Данные проспективного исследования особенностей системной красной волчанки у пациентов Кыргызстанa (Евразийский регистр РЕНЕССАНС)

Objective: to study and analyze the clinical and laboratory manifestations, course, and outcome of systemic lupus erythematosus (SLE) in patients living in Kyrgyzstan.Patients and methods. The prospective study included 150 young patients aged 34 [26, 44] years in a Kyrgyz cohort (KC) with SLE, the disease of which was 3.0 [0.7; 10] years. All clinical, laboratory, and instrumental data of patients, health-related quality of life (HRQOL) indicators, and treatment regimens were recorded in the international research base, such as British Lupus Integrated Prospective System (BLIPS). SLE activity was assessed using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K). At the end of the observation, the investigators assessed the following indicators: the number of exacerbations of SLE by the SELENA Flare Index (SFI); the onset of complete or drug-induced remission; the number of deaths; and the development of irreversible organ damages (IOD) according to the damage index (DI).Results and discussion. When seeing the physician for the first time, the KC included more patients with high (n=61 (40.66%)) and very high (n=40 (26.67%)) disease activity. Most (n=60 (40%)) patients were observed to have a subacute type of the course of the disease. At the first visit, the most common manifestations of SLE were damages to the skin (n=99 (72.67%)), serous membranes (n=91 (60.67%)), and lupus nephritis (n=79 (52.67%)). IODs were identified in 15.33% of the patients and were absent in 84.67%. IODs were more often due to the administration of glucocorticoids (GCs) in 43.48% of cases. However, GC therapy was not a predictor of organ damages (relative risk, 0.91; p>0.05). In the KC, the significant predictors of adverse outcomes were old-age onset SLE and its high activity, acute course, and frequent exacerbations.Conclusion. The KC patients had high and very high clinical and laboratory activities (40.6 and 26.6%, respectively), mainly those of acute and subacute SLE (32 and 40%, respectively), obvious immunological disorders. There was a preponderance of damages to the skin (73%), serous membranes (61%), and kidney (53%) among the clinical manifestations of SLE. IODs were found in 15.33% of patients at their study inclusion. These were more frequently represented by GC-induced changes. However, the ongoing GC therapy in the KC patients was not a predictor of organ damages. The significant predictors of an adverse outcome in our patients were old-age onset SLE and its high activity, acute course, and frequent exacerbations. Цель исследования – изучение и анализ клинико-лабораторных проявлений, течения и исхода системной красной волчанки (СКВ) у пациентов, проживающих в Кыргызстане.Пациенты и методы. В проспективное исследование включены 150 пациентов киргизской когорты (КК) с СКВ, молодого возраста (медиана возраста – 34 [26; 44] года, длительности заболевания – 3,0 [0,7; 10] года). Все клинико-лабораторные и инструментальные данные больных, показатели качества жизни, связанного со здоровьем (КЖСЗ), и схемы терапии регистрировались в международной исследовательской базе – British Lupus Integrated Prospective System (BLIPS). Активность СКВ оценивалась по индексу SLEDAI-2K. В конце наблюдения оценивались: число обострений СКВ по индексу SELENA Flare Index (SFI); наступление ремиссии (полной или медикаментозной); число летальных исходов; развитие необратимых органных повреждений (НП) по индексу повреждения (ИП).Результаты и обсуждение. При первичном обращении в КК преобладали пациенты с высокой (n=61; 40,66%) и очень высокой (n=40; 26,67%) активностью заболевания. У большинства (n=60; 40%) наблюдался подострый вариант течения болезни. Во время первого визита наиболее частыми проявлениями СКВ были поражение кожи (n=99; 72,67%), серозных оболочек (n=91; 60,67%) и волчаночный нефрит (ВН; n=79; 52,67%). НП выявлены у 15,33% больных, у 84,67% они отсутствовали. НП чаще (в 43,48% случаев) были обусловлены приемом глюкокортикоидов (ГК). Однако терапия ГК не была предиктором органных повреждений (относительный риск 0,91; p>0,05). В КК значимыми предикторами неблагоприятного исхода явились старший возраст пациентов в дебюте болезни, высокая активность, острое течение и частые обострения СКВ.Заключение. Пациенты КК отличались наличием высокой и очень высокой клинико-лабораторной активности (40,6 и 26,6% соответственно), преимущественно острого и подострого вариантов течения СКВ (32 и 40% соответственно), выраженными иммунологическими нарушениями. Среди клинических проявлений СКВ у них преобладало поражение кожи (73%), серозных оболочек (61%) и почек (53%). НП на момент включения выявлены у 15,33% больных. Чаще они были представлены изменениями, обусловленными приемом ГК. Однако проводимая терапия ГК в КК больных не была предиктором органных повреждений. Значимыми предикторами неблагоприятного исхода у наших пациентов являлись старший возраст пациентов в дебюте болезни, высокая активность, острое течение и частые обострения СКВ.

Data from a prospective study of the features of systemic lupus erythematosus in patients of Kyrgyzstan (Eurasian RENAISSANCE Register)

Objective: to study and analyze the clinical and laboratory manifestations, course, and outcome of systemic lupus erythematosus (SLE) in patients living in Kyrgyzstan.Patients and methods. The prospective study included 150 young patients aged 34 [26, 44] years in a Kyrgyz cohort (KC) with SLE, the disease of which was 3.0 [0.7; 10] years. All clinical, laboratory, and instrumental data of patients, health-related quality of life (HRQOL) indicators, and treatment regimens were recorded in the international research base, such as British Lupus Integrated Prospective System (BLIPS). SLE activity was assessed using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K). At the end of the observation, the investigators assessed the following indicators: the number of exacerbations of SLE by the SELENA Flare Index (SFI); the onset of complete or drug-induced remission; the number of deaths; and the development of irreversible organ damages (IOD) according to the damage index (DI).Results and discussion. When seeing the physician for the first time, the KC included more patients with high (n=61 (40.66%)) and very high (n=40 (26.67%)) disease activity. Most (n=60 (40%)) patients were observed to have a subacute type of the course of the disease. At the first visit, the most common manifestations of SLE were damages to the skin (n=99 (72.67%)), serous membranes (n=91 (60.67%)), and lupus nephritis (n=79 (52.67%)). IODs were identified in 15.33% of the patients and were absent in 84.67%. IODs were more often due to the administration of glucocorticoids (GCs) in 43.48% of cases. However, GC therapy was not a predictor of organ damages (relative risk, 0.91; p>0.05). In the KC, the significant predictors of adverse outcomes were old-age onset SLE and its high activity, acute course, and frequent exacerbations.Conclusion. The KC patients had high and very high clinical and laboratory activities (40.6 and 26.6%, respectively), mainly those of acute and subacute SLE (32 and 40%, respectively), obvious immunological disorders. There was a preponderance of damages to the skin (73%), serous membranes (61%), and kidney (53%) among the clinical manifestations of SLE. IODs were found in 15.33% of patients at their study inclusion. These were more frequently represented by GC-induced changes. However, the ongoing GC therapy in the KC patients was not a predictor of organ damages. The significant predictors of an adverse outcome in our patients were old-age onset SLE and its high activity, acute course, and frequent exacerbations

Myocardial tissue damage in rabbits injected with group A streptococci, types M1 and M22. Role of bacterial immunoglobuhn G-binding surface proteins

Acute rheumatic fever (ARF) and acute poststreptococcal glomerulonephritis (APSGN), two important sequelae of streptococcal throat or skin infections, according to current concepts may be elicited by autoimmune mechanisms due to molecular mimicry between group A streptococci (GAS) and human tissue. In the case of APSGN, however, Our experimental data have indicated that GAS immunoglobulin-binding surface proteins (IgG Bps) might be of pathogenic significance by triggering anti-IgG production and immune complex formation leading to renal damage. Thus, rabbits injected with IEG-binding, as opposed to non-binding, GAS strains were found to develop renal deposition of IgG and complement factor C3 and inflammatory and degenerative glomerular changes resembling the picture seen in APSGN. In the present study, cardiac tissue material from rabbits injected with GAS was investigated. After 8 or more weeks of intravenous (i.v.) injections, minimal changes were seen in those animals receiving an IgG non-binding GAS strain, type T27, whereas those animals receiving either of two IgG-binding GAS strains, types M1 or M22, developed strong inflammatory and degenerative myocardial changes accompanied by deposition of IgG and C3. Furthermore, on injecting rabbits with defined mutants of a type M22 strain, the development of myocardial tissue damage proved to be dependent on the presence of streptococcal IgG-binding activity. Our results demonstrate that myocardial tissue changes may be induced in the rabbit by i.v. injection of whole heat-killed GAS of at least two M serotypes. Conceivably, induction of immune complexes by bacterial IqG BPs may lead to myocardial deposition of IgG, in turn triggering a series of events, involving the complement system and proinflammatory cytokines, with resulting tissue damage. Though many virulence factors may be involved in the development of ARF and APSGN, and a given GAS strain will never cause both, our results may suggest a new pathogenetic mechanism common to these two major non-suppurative complications