Relationship between health-related quality of life, disease activity and disease damage in a prospective international multicenter cohort of childhood onset systemic lupus erythematosus patients.

Previously, we described associations between health-related quality of life (HRQOL) and disease-related factors among childhood onset systemic lupus erythematosus (cSLE) patients. Here we determined the relationship between HRQOL, disease activity and damage in a large prospective international cohort of cSLE. We compared HRQOL, disease activity and disease damage across different continents and examined the relationship between children's and parents' assessments of HRQOL. Patients with cSLE and their parents completed HRQOL measures at enrollment and ≥4 follow-up visits. Physicians assessed disease activity and damage. The multinational cohort ( n = 467) had relatively low disease activity and damage. Patient and parent HRQOL scores were significantly correlated. Asian and European patients had the highest HRQOL, while South and North American patients had lower HRQOL scores. Renal, CNS, skin and musculoskeletal systems exhibited the highest levels of damage. North and South American and Asian patients were more likely to have disease damage and activity scores above median values, compared with Europeans. Asians were more likely to use cyclophosphamide/rituximab. Female gender, high disease activity and damage, non-White ethnicity, and use of cyclophosphamide and/rituximab were related to lower HRQOL. HRQOL domain scores continue to emphasize that SLE has widespread impact on all aspects of children's and parents' lives.

Assessment of metastatic risk of gastric GIST based on treatment-naïve CT features.

OBJECTIVE: To study whether the CT features of treatment-naïve gastric GIST may be used to assess metastatic risk. METHODS: In this IRB approved retrospective study, with informed consent waived, contrast enhanced CT images of 143 patients with pathologically confirmed treatment-naïve gastric GIST (74 men, 69 women; mean age 61 years, SD ± 14) were reviewed in consensus by two oncoradiologists blinded to clinicopathologic features and clinical outcome and morphologic features were recorded. The metastatic spread was recorded using available imaging studies and electronic medical records (median follow up 40 months, interquartile range, IQR, 21-61). The association of maximum size in any plane (≤10 cm or >10 cm), outline (smooth or irregular/lobulated), cystic areas (≤50% or >50%), exophytic component (≤50% or >50%), and enhancing solid component (present or absent) with metastatic disease were analyzed using univariate (Fisher's exact test) and multivariate (logistic regression) analysis. RESULTS: Metastatic disease developed in 42 (29%) patients (28 at presentation, 14 during follow-up); 23 (16%) patients died. On multivariate analysis, tumor size >10 cm (p = 0.0001, OR 9.9), irregular/lobulated outline (p = 0.001, OR 5.6) and presence of a enhancing solid component (p < 0.0001, OR 9.1) were independent predictors of metastatic disease. On subgroup analysis, an irregular/lobulated outline and an enhancing solid component were more frequently associated with metastases in tumors ≤5 cm and >5-≤10 cm (p < 0.05). CONCLUSION: CT morphologic features can be used to assess the metastatic risk of treatment-naïve gastric GIST. Risk assessment based on pretreatment CT is especially useful for patients receiving neoadjuvant tyrosine kinase inhibitors and those with tumors <5 cm in size.