Distinct phenotypes of multisystem inflammatory syndrome in children: a cohort study

To characterize the clinical features and outcomes of childhood-onset primary Sjögren’s syndrome (pSS). Patients less than 18 years old who were diagnosed with pSS by paediatric rheumatologists were included, and all patients were applied the 2002 American-European Consensus Group (ACEG) criteria, the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for pSS, or the 1999 proposed juvenile pSS criteria. The electronic medical records of patients with pSS from 2013 to 2020 were collected and analysed. Thirty-nine patients were included. Of them, 27 (69.2%), 38 (97.4%) and 35 (89.7%) patients fulfilled the AECG criteria, ACR/EULAR criteria and proposed juvenile pSS criteria, respectively. The female:male ratio was 3.9:1. The median ages at first signs or symptoms and at diagnosis were 9.2 (4.7, 14.5) years and 10.9 (6.3, 15.0) years, respectively. The main clinical manifestations were rash or purpura (20, 51.3%), followed by fever (12, 30.8%), glandular enlargement/recurrent parotitis (10, 25.6%), and dry mouth and/or dry eyes (9, 23.1%). Twenty-eight (56.4%) patients had systemic damage, the most common of which was haematological involvement (14, 35.9%), followed by hepatic (13, 33.3%) and renal involvement (8, 20.5%). Thirty-eight (97.4%) patients underwent labial minor salivary gland biopsy, and all exhibited focal lymphocytic sialadenitis. All patients had a global ESSDAI score ≥ 1 at diagnosis, and the median total score at diagnosis was 8 (2, 31). Thirty-six (92.3%) patients were followed up for a median time of 23.6 (7.9, 79.5) months, and three patients developed systemic lupus erythematosus (SLE) at follow-up times of 13.3, 38.8 and 63.8 months. The presentation of childhood-onset pSS is atypical, and extraglandular manifestations and systemic involvement are more common than in adult-onset pSS. Labial salivary gland biopsy is vital for patients with probable pSS. Some patients may develop SLE over time.     

Comparison of facemask and mouthpiece interfaces for multiple breath washout measurements

Background

Different interfaces (mouthpiece/nose clip vs. facemask) are used during multiple breath washout (MBW) tests in young children.

Predictors of lipid abnormalities in children with new-onset systemic lupus erythematosus

OBJECTIVE: Lipid abnormalities in patients with systemic lupus erythematosus (SLE) are common and likely are one of the causes of premature atherosclerosis in these patients. Our aims were to determine the frequency and pattern of dyslipoproteinemia at presentation of pediatric SLE; and to determine the association between dyslipoproteinemia and markers of disease activity and inflammatory markers at presentation of pediatric SLE. METHODS: Serum lipid measurements were obtained at diagnosis before corticosteroid treatment for an inception cohort of 54 patients. Total cholesterol, triglyceride, LDL-C, and HDL-C levels were regressed on measures of inflammation, disease activity, and disease symptoms. RESULTS: At least one lipid abnormality was present in the majority of patients (63%), an elevated triglyceride level being the most common lipid abnormality (62%). Triglycerides were best predicted by fibrinogen, nephritis, and pleuritis (model R2 = 0.6). Albumin, C4, and white blood cell count were found to predict HDL-C (model R2 = 0.6). Erythrocyte sedimentation rate, central nervous system involvement, nasal ulcers, and nephritis were found as predictors for LDL-C:HDL-C (model R2 = 0.5). No significant predictors were found for total cholesterol or LDL-C. The European Consensus Lupus Activity Measure disease activity score best predicted abnormal triglyceride and HDL-C levels (OR 1.7, 95% CI 1.2-2.3). CONCLUSION: Children with newly diagnosed SLE exhibited the distinct pattern of dyslipoprotein of increased triglycerides and depressed HDL-C that was twice as common in the presence of kidney disease. This lipid profile puts them at risk for premature atherosclerosis. Good disease control and individualized use of lipid-lowering agents based on the observed pattern of lipid abnormalities may lower the risk of premature atherosclerosis in these patients.