Childhood-onset Systemic Lupus Research Articles

Relationship of regional ultraviolet index data with rash and systemic disease activity in youth with childhood-onset systemic lupus: results from the Childhood Arthritis and Rheumatology Research Alliance Registry

ObjectiveTo investigate the association between ultraviolet light index (UVI), as a marker for UV exposure, and seasonality with rash and systemic disease activity in youth with childhood-onset systemic lupus (cSLE) from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry.MethodsWe reviewed data on rash and disease activity from Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K) scores from cSLE CARRA Registry participants with visits between 2010 and 2019 and obtained zipcode level UVI data from the National Oceanic and Atmospheric Administration (NOAA). Our main exposures were UVI and season during the month of visit and one month prior to visit. We used mixed-effects logistic regression models to examine associations between regional UVI (by zipcode)/season and odds of rash and severe SLEDAI-2 K score (≥ 5 vs. 0–4), adjusting for age, sex, race and income.ResultsAmong 1222 participants, with a mean of 2.3 visits per participant, 437 visits (15%) had rash and 860 (30%) had SLEDAI-2 K score ≥ 5. There were no associations between UVI during the month prior to visit or the month of the visit and odds of rash or elevated systemic activity. However, fall season was associated with increased odds of rash (OR = 1.59, p = 0.04), but not increased disease activity.ConclusionThis study found no association between UVI and rash or UVI and disease activity. However, further studies directly measuring UV exposure and accounting for patient-level protective behavioral measures may help to better understand the complex relationship between sun exposure and SLE disease activity.

A FATAL OUTCOME OF A NEONATAL DILATED CARDIOMYOPATHY: EVOLUTIVE NEONATAL LUPUS OR EARLIER CHILDHOOD- ONSET SYSTEMIC LUPUS? A CASE REPORT

Neonatal lupus is a rare congenital syndrome resulting from placental passage of maternal antibodies against SSA/Ro and/or SSB/La to the fetus. It is a generally benign condition, the cardiac involvement determining the prognosis. NNL is rarely manifested by isolated cardiomyopathy. The subsequent evolution towards systemic lupus is rare and unpredictable. We report the case of a daughter from a non-consanguineous marriage with healthy parents who presented a dilated cardiomyopathy in the first months of her life complicated by several outbreaks of heart failure. The spontaneous evolution was marked by the occurrence of skin lesions of the face and arms at the age of 10 years, then by the installation of episodes of hydrous retention at the age of 12 years complicated by an acute renal insufficiency with a rapid fatal outcome at the age of 15 years. ANA antibodies rate was at the threshold of positivity, antiDNA and ANCA were not detectable.C3 rate was low. Although a genetic cause can never be ruled out, the diagnosis was a systemic erythematous lupus SLE (an evolution of an eventual NNL or a childhood-onset systemic lupus). We propose a decision tree for the management of neonatal cardiomyopathy NCMP as a synthesis of the literature.The search for anti SSA/SSB must be part of the check-up usually requested at the time of pregnancy. In addition, we must strengthen the knowledge of the medical profession, especially in the proximity health centers, on rare diseases for a better management of patients.

Alter bei SLE-Diagnose beeinflusst Krankheitsverlauf

Joo YB et al. Differences in Clinical Features and Mortality between Childhood-onset and Adult-onset Systemic Lupus Erythematosus: A Prospective Single-center Study.

Difference in Disease Features Between Childhood-Onset and Adult-Onset Systemic Lupus Erythematosus

Difference in Disease Features Between Childhood-Onset and Adult-Onset Systemic Lupus Erythematosus

Three cases of childhood-onset male systemic lupus erythematosus (SLE) successfully treated with a combination of pulse methylprednisolone and pulse cyclophosphamide

We reported three cases of childhood-onset male systemic lupus erythematosus (SLE), all of whom successfully treated with a combination of pulse methylprednisolone (mPSL) and pulse cyclophosphamide (IVCY). All of them had severe lupus nephritis and were complicated with other collagen diseases. Two cases were complicated with Sjögren syndrome (SS) and the other was complicated with both SS and anti-phospholipid syndrome (APS). After a combination of pulse mPSL and IVCY for a year, followed by oral predonisolone (PSL) and azathioprine (AZA), following up renal biopsy were performed in all cases, which showed histological improvement in glomerulonephritis. One case had flares a year later, but no flares were observed either in clinical symptoms or in laboratory examinations in the others. Their autoantibodies except anti-nuclear antibody (ANA) were eliminated. We suggest a combination of pulse mPSL and IVCY is effective for the patients who are suffering with severe lupus nephritis complicated with the other collagen diseases.