The undeniable progress made over the last decade in the treatment of patients with severe juvenile arthritis has ranked in the order of priorities for the provision of efficient and reliable continuity in the management tactics for patients after their transition from their follow-up by a pediatrician to adult rheumatology service. A number of objective difficulties in organizing such continuity are associated primarily with the heterogeneity of the juvenile arthritis group in its clinical manifestations and nosological outcomes. The features of physiological processes of a growing organism cause significant differences in the nature of the course, manifestations of certain clinically significant syndromes (for example, delayed axial skeleton involvement after the onset in juvenile spondyloarthritis), radiological pattern and immunological markers in juvenile-onset rheumatic diseases. Timely active therapy with increasingly available innovative technologies, biological agents in particular, provides a successful disease-modifying effect, so when a patient with juvenile arthritis is transferred from his/her follow-up by a pediatrician to adult rheumatology service, there may be a wrong opinion about the performed redundant therapy and the expediency of its cancellation or correction, which sometimes leads to irreparable negative consequences. The used indices and other tools to assess the activity and functional status are fundamentally different in children and adults with joint inflammatory diseases. The paper presents a brief review of the current state of the problem and international experience with continuity in the follow-up of patients with juvenile arthritis between pediatric and adult rheumatology service.

Glucocorticoids (GCs) play an important role in treating many inflammatory diseases due to their anti-inflammatory and immunomodulatory activities and are used in many fields of medicine. Despite their clinical benefits of GCs used to treat patients with chronic inflammatory diseases, prolonged administration of these medications, especially oral ones, frequently causes serious complications, such as bone loss and fractures. The present paper reviews the latest American College of Rheumatology clinical guidelines for the prevention and treatment of glucocorticoid-induced osteoporosis, which were published in 2017. It provides comments, by taking into account the Russian guidelines on the diagnosis, prevention, and treatment of glucocorticoid-induced osteoporosis.

Objective: to determine the efficiency and safety of therapy with tofacitinib (TOFA) in combination with methotrexate (MTX) in patients with active rheumatoid arthritis (RA) after insufficient previous therapy in real clinical practice.

Subjects and methods. The investigation enrolled a total of 33 patients with RA who met the 1987 American College of Rheumatology (ACR) and/or the 2010 ACR/European League Against Rheumatism (EULAR) criteria. All the patients received TOFA 5–10 mg administered orally twice daily in combination with MTX; 30 patients took TOFA 10 mg/day and 3 patients had TOFA 20 mg/day. Every 6 weeks, the patients were examined by a rheumatologist and laboratory-instrumental tests. RA activity changes were assessed with DAS28-CRP, SDAI, and CDAI.

Results and discussion. Results were assessed at weeks 12, 54, and 114. A significant decrease in DAS28-CRP, SDAI, and CDAI values was noted just at 12-week follow-up; at week 54, the mean values of these indices were 3.7±1.0, 14.9±8.8, and 13.4±9.0, respectively. There was a substantial decline in the levels of rheumatoid factor in 27% of the patients; while one third of them had a 60% decrease in its level and four patients achieved a negative seroconversion. Neither serious adverse events (AEs) no AEs that had not previously been described in the literature were observed during the follow-up study. Nine non-serious AEs were recorded in 8 (25.0%) patients.

Conclusion. The investigation shows that TOFA makes it possible to control the activity of the inflammatory process and, with its sufficient safety and generally good tolerance, to achieve low RA activity in 49% of cases, including patients with multidrug resistance. The high efficacy of TOFA and, in particular, its combination with MTX used in patients with refractory RA give grounds for wider use of this drug, as confirmed by our investigation.

Objective: to investigate the effect of diacerein (Diaflex) on some components of metabolic syndrome (MS) in patients with knee osteoarthritis (OA).

Subjects and methods. The multicenter open-label prospective study covered 55 patients aged 45 to 74 years with Kellgren–Lawrence Stage II–III knee OA and MS, with a pain intensity of >40 mm on a visual analogue scale, and with a disease duration of 1 to 30 years. The therapy duration was 6 months: Diaflex 50 mg/day for one month, then 50 mg twice daily for 5 months; the patients were followed up for the succeeding 3 months. During each visit, the efficiency and safety of treatment were evaluated; moreover, blood biochemical values were taken into account at the beginning and at the end of therapy.

Results and discussion. There was a statistically significant improvement in WOMAC index (all its components and total value) and quality of life using EQ-5D in the first month of therapy and throughout the follow-up. Analysis based on the OMERACT-OARSI criteria indicated high treatment response rates in 92.5% of the patients at the end of therapy and in 92.2% three months after its completion. The body mass index and levels of low-density lipoproteins, triglycerides, glucose, and uric acid significantly decreased during treatment. Adverse events were detected in a small number (5.5%) of cases.

Conclusion. Diaflex is an effective and safe drug in the treatment of knee OA in patients with MS. During therapy, there is a rapid and considerable reduction in pain and stiffness and an improvement in the functional state. In addition, the pleiotropic effects of the drug make it possible not only to effectively reduce weight, but also perhaps to improve the course of MS-associated conditions by the observed corrections of metabolic disturbances.

Objective: to assess the reliability, validity, and sensitivity of a Russian version of the LupusQol questionnaire.

Subjects and methods. The investigation enrolled 328 patients aged 18 years or older with systemic lupus erythematosus (SLE) who met the 2012 Systemic Lupus International Collaborating Clinic (SLICC) criteria. Two tools, such as the 36-item Short-Form Health Survey (SF-36) questionnaire and LupusQol questionnaire filled out by the patients, were used to study their health-related quality of life (HRQOL). Activity was estimated using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K); irreversible organ damages were evaluated by the SLICC damage index (DI). Reliability evaluation included a study of the reproducibility and internal consistency of the index. Reproducibility was evaluated using the test-retest analysis; the internal consistency was measured by calculating Cronbach’s alpha; the sensitivity was assessed by the changes in the questionnaire domains during standard therapy for SLE. Construct validity was determined in two ways: by using the known-groups method and factor analysis; criterion validity was measured by assessing the relationship with the external criteria (SF-36).

Results and discussion. A total of 108 SLE patients were included to assess the reliability of the questionnaire. The results of assessing the reproducibility of the LupusQol questionnaire by the test-retest analysis showed no significant differences between the initial and repeated measures on all 8 LupusQol domains (p > 0.05). The value of Cronbach’s alpha ranged from 0.8 to 0.9 for each of the 8 questionnaire domains. After 12 months of standard therapy, 128 patients included in the assessment of questionnaire sensitivity showed a significant improvement in HRQOL for all questionnaire domains. The most significant positive changes were achieved on the domains of pain (p = 0.002), intimate relationships (IR; p=0.01), body image (p=0.0003), and fatigue (p=0.006).

Validity was assessed in 328 patients with SLE. Construct validity was assessed by comparing the domains of the LupusQol and SF-36 questionnaires. A correlation (r > 0.4) was found on all the 8 LupusQol domains: physical functioning (PF), role-physical functioning (RPF), general health (GH), viability (V), social functioning (SF), and role-emotional functioning (REF) of the SF-36 questionnaire. The factor analysis of the LupusQol questionnaire revealed a satisfactory ratio of the distribution of its questions to its domains and a high correlation between the domains and its factor. Criterion validity was evaluated by comparing the LupuQol domains in 4 groups: 1) SLEDAI-2K < 4; 2) SLEDAI-2K ≥4; 3) SLICC DI = 0; 4) SLICC DI ≥1. Significant differences were found between the active SLE (SLEDAI-2K >4) group and inactive SLE (SLEDAI-2K <4) group on the domains of PF, pain, planning, IR, dependence, and body image (p=0.07, p=0.007, p=0.0004, p=0.03, and p=0.007, respectively). Significantly lower HRQOL scores were observed in patients with irreversible organ damages.

Conclusion. The Russian version of the disease-specific LupusQol questionnaire is a valid, reliable, and sensitive tool assessing HRQOL in SLE patients, which has good psychometric properties.

Mental disorders (MDs) of the anxiety-depressive spectrum (ADS) and cognitive impairment (CI) substantially deteriorate the course and efficiency of therapy for rheumatoid arthritis (RA). There have been practically no studies on the impact of psychopharmacotherapy (PPT) for MDs on the efficacy of standard disease-modifying antirheumatic drugs (DMARDs) and biological agents (BAs).

Objective: to investigate the impact of adequate PPT for MDs of ADS on the efficacy of DMARDs and BAs in patients with RA.

Subjects and methods. The investigation included 128 patients (13% men and 87% women) with documented RA in accordance with the 1987 American College of Rheumatology (ACR) criteria. The patients’ mean age was 47.4±0.9 years; the median duration of RA was 96 [48; 228] months. DAS28 averaged 5.34±0.17. 75.1% of the patients received DMARDs. The diagnosis of MDs was based on the ICD-10 codes, by applying a semi-structured interview and the Hospital Anxiety and Depression Scale. Changes in the pattern and severity of ADS were evaluated using the Hamilton Anxiety Scale and the Montgomery–Asberg Depression Rating Scale. Clinical and psychological procedures were used to diagnose CI. At baseline, ADS was detected in 123 (96.1%) patients: major depression in 41 (32.1%), minor depression in 53 (41.4%), and anxiety disorders in 29 (22.6%). CI was diagnosed in 88 (68.7%). PPT was offered to all the patients with MDs; 52 agreed to treatment and 71 refused. The following therapeutic groups were identified according to the performed therapy: 1) DMARDs (n = 39); 2) DMARDs + PPT (n = 43); 3) DMARDs + BAs (n = 32); 4) DMARDs + BAs + PPT (n = 9). The dynamics of MDs and the outcomes of RA were estimated in 112 (91.0%) and in 83 (67.5%) of the 123 patients at one- and five-year follow-ups, respectively. The efficiency of RA therapy was evaluated from the changes in DAS28 and SDAI.

Results and discussion. One year later, the patients who had received the complete cycle of PPT and took DMARDs achieved a satisfactory effect twice more frequently (58.1 and 32.3%, respectively; relative risk (RR) = 0.53; 95% confidence interval (CI), 0.2–1.39; p = 0.024) and did not respond to therapy 3 times less often (21.0 and 58.1%, respectively; RR = 2.41; 95% CI, 0.87–6.71; p = 0.001) according to the EULAR criteria than those who had refused PPT. The patients with MDs who received DMARDs + PPT during one year were unresponsive to therapy significantly less frequently than those who received DMARDs and BAs without PPT (21 and 44.8%, respectively; RR = 0.6; 95% CI, 0.21–1.7; p = 0.029). After 5 years of follow-up, the probability of no response to RA therapy in MD patients who received only DMARDs was 3.6 times higher than in those who had PPT (66.7% and 10.4%, respectively; RR = 3.58; 95% CI 0.82–15.5; p < 0.001). The patients adequately treated with DMARDs and BAs for MDs according to the DAS28 showed 1.3-fold more frequently good and satisfactory results (100 and 76.2%, respectively; p = 0.14) than those who refused PPT, but these differences were not statistically significant because the DMARD+BA+PPT group was small. Five-year follow-up indicated that DAS28 remission was more common in the patients receiving DMARDs and PPT than in those who had DMARDs and no PPT (34.5 and 8.3%, respectively; RR = 1.79; 95% CI, 0.34–9.24; p = 0.024). DAS28 remission was somewhat more frequently observed among the patients receiving DMARDs, BAs, and PPT than among those taking DMARDs and BAs (33.3 19.0%, respectively; RR = 1.64; 95% CI, 0.28–9.57; p = 0.34), but these differences were insignificant. Remissions according to the 2011 ACR/EULAR criteria were achieved by only the patients having DMARDs and PPT (6.9% and 13.8% after 1 and 5 years, respectively).

Conclusion. Adequate treatment of MDs in RA patients results in a significant increase in the efficiency of antirheumatic therapy.

Cardiovascular events (CVEs) are one of the most common causes of death in patients with psoriatic arthritis (PsA). The increased risk of CVEs in PsA is due to a combination of traditional cardiovascular risk factors (TCVRFs) and common inflammatory mechanisms underlying PsA and atherosclerosis.

Objective: to estimate the prevalence of TCVRFs, subclinical atherosclerosis, and coronary artery calcification in young patients with early peripheral PsA.

Subjects and methods. The investigation enrolled 25 patients (13 men, 12 women) with early PsA, who had participated in the REMARCA study. Their median age was 36 [27; 46] years; the duration of PsA and psoriasis was 5 [3; 7] and 13 [9; 84] months, respectively; DAS was 3.8 [3.4; 5.4].

All the patients underwent assessment of TCVRFs, Doppler ultrasound (DUS) of the carotid arteries (CA), evaluation of CA calcification (CAC) using multislice computed tomography (MSCT) and echocardiography, as well as 24-hour blood pressure monitoring.

Results and discussion. The patients with early PsA were found to have the following TCVRFs: hypertension in 11 (44%), abdominal obesity in 14 (56%), smoking in 16 (64%), a family history of cardiovascular disease in 6 (24%), menopause in 5 (20%), and dyslipidemia in 14 (56%). There was one TCVRF in 7 (28%), two TCVRFs in 6 (24%), three TCVRFs in 2 (8%), four TCVRFs in 4 (16%), and five TCVRFs in 6 (24%). A combination of three or more TCVRFs was found in 12 (48%) patients.

Calculation of ten-year total coronary risk scores identified low [17 (68%)], moderate [6 (24%)], high [1 (4%)], and very high [1 (4%)] risk groups among the patients with a history of acute cerebrovascular accident. Analysis of the relationship between PsA activity and total coronary risk scores revealed no significant differences. Thus, the patients with moderate PsA activity showed low, moderate, and high coronary risks in 7 (70%), 2 (20%), and 1 (10%) cases, respectively.

Those with high PsA activity had low, moderate, high, and very high risks in 10 (66.7%), 4 (26.6%), and 1 (6.7%) cases, respectively. CA DUS revealed atherosclerotic plaques (ASPs) in 8 (32%) patients and increased CA intima-media thickness (IMT) values of >0.9 mm in 8 (32%). MSCT showed signs of CAC in 4 (16%) patients. There was a correlation of IMT values with low-density lipoprotein (r = 0.48; p = 0.03), total cholesterol (r = 0.53; p = 0.01), systolic blood pressure levels (r = 0.59; p = 0.02), and abdominal obesity (r = 0.64; p = 0.001) and an inverse correlation between high-density lipoproteins and C-reactive protein levels (r = -0.52; p = 0.03).

Young patients with PsA with early-stage disease have a high prevalence of TCVRFs, CA atherosclerotic changes, and CAC signs with relatively low total coronary risk scores, which may indirectly suggest that chronic inflammation is involved in the development of atherosclerosis.

Etanercept (ETC), a soluble tumor necrosis factor-α (TNF-α) receptor, was registered for the treatment of spondyloarthritis in the Russian Federation in 2009. By now, results of prolonged treatment with this drug have been obtained in patients with ankylosing spondylitis (AS).

Objective: to evaluate the efficiency and tolerability of long-term therapy with ETC in patients with AS.

Subjects and methods. The follow-up included 60 patients with a documented diagnosis of AS (the 1984 New York criteria) and/or axial spondyloarthritis (the 2009 ASAS criteria), high activity (BASDAI >4), who received long-term (at least a year) regular therapy with subcutaneous ETC 50 mg weekly. Its effect was evaluated using the ASAS criteria.

Results and discussion. ETC was the first TNF-α inhibitor used in 29 (48%) patients, including 22 (76%) patients who achieved partial remission; 7 (24%) patients who showed ASAS40 improvement. One patient was observed to have hepatotoxicity (alanine aminotransferase elevation to 72 U/l), therefore the drug was discontinued. 31 (52%) patients were switched to ETC after infliximab (n=24) or adalimumab (n=7) due to their poor tolerance and/or loss of effect. At the same time, ETC became the second anti-TNF-α drug in 21 (35%) patients and the third one in 10 (17%). During the therapy, 21 (68%) patients switched to ETC were noted to have ASAS40 improvement; 9 (29%) patients had partial remission. The insufficient effect of the drug was observed only in one patient (3%), in whom ETC was the third TNF-α inhibitor. The tolerability of ETC was generally satisfactory after switching from anti-TNF-α monoclonal antibodies (mAbs). Three patients developed de novo psoriasis that required therapy discontinuation in one patient; another male patient developed uveitis for the first time. In one female patient with AS associated with Crohn’s disease with loss of mAb effect, ETC became the third anti-TNF-α drug, during therapy with which there was no exacerbation of Crohn’s disease. The drug was well tolerated and highly effective in 8 patients, in whom ETC treatment was initiated at the age of 60 years and older: 6 (75%) achieved partial remission, 2 (25%) had ASAS40 improvement. Nine patients with previous and/or latent tuberculosis had no exacerbation of this disease during ETC therapy.

Conclusion. During regular therapy with ETC, more than half of patients (52%) achieved partial remission according to the ASAS criteria, while this was more frequently achieved when the drug was used for the first time (76%) than when mAbs were switched to ETC (29%). Discontinuation of the latter due to adverse events was required only in 2 (3%) patients.

Objective: to compare the efficacy of methotrexate (MTX) and colchicine in patients with chronic arthritis in calcium pyrophosphate crystal deposition disease (CPPDD).

Subjects and methods. Data from a controlled prospective cross-sectional study of 10 patients (8 women and 2 men) with chronic arthritis in CPPDD are presented. In the initial period of treatment, all the patients were given colchicine 1 mg/day for 3 months, followed by a wash-out period for 1 month and then subcutaneous MTX 20 mg/week for 3 months. The diagnosis of CPPDD was made if there were calcium pyrophosphate crystals in synovial fluid and signs of chondrocalcinosis, as evidenced by joint X-ray and/or ultrasonography. DAS44, the swollen joint count (SJC) and tender joint count (TJC), pain intensity on a visual analog scale (VAS), the Health Assessment Questionnaire (HAQ) index, and serum C-reactive protein (CRP) levels were determined in all the patients at baseline, 3 months after the beginning of treatment with colchicine, after a wash-out period, and 3 months after the beginning of MTX treatment.

Results and discussion. At baseline, mean DAS44 value was 2.47±0.27; SJC and TJC were 2.0±0.6 and 2.4±1.1, respectively; pain intensity was 55.2±12.3 mm; serum CRP level – 3.89±3.82 mg/l; HAQ – 1.1±0.3. Three months after colchicine therapy initiation, mean DAS44 value decreased to 1.76±0.28 (p = 0.004), SJC – to 1.4±0.5 (p = 0.048), TJC – to 1.6±1.35 (p = 0.023), pain intensity – to 42.0±13.2 mm (p = 0.023), CRP level – to 3.13±2.85 mg/l (p = 0.75), HAQ – to 0.95±0.3 (p = 0.041). Good response was achieved in 7 patients after 3 months of colchicine therapy. After the wash-out period, the mean DAS44 value was 2.08±0.26; SJC and TJC – 1.6±0.5 and 1.7±1.4, respectively; pain intensity – 46.5±9.8 mm; CRP level – 3.38±1.74 mg/l; HAQ – 1.3±0.34. Following 3 months of MTX therapy, mean DAS44 value decreased to 1.39±0.45 (p = 0.027), SJC – to 0.7±0.5 (p = 0.023), TJC – to 0.6±0.5 (p = 0.007), pain intensity – to 26.0±18.97 mm (p = 0.045), CRP level – to 2.87±2.06 mg/l (p = 0.75), HAQ – to 0.8±0.6 (p = 0.045). Two of the 3 patients with an insufficient effect of colchicine achieved DAS44 remission after MTX treatment; two patients attained remission after therapy with colchicine and developed an exacerbation of the disease when this drug was replaced by MTX.

Conclusion. MTX 20 mg/week is as effective as colchicine in most cases and can be the drug of choice in patients with chronic arthritis in CPPDD if colchicine therapy is ineffective.

Objective: to investigate the nosological pattern of primary disability due to osteoarthritis (OA) in the Irkutsk Region in 2012–2016, its severity, age- and gender-related features.

Material and methods. A continuous method was used to analyze the database on the newly recognized as disabled due to OA in the Irkutsk Region in 2012–2018. The pattern, degree of disability, its age- and gender-related characteristics were studied.

Results and discussion. Among those who were the newly recognized as disabled due to OA in 2012–2014, there was the largest proportion of patients with knee OA (46.6 to 52.7%, respectively). There was a preponderance of the disabled due to hip OA in 2015–2016 (55.3% and 65.3%, respectively). Among the OA patients with newly established disability during the analyzed period, there was a preponderance of women, but their proportion in 2016 was smaller than that in 2012 (54.4 and 74.2%, respectively). The decrease was mainly due to women with polyarticular OA (83.5% in 2012; 67.5% in 2016), hip OA (54.3 and 42.0%), or knee OA (87.2 and 80.4%, respectively). Most of the disabled people with OA were pensioners (64.7% for an average of 5 years). Over the past 3 years, the proportion of disabled young people (aged less than 44 years) substantially increased from 7.1% in 2014 to 15.3% in 2016 and that of disabled adult people rose from 21.2% in 2014 to 30.1% in 2016. Among the disabled with hip joint damage, young and adult patients were more and pensioners were fewer than among those with polyarticular OA (16.8 and 3.1%; 31.5 and 23.5%; 51.9 and 73.4% for an average of 5 years, respectively).

Knee OA-associated disability was established mainly in pensioners (80.2%), while the proportion of young people was only 2.0%, which was much fewer than in those with hip OA, polyarticular OA, and other variants of the disease (9.2%). The proportion of disabled adult persons was also fewer (18.1% for an average of 5 years). According to the severity of disability due to OA, the largest one was group with class III disability (81.7% in 2012 to 90.9% in 2015). Group with class II disability were much less – from 16.1 to 8.3%, respectively. The minimum number of patients was recognized as having class I disability. The last five years have been marked by an improvement in the pattern of polyarticular OA and osteoarthritis-related primary disability according to its severity, by reducing the proportion of people in the most severe groups with class I and II disability and by increasing the proportion of people having class III disability. The increase in the proportion of people with class II disability due to hip OA to 22.3% in 2016 from 11.4% in 2015 was due to isolated cases of complications after hip replacement.

Objective: to clarify pregnancy outcomes in patients with ankylosing spondylitis (AS) and its course during pregnancy on the basis of a retrospective analysis.

Subjects and methods. The paper presents the results of Stage 1 of a Russian pilot study of the impact of pregnancy on AS activity, parity, and pregnancy outcomes in these patients. The basis for Stage 1 is the results of a retrospective analysis of the data of a questionnaire survey of 204 women with AS, during which they answered 19 questions regarding the presence of pregnancies and their outcomes, the reasons for the lack of pregnancies, and a subjective assessment of their health status during gestation. The respondents’ mean age was 32.0±5.8 years; the mean disease duration was 107.2±73.5 months.

Results and discussion. 84 (41.1%) women did not have pregnancies; the reasons for the lack of pregnancies were associated with AS (fear of the teratogenic effect of taken drugs; a child’ inheritance of the disease) in 48.8% of the cases, with infertility in 9.5%, and with non-medical reasons in 41.7%. A total of 120 (58.9%) women had 248 pregnancies; of whom 136 patients had the latter before and 112 – after the onset of AC. In AS versus a healthy life period, there were lower abortion rates at a woman’s will (8.9 and 35.3%, respectively; p < 0.01), more pregnancies that resulted in childbirth (75.9 and 52.2%, respectively; p < 0.01), and higher surgical delivery rates (43.5 and 28.2%, respectively; p < 0.05). The premature birth rate after the onset of AC was 13.0%. The newborn infants born before the onset of AS in mothers did not differ from those born after the onset of AS in mothers in weight and Apgar scores. Half of the respondents reported that their health status was improved in any trimester of pregnancy (slightly more often in the first trimester; but the difference was statistically insignificant). However, almost 70.0% of the respondents noted their worse health status during pregnancy, the severity of AS symptoms increased with a gestational period.

Conclusion. The reason for the lack of pregnancies in almost half of the patients with AS is associated with their subjective fear of the negative impact of the disease and therapy on a child’s future health. In AS versus a healthy life period, surgical deliveries are performed more frequently. The neonatal outcomes of pregnancies do not differ before and after the onset of AS.

The lecture presents an update on the epidemiology, pathogenesis, clinical manifestations, diagnosis, differential diagnosis, and treatment stages of polymyalgia rheumatica, a chronic inflammatory disease of unknown etiology, which affects older people.

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Authors of the article «Rheumatic polimyalgia», published in the section «Program of continuous post-graduate education of therapists» in journal «Scientific-practical rheumatology» No 2 (V 56)2018 Satybaldyev А.M et al. present their apologies for unpremeditated using for educational purposes of illustrative material on page 217 (Fig. 1) from the article by BlockmansD. Positronemission tomography and magnetic resonance imaging. In: Polymyalgia Rheumatica and Giant Cell Arteritis, Chapter 8.OxfordUniversity press. EdBh. DasguptaandCh. Dejaco; 2016:63-71 and on page 220 (Fig 2) from the article by M. Jiang,S. Navanathan“Crowned dens syndrome: a rare cause of neck pain and fever”Med J Aust 2017; 206 (5):199 || http://doi.org/10.5694/mja16.01077 Published online: 20 March 2017.

Giant cell arteritis (GCA), formerly known as Horton's disease, is among the most common diseases from a group of systemic vasculitides; its clinical significance is complemented with the potential involvement of the coronary arteries, aorta, and cranial arteries with development of ischemic optic neuropathy if there is no timely treatment that results in rapid and irreversible visual loss. The elderly age of patients is one of the key aspects of GCA. Therefore, the disease is often accompanied by various comorbidities that have considerable impact on the choice of a treatment regimen and limit the use of standard therapy with glucocorticoids (GCs). The complications due to GC treatment can be competitive in severity with GCA, especially in elderly multimorbid patients. Progress in rheumatology due to the introduction of biological agents (BAs) has created the preconditions for the development of a new area of pharmacotherapy for GCA associated with interleukin 6 (IL6) inhibition using tocilizumab (TCZ). According to the results of two randomized placebocontrolled trials (RPCTs), which were published in 2016, the rate of remission with TCZ treatment was significantly higher in patients with GCA than that in the placebo group (p = 0.03–0.0001), as is relapse-free survival after 52 weeks of TCZ treatment (85 and 20%, respectively; p ≤ 0.001), the incidence of serious adverse events (AE) was 14–35%. In 2017, the results of Phase III GiACTA RPCT became the basis for approval of the use of TCZ for the treatment of GCA in the United States and Europe. The authors present their own results of a small prospective study of TCZ in 7 patients with active GCA with severe comorbidity, including multimorbidity, that potentially increases the risk for AE due to GC therapy. The mean age of the patients was 71.3±7.6 years; among them there was one man and 6 women. The administration of TCZ in a monthly dose of 2.3–8.8 mg/kg for 1–10 months with a cumulative dose of 10–58.1 mg/kg could reduce the mean daily dose of prednisolone to 15 (5–32.5) mg, thereby preventing the development or progression of AE, and all the 7 patients could rather rapidly achieve GCA remission. A recurrence after therapy discontinuation was noted in one patient. TCZ treatment was accompanied by serious AE (purulent elbow bursitis); and other two patients had AE a few months after TCZ discontinuation. There were no fatal outcomes. Thus, the presented results suggest that the use of IL6 inhibitors in patients with GCA, including those with severe comorbidity, can be regarded as a potentially effective innovative (off-label) treatment strategy with an acceptable safety profile. The further expansion of an evidence base and the clarification of the medical and economic aspects of TCZ treatment in some groups of GCA will help justify the choice of BAs.

Anti-centromere antibody (ANCA) seropositivity is generally regarded by rheumatologists as a sign of systemic sclerosis (SS) in clinical practice. However, the literature describes many cases of ANCA-associated primary Sjogren's syndrome (PSS) that is the subtype of this disease, which differs from the classic type in a number of laboratory and clinical manifestations. According to the literature, even a long-term follow-up indicates that only one quarter of patients with ANCA-positive PSS develop documented SS. This fact raises the question of whether it is necessary to include ANCA into the list of autoantibodies pathogenetically related to PSS.

To evaluate the efficacy and safety of intraarticular hyaluronic acid (HA) in osteoarthritis (OA), the authors have analyzed the scientific publications included in foreign databases (Medline/PubMed) on the relevant laboratory, experimental, and clinical studies conducted over the past 20 years. A few in vitro laboratory studies of individual HA drugs confirm their positive effect on articular cartilage. Analysis of the literature on their in vivo use has shown that a variety of intraarticular HA drugs permitted for use in Russia have not been studied in clinical trials, which raises doubts about their efficacy. At the same time, the published works suggest that a significant clinical effect is achieved with HA therapy mainly in early OA and that this therapy for end-stage knee OA yields poor results. The data regarding the clinical efficacy of different HA drugs are very contradictory. Thus, there is a need for additional comparative postmarketing research of HA drugs permitted for use in the Russian Federation, by stratifying patients according to the characteristics of the clinical phenotype of the disease, available risk factors for OA progression, and the magnitude of morphological changes in joint tissues, as verified by magnetic resonance imaging and/or arthroscopy.

Susac's syndrome (SS), or retinocochleocerebral vasculopathy, is an extremely rare severe incapacitating disease, the basis for which is assumed to be autoimmune disorders. SS is characterized by microangiopathy with a triad of lesions of the brain, inner ear, and retina and refers to interdisciplinary problems. The results of brain magnetic resonance imaging, retinal fluorescein angiography, and audiometry and the interaction of physicians of various specialties, such as rheumatologists, neurologists, ophthalmologists, and otolaryngologists, are of key importance for the diagnosis of SS. The paper describes a case of SS and reviews the literature with emphasis on pilot recommendations for the treatment of this condition, which have been published in 2018, suggesting the combined use of high-dose glucocorticoids, intravenous immunoglobulin, cytostatic agents, and rituximab.

The paper provides a clinical description of relapsing polychondritis, a rare systemic immunoinflammatory disease that was characterized by fever episodes, cartilage damage, and reactive arthritis. Diagnostic searching could rule out an infectious disease (by determining a wide variety of potential pathogens), as well as a septic condition due to a pronounced leukemoid reaction of the myeloid type, and the presence of toxigenic neutrophil granulation in peripheral blood and bone marrow. All available clinical, functional, and radiological studies were used to make a differential diagnosis with paraneoplastic syndrome due to blood disease, primarily with myeloma. The data obtained could interpret the changes only as reactive. No increased autoantibody titers were identified. Clinical symptoms and the good effect of prednisolone therapy allowed a diagnosis of relapsing polychondritis, a rare disease that belongs to the group of immunoinflammatory diseases according to the current ideas. It is suggested that autoinflammation plays a role in its pathogenesis.