Background. The clinical and serologic heterogeneity of systemic lupus erythematosus (SLE) presents challenges for diagnosis, particularly in the earliest stages of the disease when there are insufficient signs to make a reliable diagnosis. Aim. To make a comparative assessment of sensitivity and specificity of various classification criteria of SLE on a cohort of patients of Nasonova Research Institute of Rheumatology. Materials and methods. A total of 252 patients were included in the study; 152 (60%) of 252 patients had reliable SLE (mean age 36 [29.546] years, duration of disease 9 [3.419] years). Of 252 patients, 26 (11%) had PAPS (mean age 36.5 [3142] years, duration of disease 4.6 [110.4] years). Systemic sclerosis was diagnosed in 74/252 (29%) patients, (mean age 51.5 [4259] years, duration of disease 9 [516] years). The quality of the classification function of the criteria was assessed by ROC analysis. Results. SLE was diagnosed in 131 (86%) of 152 patients using the American College of Rheumatology ACR)-1997 criteria, in 145 (95%) using the The Systemic Lupus International Collaborating Clinics (SLICC) 2012 criteria, and in 144 (94.7%) using the European League Against Rheumatism (EULAR)/ACR 2019 criteria. ANF positivity was the least statistically significant of all signs in relation to the diagnosis of SLE. The area under the curve (AUC) for ANF1/160 titers was AUC 0.654 for the ACR-97 criteria, AUC 0.616 for the SLICC-12 SLE criteria, and AUC 0.609 for the 2019 EULAR/ACR criteria. ROC analysis of the relationship between the number of criteria/points and a reliable diagnosis of SLE revealed a high diagnostic accuracy the AUC for all SLE criteria was greater than 0.940. In the ROC analysis of patients with SLE and PAFS, indicating the number of diagnostic criteria, sensitivity was 86% for ACR-1997, 95% for SLICC-2012, 95% for EULAR/ACR 2019, and specificity was 100, 62 and 62%, respectively. Conclusion. The classification criteria SLICC-2012 and EULAR/ACR 2019 are more sensitive for the diagnosis of SLE in the Russian population, and the criteria ACR-1997 are more specific. All three variants of the SLE classification criteria have sufficient sensitivity and specificity for their use in real clinical practice.

Introduction. The Global Antiphospholipid Syndrome Score (GAPSS) is a tool proposed to quantify the risk of clinical manifestations associated with antiphospholipid antibodies (aPL) and certain cardiovascular risk factors.Objective. To validate GAPSS in a cohort of patients with systemic lupus erythematosus in Russia.Material and methods. 115 patients with SLE were included in the study, including 51 (44%) patients with systemic lupus erythematosus (SLE) with antiphospholipid syndrome (APS), 14 (12%) – SLE with aPL, and 50 (44%) – SLE.Results. There was a history of thrombosis in 58 (50%) patients with 115, of them 14 (24%) had arterial thrombosis, 29 (50%) – venous, 15 (26%) – combined. Pregnancy against the background of the disease occurred in 43 women included in the study. Of them, 29 (67%) had obstetric pathology. Patients with thrombosis and obstetric pathology had a GAPSS score of 7.17±5.64, versus 4.48±4.55 without these manifestations (p=0.0003). There was a significant association between GAPSS levels and thrombosis – patients with thrombosis had a GAPSS of 7.31±5.70, those without thrombosis – 4.00±4.81 (p=0.001). GAPPS values were higher in arterial thrombosis compared to venous thrombosis (10.40±25.30 versus 5.82±5.28; p=0.01). GAPSS levels ≥6 and ≥10 were analyzed to select GAPSS values at which a high risk of recurrent thrombosis and/or obstetric pathology could be indicated. All GAPSS levels had a significant association with clinical manifestations of APS. The quality of GAPSS by ROC analysis showed an area under the curve (AUC) for GAPSS of 0.697.Conclusion. GAPSS can be used to assess the risk of recurrence or development of thrombosis and/or obstetric pathology in patients with SLE in the Russian Federation. The GAPSS ≥6 values should be used to stratify patients with SLE into high risk group for recurrence of vascular complications. Further prospective follow-up is needed to confirm the value of GAPSS.

Введение. Антифосфолипидный синдром (АФС) — аутоиммунная патология сосудов, клинически проявляющаяся рецидивирующими тромбозами сосудов любой локализации и калибра и акушерской патологией — рецидивирующими потерями плода. В последние 2 десятилетия в патогенетических аспектах АФС обсуждается роль системы комплемента. Цель исследования: определить связь между клинико-лабораторными проявлениями АФС и уровнем компонентов комплемента. Материалы и методы. Обследованы 111 пациентов: 87 (78%) женщин и 24 (22%) мужчины; 31 (28%) пациент был с первичным АФС (пАФС), 63 (57%) — с АФС в сочетании с системной красной волчанкой (СКВ) и 17 (15%) — с СКВ без АФС. У всех пациентов определяли антитела к кардиолипину (аКЛ) классов IgG и IgM и антитела к β2-гликопротеину 1 (аβ2-ГП1) классов IgG и IgM иммуноферментным анализом (ИФА), а также уровни С3 и С4 компонентов комплемента нефелометрическим методом. Результаты. У 72 пациентов в анамнезе были зарегистрированы тромбозы: у 23 пациентов с пАФС, у 44 с СКВ + АФС, у 5 с СКВ без АФС. Снижение уровня С3 было выявлено у 61 (55%) пациента из 111: у 14 (23%) пациентов с пАФС, у 35 (57%) с СКВ + АФС, у 12 (20%) с СКВ. Снижение С4 установлено у 37 (33%) из 111 пациентов: у 4 (11%) с пАФС, у 24 (65%) с СКВ + АФС, у 9 (24%) с СКВ. Снижение уровня С3 значимо чаще определено у пациентов c пАФС, позитивных по IgG-аКЛ и IgG-аβ2-ГП1 (р < 0,05). Снижение уровня С4 также ассоциировалось с позитивностью IgG-аКЛ и IgG-аβ2-ГП1 у пациентов с пАФС. У пациентов с СКВ + АФС гипокомплементемия С3 ассоциировалась с IgM-аКЛ и IgM-аβ2-ГП1, а снижение уровня С4 компонента комплемента существенно чаще зарегистрировано у пациентов с IgG-аКЛ (р = 0,002) и IgG-аβ2-ГП1 (р = 0,0001). Гипокомплементемия у пациентов с СКВ без антифосфолипидных антител имела место более чем в половине случаев: у 12 из 17 (71%) выявлено снижение С3 компонента комплемента и у 9 (53%) — снижение С4. Заключение. Зарегистрировано снижение С3 больше чем у половины (55%) и снижение С4 у трети (33%) обследованных пациентов, что свидетельствует об активации системы комплемента при АФС. Наличие IgG-аКЛ и IgG-аβ2-ГП1 ассоциировалось с гипокомплементемией, что свидетельствует о значимости этих антител в механизме активации комплемента и запуске гиперкоагуляции через систему комплемента. Background. Antiphospholipid syndrome (APS) is an autoimmune vascular pathology that is clinically manifested by recurrent vascular thrombosis and pregnancy loss. The role of complement system in the pathogenesis of APS is discussed in the last 2 decades. Objectives: to find out the relationships between the clinical and laboratory manifestations of APS and the level of complement components. Patients/Methods. We examined 111 patients: 87 (78%) women and 24 (22%) men; among them were 31 (28%) patients with primary APS (pAPS), 63 (57%) with APS and systemic lupus erythematosus (SLE) and 17 (15%) with SLE without APS. In all patients, antibodies to cardiolipin (aCL) of IgG and IgM classes and antibodies to β2-glycoprotein 1 (aβ2-GP1) components by the nephelometric method. Results. 72 patients had a history of thrombosis: 23 patients with pAPS, 44 with SLE + APS, 5 with SLE without APS. Decreased C3 level was detected in 61 (55%) of 111 patients: in 14 (23%) patients with pAPS, in 35 (57%) with SLE + APS, and in 12 (20%) with SLE. Decreased C4 level was observed in 37 (33%) of 111 patients: in 4 (11%) with pAPS, in 24 (65%) with SLE + APS, and in 9 (24%) with SLE. Decreased C3 level was significantly more often registered in pAPS-patients positive for IgG-aCL and IgG-aβ2-GP1 (p < 0.05). Decreased C4 level was also associated with positivity for IgG-aCL and IgG-aβ2-GP1 in pAPS-patients. In patients with SLE + APS, C3 hypocomplementemia was associated with IgM-аCL and IgM-аβ2-ГП1 and decreased C4 level was significantly more often registered in patients with IgG-aCL (p = 0.002) and IgG-аβ2-ГП1 (р = 0,0001). Hypocomplementemia in patients with SLE without antiphospholipid antibodies occurred in more than half of the cases: decreased C3 level was revealed in 12 of 17 (71%) patients, and decreased C4 level — in 9 (53%) patients. Conclusions. Decreased level of C3 was registered in more than half (55%) and a decreased level of C4 — in a third (33%) of examined patients, that indicated the complement system activation in APS. The presence of IgG-aCL and IgG-aβ2-GP1 was associated with hypocomplementemia that evidenced the significance of these antibodies in the mechanism of complement activation and the triggering of hypercoagulation through the complement system.

Skin lesions in systemic lupus erythematosus (SLE) are not just a cosmetic defect, accompanied by a deterioration in the quality of life and psychological discomfort, but, possibly, the first sign of a systemic course of disease. Systemic involvement can develop in patients with almost any type of cutaneous lupus erythematosus (CLE), requiring the study of CLE in combination with SLE. Dermatologists are the first to face with skin manifestations of SLE, while other organs and systems affection leads the patient to a rheumatologist. It is important to understand that skin lesions do not cause irreversible organ damage, but continuity of therapeutic approaches between a dermatologist and a rheumatologist is necessary. The review presents the clinical signs, diagnostic features and histological characteristics of CLE.

We presented two clinical cases with clinical manifestations of antiphospholipid syndrome (APS) and ankylosing spondylitis (AS). The peculiarity of these cases is the onset of diseases in childhood, as well as the presence of not only extra-skeletal manifestations, but also complications or manifestations of other pathology. In the first case, it was thrombosis of the superficial veins of the lower limbs with the development of postthrombotic syndrome. In the second case, aortic valve defect, as a result of aortitis with a dilatation of the ascending aorta, which led to aortic valve replacement and its subsequent dysfunction because of thrombosis of the valve prosthesis. The frequency of detection of antiphospholipid antibodies (aPL), APS and thrombosis in AS is discussed. The role of tumor necrosis factor α (TNFα) inhibitors in the induction of aPL synthesis and the development of APS in patients with AS is considered either. Separately, we discussed the role of TNFα inhibitors, which are the main drugs in the treatment of ankylosing spondylitis, in the induction of aPL synthesis and the development of APS. Data on the occurrence of aPL, the reasons for the development of thrombosis in APS and the role of TNFα inhibitors remains incomplete. Perhaps the combination of APS and AS is an underestimated problem, and the information available in the literature does not reflect the real numbers. It is obvious that further research is needed to improve the treatment of patients with AS with thrombosis.