BACKGROUND. The etiology of plasmacytomas in multiple myeloma (MM) remains unclear, whereas the pathogenetic mechanisms of extramedullary lesions are of particular importance because of extremely poor prognosis. The incidence of bone and extramedullary plasmacytomas complicating the course of MM as well as the efficacy of therapy for this disease are still underresearched. AIM. To assess the incidence of bone and extramedullary plasmacytomas in patients with newly diagnosed MM (ndMM) and analyze the overall survival (OS) rates depending on whether soft-tissue components were detected. MATERIALS & METHODS. This multi-center prospective study enrolled 3184 ndMM patients (1339 men and 1845 women) from 40 regions of Russia, aged 24–90 years and treated during the period from January 2015 to October 2018. The electronic patient records included demographic, clinical, laboratory, and instrumental documentation data accessible on MM diagnosis date. Bortezomib-based regimens were administered in the first-line therapy to 94 % of patients. RESULTS. Bone and extramedullary plasmacytomas were diagnosed in 763 (24 %) out of 3184 ndMM patients. At disease onset, the median hemoglobin concentration was significantly higher in patients with bone (105 g/L) and extramedullary (102 g/L) plasmacytomas than in patients without them (95 g/L; p < 0.05). The median percentage of plasma cells in the bone marrow was significantly lower in cases of bone plasmacytoma accounting for 25 % vs. 30 % in cases of extramedullary components, and it was 31 % in patients without plasmacytomas at MM onset (p < 0.05). Most commonly, plasmacytomas were detected in the thoracic spine (24.3 %; n = 132) and ribs (13.6 %; n = 74). The median OS of patients without plasmacytomas was 46 months (95% confidence interval [95% CI] 43–49 months) vs. 38 months (95% CI 42–46 months) in patients with them (p = 0.1245). The median OS was 17 months (95% CI 10–35 months) in patients with extramedullary plasmacytomas and 13 months (95% CI 1–69 months) if soft-tissue components were detected in the bones of the lower extremities. CONCLUSION. The course of ndMM is complicated by bone plasmacytomas in 23 % of cases and extramedullary plasmacytomas in 1 % of cases. The antitumor response rate as well as the OS rates are similar in patients with and without bone plasmacytomas. Extramedullary lesions in MM are associated with extremely low rate of OS with the median not exceeding 17 months.
The aim of the study – to analyze the effectiveness and tolerability of subcutaneous methotrexate (sc MTX) (Metorthrit; S.C. Rompharm Company S.R.L) in patients with rheumatoid arthritis (RA) with high disease activity and rapid dose escalation, to assess their quality of life (QoL) in real clinical practice.Material and methods. The study included 105 patients, mostly women, with a reliable diagnosis of RA with high disease activity (DAS-28 (Disease Activity Score 28) ≥5.1) aged 18 years and older and ineffectiveness of previous oral MTX therapy for at least 6 months or who had not received MTX. Sc MTX therapy was started at a dose of 15 mg/weekly. During the first month of therapy, a rapid escalation of the sc MTX dose of 2.5 mg/week was performed once a week. until the dose of 22.5 mg/week was reached, then, with insufficient response, the dose of sc MTX could be increased to 25 mg/week. The evaluation of the effectiveness of therapy, functional status, and QoL was carried out after 4–12–18–24 weeks.Results. After a rapid escalation of the sc MTX dose during the first month of the study, at all stages of follow-up, a rapid decrease in disease activity was noted for all standard indices (DAS-28 – from 5.8±0.75 to 2.93±1.05; CDAI (Clinical Disease Activity Score) – from 30.13±8.33 to 7.08±6.07; SDAI (Simplified Disease Activity Score) – from 32.78±9.64 to 7.48±6.53) and the activity index, which was evaluated by the patients themselves (RAPID-3 (Routine Assessment of Patient Index Data 3) from 16.18±4.6 to 5.56±4.66; p≤0.05). The number of patients with high disease activity according to DAS-28 decreased by 2 times by the week 4 of therapy (to 46.2%), after 12 weeks they remained 13.3%, and by week 24 high activity remained only in 4.4% of patients. There was a marked decrease in pain from 65.6±13.07 to 20.5±17.1 mm in VAS (Visual Analogue Score) (p<0.001), which contributed to an improvement in the functional state: the HAQ (Health Assessment Questionnaire) index decreased on average from 1.47±0.65 to 0.64±052 points. Population indicators of functional status (HAQ≤0.5) by the week 24 of therapy were observed in 48.9% of patients. A decrease in the level of fatigue (from 6.25±7.04 to 1.81±1.71 cm according to VAS; p<0.001) was accompanied by a decrease in anxiety (from 7.47±4.03 to 2.36±2.72; p<0.001) and depression (from 7.77±3.84 to 2.50±2.56; p<0.001), as well as improved sleep. By the week 24 of the study, 45% of patients had population-based indicators of QoL according to the EQ-5D index. Glucocorticoids (GC) were completely eliminated in 2/3 of the patients. Patients who did not receive GC had lower disease activity by 24 weeks in all indices: DAS-28 (2.7±0.1 and 3.4±0.2, respectively), CDAI (6.0±0.3 and 10.2±0.1), SDAI (6.4±0.2 and 10.9±0.3) (p<0.05). Patients receiving and not receiving GC had the same number of adverse reactions (p>0.05), however, the number of infections in patients receiving GC was significantly higher (9.5% and 0.0%, respectively; p=0.009). The need for nonsteroidal anti-inflammatory drugs (NSAIDs) at the beginning of the study was in 93.2% of patients on average 21.1 days per month, after 24 weeks, the need for NSAIDs was in 54.4% of patients on average 3.8 days per month. In general, the safety profile of MTX was acceptable.Conclusion. With high RA activity, the tactics of starting therapy with sc MTX at a dose of 15 mg per week and a rapid escalation of its dose of 2.5 mg weekly to 22.5–25 mg/week, allows achieving therapy goals by 3 months in 17.8% of patients, and by 6 months in 54.5%, to quickly improve the QoL, reduce the level of pain, reduce the dose of GC by 3 months of therapy or completely cancel them, reduce the need for NSAIDs by 7 times with an acceptable level of therapy safety.
Introduction. Divozilimab, a humanized, afucosylated monoclonal antibody that targets CD20, has been approved to treat multiple sclerosis, neuromyelitis optica spectrum disorders (NMOSD), and systemic sclerosis. The AQUARELLE clinical trial evaluates the efficacy and safety of divozilimab in patients with NMOSD. Aim. The study aimed to evaluate the efficacy and safety of divozilimab during the first year of treatment in patients with NMOSD. Materials and methods. The AQUARELLE open-label clinical trial enrolled 105 patients with NMOSD. All patients received 500 mg of divozilimab via intravenous infusion every 24 weeks. After 1 year of treatment, the following key parameters were evaluated: the annualized relapse rate (ARR), time to first relapse compared to a historical control group (the placebo group from the SakuraStar study), the proportion of patients with confirmed disability worsening (CDW), changes in Expanded Disability Status Scale (EDSS) scores, and safety parameters. Results. The ARR in patients with NMOSD who received divozilimab for one year was 0.104 (95% confidence interval [CI], 0.056–0.193), and 90.5% of patients had no confirmed relapses. The calculated weighted ratio of ARR (90% CI) for divozilimab to the historical placebo (SAkuraStar) was 0.237 (90% CI: 0.084–0.672), demonstrating superiority of divozilimab. The EDSS score remained stable, and no cases of CDW were reported. Adverse events (AEs) were observed in 76% of patients, most of which were mild or moderate in severity. The most common adverse drug reactions were lymphopenia (21%), leukopenia (11.4%), neutropenia (12.4%), and infusion-related reactions (5.7%). No serious adverse reactions were reported. Conclusion. The 1-year interim results of divozilimab therapy in patients with NMOSD from the AQUARELLE clinical trial demonstrate the sustained efficacy of therapy in reducing both the rate and risk of NMOSD relapses. Additionally, the safety profile was consistent with that expected for anti-B-cell therapy.
The results of the Russian interdisciplinary consensus on the definitions of “difficult-to-manage” and “difficult-to-treat” psoriatic arthritis/psoriasis are presented. Rheumatology and dermatovenereology experts participated in a Delphi-method voting process.
Renal angiomyolipoma is a rare, benign mesenchymal tumor composed of smooth muscle fibers, thickened-walled blood vessels, and mature adipose tissue in varying proportions. Due to its very rare prevalence, angiomyolipoma is most often an incidental finding during examination. The primary diagnostic method is renal ultrasound (US), which is supplemented by magnetic resonance imaging (MRI) if necessary. Angiomyolipoma can cause severe pregnancy complications, such as spontaneous renal rupture, which poses a danger to both mother and fetus. This article presents a clinical case of ruptured angiomyolipoma of the right kidney in a 21-year-old woman on the fourth day after a spontaneous term delivery. This case presented clinical signs of bladder tamponade, right ureter, and right renal pelvicocalyceal system, as well as the discharge of liquid blood from the urethra. An emergency right nephrectomy and comprehensive etiopathogenetic treatment were performed in the postoperative period. This clinical case demonstrates a rare combination of pregnancy and renal angiomyolipoma. Therefore, it is advisable to more carefully investigation of the urinary system during prenatal screening and consider the possibility of developing such a serious complication as spontaneous renal rupture associated with angiomyolipoma.
