Фиброзирующая артропатия при ювенильной склеродермии

The group of scleroderma diseases includes a number of clinical entities, the main symptom of which is skin tightening. Scleroderma is a prominent example of these diseases, characterized by excessive synthesis and deposition of collagen in organs and tissues. A patient with juvenile systemic scleroderma with induration of the skin and underlying tissues, and persistent contractures of large joints since childhood, is described. This clinical example illustrates disease course peculiarities and differential diagnosis of systemic and limited (focal) scleroderma and scleroderma-like conditions in pediatric patients. The feasibility of pathogenetic therapy aimed at improving patient's the quality of life with formed disease phenotype is shown.К группе склеродермических болезней относится ряд нозологий, основным признаком которых является уплотнение кожи. Склеродермия – яркий представитель этих заболеваний, характеризующихся избыточным синтезом и отложением коллагена в органах и тканях. Описана больная ювенильной системной склеродермией с индурацией кожи и подлежащих тканей, а также стойкими контрактурами крупных суставов с детского возраста. На этом клиническом примере рассматриваются особенности течения и дифференциальная диагностика системной и ограниченной (очаговой) склеродермии и склеродермоподобных состояний у пациентов детского возраста. Показаны возможности подбора патогенетической терапии, направленной на улучшение качества жизни пациентов с уже сформированным фенотипом заболевания

Комплексная терапия сосудистых нарушений у больных системной склеродермией

Scleroderma systematica (SDS) is a disease in which vascular diseases underlie the pathogenesis and presented by diverse clinical manifestations. Raynaud's syndrome and digital ulceration are the most common symptom of the diseases, which influences the quality of life in patients and requires continuous drug therapy. The paper discusses the recent European guidelines for the management of this manifestation of SDS. The proposed recommendations cannot unfortunately be realized in full measure now due to the unavailability of some drugs. The authors give their clinical experience with therapy for the vascular manifestations of SDS.Системная склеродермия (ССД) - заболевание, при котором сосудистые нарушения лежат в основе патогенеза и представлены разнообразными клиническими проявлениями. Синдром Рейно и образование дигитальных язв - наиболее частый симптом заболевания, влияющий на качество жизни пациентов и требующий постоянной лекарственной терапии. В статье обсуждаются недавно опубликованные европейские рекомендации по лечению этого проявления ССД. Выполнение предложенных рекомендаций в полном объеме, к сожалению, в настоящее время невозможно из-за недоступности некоторых препаратов. Авторами приводится собственный клинический опыт терапии сосудистых проявлений ССД

SCLERODERMA SYSTEMATICA WITH INTERSTITIAL LUNG LESION: COMPARATIVE CLINICAL CHARACTERISTICSWITH PATIENTS WITHOUT LUNG LESION

Objective. To compare disease history data and clinical and laboratory parameters in patients with scleroderma systematica (SDS) with high-resolution computed tomography (HRCT)-verified interstitial lung lesion (ILL) versus those without lung involvement. Subjects and methods. An examination was made in 138 patients with SDS who had been consecutively admitted in 2006-2008, female/male ratio, 124 : 14; limited : diffuse : mixed forms, 78 : 40 : 20; mean age, 47±13 years; median disease duration, 6 (2.5 11) years. The history data (occupational hazards, smoking, respiratory diseases) and clinical manifestations of SDS and laboratory data were studied. The diagnosis of ILL was established on the basis of chest HRCT. Results. According to HRCT data, the signs of varying ILL were found in 82% of the patients with SDS. The duration of SDS was similar in the patients with and without lung involvement; but the latter were younger at the time of disease onset. There were no significant differences between the groups compared in history data, clinical forms of SDS, the frequency of involvement of visceral organs and systems. Crepitation was heard only in the patients with ILL. The frequency of respiratory manifestations increased with a larger number of the involved lung segments. The prevalence of ILL was found to be positively correlated with age at the onset of SDS (r=0.29;

STUDY OF THE EFFICIENCY AND SAFETY OF MYCOPHENOLATE MOFETIL THERAPY IN PATIENTSWITH SYSTEMIC SCLERODERMA

Interstitial lung disease (ILD) is one of the major causes of death in systemic scleroderma (SSD). Treatment of these patients remains difficult and controversial. Mycophenolate mofetil (MPM) has been in vitro shown to inhibit overproduction of type I collagen and hence may be effective against SSD. Objective: to study the efficiency and safety of MPM therapy in patients with SSD and clinically relevant ILD in an open-label prospective study. Subjects and methods. Ten patients with SSD (7 and 3 with its diffuse and limited forms, respectively) and ILD were given MPM in combination with glucocorticoids (mean daily dose was 10+4 mg). The mean MPM therapy duration was 11.4+1.3 months. The Rodnan total skin thickness score, flexion index, forced vital capacity (FVC), diffusing capacity of the lung for carbon monoxide (DLCO), and European Scleroderma Study Group (EScSG) activity index were estimated and a 6-minute walk test (6MWT) was carried out before and after MPM therapy. Results. After therapy, the whole group showed a significant reduction in skin scores from 12.9+9.8 to 5.6+3.2 (p=0.036) and EScSG from 3.9+1.4 to 2.25+1.03 (p=0.015) and an increase in exercise tolerance from 446+155 to 535+78 m (p=0.03) as evidenced by 6MWT. The degree of flexion contractures decreased from 15+21 to 3.7+11.3 mm (p>0.05). FVC (77.8+18.7% versus 73.8+11.3%) and DLCO (45+14.4% versus 42+16.4%) were significantly unchanged. A 10% or more clinically significant fall was noted in FVC and DLCO in 3 and 1 patients, respectively. In the remaining patients, the lung functional test results remained stable. MPM tolerability was satisfactory. All the patients completed their course of treatment. Conclusion. Stabilization of lung function with higher exercise tolerance and significantly reduced skin density allow therapy with MPM in combination with low-dose glucocorticoids to be regarded as an effective and well-tolerated treatment in patients with ILD in the presence of SS

Использование международного индекса для оценки активности системной склеродермии

Up to now, it is difficult to determine systemic scleroderma (SSD) activity because of the lack of validated tools to estimate changes in the pathological process. Attempts have been made to develop unified activity assessing methods for many years. The indices proposed by the European SSD Group are most popular today. This paper gives the results of using this index in a cohort of Russian patients.Определение активности системной склеродермии (ССД) до настоящего времени затруднено из-за отсутствия валидированных инструментов для оценки изменений патологического процесса. В течение многих лет предпринимаются попытки создания унифицированных методов оценки активности заболевания. Наиболее популярными на сегодняшний день являются индексы, предложенные европейской группой по изучению ССД. В статье представлены результаты использования этого индекса у когорты российских пациентов

Comparative analysis of anxiety-depressive spectrum disorders in patients with rheumatic diseases

Research objective - comparative analysis of incidence and structure of anxiety-depressive spectrum disorders (ADD) in patients with various rheumatic diseases (RD). Materials and methods. 613 patients with RD were enrolled in the study: 180 with a reliable diagnosis of systemic lupus erythematosus (SLE), 128 with rheumatoid arthritis (RA), 110 with systemic sclerosis (SSc), 115 with Behcet's disease (BD), 80 with primary Sjögren's syndrome (pSS). Female prevailed in all groups (95% of patients with pSS, 88,2% - SSc, 87,2% - RA, 85,5% of SLE) except BD patients (70% male). The mean age was 42.3±1.54 years and was lower in patients with BD (33.3±0.98 years) and SLE (34.6±0.93 years) compared to patients with SSc (49.9±2.47 years), RA (47.4±0.99 years) and pSS (46.2±2.3 years). The mean RD duration was 130,0±8,65 months and was more at BD - 148,5±10,4 months, pSS - 141,6±8,92 months, RA - 138,4±10,1months, and less at SLE - 134,9±8,8 months and SSc - 87,0±5,04 months. The mean SLE activity index SLEDAI was 9,13±0,63 points (high), RA (DAS28) - 5,26±0,17 points (high), BD (BDCAF) - 3,79±0,2 points (moderate) and SSc by G. Valentini - 1,1±0,20 points (moderate). Glucocorticoids took 100% of patients with pSS, 91,1% - SLE, 90% - SSc, 87% - BD and 67,2% - RA patients; conventional disease modifying anti-rheumatic drugs (cDMARDs) took 90% of patients with SSc, 84% - BD, 79,6% - RA, 68% - pSS, 40,6% - SLE. Biologic DMARDs took 32% of patients with RA, 17,4% - BD, 7,3% - SSc and 7,2% - SLE. Mental disorders were diagnosed by psychiatrist as a result of screening by the hospital anxiety and depression scale (HADS) and in semi-structured interview in accordance with the ICD-10/ DSM-IV. The severity of depression was evaluated by Montgomery-Asberg Depression Rating Scale (MADRS) and anxiety - by Hamilton Anxiety Rating Scale (HAM-A). Projective psychological methods were used for cognitive impairment detection. Results. Screening of depressive disorders (HADS-D≥8) was positive in 180 (29,4%) patients with RD, including 74 (41%) patients with SLE, 38 (35%) - SSc, 29 (23%) - RA, 23 (20%) - BD and 16 (20%) - pSS; anxiety disorders (HADS-A≥8) - in 272 (44,4%) patients, including 66 (52%) patients with RA, 40 (50%) - pSS, 77 (43%) - SLE, 45 (41%) - SSc and 44 (38%) - BD. In accordance with the ICD-10/ DSM-IV depressive disorders have been identified in 389 (63%) patients, including 94 (73%) patients with RA, 71 (64,5%) - SSc, 69 (60%) - BD, 90 (50%) - SLE and 39 (49%) - pSS; anxiety disorders - in 377 (61,5%) patients, including 20 (25%) patients with pSS, 44 (24,5%) - SLE, 29 (23%) - RA, 20 (17%) - BD and 7 (6,4%) - SSc. Conclusion. Anxiety-depressive spectrum disorders are typical for most patients with RA, SLE, SSc, pSS and BD. ADDs diagnosis in RD patients with the use of the HADS did not reveal a significant proportion. To obtain objective data on the frequency and structure of ADDs, psychopathological and clinical psychological diagnosis is necessary

Функция почек на фоне длительной терапии ритуксимабом у больных системной склеродермией

   In systemic sclersis (SSc), different types of renal involvement occur. Their severity can range from asymptomatic deterioration of renal function to life-threatening damage, which is a complex therapeutic problem. Rituximab (RTM) has been used in the treatment of SSc and other autoimmune diseases with promising results, but its effect on renal function has not been adequately studied.   Objective: to evaluate the renal function during complex therapy, including RTM, in patients with SSc over a long-term follow-up (at least 1 year).   Material and methods. The study included 90 patients with SSc who were examined at least twice – before and 1–3.5 years after initiation of RTM treatment. Renal function was assessed by glomerular filtration rate (GFR) calculated according to the CKD-EPI formula. The stages of chronic kidney disease (CKD), blood pressure, daily proteinuria, skin score, activity, and indicators of lung function – forced vital capacity and diffusing capacity of the lungs – were also determined.   Results and discussion. Against the background of complex therapy with RTM, there was a statistically significant decrease in GFR in the entire group of patients at the end of observation. On the other hand, renal function remained stable in the majority of patients with initially preserved GFR and there was a 25 % decrease – from 20 to 15 patients – in the number of patients with CKD. In more than half of the patients who initially had CKD, GFR increased (n = 11) or stabilized (n = 2) after therapy, and it decreased in a statistically insignificant manner in only 7 patients, whereas the development of a more advanced stage of CKD was observed in only 2 cases. The results of the treatment of 2 patients who had previously experienced scleroderma renal crisis (SRC) are reviewed in detail.   Conclusion. In this study, there was no significant effect of RTM treatment on GFR and grade of CKD. Most patients had stable renal function; patients with an initial low grade of CKD showed a tendency toward stabilization of renal function. A significant decrease in GFR during long-term therapy noted in the entire patient group appears to be explained by an increase in renal insufficiency in patients with initially severe scleroderma renal damage, particularly due to SRC. Further studies on the effects of RTM therapy on renal function in patients with SSc are needed.   При системной склеродермии (ССД) встречаются различные типы поражения почек. Степень их выраженности может варьироваться от бессимптомного снижения почечной функции до жизнеугрожающих повреждений, которые представляют собой сложную терапевтическую проблему. Ритуксимаб (РТМ) применяется в лечении ССД и других аутоиммунных заболеваний с многообещающими результатами, однако его влияние на функцию почек изучено недостаточно.   Цель исследования – оценить состояние функции почек на фоне комплексной терапии, включающей РТМ, у больных ССД в течение длительного (не менее 1 года) наблюдения.   Материал и методы. В исследование включено 90 больных ССД, которых обследовали как минимум дважды – до и через 1–3,5 года после начала лечения РТМ. Почечную функцию оценивали по скорости клубочковой фильтрации (СКФ), рассчитанной по формуле CKD-EPI. Определяли также стадии хронической болезни почек (ХБП), уровень артериального давления, суточную протеинурию, кожный счет, активность и показатели легочной функции – форсированную жизненную емкость легких и диффузионную способность легких.   Результаты и обсуждение. На фоне комплексной терапии РТМ в конце наблюдения отмечалось статистически значимое снижение СКФ в группе больных в целом. В то же время у большинства пациентов с исходно сохранной СКФ функция почек оставалась стабильной, а число больных с ХБП уменьшилось на 25 % – с 20 до 15. Более чем у половины больных, исходно имевших ХБП, после терапии СКФ увеличилась (n = 11) или стабилизировалась (n = 2), и лишь у 7 пациентов она статистически незначимо снизилась, при этом только в 2 случаях наблюдалось развитие более продвинутой стадии ХБП. Детально рассмотрены результаты лечения 2 больных, которые ранее перенесли склеродермический почечный криз (СПК).   Заключение. В данной работе не отмечено существенного влияния лечения РТМ на СКФ и степень ХБП. Большинство больных имели стабильную функцию почек, у пациентов с исходно незначительной степенью ХБП была выявлена тенденция к стабилизации почечной функции. Значимое снижение СКФ на фоне длительной терапии, отмеченное в группе больных в целом, по-видимому, объясняется нарастанием почечной недостаточности у пациентов, исходно имевших выраженное склеродермическое поражение почек, в частности, вследствие СПК. Требуется дальнейшее изучение влияния терапии РТМ на функцию почек у пациентов с ССД