The catastrophic variant of antiphospholipid syndrome (APS) is a very rare and life-threatening condition of APS. This condition is characterized by thrombosis in multiple organs within a short period of time in the presence of positive antiphospholipid antibodies (aPL). Over the past few decades, considerable progress has been made in the treatment of patients with catastrophic APS; however, the mortality rate still remains very high. Although some cases of rituximab treatment in patients with catastrophic APS have been reported, there is no clear treatment protocol. A 14-year-old girl with systemic lupus erythematosus was diagnosed with catastrophic APS. She received several medications: corticosteroids, intravenous immunoglobulin, and plasmapheresis with anticoagulants. Unfortunately, she did not improve, and rituximab was started with four courses. After the rituximab treatment, she did not experience further thrombotic events during the follow up. This paper reports a pediatric case of catastrophic APS treated successfully with rituximab in Korea.
Adolescent ; Adrenal Cortex Hormones ; Antibodies, Antiphospholipid ; Anticoagulants ; Antiphospholipid Syndrome* ; Clinical Protocols ; Female ; Follow-Up Studies ; Humans ; Immunoglobulins ; Korea ; Lupus Erythematosus, Systemic* ; Mortality ; Plasmapheresis ; Rituximab* ; Thrombosis

OBJECTIVE: This study examined the prevalence of low-bone mineral density (BMD) and its associated factors in Korean children and adolescents with juvenile idiopathic arthritis (JIA). METHODS: Thirty-nine patients with JIA were included in this cross-sectional study. The patients were examined for their bone age (BA) and bone mineral density (BMD). The BMD was measured using dual-energy X-ray absorptiometry on the lumbar spine. Each BMD value was converted to a Z-score by comparing the reference values of the healthy control group in terms of the age and sex of each patient, which was measured using the same device. A Z-score of less than −2.0 was defined as a low BMD. Laboratory tests were performed to detect the serum calcium, phosphorus, alkaline phosphatase, and 25-hydroxyvitamin D levels. RESULTS: The mean age at the time of the examination was 12.2±3.6 years, and the study comprised a total of 39 patients (16 males, 23 females). Patients with systemic JIA had a lower BMD, which was calculated based on the CA, BA, and HA, than those with non-systemic JIA (P=0.020, P=0.049, and P=0.024, respectively); the corticosteroid user group also showed a lower BMD, which had been calculated based on the CA, BA, and HA, than the corticosteroid non-user group (p=0.002, p=0.022, and p=0.188, respectively). CONCLUSION: This study suggests that JIA patients have a lower BMD than control subjects. Therefore, treatment, and education are warranted while treating patients with JIA, particularly those requiring oral corticosteroids or those with systemic JIA and appropriate laboratory tests.
Absorptiometry, Photon ; Adolescent* ; Adrenal Cortex Hormones ; Alkaline Phosphatase ; Arthritis, Juvenile* ; Bone Density* ; Calcium ; Child* ; Cross-Sectional Studies ; Education ; Humans ; Male ; Miners ; Phosphorus ; Prevalence* ; Reference Values ; Spine