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The article is devoted to ParryRomberg syndrome, one of the rare diseases. Here we declare contemporary views on predisposing factors, pathogenesis and character of clinical performance. We describe the methods of differential diagnostics, the criteria of diagnostics as well as contemporary treatment methods. We also describe a case history of the disease.

A study of the international literature on texture analysis was performed, and the reported data was compared to the findings of radiomics studies performed by the specialists of our institute. The relevant papers were searched using a combination of the following search terms: “radiomics”, “radiology”, “texture analysis”, “perspectives”, and “clinical implementation”. The search was limited to papers published in English within the last 5 years, which essentially focused on liver and pancreas disorders. Due to the publication of new data on a fairly daily basis, the topic has not lost its relevance. The vast majority of authors confirm that radiomics can be efficiently used during diagnosis, treatment planning, and patient monitoring. However, consensus on the implementation of radiomics has not been reached yet, thereby delaying its introduction into clinical practice. The data collected in our institution reports that the clinical application of texture analysis methods may be very promising.

The article discusses one of the variants of overlap syndrome – a combination of symptoms of ankylosing spondylitis (AS) and rheumatoid arthritis (RA). Three clinical cases are described, in these cases characteristic signs of both diseases were noted and the criteria of both diseases were met. The genetic factors and immunopathogenetic mechanisms underlying AS and RA are analyzed. A review of the literature on the combination of AS and RA is presented.

The paper highlights the relationship between HLA-B27-associated uveitis and spondyloarthritis (SpA). It presents the genetic aspects of inflammation of the eyes and musculoskeletal system, the role of the HLA-B27 histocompatibility antigen, and the contribution of other genes to the development of uveitis and spondylitis. The authors characterize the general pathogenetic mechanisms of the development of these diseases: the importance of factors of innate immunity, proinflammatory cytokines in the initiation of inflammation of the eyes and joints. The detection rate of SpA is analyzed in various forms of uveitis; the predictors of SpA development are identified in the patients. An algorithm is presented, which makes it possible to timely diagnose SpA in the presence of acute anterior uveitis. HLA-B27, inflammation in the ocular anterior chamber, unilateral alternating eye lesions, an acute recurrent course, and uveitis onset at the age of less than 30 years are singled out as the most significant signs associated with SpA in patients with uveitis. The features of the course and the frequency of complications in uveitis with and without SpA are compared. It is shown that exacerbations of uveitis and associated complications occur statistically significantly more frequently in SpA. There is evidence for the need for a close interaction between ophthalmologists and rheumatologists in the management of patients with uveitis.

BackgroundUveitis is a frequent extra-skeletal manifestation of ankylosing spondylitis (AS).Data on the association of uveitis with other clinical manifestations of AS are contradictory and require further study.ObjectivesTo assess the association of uveitis in AS with demographics and genetics, spine lesions, peripheral disease, coxitis, enthesitis.Methods402 AS pts (New York criteria, 1984), were observed in V.A. Nasonova Research Institute of Rheumatology. 168 pts had at least 1 episode of uveitis (group 1), 234 pts did not have uveitis (group 2). The patients underwent standard clinical, laboratory and instrumental examination, including HLA-27- testing, Х-ray examinaiton of joints and spine, ultrasonography of joints and entheses. Two groups were compared by gender, onset age and AS duration, presence of HLA-B27, damage of the spine, hip and peripheral joints and enthesitis.ResultsBoth groups were dominated by men: 117 of 168 in group 1 and 189 of 234 in group 2. However, among patients with uveitis, the proportion of women was significantly higher: 51/168 (30.3%) and 45/234 (19.2%), p=0.01. The average duration of the disease was comparable in both groups – 14.9±10.2 and 12.8±9.9 years, respectively. The number of patients with AS onset before the age of 16 also did not significantly differ: 22/168 (13.1%) in the group with uveitis, 35/234 (14.9%) in the group without uveitis, p=0.6. HLA-B27 in the group with uveitis was in 166/168 patients (98.8%) and in 221/234 (94.4%) in the group without uveitis, p=0.04. Syndesmophytes were observed in both groups with similar frequency: in the cervical spine - in 49/168 (29.1%) in group 1 and in 78/234 (33.3%) in group 2, p>0.05; in the lumbar spine – in 87 /168 (51.8%) and in 139/234 (59.4%), respectively, p>0.05. Hip joint lesion (coxitis) was present in 75/168 (44.6%) and in 112/234 (53.8%) respectively, p=0.52; enthesitis – in 114/168 (67,9%) and in 168/234(71,8%), p>0.05. Peripheral arthritis was observed significantly more often in the group with uveitis: in 113/168 (67.2%) and in 70/234 (33.2%) respectively, pConclusionAS with uveitis is characterized by a greater proportion of women and stronger association with HLA-B27, compared with patients without uveitis. Peripheral arthritis with predominant involvement of the knee and ankle joints is significantly more common in AS pts with uveitis compared with pts without uveitis.References[1]Lee JH, Choi M, Rim THT, Lee SC, Lee CS. Clinical Characteristics and Prognostic Factors in Ankylosing Spondylitis Associated Uveitis.Ocul Immunol Inflamm. 2019;27(1):64-69. doi: 10.1080/09273948.2017.1359630.Disclosure of InterestsNone declared

This literature review is dedicated to the Behçet's disease - a multi-symptom, severe autoimmune condition, relatively rare among the population of the former Soviet republics, and to one of its three most frequent manifestations - uveitis.Данный обзор посвящен болезни Бехчета — достаточно редко встречающемуся на постсоветском пространстве многосимптомному и достаточно тяжелому аутовоспалительному заболеванию, а также одному из трех наиболее частых его проявлений — увеиту.

Anterior uveitis (AU) is one of the common extraskeletal manifestations of spondyloarthritis (SpA). The course of AU in patients with SpA is characterized by frequent relapses. The article considers the question of local and systemic use of nonsteroidal anti-inflammatory drugs (NSAIDs) in the treatment and prevention of SpA-associated uveitis exacerbations.Передний увеит традиционно является типичным внескелетным проявлением спондилоартритов (СпА). Течение переднего увеита при СпА характеризуется частыми рецидивами. В данном обзоре рассматривается вопрос местного и системного применения нестероидных противовоспалительных препаратов (НПВП) в лечении и предотвращении обострений увеита, ассоциированного со СпА.

Aim to identify outdated terms and make changes to the terminology of spondyloarthritis.At the first stage of the work, the terms divided into two categories: "outdated" definitions and terms that need to be improved or unified. Subsequently, each member of the Expert Group of Spondyloarthritis at the Association of Rheumatologists of Russia (ExSpA) presented by its own definition of the designated term or agreed with the previous term. At the next stage, the existing definitions were put together. After discussion, experts left a term that scored at least 2/3 of the votes. The special opinion of experts was recorded, whose did not coincide with the majority opinion. An open vote was conducted, when defining an "outdated" term with the unanimous decision of all group members, this term was not recommended for further clinical use.The work carried out allowed us to identify a number of terms that are not recommended for use in clinical practice. Number of terms are defined, which should be used when discussing the problem of spondyloarthritis.The Expert Group of Spondyloarthritis at the Association of Rheumatologists of Russia suggests using or, accordingly, not using a number of terms and their definitions in clinical practice.Цель исследования: выявить устаревшие термины и внести изменения в терминологию, используемую при обсуждении проблемы спондилоартритов. Материалы и методы. На первом этапе работы собраны уже употребляемые термины, их разделили на две категории: «устаревшие» определения и термины, требующие доработки или унификации. В дальнейшем каждый член Экспертной группы по изучению спондилоартритов при Ассоциации ревматологов России (ЭкСпА) представлял свое определение обозначенного термина либо соглашался с прежним термином. На следующем этапе имевшиеся определения сводились воедино, после обсуждения оставляли термин, который набрал не менее 2/3 голосов, отдельно фиксировалось особое мнение экспертов, чье мнение не совпадало с мнением большинства. При определении «устаревшего» термина проводили открытое голосование, при единогласном решении всех членов группы этот термин не рекомендовался для дальнейшего клинического использования. Результаты. Проведенная работа позволила выделить ряд терминов, которые не рекомендованы к употреблению в современных условиях, даны определения и расшифровка целого ряда терминов, которые, напротив, целесообразно использовать при обсуждении проблемы спондилоартритов. Заключение. Экспертный совет по спондилоартритам при Ассоциации ревматологов России предлагает использовать или, соответственно, не использовать ряд терминов и их определения в повседневной клинической практике и при описании клинических и иных исследований.

Extraskeletal manifestations (ESMs) are commonly observed in ankylosing spondylitis (AS). The available data on the association of ESMs with the inflammatory activity and other clinical parameters of AS are contradictory. Objective : to assess the association of ESMs with the inflammatory activity and other manifestations of AS. Patients and methods . The investigators of the V.A. Nasonova Research Institute of Rheumatology examined a total of 452 patients (363 men and 89 women) diagnosed with AS meeting the New-York criteria (1984). The patients' median age was 31.5 [24; 41] years; median disease onset age, 19.5 [15; 23] years; and disease duration, 11.5 [7; 18] years. HLA B27 was identified in 442 (97.7%) patients. In addition to standard laboratory and instrumental examinations, 172 patients underwent transthoracic echocardiography; Rehberg's test, if indicated; IgA test; histological examination of subcutaneous fat tissue or duodenal mucosa for amyloid; renal ultrasound; colonoscopy; and consultations by an ophthalmologist, a dermatologist, a nephrologist, an urologist, and a gastroenterologist. Uveitis, cardiac involvement (cardiac conduction disturbance, aortic and valvular changes), inflammatory bowel disease (IBD), glomerulonephritis, and psoriasis were considered to be ESMs. The latter were detected in 218 (48%) of the 452 patients; there was uveitis in 140 (30%), cardiac conduction disturbance in 61 (13.4%), psoriasis in 17 (3.7%), IBD in 16 (3.5%), nephritis in 16 (3.5%), and aortic and valvular changes in 71 (41.2%) of the 172 patients. The groups of patients with ESM (n = 218) and without ESM (n=234) were compared with regard to the onset age of AS, the presence of HLA- 27, peripheral arthritis, coxitis, enthesitis, syndesmophytis, fever, anemia, the need for biological agents (BAs) and/or systemic glucocorticoids (GCs), Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), and erythrocyte sedimentation rate (ESR). Results. The ESM and non-ESM groups were matched for gender, age, duration of AS, and the presence of HLA-В27. No significant differences were found in ESR, BASDAI, and the frequency of coxitis, enthesitis, and syndesmophytis in the spine. The ESM group versus non-ESM group was significantly more frequently observed to have peripheral arthritis in 148 (67.8%) of the 218 patients and in 70 (33.2%) of the 234 patients, respectively (p

Anterior uveitis (AU) and ankylosing spondylitis (AS) are associated with histocompatibility human leukocyte antigen (HLA)-B27. Previous genetic studies conducted in different populations have also demonstrated other genetic associations, including HLA, both general and individual ones for AU and AS. Objective: to investigate the association of HLA class I with AU depending on the presence or absence of spondyloarthritis (SpA). Patients and methods . The investigators used the data of HLA class I typing in the patients referred by ophthalmologists for examination to the V.A. Nasonova Research Institute of Rheumatology, as well as the previous databases of patients with AS. The investigation included retrospectively two groups of patients with AU: 1) 52 patients with a confirmed diagnosis of SpA (AU + SpA); 2) 96 patients who had other types of AU (idiopathic AU (n=52), viral uveitis (n=29), multiple sclerosis (n=2) toxoplasmosis (n=2), sarcoidosis (n=1), tuberculosis (n=1), chlamydiasis (n=3), Behcet's disease (n=2), juvenile chronic arthritis (n=3), and Fuchs' heterochromic cyclitis (n=1). A control group consisted of 150 healthy test donors. The distribution of HLA class I (A, B, and Cw) was analyzed when comparing the two groups of patients with AU and each control patient group. Results. HLA-B27 was detected in 96.1% of cases in the AU + SpA group, in 40.6% in the AU group, and in 7.3% in the controls. In HLA-27-positive patients, the risk (odds ratio (OR) for joint disease (AU + SpA) was 315.9 (95% confidence interval (CI), 61.9–2176.7); p

The paper gives the definition of remission in axial spondyloarthritides (axSpA), which has been developed by the Spondyloarthritis Study Group of Experts. The work used the Delphi technique. At stage 1, based on the analysis of the data available in the literature and on their own clinical experiences, the experts proposed some variants of the definition of remission and ways of its evaluation in patients with axSpA. At Stage 2, the definitions that had received at least 80% of the votes via anonymous voting were selected and adopted without further discussion. Those that had received an equal number of votes were reconsidered and additionally discussed; then there was repeat voting, by choosing the final definition. As a result of their discussion, the experts formulated the definitions of clinical laboratory and magnetic resonance imaging (MRI) remissions. They proposed the following remissions in axSpA: drug and drug-free, clinical laboratory, and MRI remissions, as well as a remission in the presence and absence of structural changes in the locomotor system, as evidenced by imaging techniques. Criteria for clinical laboratory remission and basic tools for its evaluation that can be used in real clinical practice and researches have been elaborated.

Background:Uveitis is a common extra-skeletal manifestation of various systemic diseases, in particular, spondyloarthritis (SpA). Uveal cataract is the frequent complication of endogenous uveitis. Surgical treatment of uveal cataracts is associated with certain problems, the main being a potential risk of recurrence of uveitis, especially in the case of intraocular lens (IOL) implantation.Objectives:To analyse the results of surgery of complicated uveal cataract in patients with SpA and other rheumatic disorders.Methods:30 pts (34 eyes) with uveal cataract as a result of systemic immuno-inflammatory diseases were assessed in the “Research Institute of Eye Diseases” in the period from 2006 to 2018. The postoperative follow-up period ranged from 2 to 12 years. 20 pts had different variants of SpA (Group 1):7 (7 eyes) - ankylosing spondylitis, 1 (1 eye) –reactive arthritis, 1 (1 eye) - psoriatic arthritis, 4 (6 eyes) – juvenile spondyloarthritis, 8 (8 eyes) non-radiological SpA. 10 patients (11 eyes) had other systemic diseases (Group 2): 1 (1 eye) Behcet’s disease, 1 (1 eye) - multiple sclerosis, 3 (3 eyes) - Fuchs heterochromic cyclitis, 2 (2 eyes) -cutaneous psoriasis, and 3 (4 eyes) -uveitis of unknown etiology. 4 pts (6 eyes) with juvenile arthritis and pronounced corneal ribbon degeneration were underwent standard intracapsular cryoextraction of the cataract, followed by optical correction of aphakia using spectacle lenses. In other cases, ultrasound phacoemulsification with IOL implantation was used as a surgical aid. All patients had a period of uveitis remission before opreation for at least 2 months.Аnti-inflammatory therapy using local glucocorticoid and local and systemic nonsteroidal antiinflammatory drugs was prescribed 2 weeks before and within a month after the operation. Intra and postoperative complications, fluctuations in intraocular pressure (IOP), improvement of visual acuity, and the frequency of uveitis flares after surgery were assessеd as the main criteria for analyzing the results of surgery.Results:А statistically significant decrease in the number of uveitis exacerbations was observed in all pts: in group 1 - from 2,5 ± 0,65 to 1, 2 ± 0,65 per year (pConclusion:Surgery of uveal cataracts in pts with SpA and other immuno-inflammatory diseases providing adequate pre- and postoperative аnti-inflammatory therapy improves visual acuity and reduces the frequency of uveitis relapses. Monitoring of IOP is necessary in the postoperative period.References:[1]Mora P., Gonzales S., Ghirardini S. et al. Perioperative prophylaxis to prevent recurrence following cataract surgery in uveitis patients a two-centre, prospective, randomized trial. Acta Ophthalmol. Scandinavica Fondation. 2016 Sept.; 94(6): e 390-394. http//doi. 10.1111/aos.12955. Epub 2016 Feb 5.Disclosure of Interests:None declared

This review is devoted to the potential application of genetically engineered biological drugs of different classes in the treatment of main forms of uveitis and other types of non-infectious ocular inflammation in immunoinflammatory rheumatic diseases. The article presents the results of studies demonstrating the influence of interleukin-17 inhibitor secukinumab on the course of uveitis in spondylarthritis, shows the possibilities of treating uveitis in juvenile idiopathic arthritis with inhibitors of interleukin-6, co-stimulation of T-lymphocytes. Inhibition of interleukin-1 is considered an effective method of influencing the ocular inflammation in monogenic autoinflammatory syndromes and Behcet's disease. Rituximab is considered as an option for immunosuppressive therapy of non-infectious uveitis and scleritis.Обзор посвящен возможностям применения генно-инженерных биологических препаратов разных классов для лечения основных форм увеита и других видов неинфекционного глазного воспаления при иммуновоспалительных ревматических заболеваниях. Представлены результаты исследований, демонстрирующих влияние ингибитора инерлейкина-17 секукинумаба на течение увеита при спондилоартритах. Показаны возможности лечения увеита при ювенильном идиопатическом артрите ингибиторами интерлейкина-6, ко-стимуляции Т-лимфоцитов. Ингибирование интерлейкина-1 рассматривается в качестве эффективного метода воздействия на глазное воспаление при моногенных аутовоспалительных синдромах, болезни Бехчета. В качестве варианта иммуносупрессивной терапии неинфекционного увеита и склерита рассматривается ритуксимаб.

The article describes clinical cases with effective use of some genetically engineered biological agents for arresting and preventing uveitis attacks, and discusses the potential usage of tumor necrosis factor (TNF)-alpha inhibitors and interleukin-17 inhibitors in the treatment of uveitis in patients with ankylosing spondylitis.В статье представлены описания клинических случаев, свидетельствующих об эффективности некоторых генно-инженерных биологических препаратов в отношении купирования и предотвращения атак увеита у больных анкилозирующим спондилитом. Обсуждается возможность применения ингибиторов фактора некроза опухоли α, а также ингибиторов интерлейкина-17 для лечения увеита при анкилозирующем спондилите.

The paper gives recommendations for the drug therapy of axial spondyloarthritides, which have been developed by the Spondyloarthritis Study Group of Experts. The recommendations describe the patient management tactic in the most common clinical situations, which is aimed at maximizing the efficacy and safety of treatment.

Involvement of the aorta and heart valves in ankylosing spondylitis (AS) is a manifestation of the systemic inflammatory process. Data on the frequency, clinical significance, and evolution of these manifestations are controversial. Objective : to estimate the time course of echocardiographic (EchoCG) changes in the aorta and heart valves from the results of a prospective follow-up study of patients with AS. Subjects and methods. In 2008 to 2015, the V.A. Nasonova Research Institute of Rheumatology followed up 45 patients (35 men and 10 women) with documented AS that was consistent with the modified New York criteria and the original EchoCG changes in the aorta and heart valves. All the patients underwent transthoracic echocardiography at baseline and after 1–5 years. At baseline, there was aortic root dilatation (>37 mm) in 15 patients, thickening of the aortic walls and aortic valve (AV) cusps in 21 and 32 patients, respectively, as well as thickening of the mitral valve (MV) cusps in 15, and valve prostheses in 5 patients (2 had AV and MV prostheses). Aortic regurgitation (AR) was recorded in 19 patients (grades 1–2 AR in 15 patients and grades 3–4 in 4 patients); grades 1–2 mitral regurgitation (MR) was seen in 20 patients. Ten patients had subaortic pectinate thickening (SPT) in the aortomitral junction area. Of the 45 patients, 16 took biological agents (BA); 29 received traditional therapy, including 14 patients who used nonsteroidal anti-inflammatory drugs, 11 and 4 patients had sulfasalazine and methotrexate, respectively. Results and discussion. Repeated examinations revealed negative changes in 27 (60%) of the 45 patients. Progressive aortic dilatation (1 to 6 mm) was found in 12 (80%) of the 15 patients; dilatation appeared during follow-up in 2 patients. Fourteen patients were noted to have negative valve changes: the emergence of cusp thickening in the valves (that in AV and MV in 9 and 4 patients, respectively; both in 1 patient). Four patients underwent valve replacement: 3 and 1 patients underwent AV and MV replacements, respectively. Ten of the 19 patients were observed to have AR progression by 2 grades in 2 (10.5%) patients and by one grade in 8 (42.0%) patients. MR progression by one grade was noted in 6 (30.0%) of the 20 people. A negative correlation was found between BA therapy and aortic dilation progression (r = -0.329; p = 0.03). SPT appeared in 3 patients. Of the 10 patients who had a baseline SPT, its reduction (8 to 3.5 mm) was seen in one patient; complete regression was identified in two patients; all received BAs. Thus, pathological changes in the aorta and heart valves in AS are progressive in most patients. SPT may regress, which reflects apparently a reduction in the severity of active inflammation of the aortic root. Active anti-inflammatory treatment may play a protective role in the progression of aortic dilatation.

The paper gives the recommendations for the assessment of disease activity and functional status in patients with ankylosing spondylitis in clinical practice, which have been developed by experts, by taking into account international and Russian experience in managing these patients.

To perform comparative analysis of the frequency of various complications of anterior uveitis associated with HLA-B27 antigen in patients with and without spondyloarthritis.Retrospective analysis included 189 patients with anterior uveitis (AU) associated with histocompatibility antigen (HLA-B27). The Follow-up period lasted 10 years.189 patients with AU associated with antigen HLA-B27 were divided into two groups. The first group included 108 patients with various diseases of the spondyloarthritis (SpA) group; the second study group consisted of 81 patient with no signs of SpA. Number of patients with complications was 1.5 times higher in the first group. Complicated cataract, glaucoma, synechiae, myodesopsia occurred significantly more frequently in patients of the first group compared to AU patients of the second group, where idiopathic AU was more prevalent. Cystoid macular edema, corneal degeneration, optic nerve atrophy were observed more often in patients with SpA, but differences between the groups were statistically insignificant. Combination of complications also occurred significantly more often in SpA patients. Comparison of the frequency of AU attacks showed that the amount of uveitis attacks per 100 patient-years was higher in patients with SpA. Mean number of AU attacks per year was also higher in the first study group, but differences between the groups were statistically insignificant.Retrospective analysis of 189 patients with AU associated with HLA-B27 antigen revealed significantly higher frequency of complications, together with more frequent acute episodes in patients with SpA in comparison with patients without it confirming the notion that a systemic inflammatory disease can influence the severity of the course of AU.Цель исследования - провести сравнительный анализ частоты развития различных вариантов осложнений передних увеитов, ассоциированных с HLA-B27-антигеном, у пациентов со спондилоартритами и без них. Материал и методы. Ретроспективно проведен анализ результатов обследований 189 пациентов с передним увеитом (ПУ), ассоциированным с антигеном гистосовместимости (HLA-B27). Срок наблюдения составил 10 лет. Результаты. 189 пациентов с ПУ, ассоциированным с HLA-B27-антигеном, были разделены на две группы. В 1-ю группу вошли 108 пациентов с различными заболеваниями, относящимися к группе спондилоартритов (СпА), 2-ю группу составил 81 пациент без признаков СпА. Число пациентов с осложнениями из 1-й группы в 1,5 раза превышало число пациентов из 2-й группы. Осложненную катаракту, глаукому, синехии, деструкцию стекловидного тела достоверно чаще наблюдали у пациентов 1-й группы по сравнению с ПУ у пациентов из 2-й группы, в которой преобладал идиопатический ПУ. Кистозный макулярный отек, дегенерация роговицы, атрофия зрительного нерва чаще отмечены при наличии СпА, однако межгрупповые различия были статистически недостоверны. Также достоверно чаще выявлено сочетание нескольких осложнений на фоне СпА. При сравнении частоты обострений ПУ оказалось, что число обострений увеита на 100 пациенто-лет было выше при наличии СпА. Среднее число обострений ПУ в год в 1-й группе также было выше, однако межгрупповые различия были статистически недостоверны. Заключение. Результаты ретроспективного анализа обследований 189 пациентов с ПУ, ассоциированным с HLA-В27-антигеном, продемонстрировали достоверно более высокую частоту развития осложнений при более высокой частоте обострений у больных СпА в сравнении с больными без СпА, что подтверждает представления о влиянии системного воспалительного заболевания на тяжесть течения ПУ.

Background HLA-B27- associated anterior uveitis (AU) can be an isolated disease or part of а systemic inflammatory process, such as spondyloarthritis. Differences in the outcome of uveitis with and without spondyloarthritisare still under researh. Objectives To compare the incidence of complications in HLA-B27- associated anterior uveitis patients with and without spondyloarthritis (SpA). Methods 189 patients with HLA-B27- associated anterior uveitis were observed in Research Institute of Eye Disease for 10 years. The pts were underwent standard ophthalmological examination, and, if necessary, computer perimetry, ultrasound examination (B-scan.), optical coherence tomography of the retina and optic nerve (OCT), fluorescence fundus angiography (FAGD) and electrophysiological studies (EFI). All pts were examined for clinical and imaging signs of SpA: inflammatory back pain, arthritis, enthesitis, sacroiliitis. Different variants of SpA were diagnosed in 108 pts: ankylosing spondylitis – in 48 (44,4%), reactive arthritis – in 9 (8,3%), psoriatic arthritis - in 9 (8,3%), inflammatory bowel disease – in 2(1,9%), juvenile SpA – in 4 (3,7%), undifferented SpA - in 36 (33,3%). In 81 pts SpA was not confirmed: 11 had a viral infection (13,5%), 7- bacterial infection (8,6%), 1 (1,2%)-multiple sclerosis, 3 (3,7%) -psoriasis, 59 (72,8%)- idiopathic AU. Results Complications were identified in 74 of 108 (68,5%) pts – 147 eyes in SpA-group and in 39of 81(48,1%) – 110 eyes in group without SpA. Cataract was identified in 89 (60,5%) of 147 eyes in SpA-group and in 35(1,8%) of 110 eyes in group without SpA, p=0,0001;glaucoma – in 28 of 147 eyes (19,0%) in SpA-group and 8 of 110 eyes (7,3%) in group without SpA, p=0,005; synechia - in 24(16,3%) and in 8 (7,3%) respectively, p=0,021;vitreous destruction - in 59(40,1%) and in 26 (23,6%), p=0,005. Maculopathy, оptic atrophy, сorneal degeneration, panuveitis were observed more often in SpA-group, but the differences were not significant. Combinations of several complications were detected in 59 (40,1%) in SpA-group and in 31 (28,2%) in group without SpA, p=0,03. Conclusion Complications of HLA-B27- associated AU were identified more often in pts with SpA than without SpA (the majority of which was idiopathic AU). It confirms the effect of systemic inflammatory disease on the severity of uveitis. Reference [1] Yang P, Wan W, Du L, et al. Clinical features of HLA-B27-positive acute anterior uveitis with or without ankylosing spondylitis in a Chinese cohort. Br J Ophthalmol. 2018Feb;102(2):215-219. doi: 10.1136/bjophthalmol-2016-309499. Epub 2017 Jun 12 Disclosure of Interests None declared

Ankylosing spondylitis (AS) is a systemic inflammatory disease involving not only the skeleton, but also other organs. The data on the frequency and clinical significance of extraskeletal manifestations are contradictory. Objective: to assess the frequency and severity of extraskeletal manifestations (ESMs) of AS in the authors' own clinical practice. Subjects and methods. 452 patients (363 men and 89 women) with AS fulfilling the 1984 relevant New York criteria were examined at the V.A. Nasonova Research Institute of Rheumatology in 2005 and 2014. The patients' median age was 31.5 [24; 41] years; median disease onset age, 19 [15; 23] years; disease duration, 11 [7; 18] years; HLA B27 was identified in 442 (97.7%) patients. In addition to standard laboratory and instrumental examinations, transthoracic echocardiography was performed in 172 patients. Rehberg's test, IgA test, histological examination of subcutaneous fat tissue or duodenal mucosa for amyloid, and renal ultrasound were made if there were urinary abnormalities and elevated creatinine levels. If indicated, there were consultations by an ophthalmologist with an appropriate instrumental examination (with evidence of uveitis in the history), a dermatologist, a nephrologist, an urologist, a gastroenterologist, and a endoscopist. Uveitis, cardiac involvement (conduction disturbance, aortic and valvular changes), inflammatory bowel disease (IBD), glomerulonephritis, and psoriasis were borne in mind as ESMs. The absolute number and percentage of patients having any ESM over the follow-up period were estimated. The number of exacerbations per year and that of complications were taken into account when evaluating uveitis; the magnitude of valvular regurgitation and the presence of prosthetic valves and a pacemaker were considered when assessing cardiac damage; the total body surface area (BSA) and the psoriasis areas and severity index (PASI) were estimated in psoriasis; the presence and stage of chronic kidney disease (CKD) and/or macrohematuria were kept in mind in nephropathy; when evaluating IBD, the Harvey–Bradshaw index (HBI) was determined on the basis of a stool frequency, the presence of blood in the stool, abdominal pain, and general well-being. Results and discussion. ESMs were detected in 218 (48%) of the 452 patients. Uveitis was present in 140 (30%) patients; in one-fourth of them, it relapsed frequently: three or more exacerbations per year and more than 10 during the disease; 41 (29%) patients had uveitis complications accompanied by visual impairment. Cardiac conduction disturbance was revealed in 61 (13%) patients. Five of them underwent pacemaker implantation. 71 (41.2%) patients were found to have aortic and valvular changes, including aortic root dilatation/thickening in 60 (34.8%) patients and aortic/mitral valve leaflet thickening in 63 (36.6%). Grades 3 and 4 valvular regurgitation was noted in 10 (5.8%) patients; valve replacement was carried out in 9 (5.2%) cases. Nephritis was diagnosed in 16 (3.5%) patients, 4 of them had Stage 2 or above of CKD. Psoriasis was present in 17 (3.7%) patients; 2 of them had severe psoriasis (BSA >10%). IBD (ulcerative colitis or Crohn's disease) was diagnosed in 16 (3.5%) patients, including 4 who showed a severe course. 79 (36%) patients were observed to have a concurrence of two or more ESMs; that of uveitis and heart disease was most commonly seen. Conclusion. ESMs are observed in nearly half (48%) of the patients with AS and can be concurrent; the most common ESMs of AS are uveitis, aortic root and heart valve lesions, and cardiac conduction disturbance. ESMs worsen the course and prognosis of AS.

Relapsing uveitis is a serious problem for patients with ankylosing spondylitis (AS). Tumor necrosis factor- α inhibitors significantly reduce the frequency of uveitis attacks in AS patients, but they are not always available. In this connection, it is appropriate to evaluate the effect of traditional disease-modifying antirheumatic drugs on the course of uveitis in AS. Objective: to compare the frequency of uveitis attacks in AS during combined therapy using sulfasalazine (SULF) and nonsteroidal anti-inflammatory drugs (NSAIDs) versus monotherapy with NSAIDs. Subjects and methods. A total of 111 patients with significant AS who had at least one uveitis attack during the disease were examined. 49 subjects received combined therapy including SULF 2 grams daily and NSAIDs for at least 1 year. Six of the 49 patients were noted to have frequently relapsing uveitis (above 3 episodes yearly); 43 had no more than 3 ones per year. The remaining 62 patients took NSAIDs only throughout the follow-up period. Nine of them had an average of 3 uveitis attacks per year; 53 had no more than 3 ones per year. Out of the 49 patients receiving combined therapy with NSAIDs and SULF, 23 took NSAIDs only at least 1 year before SULF use; 10 of them were observed to have frequent relapses in that period and 13 had three or fewer episodes. Results and discussion. The entire group showed an average of 2.06±2.04 uveitis attacks per year during monotherapy with NSAIDs and 1.41±1.83 attacks yearly during combined therapy with NSAIDs and SULF (p = 0.08). Among the patients with less than 3 uveitis attacks per year, the mean number of episodes was significantly fewer during combined therapy than during NSAID monotherapy: 0.99±0.87 and 1.37±0.91, respectively (p = 0.04). Among those with frequently relapsing uveitis (more than 3 attacks per year), the mean number of episodes yearly was not significantly different during NSAID monotherapy and combined therapy: 5.7±2.5 and 5.5±2.7, respectively (p = 0.9). In a subgroup of patients receiving sequentially NSAID monotherapy and combined therapy with NSADs and SULF, 13 patients with three or fewer baseline attacks per year displayed a significant reduction in the frequency of episodes from 1.92±0.96 to 0.4±0.44 (p = 0.00003). During combined therapy versus monotherapy, 10 patients with frequently relapsing uveitis (over 3 episodes yearly) exhibited an insignificant reduction in the number of relapses from 5.9±3.02 to 5.33±1.1 (p = 0.6). Conclusion. Combined therapy with SULF and NSAIDs does not substantially affect the frequency of uveitis episodes in AS patients with frequently relapsing uveitis (above 3 attacks per year), but significantly reduces the number of episodes among patients with its mild course (less than 3 attacks yearly).

The paper gives the draft guidelines elaborated by the Expert Spondyloarthritis Diagnosis and Treatment Group by order of the Association of Rheumatologists of Russia. The guidelines include the essentials of how to use nonsteroidal anti-inflammatory drugs in axial spondyloarthrititides, including ankylosing spondylitis, contain instructions for how long they should be administered, and describe possible patient management tactics in the most common clinical situations and a preferential algorithm for evaluating the efficiency and safety of treatment.

The paper provides guidelines for the use of tumor necrosis factor- α (TNF- α ) inhibitors in the treatment of patients with axial spondyloarthritis (axSpA), including ankylosing spondylitis. It gives data on the efficacy of TNF- α inhibitors in patients with non-radiographic axSpA. By using international and Russian guidelines, the authors lay down indications for this therapy and criteria for evaluation of its efficiency and safety.

Background: Ankylosing spondylitis (AS) leads to back pain and structural damage that may result in functional impairment1. Function is usually assessed in clinical trials conducted in developed countries, with patients receiving biological therapy2,3. Objectives: To evaluate variation in the Bath Ankylosing Functional index (BASFI) over time in a AS cohort followed in a developing country. Compare the improvement in BASFI between patients achieving or not sustained (≥12 months) ASDAS-CRP remission/low disease activity (LDA). Analyze predictors for achieving a minimum clinically important improvement (MCII) in BASFI (ΔBASFI ≤ -0.6)4. Methods: This cross-sectional analysis was conducted in a retrospective cohort. Adult patients fulfilling the New York criteria for AS and followed during at least 5 years in the Spondyloarthritis clinic were included. BASFI variation (ΔBASFI) was described as median (25th/75th). Comparison of ΔBASFI between patients fulfilling or not sustained ASDAS-CRP remisson/LDA was done using the Mann-Whitney test. Hierarchical Poisson model was used to identify predictors for achieving a MCII in BASFI. Results: 69 patients were analyzed, 53.6% were men, the mean age was 48.9±11.4 years, and the mean follow-up time was 6.1±0.5 years, median (25th/75th) disease duration of 10 (5-18) years; 14.5% of the patients were on biological therapy at baseline. The median (25th/75th) ΔBASFI was low: -0.1 (-1.9 /+1.1) but 46.4% (N= 32) presented a MCII in BASFI during follow-up. Patients who achieved sustained ASDAS-CRP remission/LDA had a significant improvement in BASFI over time compared with those who did not achieve this target (p=0.026) (Figure 1). Patients with higher BASFI scores at baseline had a greater probability of achieving a MCII in BASFI (RR1.13 95%CI 1.00-1.27 p=0.047). Achieving and maintaining ASDAS-CRP remission/LDA during at least 12 months increased in 82% the probability to obtain a MCII in BASFI (RR 1.82 95% CI 1.14-2.91, p=0.012). Conclusion: Patients achieving sustained ASDAS-CRP remission/LDA had better functional outcomes over time compared to those not achieving this target. Higher BASFI scores at baseline and sustained ASDAS remission/LDA were predictors of a MCII in BASFI. References: [1] Madsen OR: Stability of fatigue, pain, patient global assessment and the Bath Ankylosing Spondylitis Functional Index (BASFI) in spondyloarthropathy patients with stable disease according to the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Rheumatol Int. 2018;38(3):425-432. [2] Baraliakos X, Kivitz AJ, et al: Long-term effects of interleukin-17A inhibition with secukinumab in active ankylosing spondylitis: 3-year efficacy and safety results from an extension of the Phase 3 MEASURE 1 trial. Clin Exp Rheumatol. 2018; 36(1):50-55 [3] Deodhar A, Reveille JD, et al: Safety and Efficacy of Golimumab Administered Intravenously in Adults with Ankylosing Spondylitis: Results through Week 28 of the GO-ALIVE Study. J Rheumatol. 2018; 45(3):341-348 [4] Kviatkovsky MJ, Ramiro S, Landewe R, et al: The Minimum Clinically Important Improvement in Patient-acceptable Symptom State in the BASDAI and BASFI for Patients with Ankylosing Spondylitis. J Rheumatol 2016; 43(9): 11680-1686. Disclosure of Interests: Juliana Maria Kerber Grant/research support from: This work was sponsored by the regional society of rheumatology (Sociedade de Reumatologia do Rio Grande do Sul)., Juliana Dias de Mello Grant/research support from: This work was sponsored by the regional society of rheumatology (Sociedade de Reumatologia do Rio Grande do Sul)., Penelope Palominos Grant/research support from: This work was sponsored by the regional society of rheumatology (Sociedade de Reumatologia do Rio Grande do Sul)., Andrese Aline Gasparin Grant/research support from: This work was sponsored by the regional society of rheumatology (Sociedade de Reumatologia do Rio Grande do Sul)., Franciele de Almeida Menegat Grant/research support from: This work was sponsored by the regional society of rheumatology (Sociedade de Reumatologia do Rio Grande do Sul)., Claiton Viegas Brenol Grant/research support from: This work was sponsored by the regional society of rheumatology (Sociedade de Reumatologia do Rio Grande do Sul)., Charles Kohem Grant/research support from: This work was sponsored by the regional society of rheumatology (Sociedade de Reumatologia do Rio Grande do Sul).

Background Anterior uveitis (AU) and spondyloarthritis (SpA) are associated with HLA-B27. Previous genetic studies in different populations demonstrated other associations, in particular, HLA, both common and different for AU and SpA Objectives To assess the relationship of HLA class I antigens with AU depending on the presence or absence of SpA Methods 148 pts with AU were typed. All pts were divided into 2 groups: 1–52 pts with confirmed diagnosis of SpA (AU +SpA), 96 pts – with other forms of AU (52 – idiopathic AU, 29 – viral AU, 2- multiple sclerosis,, 2 - toxoplasmosis, 1- sarcoidosis, 1- tuberculosis, 3- chlamydial AU, 2 - Behcet’s disease, 3 - juvenile chronic arthritis, 1 - Fuchs′s heterochromic iridocyclitis). 150 healthy donors formed control group. Analysis of the distribution of HLA- class I antigens (loci A, B, Cw) was performed comparing 2 groups of patients with AU and each group with control. Results HLA-27 in the group of pts AU +SpA identified in 96.1% (50/52), in the group AU – in 40,6% (39/96), in control – in 7.3% (11/150). If HLA-27 was present in the genotype, the risk of developing of common pathology (AU +SpA) was OR=315,9 [95% CI 61,9–2176,7], p Conclusions The analysis of the distribution of HLA class I antigens confirmed association of HLA- B27 with AU in Russian population. Other associations, except Cw2, were not identified. Such significant increase of Cw2 in two groups of pts with high frequency of B27 is natural due to the phenomenon of linkage disequilibrium for HLA-B27 and Cw2 antigens. Cw7 antigen can play a ”protective” role in relation to SpA, because the frequency of this gene in the group AU was not reduced compared to control. Reference [1] Robinson PC, Claushuis TA, Cortes A, et al. Genetic dissection of acute anterior uveitis reveals similarities and differences in associations observed with ankylosing spondylitis. Arthritis Rheumatol.2015Jan;67(1):140–51. doi:10.1002/art.38873 Disclosure of Interest None declared