FRONTIERS
Osteoporosis (OP) is the most common bone disorder associated with an increase bone fragility and a high fracture risk, which can be an isolated condition or a comorbidity of immuno-inflammatory rheumatic diseases. A great contribution to the study of OP in the Russian Federation was made by V.A. Nasonova, L.I. Benevolenskaya and scientific researchers of the Institute of Rheumatology. The article presents the main achievements that have occurred over the past 30 years in the development of this problem in our country and abroad, and the perspectives of osteoporosis treatment.
INTERNATIONAL AND RUSSIAN GUIDELINES FOR THE TREATMENT OF RHEUMATIC DISEASES
The article presents updated guidelines developed by the American College of Rheumatology and the American Association of Hip and Knee Surgeons on the perioperative treatment of patients with rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, juvenile idiopathic arthritis and systemic lupus erythematosus undergoing elective total hip or total knee arthroplasty. The perioperative use of anti-rheumatic drug therapy, including traditional disease-modi fying antirheumatic drugs, biologic agents, targeted synthetic small-molecule drugs and glucocorticoids. All recommendations are conditional and based on the results of retrospective clinical studies, which should be taken into account in decisionmaking when choosing perioperative antirheumatic therapy.
PROBLEMS OF RHEUMATOLOGY DURING THE 2019 CORONAVIRUS PANDEMIC
The aim of the study was to assess the safety of the combined vector vaccine Gam-COVID-Vac (Sputnik V) and to determine the risk factors for the development of adverse events in patients with immuno-inflammatory rheumatic diseases (IIRD).
Patients and methods. A single-stage study of patients with IIRD who were on inpatient treatment or who applied to the consultative and diagnostic center of the V.A. Nasonova Research Institute of Rheumatology was conducted, who received both components of the Sputnik V vaccine. The control group included immunized persons without IIRD. All participants were interviewed by a research doctor with filling out a unified questionnaire, additional information was obtained from medical documentation.
Results. The study included 325 patients with IIRD and 138 healthy controls. After vaccination with the first component, the number of patients with IIRD, in whom the development of local and systemic adverse events (AEs) was noted, was significantly lower compared to the control (20.3% and 38.4% respectively; p<0.001). These differences also persisted after immunization with the second component (12.3% and 28.3% respectively, p<0.001). After complete vaccination, no AEs were documented in 40.3% of patients and 22.5% of the control group (p<0.001). Female sex and, possibly, methotrexate therapy increases the risk of developing local and systemic AEs on the first component of the vaccine, rituximab therapy - on the second. A lower incidence of AEs is typical for elderly patients, patients with a disease duration of more than 10 years and obesity. Exacerbation of IIRD was registered in 1 (0.3%) case, the occurrence of new autoimmune phenomena was not observed.
Conclusions. According to the data obtained, the use of Gam-COVID-Vac (Sputnik V) in patients with IIRD is safe.
PROGRESS IN RHEUMATOLOGY IN THE XXI CENTURY
Rheumatoid arthritis (RA) is the most common immune mediated (autoimmune) rheumatic disease, manifested by chronic erosive arthritis and systemic internal organ damage. Currently, RA is considered as a syndrome characterized by clinical and pathogenetic heterogeneity associated with a variety of mechanisms of pathological activation of innate and acquired immunity, determining the variability of the course and outcome of the inflammatory process and effectiveness of therapy. Based on the detection or absence of rheumatoid factor (RF) IgM and antibodies to cyclic citrullinated peptides (ACCP), RA can be conventionally divided into two subtypes (phenotypes): seropositive RA and seronegative RA, but thanks to improvement of laboratory diagnostic methods the spectrum of autoantibodies detected in RA has increased significantly. Diagnosis of seronegative RA based on classification (rather than diagnostic) criteria can be difficult, especially in the early stages of the disease, and the diagnosis is made only during long-term follow-up of patients. It complicates the timely prescription of adequate anti-inflammatory therapy. This article summarizes the data on genetic predisposition, immunopathogenesis, biomarkers, clinical spectrum, instrumental diagnosis and pharmacotherapy of seronegative RA.
REVIEWS AND LECTURES
Systemic lupus erythematosus (SLE) is a disease of women of reproductive age. Up to a certain time, pregnancy was contraindicated in patients with SLE, improving the management of the disease itself (monitoring), as well as understanding the safety of drugs make pregnancy possible for most patients with SLE. Careful pregnancy planning is crucial when the disease is well controlled with pregnancy-compatible medications. This is also facilitated by the management of patients jointly by doctors of different specialties (rheumatologist, neurologist, endocrinologist, etc.) with obstetricians. The article discusses the achievements of managing women with SLE during pregnancy planning, during pregnancy and after delivery.
YOUNG SCIENTISTS FORUM
The involvement of the cardiovascular system is a typical manifestation of systemic lupus erythematosus (SLE), which determines the high level of mortality and disability of patients. A serious clinical problem is the development of heart failure (HF), which frequency in SLE is 3–4 times more than in the population. The development of this pathology is a complex process that occurs under the influence of systemic autoimmune inflammation and associated with heart damage (pericarditis, myocarditis, endocarditis, coronary artery disease, myocardial infarction), disorders of the cardiac conduction system (various arrhythmias), atherosclerosis, arterial hypertension, pulmonary hypertension, thrombosis against connected with bleeding disorders (especially associated with antiphospholipid syndrome), traditional risk factors, as well as the negative effect of anti-rheumatic therapy. Mostly HF in SLE occurs in a subclinical form with a preserved ejection fraction, and is detected using instrumental methods in more than 60% of patients.
The management of patients with SLE and HF requires early diagnosis of this pathology, to do this, various diagnostic methods are used (particularly, echocardiography with speckle tracking imaging technique) and the identification of biomarkers such as NT-proBNP. HF therapy in SLE patients is based on the maximal reduction o f the activity of the disease due to rational pathogenetic therapy, also the control of traditional risk factors – antihypertensive therapy, the use of statins and the prevention of arterial and venous thrombosis.
ORIGINAL RESEARCH
Aim. Switching to another biologic with the same mode of action provides greater opportunity for long-term management of patients with rheumatoid arthritis (RA). In clinical practice, especially in the context of the COVID-19 pandemic, such switching occurred for non-medical reasons as well. However, there is no information about switching from interleukin 6 (IL-6) receptor (R) inhibitor to direct IL-6 inhibitor.
Objective – to assess the efficacy and safety of therapy in RA patients, after switching from IL-6R inhibitors (tocilizumab (TOC), sarilumab (SAR)) to olokizumab (OKZ) for reasons not related to the loss of their efficacy or adverse events.
Material and methods. In this retrospective cohort study efficacy parameters and routine biochemical data were analyzed using descriptive statistics – mean values with standard deviation for continuous parameters and absolute and relative frequency for binary variables. Adverse events (AE) were reported according to patient’s files. The statistical significance and changes of the analyzed variables by visits were determined using paired t-test. Fisher’s exact test or chi-square test was used to compare the proportion of patients with improvement/no change and of patients with worsening. All tests were 2-sided, and p<0.050 was considered statistically significant. As this was an observational study, the statistical criteria have not been pre-specified.
Results. We analyzed results obtained during 5 visits (2 visits before switching, switching visit and 2 visits after switching) in 110 RA patients who switched to OKZ 64 mg every 4 weeks subcutaneously (SC). Most patients (79.1%) were women, and 70% of patients were both positive by rheumatoid factor and antibodies to cyclic citrullinated peptide. Mean RA duration was 11 [6; 16] years, previous treatment duration was 44 [27; 62] months and mean interval before switching to OKZ was 35 [31; 68] days. This relatively long interval led to an increase in DAS28-ESR (Disease Activity Score 28 with determination of erythrocyte sedimentation rate) from 2.4 [1.9; 3.0] to 2.6 [2.1; 3.5] and DAS28-CRP (DAS28 with determination of C-reactive protein level) from 2.8 [2.0; 3.3] to 2.9 [2.2; 4.0] (the trends were similar in patients who received combined therapy and monotherapy).
After switching, all of RA symptoms and indexes have been improved compared with the switching visit (some of them were significantly better even compared with stable therapy period e. g. DAS28-CRP was 2.4 [2.0; 3.1] in the overall group and 2.4 [2.1; 2.7] in the monotherapy group).
AEs were registered in only 7 (6.4%) patients, of which 1 (0.9%) case (an exacerbation of herpes infection) was considered as serious. The most frequent AEs were arthralgia and mild transient leukopenia (2 patients each). There were no deaths.
Conclusion. OKZ effectively maintained remission/low activity of RA after switching in both regimens: as add-on to disease modifying anti-rheumatic drugs and as monotherapy, and did not cause any additional safety concerns. The optimal results were reported when intervals before switching to OKZ were closer to those indicated in the instructions for IL-6R inhibitors.
According to the European guidelines for osteoporosis, the same FRAX intervention threshold is suggested for men as for women. At the same time, in the Russian Federation, according to research data, an extremely low proportion of identified men who are subject to the initiation of osteoporosis therapy. The female intervention threshold identifies only 1.1 to 4% of men for treatment.
Aim – to develop and evaluate various options for the intervention threshold using the FRAX calculator for men in the Russian Federation and adopt the most acceptable intervention threshold by consensus.
Material and methods. Delphi voting was conducted among 18 Russian experts who have publications and personal reports about their experience with the FRAX calculator.
For discussion, 5 intervention threshold options with the corresponding rationale based on the literature reference were presented, as well as the proportion of men of different ages to be initiated in each of the options (based on several Russian population-based studies).
Anonymous voting was carried out using the Delphi method with questionnaire placed in the Google form. It was proposed to evaluate all options for intervention thresholds on a 9-point Likert scale. Consensus was considered reached if the intervention threshold reached a Likert score of 7 or more points in 80% or more of the experts. The rating of each intervention threshold option was expressed as mean and standard deviations.
Results. In the first round of voting, the maximum rating and percentage of agreement is reached for the 9% fixed interference threshold option based on the FRAX calculation. The rating was 7.72±1.6 points, the percentage of experts’ agreement was 88.9%. A fixed threshold of 9% determined 13–19.5% of men aged 50 years and older to be treated for osteoporosis, while their proportion increased to 26–38% at the age of 85 years and older.
Conclusion. The consensus of experts of the Russian association on osteoporosis suggests initiating treatment of osteoporosis in Russian men with a 10-year probability of major osteoporotic fractures according to FRAX of 9% or higher.
Infections remain one of the main causes of morbidity and mortality in patients with immuno-inflammatory rheumatic diseases.
Objective – to study the efficacy, immunogenicity and safety of the 23-valent polysaccharide pneumococcal vaccine (PPV-23) in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (AРS).
Materials and methods. 91 patients were included in the study: 78 with SLE, of which 18 (23 %) – with secondary AРS, 13 – with primary AРS. 85 patients received immunosuppressive therapy, including 30 – genetically engineered biological drugs (bDMARD); 23 – anticoagulants. PPV-23 was administered subcutaneously, patients were observed for a year after vaccination.
Results. Local reactions were observed in 49% of patients with SLE and secondary AРS, in 23% of patients with primary AРS. General reactions were noted in isolated cases, were short-term and did not require additional prescriptions. During the follow-up period, no exacerbations of SLE, relapses of thrombosis and thromboembolism associated with vaccination were detected; no development of new autoimmune diseases was registered. 10 (13%) patients with SLE were immunized against the background of high activity of the disease, no adverse reactions were recorded. In some patients, a transient increase in a-DNA and ANF was observed during the year without signs of exacerbation of the disease. 56% of patients with SLE and secondary AРS, 15.4% with primary AРS were “responders” to the vaccine. There was no negative effect on the immune response of the dose of GC >10 mg/day, age, duration and activity of the disease. With the treatment of bDMARD, a full-fledged vaccine response was recorded much less frequently than with standard therapy (38% and 67.4%, respectively; p=0.01). After vaccination, there was a significant decrease in the number of lower respiratory tract infections (LRTI) (p=0.0001), including community-acquired pneumonia (PN) (p=0.03) and acute bronchitis (p=0.04), ENT infections (p=0.001). In the treatment of rituximab (RTM), compared with belimumab (BLM), a greater number of LRTI was observed, mainly due to PN. After vaccination on RTM therapy, the number of INDP in general (p=0.008) and PN in particular (p=0.03) decreased, isolated cases of LRTI and ENT organs were recorded on BLM therapy. Within 4–6 years after vaccination, 30 patients with SLE retained the clinical effect of vaccination, while immunogenicity decreased to 18%.
Conclusion. Safety, sufficient immunogenicity, and clinical efficacy of PPV-23 in patients with SLE and AРS have been shown. The use of bDMARD reduces the vaccine response. Immunization performed prior to or during treatment with bDMARD lasting <1 year increases the number of vaccine responders.
Objective – to identify different phenotypes of overweight in women with systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) based on body mass index (BMI) and serum leptin levels, as well as to determine the frequencies of various metabolic disorders, hypertension and cardiovascular complications in individual phenotypes.
Material and methods. The study included 50 women with RA and 46 with SLE aged 18 to 65 years without a history of diabetes and fasting hyperglycemia. The concentration of leptin (ELISA), insulin (electrochemiluminescence analysis) was determined in all patients, and the HOMA-IR index was calculated. Hyperleptinemia was diagnosed at leptin concentrations >11,1 ng/ml, insulin resistance (IR) – at HOMA-IR values ≥2,77. Three main phenotypes of overweight were distinguished: “classic” (BMI≥25 kg/m2 + hyperleptinemia), “healthy” (BMI≥25 kg/m2 , without hyperleptinemia), “hidden” or “latent” (BMI<25 kg/m2 + hyperleptinemia), as well as “normal weight” (BMI<25 kg/m2 , without hyperleptinemia).
Results. Patients with RA and SLE were similar in age (p=0.4), disease duration (p=0.2) and BMI (p=0.5). Hyperleptinemia was found in 46% of women with RA and 74% – with SLE (p=0.005), IR – in 10% and 22% of patients, respectively (p=0.2). The “classic” phenotype of overweight was diagnosed in 30%, “healthy” – in 8%, “hidden” – in 16% of cases with RA and in 44%, 0% and 30% of cases with SLE, respectively. IR was found in 3%, hypertension – in 6% of patients with “normal weight”. With the “classical” phenotype, IR (29%) and hypertension (66%) were more common than with “normal weight” (p<0.01 in all cases), with the “hidden” phenotype, significant differences were obtained only in hypertension frequency (45%; p=0.0012), but not IR (18%). 3 out of 4 women with a history of cardiovascular complications suffered from “classic” overweight, one patient had a “normal weight”.
Conclusion. In women with SLE up to 65 years of age, the frequency of hyperleptinemia, but not IR, is higher than in patients with RA. In both diseases, the “classic” overweight phenotype is most common. In RA, a “hidden” phenotype was detected less often than in SLE, at the same time, a “healthy” phenotype is not characteristic of SLE. The frequencies of metabolic disorders and hypertension is low with the “normal weight” and “healthy” phenotype, high – with the “classic”, intermediate – with the “hidden” phenotype.
Objective. To evaluate the ovarian reserve in women with systemic sclerosis (SSc) and to analyze the relationship of the concentration of anti-Müllerian hormone (AMH) with the main manifestations of the disease and therapy.
Material and methods. The study included 74 SSc patients aged 18 to 40 years; the control group consisted of 32 healthy women, matched by age. The concentration of follicle-stimulating hormone (FSH), luteinizing hormone (LH), prolactin, estradiol (E2) and testosterone was determined by enzyme immunoassay (ELISA), AMH quantitatively using standard chemiluminescent analysis on paramagnetic particles in blood serum. The AMG level of 1.0– 10.6 ng/ml was taken as normative values. Values <1.0 were regarded as a decrease in ovarian reserve.
Results. In patients with SSD, the levels of AMH and testosterone were significantly lower than 1.4 [0.5; 2.3] and 0.45 [0.2; 0.96], respectively, versus 2.4 [1.8; 3.3] (p=0.002) and 1.6 [0.97; 2.5] (p=0.0001) in the control. The concentration of prolactin and E2 was recorded higher with SSDs – 22.23 [14.08; 31.18] and 140.2 [102.43; 179.74], respectively, against 10.2 [7.11; 16.68] (p=0.000002) and 95.3 [64.50; 130.0] (p=0.002) in the control. A decrease in the ovarian reserve by the level of AMH was significantly more often detected in patients with SSD in 43% versus 9.4% in the control (p=0.002). The risk of AMH reduction in patients with SSD was 7 times higher compared to the control (OR=7.030; 95% CI: 1.97–25.11). The levels of the hormones studied were comparable in patients with low and normal ovarian reserve. Diffuse form (46.9%) and subacute course of the disease (53.1%) were more often detected in patients with SSD and with low ovarian reserve compared to those with normal ovarian reserve (23.8% (p=0.033); 23.4% (p=0.004)). The frequency of organ lesions of SSDs, immunological disorders, inflammatory markers, and the lipid spectrum in the groups did not differ depending on the level of AMH. There were also no differences in the regimens and doses of treatment with basic anti-inflammatory drugs and glucocorticoids. Menstrual cycle disorders were noted by 31% of patients with SSD versus 6.2% in the control (p=0.004). Premature ovarian insufficiency (POI) was detected in 6.8% of patients with SSD and none in the control group (p=0.02). Patients with SSD and POI did not differ in age, duration of illness, clinical manifestations and therapy of them without POI.
Conclusion. The concentration of AMH and testosterone was significantly lower in patients with. A decrease in ovarian reserve was significantly more often detected in women with SSs. Low ovarian reserve was more often detected in patients with diffuse form and subacute course of the disease. POI was more often observed in the group of SSc.
The study of pain in the projection of joints occurrence is an important medical and social task, the solution of which makes it possible to determine the amount of necessary forces and means for the organization of medical care for these patients. For the first time in Russian Federation on the basis of Saint-Petersburg Research Institute of Emergency Care named after I.I. Dzhanelidze the concept of providing medical care to patients with pain in the projection of joints was introduced into clinical practice. The study of the prevalence of pain in the projection of joints data, places and roles of rheumatological diseases (RD) allows to outline ways to optimize diagnostic and therapeutic algorithms, reducing the time of diseases diagnosis and optimizing the inpatient stage of treatment duration. An increase in the proportion of patients referred to the outpatient stage allows rational use of specialized beds in the hospital and gives a significant economic effect for the healthcare system.
RD are the second most common cause of pain in the projection of joints. Its average percentage of patients referred for hospitalization was 2.69% of the total number of patients in the Saint-Petersburg Research Institute of Emergency Care named after I.I. Dzhanelidze. The structure of the incoming flow of patients with pain in the projection of joints was analyzed, the nosological structure of patients with RD was evaluated. Osteoarthritis, rheumatoid arthritis and gouty arthritis are the leading causes of pain in the projection of joints.
Achieving a good treatment outcome in rheumatic diseases (RD) requires regular, dynamic patient monitoring and therapy correction if it is not effective or intolerant. The patient assessment must be based on clear criteria to objectify the main manifestations of the disease. For this purpose, the calculation of standard activity and severity indices (DAS28, CDAI, SDAI, BASDAI, ASDAS, DAPSA, PsARC, PASI, etc.) is used. However, this methodology does not always allow the assessment of the fundamentally important parameters of treatment outcome – patient satisfaction and well-being. According to a series of studies, poor therapy satisfaction may be observed in ≈25% of patients with systemic RD who are in remission/low disease activity according to standard indices. Moreover, in 20–30% of cases there is a major discrepancy in the assessment of therapy outcome between the patient and physician.
Therefore, a more accurate assessment of the patient’s condition requires, in addition to the calculation of standard indices, the mandatory analysis of patient-reported outcomes – pain, functional impairment, general assessment of disease activity, fatigue, etc. A valuable tool for determining well-being and good therapy outcome from the patient’s point of view is the PASS (“patient acceptable symptom state”). This simple and quite informative index correlates well with core symptoms and indicators of remission/low disease activity. PASS analysis can be used in telemedicine follow-up of patients when an objective examination is not possible. The combined use of PASS and standardized indices can better assess treatment outcomes and improve the quality of life of patients with RD.
PEDIATRIC RHEUMATOLOGY
Seropositive juvenile idiopathic arthritis (JIA) is one of the rarest and most unfavorable subtypes of juvenile arthritis, characterized by an increased frequency of inefficacy of therapy.
Objective – to characterize biologic therapy in patients with seropositive JIA, to identify factors influencing the choice of a biological agents (BA) and the need to replace it, to evaluate the value of the JADI damage index for predicting the response to BA.
Material and methods. The diagnosis of seropositive JIA for the period from 2010 to 2022 was verified in 92 patients, 10.9% were boys. The median age of JIA onset in the study group was 12.0 [7.7; 14.0] years. BA were prescribed to 89.1% of patients in the study group, 31.7% of them for a period of less than 1 year from the onset. The median number of active joints at the time of BA initiation was 15 [10; 22], median ESR – 29 [18; 43] mm/h, CRP – 15.0 [5.3; 31.0] mg/l. Extra-articular manifestations at the time of prescribing BA occurred in 29.0% of patients. The analysis of factors that could influence the need to switch BA was carried out: age of onset, timing of diagnosis verification and initiation of BA, gender, the number of active joints at the start of BA, ACCP positivity, RF, ACCP, ESR and CRP values – at the time of BA appointment, the presence of secondary Sjögren’s syndrome. Since 2021, the complex of examinations included the calculation of the JADI (The Juvenile Arthritis Damage Index) damage index in all patients from the study group who were admitted to the hospital (28 in total; 17.9% – boys). The median age of JIA onset among them was 10.5 [6.31; 13.0] years, 81.2% received BA. The JADI index was compared with the ACCP, RF, CRP, ESR and the need to prescribe and switch BA. The design of the study was a retrospective, open-label, non-randomized, uncontrolled study.
Results. In the study group of patients, 29% had experience with more than 1 BA. Abatacept (45.1%), TNF-inhibitors (40.3%) were most often used as the first BA; tocilizumab and rituximab were predominantly used in the 2nd–4th line of therapy, with a trend towards their more frequent prescription in recent years. The main reason for switching from one BA to another is the secondary failure of therapy, 4.9% of patients have serious adverse reactions (AE). In general, AEs that did not require discontinuation of therapy were recorded in 24.6% of patients. Patients who received more than 1 BA had relatively higher values of RF, ACCP and significantly higher CRP. The mean value of JADI-A was 2.39 points, 50% of patients had significant JADI-A scores, 92.8% of whom received BA with experience of more than 1 prescription of BA in 28.6% of them. A direct correlation of the JADI index with ACCP, ESR and CRP was revealed.
Conclusions. Seropositive JIA is characterized by a high need for prescribing BA, the frequency of prescribing BA is associated with significant indicators of the JADI damage index. The choice of a specific BA is determined, first of all, by the presence of systemic manifestations or secondary Sjögren’s syndrome. In patients with high surrogate measures of activity (especially CRP), given the high risk of secondary failure of TNF-inhibitors, tocilizumab in the first line of therapy may be considered as the preferred choice. Our data did not reveal an effect of ACCP positivity on the preferred choice or frequency of BA replacement. Attention was drawn to the trend towards higher RF and ACCP values in patients treated with more than one BA. A correlation was established between the JADI index and ACCP, ESR, and CRP, which indirectly leads to the conclusion that it is necessary to prescribe BA earlier in this category of patients in order to avoid permanent damage and increase the effectiveness of thera py. The use of BA had an acceptable safety profile.
ORTHOPEDIC RHEUMATOLOGY AND REHABILITATION
Injuries cause a systemic neurohumoral and behavioral response of the body, aimed at restoring damaged tissues and correcting biomechanical disorders. However, in many cases, full-fledged repair is impossible – traumatic injury, inflammation that occurs against its background, and degenerative processes (fibrosis, neoangiogenesis, heterotopic ossification) lead to severe structural changes and a progressive decrease in functional ability. The most common complications of trauma include chronic post-traumatic pain and post-traumatic osteoarthritis (PTOA). These complications are interrelated – pain (accompanied by stiffness and dysfunction) that occurs in 10–50% of people who have suffered a joint injury may indicate the formation of early (pre-radiological) stages of PTOA. The development of typical structural changes in PTOA is observed 10–15 years after a knee injury (in >30% of patients). PTOA of large joints is more aggressive, often accompanied by synovitis, and requires arthroplasty on average 10–15 years earlier than primary osteoarthritis. Early diagnosis of PTOA is based on the analysis of the dynamics of clinical manifestations (primarily post-traumatic pain), visualization of early changes in the structure of the joint (magnetic resonance imaging), as well as the study of the level of biomarkers of inflammation and osteochondral destruction. As additional risk factors for PTOA, genetic features are considered that determine the chronicity of inflammation, pain, and impaired repair of cartilage and bone tissue.
CLINICAL OBSERVATION
Susac syndrome (SS) or retino-cochleo-cerebral vasculopathy is an extremely rare, severe, and potentially disabling condition. Underlying occlusive microangiopathy in SS is clinically characterized by the triad of encephalopathy, sensorineural hearing loss and branch retinal arterial occlusion. SS therapy envisages simultaneous use of high doses of glucocorticoids, intravenous immunoglobulins, cyclophosphamide and rituximab (RTХ). This article presents a case of remitting-relapsing slow-progressive SS with typical clinical manifestations demonstrating successful treatment SS with RTХ monotherapy; it also discuss the focus of RTХ monotherapy should be targeted at SS cases with contraindications for glucocorticoids and cytostatics use, slow-progressive SS or at early stages.
Articles
ISSN 1995-4492 (Online)