Systemic Lupus Erythematosus: Clinical Recommendations. Part 2

Алексеева, Е. И.; Дворяковская, Т. М.; Nikishina, Irina; Денисова, Р. В.; Подчерняева, Н. С.; Сухоруких, О. А.; Шубина, Л. С.; Часнык, В. Г.; Костик, М. М.

doi:10.15690/vsp.v17i2.1877

Cited by 4 publications

(1 citation statement)

References 16 publications

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“…В то же время имеются многочисленные данные, свидетельствующие об успешном применении РТМ в педиатрической ревматологии для лечения ИВРЗ и других аутоиммунных заболеваний [5][6][7]. РТМ включен в Международные рекомендации по диагностике, мониторингу и терапии СКВ с ювенильным началом [8], отечественные клинические рекомендации по лечению СКВ у детей [9], а также в клинические рекомендации по лечению ЮИА, серопозитивного по РФ [10], ЮИА с системным началом (сЮИА) [11]. В рекомендациях ACR (American College of Rheumatology, Американская коллегия ревматологов) по терапии отдельных субтипов ЮИА подчеркивается необходимость обсуждения возможности раннего назначения РТМ как перспективного подхода к терапии серопозитивного ЮИА [12].…”

Section: Discussionunclassified

Rituximab for rheumatic diseases in children: Results of a retrospective study of the safety of therapy

Каледа¹,

Nikishina²,

Николаева³

et al. 2021

Naučno-praktičeskaâ revmatologiâ

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Objective: to analyze the safety of rituximab (RTM) in children with various rheumatic diseases.Materials and methods. The retrospective study included 81 pediatric patients with a confirmed diagnosis of rheumatic disease. Data on the safety of RTM were analyzed for all patients who received at least one infusion of the drug. All patients underwent a standard clinical, laboratory and instrumental examination in accordance with the verified diagnosis before the appointment of RTM therapy. The dose of RTM for administration was calculated based on 375 mg/m 2 of body surface area, the drug was administered once a week for 1 to 4 consecutive weeks, depending on the number of CD19 lymphocytes determined after the infusion, and the tolerability of therapy.Results. Among the patients included in the study, 38 (46.9%) were with systemic lupus erythematosus (SLE), 16 (19.75%) – with juvenile idiopathic arthritis (JIA), polyarticular variant (14 (87,5%) of them – RF-positive), 9 (11,1%) – with juvenile idiopathic arthritis with systemic onset (sJIA), 6 (7.4%) – with systemic sclerosus (SSc), 5 (6.2%) – with primary Sjogren’s syndrome, 2 (2.5%) – with juvenile dermatomyositis, 4 (4.9%) – with mixed connective tissue disease, and 1 – with livedoid vasculopathy. 53 (65.4%) patients underwent more than one course of RTM therapy, with a maximum of 10 courses. The total number of infusions was 198. The median time between each course was 182 [156–315] days. RTM was effective in 67 (95%) patients, ineffective in 2 (2.5%) patients with sJIA, 2 (2.5%) patients with SLE and macrophage activation syndrome (MAS).Adverse reactions (AE) of mild to moderate severity were reported in 23 (28.4%) patients, including upper respiratory tract infections – in 7 (8.6%), urinary tract infections – in 2 (2.5%), mild infusion reactions that did not require discontinuation of therapy – in 2 (2.5%), clinically insignificant neutropenia (I–II degree) – in 4 (4.9%), a decrease in IgG levels – in 14 (17.5%) patients (median – 5.5 [4.0; 6.9] g/l). Two patients with sJIA had persistent hypogammaglobulinemia for 3 and 5 years after the last RTM infusion, respectively. The incidence of infections in patients with low IgG levels was 35.7%, and no cases were registered in patients with neutropenia. Serious AE was reported in 16 (19.7%) patients: sepsis – in 4 (4.9%), pneumonia – in 3 (3.7%), herpes zoster – in 1 (1.2%), serious infusion reactions – in 2 (2.5%), serious postinfusion reactions within 3–10 days – in 4 (4.9%) (in 3 patients (3.7%) – MAS, in 1 (1.2%) – hemorrhagic vasculitis); death was registered in 2 cases of SLE and MAS (therapy of RTM was inefficient). In general, various AE were reported in 55.6% of patients with sJIA, 52.6% of patients with SLE, 50% of patients with SSc and juvenile dermatomyositis, and 80% of patients with primary Sjogren’s syndrome. Discontinuation of therapy due to serious AE was observed in 15 (18.5%) patients.Conclusion. Our study demonstrated that RTM therapy is highly effective with an acceptable safety profile in children with rheumatic diseases. The safety data obtained indicate the need for careful monitoring of therapy, primarily taking into account the risk of infection, despite the fact that in this study the frequency of infectious complications was not high. A decrease in IgG level was observed in a small proportion of patients and did not correlate with the incidence of infections.

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Section: Discussionunclassified

Rituximab for rheumatic diseases in children: Results of a retrospective study of the safety of therapy

Каледа¹,

Nikishina²,

Николаева³

et al. 2021

Naučno-praktičeskaâ revmatologiâ

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Belimumab in a Patient with Systemic Lupus Erythematosus with Juvenile Onset and Steroid-induced Diabetes: Clinical Case

Kaleda,

Nikishina,

Firsa

2024

Vopr. sovr. pediatr.

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Background. The management of children with systemic lupus erythematosus (SLE) is usually associated with lifelong systemic glucocorticoids administration and, thereby, high risk of serious side effects, including steroid-induced diabetes. The belimumab (B-lymphocyte stimulator inhibitor) administration significantly reduces the glucocorticoids dose, the risk and severity of steroid therapy complications. Clinical case description. The patient was diagnosed with SLE at the age of 16 years. Therapy with hydroxychloroquine and oral glucocorticoid at a high dose (methylprednisolone 56 mg per day) was initiated. Steroid-induced diabetes was diagnosed 1 month after the therapy start; avascular necrosis sites were revealed in 2 months. Mycophenolate mofetil made it possible to achieve the disease activity control. However, the belimumab was prescribed 5 months after diagnosis verification due to continuous insulin requirement and avascular necrosis progression. Conclusion. Belimumab is the only genetically engineered biologic drug approved for the treatment of children with SLE. As a result of its use, it was possible to stabilize the patient's condition quickly (within 3 months), to reduce significantly the dose of oral glucocorticoid, methylprednisolone (from 24 to 8 mg/day), to achieve remission of steroidinduced diabetes with further insulin withdrawal, and also to relieve avascular necrosis clinical symptoms.

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Сlinical cases of newly diagnosed systemic lupus erythematosus at the appointment with a dermatologist

Morozova

Bausheva

2022

Russian Journal of Skin and Venereal Diseases

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Lupus erythematosus is a multisystemic connective tissue disease that has a wide range of manifestations affecting the skin and mucous membranes, joints, kidneys, heart, lungs, and central nervous system. In dermatology, cutaneous forms of lupus erythematosus can be classified into discoid, disseminated, centrifugal erythema, lupus panniculitis, subacute and chronic lupus, and systemic lupus erythematosus with damage to internal organs. It can develop as an independent disease or can be a result of progression of cutaneous forms. The skin is the second most commonly affected organ in systemic lupus erythematosus folowing the joints, with skin manifestations observed in 80% of patients throughout the course of the disease. And in 2030% of cases, the disease debuts with skin rash. Thus, these patients primarily present to dermatologists. Knowledge of the criteria for making a diagnosis, the clinical characteristics, in combination with performance of advanced laboratory diagnostic tests allow a dermatologist to suspect a systemic process in a timely manner and prescribe appropriate therapy. The article presents two clinical observations of male patients with systemic lupus erythematosus, the diagnosis of which could not have been established for several years. The diagnosis in these cases met the 2019 European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) classification criteria for systemic lupus erythematosus, confirmed by laboratory tests. Patients had a positive trend to response to therapy.

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Systemic Lupus Erythematosus: Clinical Recommendations. Part 2

Cited by 4 publications

References 16 publications

Rituximab for rheumatic diseases in children: Results of a retrospective study of the safety of therapy

Rituximab for rheumatic diseases in children: Results of a retrospective study of the safety of therapy

Belimumab in a Patient with Systemic Lupus Erythematosus with Juvenile Onset and Steroid-induced Diabetes: Clinical Case

Сlinical cases of newly diagnosed systemic lupus erythematosus at the appointment with a dermatologist

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