EVOLUTION OF SYSTEMIC JUVENILE ARTHRITIS DURING LONG-TERM FOLLOW-UP

Objective: to study the evolution of systemic juvenile arthritis (JA) in adult patients. Subjects and methods. The trial covered 19 patients (7 men, 12 women; mean age 28.8+9.1 years) with systemic JA who had fallen ill in childhood (at the age of 1 to 16 years). The duration of the disease was 11 to 53 years (mean 24.7+11.4 years). Its systemic manifestations at the onset included fever (n = 18), rash (n = 8), lymphadenopathy (n = 7), hepatolienal syndrome (n = 8), pericarditis (n = 1). At the onset, oligoarthritis was present in 9 (47.4%) patients; polyarthritis was in 7 (36.8%) and 3 patients had only arthralgies. Results and discussion. The rate of systemic manifestations was decreased over the course of the disease and they were detected only in 3 patients by the moment of the trial. At the examination, almost half (n = 9) had no active arthritis, 10 had inflammatory changes in the joints. At the trial, the total scores of inflammatory disease activity were retained in the vast majority (n = 17); however, its magnitude was low or modest. The mean HAQ score was 1.2. More than half of the patients had retarded physical development or disproportionate body build. X-ray changes were absent or minimal in 4 patients; there were erosions in 5, ankyloses in 3, and Stage IV sacroiliitis in 2. Six patients presented with aseptic necrosis of the heads of the femur and humerus. Nine patients received nonsteroidal anti-inflammatory drugs; 7 took glucocorticoids, and 7 had disease-modifying anti-rheumatic drugs, mainly methotrexate. At the study, the disease activity steadily remained low in 5 patients; 1 patient achieved complete remission; an exacerbation was seen in 13 of the 19 patients. 5-to-23-year remissions were recorded in 11 (57.9%) patients over the course of the disease. Conclusion. The patients with long-term JA tend to develop polyarthritis. Despite the severity, remaining inflammatory activity, and recurrent course of arthritis, the magnitude of its systemic and articular manifestations is reduced in most patients over time. During the disease, there may be long-term remissions; however, one third of the patients develop an erosive process and almost half do aseptic necrosis, which is the ground to continue active therapy

Функциональный статус и качествожизни взрослых больных ювенильнымартритом при длительном течениизаболевания

Objective: to assess the functional status and quality of life of adult patients long suffering from juvenile arthritis (JA). Subjects and methods. The study included 213 adult patients with JA, the majority (84.5%) of whom had been treated at the Childhood Department, Research Institute of Rheumatology, Russian Academy of Medical Sciences, in 1968 to 2008 or sought medical advice for the first time. Ninety-seven of the 213 patients were consulted or examined in the outpatient setting or at the clinic of the Research Institute of Rheumatology, Russian Academy of Medical Sciences. Among the patients enrolled into the study, women were prevalent (72.8%); the patientsmean age was 23.9±6.5 years (range 18 to 60 years); the disease duration was 17.4±6.8 years (range 10 to 53 years). The patients' functional capacity was determined by O. Steinbrocker's classification (functional class - FC) and HAQ questionnaire. The patients were asked (by mail, telephone, or personal interview) to fill in the questionnaire, by answering the questions concerning the functional and social status. Results. A third of the patients had no functional limitations (FC 1), 36% had minimal functional limitations (FC 2); 29% needed help with self-service, and 6.2% were severely disabled. According to the questionnaire survey data, the mean HAQ score was 0.8. Half of the respondents were found to have no functional limitations (HAQ score was 0). HAQ scores of 0.1-1.0 were observed in 33.5% of the respondents. 6.6% of the patients had the most serious problems with self-service (HAQ scores of 2.1-3.0) HAQ scores correlated with age and disease duration (p<0.001). The health status was appraised as good or very good, satisfactory, or bad by 43.3, 39.8, and 16.9% of the patients, respectively. There was a correlation between the health status and the HAQ scores (R = 0.6; p<0,001), age (R = 0.3; p<0,001), and JA duration (R = 0.3; p<0.001). At the moment of the study, most (78.9%) patients worked or studied at the moment; 48.2% had a higher education; 6.8% could not study or work because of the disease. A third of the examinees had families; 46 patients had children, which did not depend on the patients' health status and functional capacity. Conclusion. Most adult patients, long suffering from JA, have a good prognosis for their functional status and quality of life. At the same time some patients were observed to have a severer course of the disease and its outcomes with significant functional limitations; in this connection, they need treatment and a continued follow-up.Ц ел ь - оценить функциональный статус и качество жизни взрослых пациентов, длительно страдающих ювенильным артритом (ЮА). М ат ер иа л им ет од ы. В исследование были включены 213 взрослых больных ЮА, большинство из которых (84,5%) находились на стационарном лечении в детском отделении НИИ ревматологии РАМН с 1968 по 2008 г. или обратились впервые. 97 из 213 были консультированы и обследованы амбулаторно или в стационаре НИИ ревматологии РАМН. Среди включенных в исследование преобладали лица женского пола (72,8%), средний возраст больных составил 23,9±6,5 года (от 18 до 60 лет), длительность болезни - 17,4±6,8 года (от 10 до 53 лет). Функциональная способность пациентов определялась по классификации O. Steinbrocker (функциональный класс - ФК) и опроснику HAQ. Пациентам были предложены (путем рассылки, телефонного или очного опроса) для заполнения анкеты, содержащие вопросы о функциональном и социальном статусе. Р ез ул ьт ат ы и сс ле до ва ни я. Треть пациентов не имели функциональных ограничений (ФК 1), у 36% они были минимальными (ФК 2), 29% нуждались в помощи при самообслуживании, а 6,2% являлись тяжелыми инвалидами. По данным анкетирования, среднее значение индекса HAQ составило 0,8 балла. У половины респондентов не отмечалось функциональных ограничений (HAQ=0). HAQ, равный 0,1-1,0, имели 33,5% опрошенных. Самые серьезные затруднения при самообслуживании (HAQ=2,1-3,0) испытывали 6,6больных. Значения HAQ коррелировали с возрастом больных и длительностью заболевания (p<0,001). 43,3% опрошенных оценили состояние своего здоровья как хорошее или очень хорошее, 39,8% - как удовлетворительное и только 16,9% - как плохое. Отмечалась корреляция между состоянием здоровья и значениями HAQ (R=0,6, p<0,001), возрастом больных (R=0,3, p<0,001), длительностью ЮА (R=0,3, p<0,001). Большинство пациентов (78,9%) на момент исследования учились или работали, 48,8% имели высшее образование, 6,8% не могли учиться или работать из-за болезни. Треть обследованных имели семью, у 46 родились дети, что не зависело от состояния здоровья и функциональной способности пациентов. З ак лю че ни е. Большинство взрослых пациентов, длительно страдающих ЮА, имеют благоприятный прогноз в отношении функционального статуса и качества жизни. В то же время у части пациентов отмечены более тяжелое течение и исходы болезни с формированием выраженных функциональных ограничений, в связи с чем они нуждаются в лечении и продолжении наблюдения

Нозологическая диагностика ювенильного артрита на разных этапах наблюдения

Objective: to define the nosological category of juvenile arthritis (JA) in patients of different ages with different disease stages and follow-up periods. Subjects and methods. Group 1 included 135 children, mainly girls (60.7%), aged 1 to 16 years (range 7.9±5.0 years) with early-onset JA, the duration of which was 2 weeks to 6 months (range 2.9±1.6 months) who were further followed up for 2 years. Group 2 comprised 97 adult patients, mainly women (80.4%) aged 18 to 60 years (range 26.8±7.9 years) who had suffered from JA since childhood. The history of the disease varied from 10 to 53 years (range 20.5±8.2 years). The diagnosis in the children was made in accordance with the working criteria developed at the Pediatric Department of the Research Institute of Rheumatology, Russian Academy of Medical Sciences, for juvenile rheumatoid arthritis (JRA) and chronic JA (CJA). That in the adults was established by the diagnostic criteria for rheumatoid arthritis (RA), spondylarthritis (SA), ankylosing spondylitis (AS), and psoriatic arthritis (PA). Results. In the early-onset arthritis group, nonrheumatic diagnoses were made in 5 patients just in the first stage, which was a basis for their exclusion from the investigation. Reactive arthritis was diagnosed most frequently (46.3%) in the place of primary observation, the proportion of patients with this condition, when included into the study, was 4.4%; the diagnoses of JRA and CJA were established in 12.6 and 44.4% of cases, respectively. Later on, over 2 years of follow-up, the predominance (74.1%) of patients with CJA was retained. One patient with CJA developed obvious juvenile AS (JAS); JRA was verified in 3 children; acute rheumatic fever (ARF) and Kawasaki disease were in 2 cases; in 3 patients, rheumatic diagnoses (dysplastic coxarthrosis in 1 case, meniscopathy in 1, and leukoencephalitis in 1) were rejected. Ten years or more after disease onset, the adult patients with JA were diagnosed as having RA in 52% of cases; 11.5% had one of the types of SA; AS, PS, Crohn's disease- associated arthritis being in 4, 2, and 1 cases, respectively. Other diseases accounted for 11.5%. A diagnosis could not be verified in 25% of the patients; it was formulated as CJA or undifferentiated arthritis (UA). Among the patients with uveitis, the equal number (37.5%) RA or one of the types of SA; a diagnosis could not be specified in 25%, it was formulated as CJA (UA) or other diseases. Conclusion. This investigation has demonstrated that the diagnosis of JA presents certain difficulties and requires a thorough differentiated approach. Nosologically, the evolution of JAfollows the ways various joint diseases, such as JRA (RA), JAS (AS), other types of SA, develop; however, their diagnosis may be ultimately verified in different periods of the course of disease and a follow-up. The elaboration of current diagnostic criteria for JA, including those for JRA, will facilitate early disease recognition and timely selection of adequate and the most optimal patient management tacticsЦель - определить нозологическую принадлежность ювенильного артрита (ЮА) у пациентов различного возраста с различными стадиями болезни и сроками наблюдения. Материал и методы. В 1-ю группу включено 135 детей, преимущественно девочек (60,7%), с ранним ЮА длительностью от 2 нед до 6 мес (2,9±1,6 мес) в возрасте от 1 года до 16 лет (7,9±5,0 года), которые в дальнейшем находились под наблюдением в течение 2 лет. 2-ю группу составили 97 взрослых больных, преимущественно женщин (80,4%), страдающих ЮА с детства, в возрасте от 18 до 60 лет (26,8±7,9 года). Давность заболевания варьировала от 10 до 53 лет (20,5±8,2 года). Диагноз у детей определялся в соответствии с разработанными в детском отделении НИИ ревматологии РАМН рабочими критериями для ювенильного ревматоидного артрита (ЮРА) и ювенильного хронического артрита (ЮХА). У взрослых диагноз ставили на основании диагностических критериев для ревматоидного артрита (РА), спондилоартритов (СА), анкилозирующего спондилита (АС), псориатического артрита (ПА). Результаты исследования. В группе раннего артрита уже на первом этапе обследования 5 больным были поставлены неревматические диагнозы, что явилось основанием для исключения их из исследования. Наиболее часто (46,3%) по месту первичного наблюдения диагностировался реактивный артрит, доля которого при включении в исследование составила 4,4%, диагноз ЮРА был поставлен в 12,6% случаев, ЮХА - в 44,4%. В последующем за 2 года наблюдения преобладание больных с ЮХА сохранилось (74,1%). У 1 больного с ЮХА в течение 2 лет развился достоверный ювенильный анкилозирующий спондилит (ЮАС), у 3 детей верифицирован ЮРА, у 2 - острая ревматическая лихорадка (ОРЛ) и болезнь Кавасаки, у 3 больных ревматические диагнозы были отвергнуты (диспластический коксартроз - у 1, менископатия - у 1, лейкоэнцефалит - у 1). У взрослых больных ЮА через 10 лет и более после начала заболевания диагноз РА поставлен в 52% наблюдений, у 11,5% диагностирован один из вариантов СА, причем у 4 - АС, у 2 - ПА, у 1 - артрит, ассоциированный с болезнью Крона. 11,5% составили другие заболевания. У 25% больных диагноз верифицировать не удалось, и он формулировался как ЮХА или недифференцированный артрит (НА). Среди больных с увеитом равное количество пациентов (по 37,5%) имели РА или один из вариантов СА, у 25% диагноз уточнить не удалось, он формулировался как ЮХА (НА) или другие заболевания. Заключение. Наше исследование показало, что диагностика ЮА представляет определенные трудности и требует тщательного дифференцированного подхода. Эволюция ЮА в нозологическом плане идет по пути формирования различных заболеваний суставов, таких как ЮРА (РА), ЮАС (АС), другие варианты СА, однако окончательная верификация диагноза может быть осуществлена в разные периоды течения болезни и наблюдения. Создание современных диагностических критериев ЮА, в том числе для ЮРА, будет способствовать более раннему распознаванию болезни и своевременному выбору адекватной и наиболее оптимальной тактики ведения больных

The Russian version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR)

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Russian language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach\u2019s alpha, interscale correlations, test\u2013retest reliability, and construct validity (convergent and discriminant validity). A total of 100 JIA patients (25% systemic, 19% oligoarticular, 38% RF-negative polyarthritis, 18% other categories) and 198 healthy children, were enrolled in two centres. The JAMAR components discriminated healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the Russian version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research

Циклоспорин А в терапии ювенильных артритов

The paper describes approaches to using cyclosporin A (CsA) in juvenile arthritis (JA). It shows the benefits of combination basic therapy with CsA and methotrexate included into a treatment regimen mainly for systemic JA and JA involving the eye (uveitis) versus monotherapy with the above drugs. Attention is drawn to that the oral dose of glucocorticoids may be decreased when CsA is incorporated into the treatment regimen. CsA is shown to be of value as the drug of choice for the therapy of such a menacing complication of systemic JA as the macrophage activation syndromeИзложены подходы к применению циклоспорина А (ЦсА) при ювенильном артрите (ЮА). Показано преимущество комбинированной базисной терапии с включением в схему лечения ЦсА и метотрексата в первую очередь у больных с системной формой ЮА и ЮА с поражением глаз (увеит) по сравнению с монотерапией указанными препаратами. Обращено внимание на возможность снижения пероральной дозы глюкокортикоидов при включении в схему лечения ЦсА. Показано значение ЦсА как препарата выбора для терапии такого опасного осложнения системного варианта ЮА, как синдром активации макрофаго

Ингибитор интерлейкина 1 канакинумаб в лечении криопирин-ассоциированных периодических синдромов (CAPS). Клинический опыт

Interleukin (IL) 1βis a major mediator of cryopyrin-associated periodic syndrome (CAPS). In this connection, the experience with IL-1 inhibitors used in patents with CAPS is being accumulated worldwide. Canakinumab was approved by FDA and EMEA in 2009 to treat CAPS and registered in the Russian Federation for this in 2011. The drug has been shown to be highly effective and well tolerated by patients with CAPS.Objective: to present Russia's experience in using the IL-1 inhibitor canakinumab in children with CAPS.Subjects and methods. The trial enrolled 6 CAPS patients, including 4 with Muckle-Wells Syndrome (MWS) and 4 with chronic infantile onset neurologic cutaneous articular/neonatal onset multisystem inflammatory disease (CINCA/NOMID), among whom there were 5 female patients aged 3.5 to 40 years and 1 male patient aged 17 years. Two patients (a 17-year-old daughter and her 40-year-old mother) were stated to have a familial MWS case. The duration of the disease was 3.5 to 33 years. All the patients underwent a molecular genetic analysis for mutations in the NLRP3 (CIAS1) gene. Four patients with MWS were found to have Thr436Ile and Thr438Ile mutations; the mother and her daughter had Thr350Met mutations; no mutations were detected in 2 patients with CINCA/NOMID. At the study, one female patient with MWS took gluco-corticoids (GC) in a dose of 0.1 mg/kg; the others received symptomatic therapy with nonsteroidal antiinflammatory drugs. Canakinumab was injected subcutaneously every 8 weeks in a dose of 4 mg/kg for patients with a body weight of <15 kg and in a dose of 2 mg/kg for those with a body weight of >15 kg. By now, 2 patients with MWS received 7 injections of the drug (a 48-week follow-up); 2 patients with CINCA/NOMID had its 6 injections (a 40-week follow-up) and 2 patients with MWS had 2 injections (a 10-week follow-up).Results. All the patients showed a significant clinical improvement: recovery; elimination of fever, rash, and eye symptoms; and a reduction in the levels of acute-phase markers. The effect retained throughout the follow-up. GC could be completely discontinued in the female patient with MWS. No adverse events were observed in any case.Conclusion. The experience in using canakinumab in the patients with CAPS showed the high efficacy and good tolerability of the drug. The decrease in acute-phase markers was slower in the patients with CINCA/NOMID, the most severe form of CAPS.Основным медиатором для криопирин-ассоциированных периодических синдромов (Сryopyrin Аssociated Рeriodic Syndrome – CAPS) является интерлейкин (ИЛ) 1β. В связи с этим в мире накапливается опыт применения ИЛ1-ингибиторов у пациентов с CAPS. Канакинумаб одобрен FDA и EMEA с 2009 г. для лечения CAPS, в Российской Федерации препарат зарегистрирован для применения при CAPS с 2011 г. Показана высокая эффективность и хорошая переносимость канакинумаба у пациентов с CAPS.Цель исследования – представить российский опыт применения ИЛ1-ингибитора канакинумаба у детей с CAPS.Материал и методы. В исследование включено 6 пациентов с CAPS: 4 с Muckle – Wells Syndrome (MWS) и 2 с синдромом Chronic Infantile Onset Neurologic Cutneous Articular/Neonatal Onset Multisystem Inflammatory Disease (CINCA/NOMID), среди них было 5 больных женского пола в возрасте от 3,5 до 40 лет и 1 пациент мужского пола 17 лет. У 2 пациенток (дочери 17 лет и матери 40 лет) констатирован семейный случай MWS. Давность заболевания составила от 3,5 до 33 лет. У всех пациентов проведен молекулярно-генетический анализ на мутацию гена NLRP3(CIAS1). У 4 больных с MWS выявлены мутации Thr436Ile и Thr438Ile, у матери и дочери – Thr350Met, у 2 больных с CINCA/NOMID мутаций не обнаружено. На момент исследования 1 больная с MWS принимала глюкокортикоиды (ГК) в дозе 0,1 мг/кг, остальные получали симптоматическое лечение нестероидными противовоспалительными препаратами. Канакинумаб вводили каждые 8 нед подкожно в дозе 4 мг/кг при массе тела <15 кг, в дозе 2 мг/кг при массе тела >15 кг. К настоящему времени 2 больных с MWS получили 7 инъекций препарата (48 нед наблюдения), 2 больных с CINCA/NOMID – 6 инъекций (40 нед наблюдения) и 2 пациента с MWS – 2 иньекции (10 нед наблюдения).Результаты исследования. У всех пациентов отмечено достоверное клиническое улучшение: нормализация самочувствия, купирование лихорадки, сыпи, глазных симптомов, снижение уровня острофазовых маркеров. Эффект сохранялся на протяжении всего периода наблюдения. У пациентки с MWS удалось полностью отменить ГК. Нежелательных явлений не выявлено ни в одном случае.Выводы. Опыт применения канакинумаба у пациентов с CAPS показал высокую эффективность и хорошую переносимость препарата. Более медленным оказалось снижение острофазовых маркеров у пациентов с синдромом CINCA/NOMID – наиболее тяжелой разновидностью CAPS

FUNCTIONAL STATUS AND QUALITY OF LIFE IN ADULT PATIENTS WITH JUVENILE ARTHRITIS DURING THE LONG-TERMCOURSE OF DISEASE

Objective: to assess the functional status and quality of life of adult patients long suffering from juvenile arthritis (JA). Subjects and methods. The study included 213 adult patients with JA, the majority (84.5%) of whom had been treated at the Childhood Department, Research Institute of Rheumatology, Russian Academy of Medical Sciences, in 1968 to 2008 or sought medical advice for the first time. Ninety-seven of the 213 patients were consulted or examined in the outpatient setting or at the clinic of the Research Institute of Rheumatology, Russian Academy of Medical Sciences. Among the patients enrolled into the study, women were prevalent (72.8%); the patientsmean age was 23.9±6.5 years (range 18 to 60 years); the disease duration was 17.4±6.8 years (range 10 to 53 years). The patients' functional capacity was determined by O. Steinbrocker's classification (functional class - FC) and HAQ questionnaire. The patients were asked (by mail, telephone, or personal interview) to fill in the questionnaire, by answering the questions concerning the functional and social status. Results. A third of the patients had no functional limitations (FC 1), 36% had minimal functional limitations (FC 2); 29% needed help with self-service, and 6.2% were severely disabled. According to the questionnaire survey data, the mean HAQ score was 0.8. Half of the respondents were found to have no functional limitations (HAQ score was 0). HAQ scores of 0.1-1.0 were observed in 33.5% of the respondents. 6.6% of the patients had the most serious problems with self-service (HAQ scores of 2.1-3.0) HAQ scores correlated with age and disease duration (p<0.001). The health status was appraised as good or very good, satisfactory, or bad by 43.3, 39.8, and 16.9% of the patients, respectively. There was a correlation between the health status and the HAQ scores (R = 0.6; p<0,001), age (R = 0.3; p<0,001), and JA duration (R = 0.3; p<0.001). At the moment of the study, most (78.9%) patients worked or studied at the moment; 48.2% had a higher education; 6.8% could not study or work because of the disease. A third of the examinees had families; 46 patients had children, which did not depend on the patients' health status and functional capacity. Conclusion. Most adult patients, long suffering from JA, have a good prognosis for their functional status and quality of life. At the same time some patients were observed to have a severer course of the disease and its outcomes with significant functional limitations; in this connection, they need treatment and a continued follow-up

NOSOLOGICAL DIAGNOSIS OF JUVENILE ARTHRITIS AT DIFFERENT FOLLOW-UP STAGES

Objective: to define the nosological category of juvenile arthritis (JA) in patients of different ages with different disease stages and follow-up periods. Subjects and methods. Group 1 included 135 children, mainly girls (60.7%), aged 1 to 16 years (range 7.9±5.0 years) with early-onset JA, the duration of which was 2 weeks to 6 months (range 2.9±1.6 months) who were further followed up for 2 years. Group 2 comprised 97 adult patients, mainly women (80.4%) aged 18 to 60 years (range 26.8±7.9 years) who had suffered from JA since childhood. The history of the disease varied from 10 to 53 years (range 20.5±8.2 years). The diagnosis in the children was made in accordance with the working criteria developed at the Pediatric Department of the Research Institute of Rheumatology, Russian Academy of Medical Sciences, for juvenile rheumatoid arthritis (JRA) and chronic JA (CJA). That in the adults was established by the diagnostic criteria for rheumatoid arthritis (RA), spondylarthritis (SA), ankylosing spondylitis (AS), and psoriatic arthritis (PA). Results. In the early-onset arthritis group, nonrheumatic diagnoses were made in 5 patients just in the first stage, which was a basis for their exclusion from the investigation. Reactive arthritis was diagnosed most frequently (46.3%) in the place of primary observation, the proportion of patients with this condition, when included into the study, was 4.4%; the diagnoses of JRA and CJA were established in 12.6 and 44.4% of cases, respectively. Later on, over 2 years of follow-up, the predominance (74.1%) of patients with CJA was retained. One patient with CJA developed obvious juvenile AS (JAS); JRA was verified in 3 children; acute rheumatic fever (ARF) and Kawasaki disease were in 2 cases; in 3 patients, rheumatic diagnoses (dysplastic coxarthrosis in 1 case, meniscopathy in 1, and leukoencephalitis in 1) were rejected. Ten years or more after disease onset, the adult patients with JA were diagnosed as having RA in 52% of cases; 11.5% had one of the types of SA; AS, PS, Crohn's disease- associated arthritis being in 4, 2, and 1 cases, respectively. Other diseases accounted for 11.5%. A diagnosis could not be verified in 25% of the patients; it was formulated as CJA or undifferentiated arthritis (UA). Among the patients with uveitis, the equal number (37.5%) RA or one of the types of SA; a diagnosis could not be specified in 25%, it was formulated as CJA (UA) or other diseases. Conclusion. This investigation has demonstrated that the diagnosis of JA presents certain difficulties and requires a thorough differentiated approach. Nosologically, the evolution of JAfollows the ways various joint diseases, such as JRA (RA), JAS (AS), other types of SA, develop; however, their diagnosis may be ultimately verified in different periods of the course of disease and a follow-up. The elaboration of current diagnostic criteria for JA, including those for JRA, will facilitate early disease recognition and timely selection of adequate and the most optimal patient management tactic

CYCLOSPORIN A IN THERAPY FOR JUVENILE ARTHRITIS

The paper describes approaches to using cyclosporin A (CsA) in juvenile arthritis (JA). It shows the benefits of combination basic therapy with CsA and methotrexate included into a treatment regimen mainly for systemic JA and JA involving the eye (uveitis) versus monotherapy with the above drugs. Attention is drawn to that the oral dose of glucocorticoids may be decreased when CsA is incorporated into the treatment regimen. CsA is shown to be of value as the drug of choice for the therapy of such a menacing complication of systemic JA as the macrophage activation syndrom

Increasing the Interval of Canakinumab Administration Effectively Supports the Remission of Schnitzler’s Syndrome

Schnitzler’s syndrome (SchS) is a rare, disabling, autoinflammatory disorder characterized by recurrent urticarial rash and monoclonal IgM gammopathy. Interleukin-1 beta (IL-1β) plays an important role in the pathophysiology of SchS. Only anecdotal reports demonstrate the efficiency and safety of human monoclonal anti-human IL-1β antibody (canakinumab) use in SchS therapy. However, there are no generally accepted recommendations concerning the scheme (or frequency) of canakinumab use for this disease. Here, we report the effective long-term treatment of SchS in a 44-year-old male with a standard canakinumab dose (150 mg) but with an increased 4-month injection interval