These are considered the first guidelines using the GRADE method for the monitoring of SLE. Existing evidence is largely of low to moderate quality, resulting in more conditional than strong recommendations. Additional rigorous studies and special attention to pediatric SLE populations and patient preferences are needed.

Современные международные рекомендации по диагностике и лечению системной красной волчанки с ювенильным дебютом Каледа М.И., Никишина И.П.Системная красная волчанка (СКВ) с дебютом в детском и подростковом возрасте представляет существен-ную проблему в практике педиатров и ревматологов ввиду диагностических сложностей, особенностей тече-ния и большей вероятности неблагоприятного прогноза. До 20% больных СКВ заболевают в возрасте млад-ше 18 лет. Только у 13% пациентов с ювенильным дебютом во взрослом состоянии имеется безмедикамен-тозная ремиссия при более низком, чем в популяционном контроле, качестве жизни. Статья посвящена об-суждению последних международных рекомендаций по диагностике, мониторингу и лечению СКВ у детей и подростков, опубликованных в 2017 г., с комментариями, основанными на данных литературы и на прак-тическом опыте ведения подобных пациентов. Ключевые слова: системная красная волчанка; клинические рекомендации; детский возраст. Для ссылки: Каледа МИ, Никишина ИП. Современные международные рекомендации по диагностике и ле-чению системной красной волчанки с ювенильным дебютом. Научно-практическая ревматология. 2018;56(4):405-415. CURRENT INTERNATIONAL GUIDELINES FOR THE DIAGNOSIS AND TREATMENT OF JUVENILE-ONSET SYSTEMIC LUPUS ERYTHEMATOSUSKaleda M.I., Nikishina I.P.Systemic lupus erythematosus (SLE) with its onset in childhood or adolescence is a significant problem in the practice of pediatricians and rheumatologists due to diagnostic difficulties, clinical features, and the greater likelihood of unfavorable prognosis. About 20% of SLE patients fall ill at the age of 18 years. Only 13% of patients with juvenile-onset SLE have a drug-free remission in adulthood and have a lower quality of life than population controls. The paper discusses the latest international guidelines for the diagnosis, monitoring, and treatment of SLE in children and adolescents, which were published in 2017, with comments based on the data available in the literature and on practical experiences in managing these patients.

Patients with cSLE with severe organ involvement including nephritis and CNS disease and higher maximal daily dose of PRED are more likely to develop symptomatic AVN.

Objective. To determine the longitudinal damage trajectory of patients with juvenile-onset systemic lupus erythematosus (SLE), and to identify baseline and disease course predictors of damage trajectory. Methods. This is a retrospective inception cohort. Longitudinal pediatric-age data were obtained from a juvenile-onset SLE research database, while adult-age data were obtained from either a research database or patients' charts. Baseline factors were tested as predictors. Time-varying factors were lagged 6-24 months before a visit for testing their predictive effects. The longitudinal damage trajectory was modeled using a weighted generalized estimating equation.Results. This study cohort consisted of 473 subjects, with followup to 26 years. A total of 65% of patients were ages >18 years at last followup. Cataracts (14%), avascular necrosis (10%), and osteoporosis (5%) were the most common items of damage. Two patients had myocardial infarctions. Baseline features, self-reported ethnicity (Afro-Caribbean), earlier time periods of diagnosis, and the presence of a life-threatening major organ manifestation (lupus nephritis class III-V, cerebrovascular accidents, major organ vasculitis, pulmonary hemorrhage, or myocarditis), were associated with greater damage. Throughout the disease course, an acute confusional state, lupus headache, and fever predicted subsequent increases in the damage trajectory. A higher prednisone dose and exposure to cyclophosphamide also predicted subsequent increases in the damage trajectory. Antimalarial exposure was protective against an increase in damage trajectory. Conclusion. Patients with juvenile-onset SLE accrue damage steadily into adulthood. Baseline factors predict greater damage and/or influence the evolution of the damage trajectory. Additionally, SLE clinical features and therapies during the course of disease predict additional changes in the damage trajectory.

Most patients responded to immunosuppressive treatment, although median time to remission was > 1 year. Roughly half the patients acquired a new damage item, most of which did not interfere with functional abilities. Fewer than 20% of patients developed neuropsychiatric damage. Both phenotypes of psychiatric pSLE responded equally well to current treatment.

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There are 5 distinct latent classes of disease trajectory in patients with cSLE. Membership within disease trajectories is predicted by baseline clinical and demographic factors. Membership in different disease activity trajectory classes is associated with different damage trajectories.

The LS BMD of pSLE patients followed a general deteriorating trend over time and could be predicted by a combination of pubertal status at diagnosis, interval cumulative doses of steroids and weight z-scores. Interval cumulative steroid dose represents an important target that clinicians may modify to ameliorate deteriorating BMD trajectory over time.