M. M. Kulshymanova

Objective – to evaluate the features of clinical manifestations, course, outcomes and quality of life related to health in patients with systemic lupus erythematosus in the Republic of Kazakhstan.Patients and methods. The study included 102 patients with systemic lupus erythematosus (SLE) with a reliable diagnosis according to SLICC (2012). Disease activity was assessed by the SLEDAI 2K index, organ damage (IOD) by SLICC/ACR (2000). Statistical processing was carried out using SPSS 13 software (IBM Corp., USA). Variables with a parametric distribution are presented as М±SD, nonparametric – as a median (Me) [25th; 75th percentile].Results and discussion. The cohort was dominated by female patients (98%), Asians (83.33%), young patients (33.85±10.58 years) with a disease duration of 5 [2; 9] years with high (30.8%) and very high (39.2%) degree of activity (SLEDAI-2K – 17.64±8.80 points). The debut of the disease was in 18.6% of patients in adolescence, it was characterized by an unfavorable course. Clinical manifestations of the disease: skin lesions (acute active and chronic forms) (98%), joints (79.4%), non-scarring alopecia (75.5%), neuropsychiatric disorders (49%), mucous membranes (46.1%), hematological (54.9%) and immunological disorders (100%). IOD: low – in 20.6%, medium – in 59.8%, high – in 9.8% of patients, 0 – in 9.8%, Risk factors for poor outcome were in 93.1% of patients. Assessment of health-related quality of life (HRQOL) in SLE patients showed a significant decrease on all scales. Correction of the treatment program, taking into account the factors of adverse outcome (FRNI), consisted in strengthening therapy with the inclusion of genetically engineered biological drugs (GEBP).Conclusion. SLE is a socially significant disease in Kazakhstan with a high incidence rate (101%) over 10 years (2009–2018). The cohort of SLE patients is dominated by young people, females. The duration of the disease is up to 5 years with a delayed verification of the diagnosis of SLE. Organ damage is already in the onset of the disease and the presence of FRNI of the disease in 93.1% of patients, which indicates the severity of the course, which requires early diagnosis and active involvement of pathogenetic treatment, including GEBD.

Objective: to study and analyze the dynamics of prevalence, demographic, social, and clinical manifestations of systemic lupus erythematosus (SLE) in patients living in Kazakhstan.Materials and methods. Official materials of the Ministry of Health of the Republic of Kazakhstan (2009–2018): statistical compendiums, a consolidated reporting form for medical treatment (N 12). 102 patients (100 women and 2 men) were included in the register of SLE with reliable SLE according to SLICC (ACR, 2012). Assessment of the debut of the disease was carried out according to archival material (medical history, outpatient records) of patients. The activity of the process was evaluated according to SLEDAI-2K, organ damage according to SLICC/ACR (2000).Results. The total number of patients with SLE over 10 years (2009–2018) more than doubled, the growth rate was 101%. The overwhelming majority of patients with SLE are women (91%). In the study group, Asians (83.33%) predominated by race, of which Kazakhs (76.47%) were young (mean age – 33.85±10.58 years), with a disease duration of 5 (2; 9) years, time from the appearance of the first signs to verification of SLE – 4.5 (3; 12) years. The prevailing acute (49.0%), subacute (33.3%), rather than chronic (18.7%) variants of the course, with high activity according to SLEDAI-2K – 17.64±8.80. The most common clinical manifestations of the disease: skin lesions (98%), joints (79.4%), nervous system (49%), hematological (54.9%) and immunological (100%) disorders. The absence of organ damage (0 points) was detected in 10 (9.8%) cases, low (1 point) – in 21 (20.6%), medium (2–4) – in 61 (59.8%), high (over 4) – in 10 (9.8%) patients.Conclusion. SLE remains a socially significant disease in Kazakhstan, as evidenced by the dynamics of the increase in incidence (101%) over 10 years (2009–2018). The prevalence of SLE is 24.7 per 100 thousand of the country’s population, lower than in other countries with a predominant Asian population or in comparison with Asian populations. A cohort of patients with SLE was represented by people of the Asian race (83.33%), Kazakhs (76.47%), and young people (33.85±10.58). The analysis revealed a delayed verification of the diagnosis of SLE (on average 4.5 (3; 12) years). Acute variants of the course of the disease with high disease activity according to SLEDAI-2K prevail. Common clinical manifestations of SLE are skin lesions, both acute and chronic (98%), joints (79.4%), damage to the nervous system (49%), hematological (54.9%) and immunological disorders (100%).