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Biomedical subjects

M Abu-Shakra

Publications and source records attributed to M Abu-Shakra.

At least 55 records · Page 3Linked to original sources

Malignancy in systemic lupus erythematosus.

OBJECTIVE: To estimate the risk of cancer in patients with systemic lupus erythematosus (SLE). METHODS: Patients with SLE (n = 724) have been followed prospectively, for 24 years, at the University of Toronto Lupus Clinic. The diagnosis of cancer was confirmed by histologic or autopsy reports. Standardized rates of cancer and standardized incidence rates (SIR) (ratio of observed-to-expected cancers) were used to estimate the risk for cancers. RESULTS: Twenty-four cancers were identified in 23 SLE patients (3.2%) during 7,233 patient-years of followup. Compared with the Ontario population, the overall estimated risk for all cancers was not increased in the lupus cohort (SIR 1.08, 95% confidence interval 0.70-1.62). A 4.1-fold increased risk for hematologic cancers was observed, due mainly to an increased risk of non-Hodgkin's lymphoma. The risk for cancer was significantly lower in the SLE cohort compared with patients with rheumatoid arthritis (RA) and systemic sclerosis (SSc). CONCLUSION: SLE is associated with a lower risk of all cancers compared with RA and SSc, but an increased risk for non-Hodgkin's lymphoma compared with the general population.

Adolescent↗

Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients.

A retrospective chart review was carried out on 344 patients with systemic sclerosis (SSc) followed prospectively for the occurrence of pulmonary hypertension (PHT). Seventeen patients (4.9%) were found to have PHT. Eight patients had isolated PHT, while in nine PHT was associated with restrictive lung disease (RLD). The subset with RLD developed PHT earlier, but had longer survival than patients with isolated PHT. Patients with limited scleroderma tend to develop isolated PHT, while in those with diffuse disease PHT is associated with RLD. Irrespective of disease type, PHT in SSc has an extremely poor prognosis with a median survival of 12 months following diagnosis.

Adult↗

The significance of anticardiolipin antibodies in patients with lupus nephritis.

The objective of this study was to determine whether anticardiolipin antibodies (ACL) in SLE patients are associated with a specific pattern of lupus nephritis and/or with renal microvascular changes. Patients with SLE, followed prospectively between June 1991-May 1994 at The Wellesley Hospital Lupus Clinic, who underwent a renal biopsy were included. The ACL was measured by the ELISA according to international standardized method. Renal biopsy morphology was assessed using the WHO criteria for the classification of lupus nephritis. Renal vascular changes included glomerular hyaline thrombi, intimal fibrosis and intraluminal thrombi of renal arterioles. There were 23 SLE patients. The mean age at diagnosis of SLE was 28.2 years and the mean disease duration was 6.3 years. Of these 10 (43%) had high levels of ACL. No difference in the frequency of severe nephritis (Class III and IV) was identified amongst patients with and without ACL. Mesangial nephritis was more common in patients with ACL 40% vs 0, p = 0.02). Glomerular hyaline thrombi occurred in 3 (13%) patients. None of them had positive ACL. Renal vascular lesions included intimal proliferation in 4 (ACL + , 1) occluded lumens by thrombi in 2 (ACL + 1). Our data indicate that the development of glomerular and/or microvascular changes is not related to the presence of ACL.

Adolescent↗

Superior sagittal sinus thrombosis in a patient with systemic lupus erythematosus.

Cerebral venous thrombosis is a rare cause of focal neurological damage in patients with SLE. We describe a patient with SLE who presented with a focal neurological deficit and subsequently developed increased intracranial pressure. A superior sagittal sinus thrombosis was diagnosed by MRI. The association between SLE and superior sagittal sinus thrombosis is discussed. Early diagnosis is essential because treatment leads to a positive clinical outcome.

Adult↗

Anticardiolipin antibodies in systemic lupus erythematosus: clinical and laboratory correlations.

PURPOSE: To examine the link between anticardiolipin antibodies and the features of antiphospholipid syndrome in patients with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: In this prospective cohort study conducted in a single center, 390 SLE patients were followed up between June 1991 and 1994. At each assessment, a complete history, physical examination, and laboratory evaluation (including measurement of anticardiolipin antibodies) were performed according to a standard protocol. RESULTS: Forty-seven percent of the patients had an elevated level of anticardiolipin antibodies. In the univariate analysis, elevated anticardiolipin antibody levels were found to correlate with thrombocytopenia (P = 0.006), prolonged activated partial thromboplastin time (aPTT) (P = 0.003), and positive direct and indirect Coombs' test result's (P < 0.001). No correlation was identified with any of the clinical features of antiphospholipid syndrome. In the multivariate analysis, anticardiolipin antibodies remained highly associated with thrombocytopenia (odds ratio [OR] 4.05, P = 0.02), positive direct Coombs' test (OR 2.31, P < 0.001), and prolonged aPTT (OR 1.73, P = 0.03). In the multivariate model using venous/arterial thrombosis as the outcome variable, only prolonged aPTT was associated with venous/arterial thrombosis (OR 7.9, P < 0.001). None of the laboratory variables were found to correlate with fetal loss. CONCLUSIONS: The presence of anticardiolipin antibodies in patients with SLE is associated with prolonged aPTT, thrombocytopenia, and positive Coombs' test result, but not with antiphospholipid syndrome. Prolonged aPTT is strongly associated with venous/arterial thrombosis.

Adolescent↗

Mortality studies in systemic lupus erythematosus. Results from a single center. I. Causes of death.

OBJECTIVE: To study the causes of death in patients with SLE, followed prospectively in a single center. METHODS: The study population comprised 665 patients with systemic lupus erythematosus (SLE). Causes of death were determined by review of hospital files, autopsy reports, and death certificates. Nonparametric lifetable models were used to calculate Kaplan-Meier estimates of survival probabilities. RESULTS: One hundred and twenty-four patients (18.6%) had died. The primary causes of death were active SLE in 20 (16%), infection in 40 (32%), acute vascular event in 19 (15.4%), sudden death in 10 (8.1%), organ failure in 6 (4.8%), malignancy in 8 (6.5%), others in 8 (6.5%), and unknown in 13 (10.5%). Death as a result of active SLE was more common in patients who died within 5 years of diagnosis compared to those dying after 5 years (p = 0.021), and deaths due to vascular events and end organ failure not related to active lupus were more frequent in the late death group (p = 0.028). The overall 5, 10, 15, and 20 year survival rates were 93, 85, 79, and 68%, respectively. Patients with SLE had a 4.92 fold increased risk for death compared with the general population. CONCLUSION: Survival rates continue to improve in SLE but causes of mortality vary at different stages.

Adolescent↗

Mortality studies in systemic lupus erythematosus. Results from a single center. II. Predictor variables for mortality.

OBJECTIVE: To analyze the factors associated with mortality in patients with systemic lupus erythematosus (SLE), followed prospectively in a single center. METHODS: The study included 665 patients with SLE followed over a 20-year period according to a standard protocol. Clinical laboratory information has been entered into a database. Univariate analysis was carried out to identify prognostic factors of death. The Cox proportional hazard regression model was used to estimate risk ratio of death. RESULTS: Renal damage, thrombocytopenia, lung involvement, systemic lupus erythematosus disease activity index (SLEDAI) > or = 20 at presentation, and age > or = 50 at diagnosis were predictive factors for mortality in the univariate as well as in the multivariate analyses. Hypertension and ischemic heart disease were significantly associated with death only in the univariate analysis. CONCLUSION: Renal damage, thrombocytopenia, SLEDAI > or = 20 at presentation, lung involvement, and age > or = 50 at diagnosis are prognostic factors associated with mortality.

Adolescent↗

Pericardial effusion and vasculitis in a patient with systemic sclerosis.

We describe a case of diffuse scleroderma with a large pericardial effusion, pleural effusions, and subsequent oliguric renal failure. Histology of the pericardium and pleura revealed the presence of leukocytoclastic vasculitis. Pleuropericarditis in systemic sclerosis may occur on the basis of vasculitis. Large pericardial effusions may predispose to subsequent renal failure.

Female↗

Longterm methotrexate therapy in psoriatic arthritis: clinical and radiological outcome.

OBJECTIVE: To determine whether methotrexate (MTX) therapy for 24 months prevents progression of radiographic damage in psoriatic arthritis (PsA). METHODS: Patients who were given MTX during their attendance at the psoriatic arthritis clinic were enrolled in the study. Patients who had never had MTX and who were matched by damage, actively inflamed joints, sex, and disease duration were identified from the PsA database as controls. The outcome measure was increase in the number of damaged joints. RESULTS: The study population comprised 38 patients (16 F, 22 M) with a mean age of 44.6 years and disease duration of 11.4 years. Twenty-three patients continued therapy for 24 months. Clinical evaluation revealed that 45% of the patients had > or = 40% improvement in actively inflamed joint count at 6 and 24 months. Radiographs were available for 19 of the 23 patients who took MTX for 24 months, and they were compared to their respective controls. Radiographic damage scores at 24 months showed an increase in the damage score in 63% of the patients. Compared to the matched controls, there was no statistically significant difference in the progression in damage. CONCLUSION: Our results suggest that compared to other regimens, MTX conferred no advantage with respect to clinical response or longterm damage even after 24 months of therapy.

Adult↗

Mortality in systemic sclerosis: a comparison with the general population.

OBJECTIVE: To compare the mortality rate in patients with systemic sclerosis (SSc) with that of the general population. METHODS: Standardized mortality ratios (SMR) were calculated for 237 patients with SSc followed prospectively, using age and sex specific mortality rates in Ontario for the period 1976-1990. RESULTS: The overall SMR for the SSc cohort was 4.69. The mortality rate was greater with diffuse than with limited scleroderma (SMR 6.18 and 3.80, respectively), but not different between men and women. CONCLUSION: The mortality rate in SSc is increased compared to that of the general population, especially in the subset with diffuse disease.

Adult↗

Outcome of polyarteritis nodosa and Churg-Strauss syndrome. An analysis of twenty-five patients.

OBJECTIVE: To compare outcome in patients with polyarteritis nodosa (PAN) and patients with Churg-Strauss syndrome (CSS) followed up at a single center. METHODS: A retrospective data review of 13 patients with PAN and 12 patients with CSS who were followed up at a vasculitis clinic. Outcome measures included a global damage index, disability and pain dimensions of the Health Assessment Questionnaire, and mortality rate. RESULTS: Compared with patients with CSS, patients with PAN had a significantly higher mean damage index score (5.15 versus 2.42; P = 0.011), a higher disability score (1.09 versus 0.16; P = 0.007), and a higher pain score (1.04 versus 0.01; P = 0.017). Patients with PAN had more relapses (relative risk = 5.07; P < 0.000) and a higher mortality rate (31%) compared with patients with CSS (8%). CONCLUSION: PAN and CSS differ in their morbidity and mortality; therefore, they should be considered as distinct clinical entities.

Adult↗

Gastrointestinal manifestations of systemic sclerosis.

Gastrointestinal (GI) manifestations of systemic sclerosis (SSc) were found in 82% of 262 patients followed up prospectively. Esophageal dysmotility, lower esophageal sphincter laxity, bacterial overgrowth, and wide mouth diverticuli were the most common findings. The disease is usually diffuse with multiple levels of involvement. Gastrointestinal involvement was not significantly correlated with gender, age at SSc diagnosis or disease type (limited or diffuse scleroderma). Upper GI symptoms develop early in the course of SSc and may not correlate with objective findings. Various investigations, treatment regimens, and less frequent disease manifestations are reviewed and discussed.

Adult↗

Primary angiitis of the CNS diagnosed by angiography.

Sixteen patients (8 female, 8 male) with primary angiitis of the CNS (PACNS), were followed prospectively in a vasculitis clinic. Diagnosis was by angiography in patients without underlying disease. Median age at diagnosis was 36.5 years, and median duration of follow-up was 28 months. Onset was acute in 14 patients (88%), with 3.5 weeks (median) from onset symptoms to diagnosis. Three women developed symptoms within 3 weeks postpartum. The most frequent symptoms were severe headaches (12, 75%), stroke (6, 30%), transient ischaemic attack (TIA) (4, 28%), seizures (7, 44%), visual aberration (3, 19%), and cognitive impairment (5, 31%). Laboratory data included high ESR (2, 13%), leucocytosis (8, 80%), thrombocytosis (1, 6%), positive antinuclear antibody titre (3, 15%), and high levels of complement (5, 31%). Lumbar puncture was performed in 12 patients (75%). CSF analysis was abnormal in five patients (42%). EEG was abnormal in 5/9 patients. The major CT/MRI scan findings were cerebral haemorrhage (4, 25%), brain infarcts (5, 31%), brain atrophy (2, 13%) and non-specific lesions (2, 13%). Four patients had normal studies. All patients received corticosteroids (CS), and five were treated with oral cyclophosphamide. Two patients relapsed despite CS and cyclophosphamide therapy. All patients are alive, and at the last assessment, eight had a permanent neurological deficit, which included paresis (3, 19%), neurocognitive abnormalities (2, 13%), visual loss (2, 13%) and seizure activity (5, 31%). Our data suggest a non-progressive, non-fatal course in those PACNS patients diagnosed angiographically and treated with CS with or without cyclophosphamide.

Adult↗

Accelerated nodulosis, pleural effusion, and pericardial tamponade during methotrexate therapy.

We describe a patient with seropositive erosive rheumatoid arthritis (RA) who developed accelerated nodulosis, pleural effusion, and pericardial tamponade during methotrexate (MTX) therapy while her arthritis remained inactive. MTX is an effective therapy for the articular manifestations of RA. However, on occasion it may result in triggering the development of extraarticular manifestations of RA.

Arthritis, Rheumatoid↗

Cancer in systemic sclerosis.

OBJECTIVE: To determine whether the frequency of cancer is increased among patients with systemic sclerosis (SSc). METHODS: A retrospective chart review of 248 patients who were followed up prospectively was conducted. RESULTS: Cancers developed in 18 patients (7.3%) during 2,001 patient-years at risk. The most frequent types were cancers of the lung (7 patients) and breast (5 patients). Older age at diagnosis of SSc was a significant risk factor for cancer. Lung cancer was associated with the presence of pulmonary fibrosis. The age-standardized incidence rate for all cancers (7.9/1,000) was 2.1 times the overall rate in the Ontario population (P < 0.0001). CONCLUSION: The frequency of cancer is increased in patients with SSc.

Adult↗

Exaggerated fibrosis in patients with systemic sclerosis (scleroderma) following radiation therapy.

We describe 2 patients with systemic sclerosis (scleroderma) who developed severe localized skin thickening following radiation therapy for cancer. Radiation therapy may result in intense localized areas of fibrosis in patients with scleroderma. The fibrosis may extend beyond the irradiation portals and result in damage to underlying viscera. Radiotherapy may precipitate scleroderma or cause a generalized worsening of the underlying disease.

Adult↗