Systemic Lupus Erythematosus in Filipino children: A 10-year retrospective analysis of mortality, morbidity and survival

@#Background. Systemic Lupus Erythematosus (SLE) in children has been estimated to account for 15 to 20% of all SLE cases worldwide. It was described to have more severe disease at presentation including renal, neuropsychiatric, and hematologic involvements; more disease activity over time, and a significantly higher risk of organ damage. Thus, considered a significant risk factor for mortality among adult SLE patients. Objective. This is a retrospective cohort study aimed to determine the clinical profile, outcome, and survival of SLE among Filipino children. Methods. All SLE patients, less than 19 years old, diagnosed in the pediatric department of a tertiary hospital from January 2008 to December 2017 were included in the study. Their medical charts were retrieved for data gathering. Demographics, and clinical disease characteristics were collected from admission and on subsequent follow ups. Lost to follow up patients were contacted for updates of their current clinical status. Results. A total of 261 pediatric SLE patients were gathered. Average age at diagnosis is 14.5 years old (±2.7), with female to male ratio of 16:1. Symptoms starts at 3 months prior to consult (±2.1). Upon diagnosis, most of the patients have fever, malar rash, alopecia, oral ulcers, and proteinuria. Most common systemic organ involvement through time were mucocutaneous, hematologic, and renal. Steroids were the mainstay management for all patients, in which 95% started on oral Prednisone, while 71% needed IV Methylprednisolone in at least once during the disease course. Two patients received biologic treatment. Overall mortality rate was 14.9%, identified to be secondary to sepsis and/or SLE activity. Myocarditis, pleural effusion, and seizures were identified as significant risk factors for mortality. Survival rate at 1 year and 10 years were 92% and 79%, respectively. Conclusion. SLE in Filipino children mostly presents with mucocutaneous symptoms. Presence of seizures, myocarditis, and pleural effusion at any time of the disease entails risk for mortality. SLE nephritis is a substantial cause of morbidity due to its chronicity. The survival rate of Filipino children with SLE is comparable with the data from other developing countries.

Isolated Splenic Infarction in a Girl with Systemic Lupus Erythematosus

Splenic infarction is a rare disease and usually detected as a complication of SBE, atrial fibrillation, hemoglobinopathy, or myeloproliperative disorders. Although thrombotic event, such as deep vein, cerebral or renal thrombosis, is a frequent complication of SLE, there have been only a couple of cases documenting splenic infarction complicated in SLE. This is, to our knowledge, the first case report describing isolated splenic infarction in pediatric SLE. The splenic infarction was diagnosed on abdominal CT, 99mTc-RBC splenic scan, and abdominal USG, and resolved with anticoagulant therapy. And we discussed about the possibility of antiphospholipid syndrome as a cause of the infarction in this case.