E V Bolotova, E V Yakovleva, E K Ilinykh, Т A Rassovskaya
{"title":"[Efficacy and safety of levilimab in the treatment of patients with rheumatoid arthritis].","authors":"E V Bolotova, E V Yakovleva, E K Ilinykh, Т A Rassovskaya","doi":"10.26442/00403660.2024.07.202907","DOIUrl":"https://doi.org/10.26442/00403660.2024.07.202907","url":null,"abstract":"<p><strong>Aim: </strong>Evaluation in real clinical practice of the effectiveness and safety of levilimab therapy in patients with highly and moderately active rheumatoid arthritis (RA).</p><p><strong>Materials and methods: </strong>A prospective observational study (6 months) involving 35 patients with RA (29 women and 6 men, mean age 53.17±13.2 years) who were treated at the Ochapovsky Regional Clinic Hospital of Krasnodar Region. All patients included in the study were prescribed the drug levilimab (Ilsira).</p><p><strong>Results: </strong>After 1 month of observation, there was a decrease in the clinical and laboratory activity of the process in the form of a decrease in the number of painful joints - 17.0 (14.0; 20.0) vs 8.0 (6.0; 10.0); <i>p</i>=0.000001, number of swollen joints - 3.0 (2.0; 4.0) vs 0.0 (0.0; 0.0); <i>p</i>=0.000002, reduction in pain intensity according to visual analog scale - 60.0 (60.0; 70.0) mm vs 30.0 (20.0; 40.0) mm (<i>p</i>=0.000001). Also, by the end of the first month of therapy, there was a decrease in clinical activity indices DAS28-ESR by 43%, SDAI by 60%, CDAI by 55%. Positive dynamics of laboratory parameters were noted - a decrease in erythrocyte sedimentation rate by 76%, a decrease in C-reactive protein level by 98%. By the 6th month of therapy, a decrease in RF by 36% and ACCP by 11% was recorded, but the dynamics of these indicators did not reach statistical significance. By the end of 4 weeks of treatment, 24 (68.6%) patients showed an increase in the level of total blood cholesterol - 5.1 (3.91; 6.0) mmol/L vs 6.1 (4.99; 7.07) mmol/L (<i>p</i>=0.000006), while 11 (45.8%) patients from this group had initially elevated cholesterol levels (6.4±0.6 mmol/L). In 5 (14.3%) patients, an increase in alanine aminotransferase (ALT) was recorded in the 4th week - 17.0 (11.0; 25.0) U/L vs 32.0 (22.0; 43.0) U/L (<i>p</i>=0.000062) and aspartate aminotransferase (AST) - 19.0 (14.0; 24.0) U/L vs 25.0 (18.0; 36.0) U/L (<i>p</i>=0.000171), in 1 (2.85%) of the patient, an increase in ALT and AST above normal was noted (ALT 144 U/L, AST 52 U/L), which required discontinuation of levilimab. In 2 (5.7%) patients, by the end of the 4th week a decrease in the absolute number of neutrophils was registered - 3.2 (2.6; 4.0)×10E<sup>9</sup>/L vs 2.3 (2.0; 2.5)×10E<sup>9</sup>/L (<i>p</i>=0.002), which did not require discontinuation of treatment, since the number of cells remained more than 1×10E<sup>9</sup>/L. During treatment with levilimab 162 mg subcutaneously once a week, the proportion of patients taking prednisolone decreased from 46% at the start of therapy to 11% at the end of 6 months of therapy.</p><p><strong>Conclusion: </strong>Levilimab is a highly effective drug for the treatment of patients with highly and moderately active RA and has a favorable tolerability and safety profile.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":null,"pages":null},"PeriodicalIF":0.3,"publicationDate":"2024-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141898328","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
V I Podzolkov, M V Vetluzhskaya, I D Medvedev, A A Abramova, G A Kislenko
{"title":"[Dyspnea in post-COVID-19 patients: A review].","authors":"V I Podzolkov, M V Vetluzhskaya, I D Medvedev, A A Abramova, G A Kislenko","doi":"10.26442/00403660.2024.07.202785","DOIUrl":"https://doi.org/10.26442/00403660.2024.07.202785","url":null,"abstract":"<p><p>New coronavirus infection may lead to long-term consequences, particularly to post-COVID syndrome, one of the most common manifestations of which is dyspnea. Post-COVID-19 shortness of breath may persist from one to several months and even years that results in low quality of life of patients. The review highlights possible risk factors and causes of dyspnea in post-COVID period such as lung damage, cardiovascular pathology, hyperventilation syndrome, dysfunction of the autonomic nervous system, detraining, anemia, etc. The authors present data about COVID-19-associated causes of dyspnea and severity of acute COVID-19. The review emphasizes the importance of a multidisciplinary approach to the diagnosis and treatment of patients with shortness of breath in post-COVID-19 period.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":null,"pages":null},"PeriodicalIF":0.3,"publicationDate":"2024-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141898286","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[4D flow MRI: value and clinical perspectives in patients with pathology of the heart and great vessels (part 2): A review].","authors":"L A Yurpolskaya","doi":"10.26442/00403660.2024.07.202786","DOIUrl":"https://doi.org/10.26442/00403660.2024.07.202786","url":null,"abstract":"<p><p>The study of blood flow is becoming a new trend in cardiology and cardiovascular surgery. Based on the literature and our own data, a review is presented on the use of 4D flow in diseases of the heart and blood vessels. The main state of the question about the features of the application of the technique in various pathologies of the cardiovascular system is described in detail, the priorities, limitations and promising directions of the technique application are considered taking into account the goals of practical medicine. The review consists of two parts. The first is devoted to general issues, limitations of the technique, and issues of 4D flow mapping in patients with lesions of the great vessels. In the second part, the emphasis is on the use of 4D flow MRI in the study of intraventricular blood flow and the application of the technique in congenital heart and vascular diseases.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":null,"pages":null},"PeriodicalIF":0.3,"publicationDate":"2024-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141898282","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
E M Mezhonov, O M Reitblat, Y A Vyalkina, A A Airapetian, N V Lazareva, F T Ageev, Z N Blankova, O N Svirida, Y S Prints, I V Zhirov, S N Tereshchenko, S A Boytsov
{"title":"[Chronic kidney disease and chronic heart failure: impact on prognosis and choice of pathogenetic therapy].","authors":"E M Mezhonov, O M Reitblat, Y A Vyalkina, A A Airapetian, N V Lazareva, F T Ageev, Z N Blankova, O N Svirida, Y S Prints, I V Zhirov, S N Tereshchenko, S A Boytsov","doi":"10.26442/00403660.2024.07.202781","DOIUrl":"https://doi.org/10.26442/00403660.2024.07.202781","url":null,"abstract":"<p><strong>Aim: </strong>To evaluate the impact of a decrease in glomerular filtration rate (GFR) on the prognosis of patients with chronic heart failure (CHF), to analyze real clinical practice regarding the frequency of prescribing pathogenetic therapy for CHF, achieving target dosages depending on the gradation of GFR in patients included in the CHF Register of the Tyumen region.</p><p><strong>Materials and methods: </strong>The analysis included medical data of 4077 patients (1662 men and 2415 women) with NYHA class I-IV CHF who underwent examination and treatment in medical organizations of the Tyumen region for the period from January 2020 to May 2023. Criteria for inclusion in the register: proven heart failure. Chronic kidney disease (CKD) was assessed by GFR calculated using the CKD-EPI formula (ml/min/1.73 m<sup>2</sup>). The primary end point was defined as death from all causes.</p><p><strong>Results: </strong>GFR<60 ml/min/1.73 m<sup>2</sup> was recorded in 34.6% of patients, more common in women (40.2 and 26.6%, respectively; <i>p</i><0.001). When dividing patients into phenotypes according to LVEF, no statistically significant differences were found in the distribution of patients according to GFR. In patients with HFrEF and HFpEF GFR<45 ml/min/1.73 m<sup>2</sup> was associated with an increased risk of meeting the endpoint. Analysis of prescribed pathogenetic therapy showed that in patients with HFrEF, the frequency of prescription of ACE inhibitors, â-blockers and MRA decreased (<i>p</i>=0.023, 006 and 0.01, respectively), and ARNI, on the contrary, increased with a decrease in GFR (<i>p</i>=0.026). In patients with HFpEF, a similar trend towards a decrease in the frequency of prescription of ACEIs and MCBs with a decrease in GFR (<i>p</i><0.001) remained, but it was compensated by an inversely proportional increase in the frequency of prescription of ARBs (<i>p</i><0.001). 100% of the target dosage is achieved in more than 90% of patients taking MRA across the entire LVEF range. While for â-blockers and ARNI/ACE/ARB the percentage of patients receiving the full therapeutic dosage of drugs is significantly lower. When analyzing target dosages of pathogenetic drugs, gradations of achieved doses were distributed evenly throughout the entire range of GFR.</p><p><strong>Conclusion: </strong>GFR<60 ml/min/1.73 m<sup>2</sup> occurs in every 3 patients with CHF across the entire range of LVEF. A decrease in GFR worsens the prognosis of patients with both HFrEF and HFpEF, increasing in direct proportion with the severity of the stage of CKD. Inclusion of patients in the monitoring program within the framework of the CHF service allows the treatment to be significantly brought closer to optimal drug therapy, at the same time, certain efforts are required to overcome difficulties with titration to target dosages.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":null,"pages":null},"PeriodicalIF":0.3,"publicationDate":"2024-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141898283","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A A Safiullina, T M Uskach, O V Sapelnikov, M A Saidova, A A Ansheles, V B Sergienko, V A Amanatova, I R Grishin, D I Cherkashin, R S Akchurin, S N Tereschenko
{"title":"[Improvement of the technique of positioning the endocardial electrodes of the cardiac contractility modulation device in patients with CHF with reduced ejection fraction and atrial fibrillation].","authors":"A A Safiullina, T M Uskach, O V Sapelnikov, M A Saidova, A A Ansheles, V B Sergienko, V A Amanatova, I R Grishin, D I Cherkashin, R S Akchurin, S N Tereschenko","doi":"10.26442/00403660.2024.07.202782","DOIUrl":"https://doi.org/10.26442/00403660.2024.07.202782","url":null,"abstract":"<p><strong>Aim: </strong>To evaluate the efficacy and safety of the advanced technique for positioning the endocardial electrodes of a cardiac contractility modulation (CCM) device.</p><p><strong>Materials and methods: </strong>The CCM system was implanted in 100 patients, of which 60 CCM electrodes were positioned in the most optimal zones of myocardial perfusion, in particular, in the zone of the minor focal-scar/fibrotic lesion (the Summed Rest Score of 0 to 1-2, the intensity of the radiopharmaceutical at least 30%), and in 40 patients according to the standard procedure. Before the implantation of the CCM system, 60 patients underwent tomography (S-SPECT) of the myocardium with <sup>99m</sup>Tc-methoxy-isobutyl-isonitrile at rest to determine the most optimal electrode positioning zones and 100 patients underwent transthoracic echocardiography at baseline and after 12 months to assess the effectiveness of surgical treatment.</p><p><strong>Results: </strong>Improved ventricular electrode positioning technique is associated with the best reverse remodeling of the left ventricular myocardium, especially in patients with ischemic chronic heart failure, with less radiation exposure to the surgeon and the patient, and without electrode-related complications.</p><p><strong>Conclusion: </strong>At the preoperative stage, it is recommended to perform a synchronized single-photon emission computed tomography of the myocardium with <sup>99m</sup>Tc-methoxy-isobutyl-isonitrile at rest before implantation of the CCM device to assess the presence of scar zones/myocardial fibrosis in the anterior and inferior septal regions of the interventricular septum of the left ventricle, followed by implantation of ventricular electrodes in the zone of the minor scar/fibrous lesion, which will allow to achieve optimal stimulation parameters, increase the effectiveness of CCM therapy, reduce the radiation exposure on medical personnel and the patient during surgery.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":null,"pages":null},"PeriodicalIF":0.3,"publicationDate":"2024-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141898331","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
D P Golubovskaya, E V Dren', A V Yurkina, T B Pecherina, O L Barbarash
{"title":"[Clinical case of generalized amyloidosis (ATTR-amyloidosis) with a progressive course of chronic heart failure. Case report].","authors":"D P Golubovskaya, E V Dren', A V Yurkina, T B Pecherina, O L Barbarash","doi":"10.26442/00403660.2024.07.202784","DOIUrl":"10.26442/00403660.2024.07.202784","url":null,"abstract":"<p><p>Despite the presence of various signs of cardiac amyloidosis (\"red flags\"), the introduction into routine practice of new non-invasive diagnostic methods (Speckle Tracking technology using echocardiography, myocardial scintigraphy with technetium pyrophosphate, genetic testing, screening for free light chains of immunoglobulins to exclude AL-amyloidosis), which have high specificity and sensitivity, transthyretinic (ATTR) cardiomyopathy is still a difficult to diagnose disease, especially in the early stages when treatment is most effective. The article presents a clinical case of ATTR-amyloidosis with predominant heart damage, manifested by severe diastolic heart failure resistant to treatment. The timing, from the moment of the first episode of decompensation of heart failure to death, is 4 months, which confirms the rapid progression of severe biventricular dysfunction of the heart. Despite the presence of cardiac and extracardial \"red flags\" of ATTR-amyloidosis in the patient, the diagnosis was established at autopsy. The paper analyzes possible errors of early diagnosis at the outpatient and inpatient stages of patient management.</p>","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":null,"pages":null},"PeriodicalIF":0.3,"publicationDate":"2024-07-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141898284","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
E. Kamyshova, I. N. Bobkova, P. Kakhsurueva, Amnat S. Abdulaeva, T. Rudenko, E. Stavrovskaya, E. Y. Andreeva, O.A. Li, A. Suvorov
{"title":"Idiopathic membranous nephropathy with focal segmental sclerosis","authors":"E. Kamyshova, I. N. Bobkova, P. Kakhsurueva, Amnat S. Abdulaeva, T. Rudenko, E. Stavrovskaya, E. Y. Andreeva, O.A. Li, A. Suvorov","doi":"10.26442/00403660.2024.06.202725","DOIUrl":"https://doi.org/10.26442/00403660.2024.06.202725","url":null,"abstract":"Aim. To evaluate the clinical and pathological features and prognosis of idiopathic membranous nephropathy (IMN) with focal segmental sclerosis (FSGS) in a group of Russian patients. \u0000Materials and methods. 101 patients with morphologically verified IMN were enrolled in our single-center cohort retrospective study. The patients were divided into IMN group and IMN+FSGS group. The primary and secondary outcomes were analyzed in 59 patients, which had follow-up data for period more than 6 months. \u0000Results. At the time of renal biopsy the median age was 46.0 (33.0; 55.0) years and the median follow-up was 6.8 (4.0; 15.6) months. Secondary FSGS was revealed in 15 (14.9%) patients with IMN. The IMN and IMN+FSGS groups did not differ in gender, age of onset IMN and age of renal biopsy. In the IMN+FSGS group proteinuria was higher and estimated glomerular filtration rate was lower than that in the IMN group (p0.05). The systolic arterial pressure and creatinine levels in the IMN+FSGS group were slightly higher than in the IMN group, but the difference was not significant. Anti-PLA2R positivity was similar in both groups. Chronic kidney disease (CKD) progression was observed in 10/52 (19.2%) and 5/7 (71.4%) patients in IMN and IMN+FSGS groups, respectively. In a multivariate Cox regression model, age of renal biopsy (odds ratio – OR 1.12, 95% confidence interval – CI 1.03–1.22; р=0.07), FSGS (OR 0.05, 95% CI 0.01–0.34; р=0.002) и response to initial course of immunosuppression (OR 0.33, 95% CI 0.12–0.95; р=0.039) were associated with the CKD progression. \u0000Conclusion. In patients with IMN secondary FSGS is associated with a greater severity of proteinuria and a decrease in estimated glomerular filtration rate, and is also an independent factor of the CKD progression.","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":null,"pages":null},"PeriodicalIF":0.3,"publicationDate":"2024-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141671607","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
N. Chebotareva, Evgenia A. Kharionovskaya, Evgenia A. Biryukova, S. Berns, T. Vuimo
{"title":"Comparison of thrombodynamic methods and routine hemostasis tests in the evaluation of hypercoagulable syndrome in chronic glomerulonephritis","authors":"N. Chebotareva, Evgenia A. Kharionovskaya, Evgenia A. Biryukova, S. Berns, T. Vuimo","doi":"10.26442/00403660.2024.06.202723","DOIUrl":"https://doi.org/10.26442/00403660.2024.06.202723","url":null,"abstract":"Background. Nephrotic syndrome (NS) is associated with a high risk of thrombotic complications. In this group of patients, routine local tests for assessing hemostasis do not accurately reflect hypercoagulable state. Global functional tests for assessing hemostasis, including thrombodynamics (TD), are considered promising for assessing disorders in the blood coagulation system of these patients. \u0000Aim. To compare the rate of hypercoagulability according to routine hemostatic tests and TD and to evaluate the factors associated with increased risk of thrombotic complications in patients with chronic glomerulonephritis (CGN). \u0000Materials and methods. The study included 94 patients with active CGN who were not receiving anticoagulant therapy; 63 (80.3%) patients had NS, and 31 (19.7%) had active CGN without NS. Hemostasis parameters were assessed using local coagulation tests and TD test. Using logistic regression analysis, factors associated with the risk of thrombosis were assessed. \u0000Results. Of the 94 patients with active CGN in 63 without preventive anticoagulant therapy, hypercoagulability according to routine tests was detected in 6 (9.5%) patients with NS and in 3 (9.7%) patients without NS (p0.05). Hypercoagulability according to the TD test was detected in 24 (53.9%) patients with NS and in 5 (32.2%) without NS (p0.05). The formation of spontaneous clots was observed in 29 (30.9%) of patients with CGN, most of them 24 (83%) with NS. 10.6% of patients in our cohort experienced thromboembolic events. The risk of thromboembolic events according to the univariate regression analysis was associated with older age, higher lipid levels, use of glucocorticosteroids and detection of spontaneous clots by the TD test. No association of thromboembolic events with abnormalities in routine hemostasis tests was obtained. \u0000Conclusion. In patients with CGN with nephrotic syndrome, hypercoagulability is detected in 9.5% of cases with routine coagulation tests and in 53.9% of cases with TD test. Detection of spontaneous clots by TD test is associated with a risk of thromboembolic events.","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":null,"pages":null},"PeriodicalIF":0.3,"publicationDate":"2024-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141670845","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Akaeva, N. Kozlovskaya, L. Bobrova, Olga A. Vorobyeva, E. S. Stoliarevich, P. Shatalov, Tatiana V. Smirnova, Anastasiia O. Anan'eva
{"title":"Clinical characteristics and genetic profile of complement system in renal thrombotic microangiopathy in patients with severe forms of arterial hypertension","authors":"M. Akaeva, N. Kozlovskaya, L. Bobrova, Olga A. Vorobyeva, E. S. Stoliarevich, P. Shatalov, Tatiana V. Smirnova, Anastasiia O. Anan'eva","doi":"10.26442/00403660.2024.06.202724","DOIUrl":"https://doi.org/10.26442/00403660.2024.06.202724","url":null,"abstract":"Background. The spectrum of diseases characterized by the development of renal thrombotic microangiopathy (TMA) encompasses the malignant hypertension (MHT). TMA in MHT has conventionally been regarded as a variation of secondary TMA, the treatment of which is restricted to the stabilization of blood pressure levels, a measure that frequently fails to prevent the rapid progression to end-stage renal disease in patients. Nevertheless, there exists a rationale to suggest that, in certain instances, endothelial damage in MHT might be rooted in the dysregulation of the complement system (CS), thereby presenting potential opportunities for the implementation of complement-blocking therapy. \u0000Aim. To study clinical manifestations and genetic profile of CS in patients with morphologically confirmed renal TMA combined with severe AH. \u0000Materials and methods. 28 patients with morphologically verified renal TMA and severe AH were enrolled to the study. Patients with signs of microangiopathic hemolysis and thrombocytopenia were not included in the study due to possible compliance with the criteria for atypical hemolytic uremic syndrome (aHUS). The prevalence of rare genetic defects (GD) of the CS was assessed by molecular genetic analysis (search for mutations in the clinically significant part of the human genome – exome) by next-generation sequencing technology (NGS). \u0000Results. GD of CS were detected in a quarter of patients. Rare genetic variants classified as “likely pathogenic” including defects in CFI, C3, CD46, CFHR4, CFHR5 genes were detected in five cases. Two patients were found to have chromosomal deletions containing CFH-related proteins genes (CFHR1, CFHR3). \u0000Conclusion. Rare variants of CS genes linked to aHUS were found in 25% of patients with renal TMA, the genesis of which was originally thought to be secondary and attributed to MHT, with partial or complete absence of hematological manifestations of microangiopathic pathology. The key to confirming TMA associated with MHT, particularly in the absence of microangiopathic hemolysis and thrombocytopenia, elucidating its nature, and potentially effective complement-blocking therapy in patients with GD of CS, appears to be a genetic study of CS combined with a morphological study of a renal biopsy.","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":null,"pages":null},"PeriodicalIF":0.3,"publicationDate":"2024-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141670260","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Lupus nephritis and thrombotic microangiopathy: A review","authors":"L. Bobrova, N. Kozlovskaya","doi":"10.26442/00403660.2024.06.202731","DOIUrl":"https://doi.org/10.26442/00403660.2024.06.202731","url":null,"abstract":"Lupus nephritis (LN) is one of the most common organ-specific manifestations of systemic lupus erythematosus (SLE). Various clinical signs of LN develop in at least 50% of patients with SLE. In addition to LN, the spectrum of renal lesions associated with SLE also includes vascular pathology. One of the variants of renal microvascular injury is thrombotic microangiopathy (TMA), the mechanisms of which are diverse. The review focuses on the main forms of TMA, including antiphospholipid syndrome and nephropathy associated with antiphospholipid syndrome, TMA caused by complement system regulation disorders and deficiency of ADAMTS13. In most cases, these forms of TMA are combined with LN. However, they may also exist as a single form of kidney damage. This article discusses the TMA pathogenesis, the impact on kidney prognosis, and treatment options.","PeriodicalId":22209,"journal":{"name":"Terapevticheskii Arkhiv","volume":null,"pages":null},"PeriodicalIF":0.3,"publicationDate":"2024-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141670851","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}