and anti-dsDNA antibody titer. Renal patients (n = 35) showed a significant reduction in urinary 24 hour protein excretion. Levels of serum creatinine, creatinine clearance, and EDTA-GFR showed no significant change during treatment. Thirty-seven patients (42.8%) developed adverse events. Gastrointestinal intolerance in 25 (29%) and infections in 20 (23.2%) were the most frequent. The drug was discontinued in 14 (16.3%) patients due to side effects and 6 patients discontinued MMF because they achieved disease remission and were trying to conceive. MMF was stopped due to lack of efficacy in 12 patients. CONCLUSION: Our data suggest that MMF is a good therapeutic alternative for patients with SLE and renal involvement or refractory disease activity.
OBJECTIVE: Transverse myelitis (TM) is a rare complication of systemic lupus erythematosus (SLE). Although usually a late manifestation of SLE, it can occur at presentation. We investigated the clinical presentation, treatment and outcome of 15 patients with TM as the presenting manifestation of SLE or lupus-like disease. METHODS: All patients received corticosteroids, while 13 also received immunosuppressive therapy. Five patients were fully anticoagulated with warfarin. RESULTS: A sensory level with spastic lower limb weakness and sphincter disturbance was the most common presentation: 14/15 patients had a thoracic or cervical sensory level. Cerebrospinal fluid examination showed high protein concentrations in 3 patients and oligoclonal bands in 8. Eleven of the 15 (73%) had antiphospholipid antibodies (aPL). Of the 15 patients, 3 had complete resolution of the symptoms, 6 had good functional improvements, 5 had good to fair outcome with some functional deficit, and one patient who received corticosteroids alone later died from pneumonia. CONCLUSION: We describe 15 patients with TM as the presenting manifestation of SLE or lupus-like disease with a high prevalence of aPL. Our data support the view that early diagnosis and immunosuppressive therapy may be superior to corticosteroids alone in improving functional outcome. In those patients with aPL, antiplatelet agents and/or warfarin should also be considered.