Eyal Muscal

Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study

To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegeners) (GPA).

Comparing presenting clinical features of 48 children with microscopic polyangiitis (MPA) against 183 having granulomatosis with polyangiitis (GPA). An ARChiVe study

To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegeners) (GPA).

MR imaging findings suggestive of posterior reversible encephalopathy syndrome in adolescents with systemic lupus erythematosus

BackgroundEndothelial damage, hypertension and cytotoxic medications may serve as risk factors for the posterior reversible encephalopathy syndrome (PRES) in systemic lupus erythematosus. There have been few case reports of these findings in pediatric lupus patients.ObjectiveWe describe clinical and neuroimaging findings in children and adolescents with lupus and a PRES diagnosis.Materials and methodsWe identified all clinically acquired brain MRIs of lupus patients at a tertiary care pediatric hospital (2002–2008). We reviewed clinical features, conventional MRI and diffusion-weighted imaging (DWI) findings of patients with gray- and white-matter changes suggestive of vasogenic edema and PRES.ResultsSix pediatric lupus patients presenting with seizures and altered mental status had MRI findings suggestive of PRES. In five children clinical and imaging changes were seen in conjunction with hypertension and active renal disease. MRI abnormalities were diffuse and involved frontal regions in five children. DWI changes reflected increased apparent diffusivity coefficient (unrestricted diffusion in all patients). Clinical and imaging changes significantly improved with antihypertensive and fluid management.ConclusionMRI changes suggestive of vasogenic edema and PRES may be seen in children with active lupus and hypertension. The differential diagnosis of seizures and altered mental status should include PRES in children, as it does in adults.

Neurological manifestations of the antiphospholipid syndrome

The antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterised by autoantibody production and vascular thrombosis or pregnancy morbidity. Autoantibodies generated against phospholipid and phospholipid‐binding proteins often impair phospholipid‐dependent clotting assays (lupus anticoagulants). These autoantibodies activate endothelial cells, platelets and biochemical cascades and can exist in autoimmune disorders such as lupus. Consistently positive antibodies may worsen the severity of thrombo‐occlusive disease. The most common neurological manifestations of APS include stroke and transient ischaemic attacks due to arterial thromboses. Antiphospholipid antibodies may cause additional neurological impairments through both vascular and immune mechanisms. Antiaggregant or anticoagulant therapies are indicated for APS‐related ischaemic strokes. Treatment regimens for asymptomatic antibody‐positive patients and those with refractory disease remain controversial. There is scant literature on neurological APS manifestations in paediatric patients. Assessment of traditional cardiovascular and inherited thrombophilia risk factors is essential in patients with APS. Modifiable risk factors and valvular heart disease may worsen thrombotic and cerebrovascular outcomes. Alternative therapies such as statins, anti‐malarials, angiotensin‐converting enzyme inhibitors and thrombin inhibitors warrant further research.

Cognitive Performance Scores for the Pediatric Automated Neuropsychological Assessment Metrics in Childhood-Onset Systemic Lupus Erythematosus.

To develop and initially validate a global cognitive performance score (CPS) for the Pediatric Automated Neuropsychological Assessment Metrics (PedANAM) to serve as a screening tool of cognition in childhood lupus.

Efficacy of an Interinstitutional Mentoring Program Within Pediatric Rheumatology.

The small size of many pediatric rheumatology programs translates into limited mentoring options for early career physicians. To address this problem, the American College of Rheumatology (ACR) and the Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed a subspecialty‐wide interinstitutional mentoring program, the ACR/CARRA Mentoring Interest Group (AMIGO). We sought to assess the impact of this program on mentoring within pediatric rheumatology.

Healthcare utilization for antinuclear antibody and rheumatoid factor labs in the assessment of musculoskeletal pain in children and adolescents – A claims analysis and chart review

According to the National Bureau of Economics Research, Canada spends far less of its gross domestic product on health care (10. 4% versus 16% in the U. S. ) yet performs better than the U. S. on infant mortality rate and life expectancy [1]. Lowering healthcare utilization and costs are important goals for primary care and subspecialty providers. In March 2012, the Agency for Healthcare Research and Quality (AHRQ) published a meta-analysis of the utility of lab testing in musculoskeletal pain evaluation and screening of rheumatic diseases in children and adolescents2. The review analyzed 28 pediatric articles and concluded that current evidence does not support the use of antinuclear antibody (ANA) and rheumatoid factor (RF) testing as a screening for rheumatic diseases in the primary pediatric care setting. Despite this and other articles with similar recommendations, it seems that inappropriate use of these labs continues to be a persistent problem inpediatrics [2-4]. Referring pediatric patients to rheumatology for positive labs with a normal exam and complaints of intermittent joint pain generates multiple unnecessary referrals that prolong wait times for new patients with active pathology. Additionally, new patients are seen with severe joint damage and contractures that are not referred earlier as their “arthritis labs are negative.” This practice may waste a significant amount of money annually and contributes to higher healthcare utilization [5].

Task Force Report on Brain Involvement in Antiphospholipid Syndrome

Many neurological manifestations have been described in Antiphospholipid Syndrome (APS). More work is needed to identify those individuals with antiphospholipid antibodies (aPL) who are at greatest risk for nervous system manifestations. Antiphospholipid antibodies are established risk factors for a first ischemic stroke in patients with and without systemic lupus erythematosus (SLE), and recurrent cerebrovascular ischemia, particularly in adults with SLE; however, subgroups of these patients at high-risk for recurrent events have not been clearly identified. Persistently positive aPL are associated with cognitive dysfunction in adults with SLE. There is no evidence to support an association between aPL and headache. Antiphospholipid antibodies may be associated with a worse clinical course in adults with SLE who also have Multiple Sclerosis (MS) or an “MS-like” illness and anticardiolipin antibody IgM may be increased during an exacerbation in adults with definite MS, but without SLE. Antiphsopholipid antibodies are associated with seizures and epilepsy in patients with and without SLE. Particular attention needs to be paid to differentiating those manifestations that are mediated through a thrombotic mechanism and those that are mediated through another immune-mediated mechanism. Additional studies that help determine the pathophysiology of aPL-mediated nervous system effects are needed to target appropriate therapies.