CLINICAL AND LABORATORY FEATURES OF ANTICENTROMERE ANTIBODY-POSITIVE SJö GREN’S SYNDROME

Чальцев, Б. Д.; Васильев, В. И.; Palshina, S.; Torgashina, A.; Sokol, E.; Хван, Ю. И.; Safonova, T N; Rodionova, E B; Гайдук, И. В.; Borozdkin, L. L.

doi:10.14412/1995-4484-2019-431-439

Cited by 2 publications

(4 citation statements)

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“…После первого описания СШ у 3 пациентов с ССД [16] и ранних исследований взаимосвязи этих двух АЗ установлено, что различные клинические проявления, характерные для СШ, могут фиксироваться у 11-84% пациентов с ССД [17], однако частота диагностики СШ при ССД колеблется от 12 до 30%, а наиболее часто СШ диагностируется при лимитированной форме ССД с антицентромерными антителами (АЦА) [17][18][19]. Начиная с первого описания АЦА у пациентов с ССД [20], большинство исследователей относят их к «болезнь-специфическим» аутоантителам и связывают их наличие с лимитированной формой заболевания [18,19,[21][22][23], однако последующее изучение этих аутоантител выявило их встречаемость при БШ, первичном билиарном циррозе, РА, СКВ, изолированном феномене Рейно (ФР), дигитальной гангрене и некоторых опухолях [24,25]. АЦА определяются у 3,5-27% пациентов с БШ без каких-либо признаков ССД как единственный вариант аутоантител при этом заболевании, так и в комбинации с другими антинуклеарными антителами (антиRo/SS-A, ан-тиLa/SS-B), ревматоидным фактором (РФ) и прочими антителами [19,20,[25][26][27].…”

Section: ââåäåíèåunclassified

“…Начиная с первого описания АЦА у пациентов с ССД [20], большинство исследователей относят их к «болезнь-специфическим» аутоантителам и связывают их наличие с лимитированной формой заболевания [18,19,[21][22][23], однако последующее изучение этих аутоантител выявило их встречаемость при БШ, первичном билиарном циррозе, РА, СКВ, изолированном феномене Рейно (ФР), дигитальной гангрене и некоторых опухолях [24,25]. АЦА определяются у 3,5-27% пациентов с БШ без каких-либо признаков ССД как единственный вариант аутоантител при этом заболевании, так и в комбинации с другими антинуклеарными антителами (антиRo/SS-A, ан-тиLa/SS-B), ревматоидным фактором (РФ) и прочими антителами [19,20,[25][26][27]. При РЗ серопозитивность по тому или иному типу аутоантител нередко связана с определенными клиническими проявлениями, течением и последующим прогнозом заболевания [11,22,24].…”

Section: ââåäåíèåunclassified

“…Принятые американо-европейские классификационные критерии БШ с включением в качестве серологического признака только антиRo/SS-A-антител не позволяют диагностировать заболевание на ранней стадии, поэтому нуждаются в будущем пересмотре. Многие исследователи рассматривают БШ, ассоциированную с АЦА, как определенный субтип заболевания [25,27], тогда как другие предлагают объединить группу БШ с АЦА, а также группу ССД с АЦА и СШ в определенную нозологическую единицу под названием «overlap-синдром АЦА-позитивной лимитированной ССД с СШ», характеризуемую доброкачественным течением ССД и более частым развитием НХЛ [26]. В настоящем исследовании представлен накопленный опыт диагностики НХЛ при РЗ в лаборатории интенсивной терапии РЗ ФГБНУ «НИИ ревматологии им.…”

Section: ââåäåíèåunclassified

“…Интересно отметить, что в группе АЦА-позитивных лиц с БШ также развивались экстранодальные MALT-лимфомы слюнных желез. Пациенты с БШ, позитивные по АЦА [25], случаи заболевания которых описаны нами ранее, очень схожи по клинико-лабораторным проявлениям с объединенной группой пациентов БШ с АЦА и АЦА-позитивной ССД с СШ из итальянского исследования [26]. У описанной в данной статье группы пациентов с сочетанием БШ и лимитированной формы ССД при сравнении с группой пациентов с БШ и АЦА, описанной в итальянском исследовании, а также нами ранее, имеется ряд существенных различий: 1) все пациенты удовлетворяли критериям постановки диагноза БШ и 91% -лимитированной формы ССД;…”

Section: îáñóAeäåíèåunclassified

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The relationship between Sjogren’s syndrome, systemic sclerosis and lymphoproliferative diseases

Васильев

Чальцев

Городецкий

et al. 2020

Terapevticheskii arkhiv

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Despite the large number of studies devoted to the study of systemic sclerosis (SSc), the high risk of developing lymphomas in this disease, the relationship of their development with certain subtypes of SSc and specific SSc-associated autoantibodies is still debated in the literature. Aim.To study demographic, clinical, laboratory and immunological characteristics of patients with a combination of primary Sjogrens syndrome (pSS) and SSc and diagnosed lymphoproliferative diseases (LPDs); to characterize morphological/immunomorphological variants and course of non-Hodgkins lymphomas (NHL), developing in patients with these rheumatic diseases (RDs). Materials and methods.In 19982018 at the Nasonova Research Institute of Rheumatology, 13 patients with clinical and laboratory manifestations of pSS (12) and SSc (13) were diagnosed with various lymphoproliferative diseases (LPDs). In 3 cases, an induced RD was observed: 1 case of a diffuse, rapidly progressive form of SSc, 2 cases of pSS in combination with a limited form of SSc after chemotherapy and radiation therapy of Hodgkins lymphoma (1), B-cell NHL (1) and CR of the breast (1) respectively. The first 2 cases were excluded from the analysis, since the development of lymphomas is not pathogenetically associated with RD. Results.Of 11 patients with LPDs, 10 after a long course of RDs were diagnosed with NHL [MALT lymphoma of the parotid salivary glands 7, disseminated MALT lymphoma 2, disseminated MALT lymphoma with transformation into diffuse large B-cell lymphoma (DLBCL) 1]. RDs debuted with Raynauds phenomenon (RP) in 64.5% and pSS manifestations in 45.5% of patients. Stomatological manifestations of pSS were characterized by recurrent parotitis in 36%, significant parotid gland enlargement with massive infiltration of labial salivary glands (focus score 4) in 100%, severe xerostomia in 70%, extraglandular manifestations and lymphadenopathy in 50% of patients. The course of the SSc was characterized by mild RP with various types of capillaroscopic changes and mild lung changes and non-significant progression during long-term follow-up (median 22 years). The entire spectrum of SSс specific antibodies (anticentromere antibodies 60%, antibodies to ribonucleoprotease III 30%, Pm/Scl 10%), excepting antibodies to topoisomerase I, as well as pSS specific autoantibodies (antiRo/La 70%, RF (rheumatoid factor) 90%), were detected in patients with a combination of these RDs. Conclusion.pSS is often combined with a limited form of SSc regardless of the type of autoantibodies detected. The presence of pSS, rather than SSc, is a high-risk factor for the development of NHL in this group of patients. The patients with pSS and SSc are characterized by a steady progression of pSS with a slow and mild course of SSc throughout the observation period. The development of severe stomatological manifestations and high immunological activity of pSS contribute to the development of localized MALT lymphomas (70%) and disseminated MALT lymphomas (30%) with primary lesions of the salivary glands and transformation into DLBCL in case of their late diagnosis. The optimal method for preventing the development of NHL in this group of patients is the early diagnosis of pSS, the appointment of alkylating cytotoxic agents and/or anti-B-cell therapy in the early stages of pSS. Given the possibility of transformation of localized NHL into DLBCL, for early diagnosis, minimally invasive surgical biopsies of significantly enlarged parotid salivary glands should be performed before glucocorticoids are prescribed. Detection of positive B-cell clonality and lymphoepithelial lesions in the parotid salivary gland is considered a predictor of MALT lymphoma development during follow-up. Localized and disseminated MALT lymphomas in patients with pSS and SSc respond well to therapy, in contrast to MALT lymphomas transformed into DLBCL.

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Section: îáñóAeäåíèåunclassified

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The relationship between Sjogren’s syndrome, systemic sclerosis and lymphoproliferative diseases

Васильев

Чальцев

Городецкий

et al. 2020

Terapevticheskii arkhiv

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Clinical and laboratory characteristics of lymphoproliferative diseases in primary Sjogren's syndrome associated with anticentromere antibodies

Чальцев¹,

Vasiliev

Palshina³

et al. 2021

jour

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Purpose of the study. To study the characteristics and frequency of lymphomas in patients with Sjogren's disease (SD) and anticentromere antibodies (ACA); to evaluate the predictors of the development of lymphoproliferative diseases (LPD) in this group of patients. Material and methods. Over the period from 1998 till 2019, 131 ACA-positive patients were under medical supervision at the Research Institute of Rheumatology named after Nasonova V.A. Isolated SD was diagnosed in 82 patients (62.6%), isolated limited form of SSc — in 12 patients (9.2%), combination of SD and limited form of SSc — in 37 patients (28.2%). Lymphoproliferative diseases (LPD) were diagnosed in 20 ACA-positive patients: in 15 — with SD, in 5 — with SD and SSc; no lymphomas were found in the group of patients with isolated SSc. All lymphomas were diagnosed on the basis of histological, immunohistochemical and PCR examination with of B-cell clonality determination in the tissue, and were classified on the base of haematopoietic and lymphoid tissue tumors classification by the World Health Organization. Further analysis included 15 ACA-positive patients with isolated SD and lymphomas. Results. In our study, 18.3% of patients with isolated ACA-positive SD were diagnosed with LPD, represented by MALT lymphomas of the salivary glands (subsequent transformation into aggressive diffuse large B-cell lymphoma (DLBCL) was noted in one patient) in most cases. The course of SD before the diagnosis of LPD was characterized by a gradual progression of dental manifestations of SD with the development of late stages of parenchymal parotitis, severe xerostomia, and significant enlargement of the salivary glands with a minimum number of systemic manifestations of the disease. Significant enlargement of salivary glands, severe infiltration of minor salivary glands, severe xerostomia, decreased level of C4-complement component, monoclonal secretion, low content of CD19+B-cells in peripheral blood, positive B-cell clonality in biopsy material were the main signs of LPD in this study. When diagnosing MALT lymphomas, a focal damage of the salivary glands with no signs of dissemination, no symptoms of B-cell intoxication, and minimal changes in laboratory assessment were found in patients with ACA-positive SD. Conclusion. The natural course of ACA-positive SD and the absence of pathogenetic therapy at an early stage contribute to the development of salivary gland lymphomas in the first 10 years of the disease. Persistent enlargement of the salivary glands in SD, especially in the presence of other predictors of lymphoproliferation, is a direct indication for biopsy followed by the research to exclude the presence of lymphoma.

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CLINICAL AND LABORATORY FEATURES OF ANTICENTROMERE ANTIBODY-POSITIVE SJö GREN’S SYNDROME

Cited by 2 publications

References 29 publications

The relationship between Sjogren’s syndrome, systemic sclerosis and lymphoproliferative diseases

The relationship between Sjogren’s syndrome, systemic sclerosis and lymphoproliferative diseases

Clinical and laboratory characteristics of lymphoproliferative diseases in primary Sjogren's syndrome associated with anticentromere antibodies

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