Retinal vasculitis in two pediatric patients with systemic lupus erythematosus: a case report.

We report two pediatric female patients with systemic lupus erythematosus (SLE) who presented with decreased vision. Both patients were found to have retinal vasculitis and occlusive disease. The first patient also presented with vitreous hemorrhage and later non-arteritic ischemic optic neuropathy. She was treated with panretinal photocoagulation and steroid therapy and later in her disease course was treated with rituximab and cyclophosphamide. Her vision remained decreased. The second patient was treated with rituximab and monthly cyclophosphamide infusions early in her disease course, and her vision improved dramatically. The difference in the presentations and outcomes of these two pediatric patients with SLE highlights the spectrum of severity of SLE retinopathy. We suggest that early recognition of disease and early intervention with B-cell depletion therapy in addition to a traditional cytotoxic agent should be considered in pediatric patients with SLE and occlusive retinopathy.

Management of pediatric patients with pseudotumor cerebri.

INTRODUCTION: The care of patients with pseudotumor cerebri (idiopathic intracranial hypertension) involves ophthalmologists, neurologists and neurosurgeons. Its clinical characteristics in the pediatric population are distinct from those in adult patients. PATIENTS AND METHODS: Fifty-nine patients diagnosed with pseudotumor cerebri were identified from the neurosurgery and neurology databases at Children's Hospital, Birmingham, AL, USA. Clinical data were collected from the ophthalmology, neurology and neurosurgery departments. RESULTS: The average age of patients at diagnosis was 11.4 years (range 3-17). The average opening pressure of lumbar puncture (LP) was 37 cm of water. Most of the patients responded well to therapeutic LPs and medical management. Neurosurgical interventions included intracranial pressure monitoring and shunt insertion in nine patients. In three patients who presented with acute visual decline, two recovered and one remains legally blind. CONCLUSIONS: The care of patients with pseudotumor cerebri requires a multiple-disciplinary approach. Neurosurgical interventions are sometimes needed for diagnostic and treatment purpose. Prompt and accurate communication among specialists is necessary to ensure timely treatment and optimal outcomes.

Attainment of inactive disease status following initiation of TNF-α inhibitor therapy for juvenile idiopathic arthritis: enthesitis-related arthritis predicts persistent active disease.

OBJECTIVE: To analyze the attainment of inactive disease following initiation of tumor necrosis factor-α (TNF-α) inhibitors in a heterogeneous cohort of children with juvenile idiopathic arthritis (JIA). METHODS: We performed retrospective chart review of all children with JIA at 1 academic center who had started TNF-α inhibitor therapy. We retrospectively determined inactive disease status according to the 2004 criteria of Wallace, et al. We evaluated inactive disease status at 1 year after initiation of TNF-α inhibitor and attainment of inactive disease at any point during the study period. Predictors of inactive disease were determined using univariate analyses and multivariable logistic regression models. RESULTS: A total of 125 patients started TNF-α inhibitors, and 88 patients had data available for the 1-year followup visit. Many patients (49%) started TNF-α inhibitors within 6 months of the diagnosis of JIA. Diverse JIA phenotypes were represented: at baseline, 29% of all patients had active enthesitis and only 23% had active polyarthritis. At the 1-year followup, 36 of 88 (41%) patients had inactive disease. Overall, 67 of 125 (54%) patients ever attained inactive disease status during the study period. In multivariable models, enthesitis-related arthritis (ERA) and higher Childhood Health Assessment Questionnaire (CHAQ) scores at baseline were independently associated with failure to later attain inactive disease status. CONCLUSION: Treatment with TNF-α inhibitors appears to be less effective for attaining inactive disease status in patients with ERA or higher baseline CHAQ scores. Further studies are needed regarding the clinical effectiveness of TNF-α inhibitor therapy and the optimal treatment of ERA.

Performance on rheumatoid arthritis quality indicators in an Alaska Native healthcare system.

OBJECTIVE: Rheumatoid arthritis (RA) is highly prevalent in some Alaska Native and American Indian populations. Quality indicators for RA have been proposed, but these have not been widely implemented or used to assess RA care in Alaska Native or American Indian populations. METHODS: Medical records were included if they met the following criteria: RA diagnosed before October 2000 fulfilling American College of Rheumatology classification criteria; all care for RA at the Alaska Native Medical Center. Records were reviewed for a 5-year period to determine compliance with eight quality indicators defined by the Arthritis Foundation Quality Indicator Program. Multivariate logistic regression was performed to analyse associations with quality of care. RESULTS: There were 106 individuals included in the study. The highest-scoring measures were folic acid prescription if on methotrexate (93.3%) and disease-modifying antirheumatic drug prescription (90.6%). The lowest scoring measure was radiographs of both hands and feet (16.0%). In multivariate analysis, the factor most strongly associated with disease-modifying antirheumatic drug prescriptions, annual exam for RA and hand radiographs was at least one visit with a rheumatologist. CONCLUSIONS: Quality of care for RA varies by measure and is better for patients who see a rheumatologist. These data provide an initial evaluation of RA quality of care in a unique minority population with an integrated healthcare system.

Rituximab therapy for severe refractory chronic Henoch-Schönlein purpura.

To report on the efficacy of rituximab (RTX) therapy in standard treatment-refractory, chronic Henoch-Schönlein purpura, a retrospective chart review of 3 pediatric patients treated with RTX for severe refractory chronic Henoch-Schönlein purpura was performed. All 3 patients responded to 1 or 2 courses of RTX without serious adverse events.