Aim: to propose diagnostic algorithm of IgG4-related disease (IgG4-RD). Materials and methods. One center retrospective research. 52 pts with IgG4-RD were included. The diagnosis was proved histologically and immunohistochemically. 48 out of 52 pts received treatment. Treatment included one of the following schemes (along with low oral glucocorticoids): rituximab monotherapy, cyclophosphamide monotherapy or their combination. Results. The mean age was 47.4±5.9 years, the mean age of the disease onset was 43.9±16.0 years. Median time before the diagnosis was 24 months. The most often sites of IgG4-RD were lacrimal (63.5%), salivary (46.2%) glands, lungs (48%), lymph nodes (34.6%) and retroperitoneum (17.3%). In clinical picture the leading complain was organ enlargement, but not its dysfunction. Pain was characteristic for retroperitoneum localization. In 56.8% of pts with IgG4-related syalo - and/or dacryoadenitis there was association with ear - nose - throat organs affection. In 4 pts (7.7%) IgG4-RD was combined with some malignant disease, including MALT-lymphoma of lacrimal glands. Irreversible organ damage as an IgG4-RD outcome had 15.4% of pts. The main laboratory markers of IgG4-RD were ESR elevation (38.5%), blood eosinophilia (9.6%), immunological disturbances (serum total IgG and IgG4 elevation, IgE elevation, antinuclear antibodies, rheumatoid factor detection, hypocomplementemia). Serum IgG4 level >1.35 g/l was elevated in 88% of pts and correlated with the number of affected organs (Spearman correlation coefficient 0.39, Student’s test, р=0.0056). Monoclonal serum secretion and B-cell clonality in the tissue was detected in 4 (23.5%) out of 17 pts, but not all of them had both signs. Conclusion. Based on the analysis of clinical and laboratory characteristics of IgG4-RD a diagnostic algorithm was proposed that enhances the detection and examination of the patients with suspected IgG4-RD.
Objective: to study the clinical and laboratory features of patients with anticentromere antibody (ACA) positive Sjö gren’s syndrome (SjS); to assess the spectrum of autoantibodies in patients of this group; to determine the frequency with which the SjS patients who are highly positive for ACA, meet the international classification criteria for SjS and systemic sclerosis (SS); to reveal the incidence of MALT lymphomas in this patient group; to estimate the incidence of primary biliary cirrhosis (PBC)/biliary lesions as part of autoimmune epithelitis in SjS in this patient group.Material and methods. A total of 83 patients with ACA positive SjS were comprehensively examined at the V.A. Nasonova Research Institute of Rheumatology during the period 2012 to 2018. The inclusion criteria were con formity to the 2001 Russian SjS criteria and a high ACA level. MALT lymphomas were diagnosed on the basis of histological and immunohistochemical studies and polymerase chain reaction-based determination of B-cell clonality in the biopsy samples of affected organs according to the World Health Organization classification of Hematopoietic Tumors. The diagnosis of PBC/biliary lesions was made on the basis of histological and immunohistochemical studies of liver biopsy specimens.Results and discussion. The investigation revealed low detection rates for anti-Ro antibodies (32.5%), anti-La antibodies (7.2%) and rheumatoid factor (RF) (21.7%), which were typical for the classical SjS immunophenotype), increased ESR (14%), leukopenia (7%), hypergammaglobulinemia (17.6%), elevated levels of IgG (9.5%) and IgA (18.7%), and hypocomplementemia (16.1%) in the ACA positive SjS patients. Despite the low detection rate of RF, 15 (18%) patients in this group developed MALT lymphomas: 14 patients had salivary gland MALT lymphoma and one patient had tonsil MALT lymphoma with peripheral lymph node involvement (generalized marginal zone lymphoma). Also, the patients of this group showed high detection rates for AMA antibodies (34.6%), increased IgM level (29.7%) and a higher risk for PBC/biliary lesions as a manifestation of autoimmune epithelitis in SjS (14.5%). AMA-antibodies were absent in only two patients who were diagnosed with liver disease according to biopsy specimens. Nervous system and renal lesions, antiphospholipid syndrome, rheumatoid arthritis, hypergammaglobulinemic purpura, and cryoglobulinemic vasculitis were much less common and sporadic. Also ACA-positive SjS patients often have Raynaud’s phenomenon (54.9%) with scleroderma-type capillaroscopic changes (68%) and a limited form of SS (24%) according to the 2013 ACR criteria.Conclusion. ACA-positive SjS is a subtype of the disease, which is significantly different from the classic one in a number of clinical and laboratory signs and characterized by an increased risk for SS, MALT lymphomas, and PBC/biliary lesions as a manifestation of autoimmune epithelitis in SjS which in some cases leads to the underdiagnosis of SjS. ACA should be considered as pathogenetically related to SjS autoantibodies; and all patients who are seropositive for ACA should be examined for SjS and PBC/biliary lesions as a manifestation of autoimmune epithelitis in SjS regardless of whether they have SS or not, as well as complaints of dry mouth and eyes. Patients with significantly enlarged salivary glands should undergo biopsy to rule out or confirm MALT lymphoma before initiating hormonal, antilymphoproliferative, and anti-B-cell therapy.
РезюмеЦель исследования. Предоставить демографическую, клиническую, лабораторную, рентгенорадиологическую, морфоло-гическую и иммуноморфологическую характеристику сиалоаденитов, связанных с IgG4 (IgG4-С), позволяющую прово-дить дифференциальную диагностику с нейроэндокринными, гранулематозными и онкогематологическими поражениями слюнных желез (СЖ). Материалы и методы. В период с 2004 по 2014 г. у 32 (11%) из 289 больных со значительным увеличением околоушных СЖ (ОУСЖ) и поднижнечелюстных СЖ (ПНЧСЖ) диагностирован IgG4-С. Только у 3 (9%) больных имелось изолированное заболевание, связанное с IgG4 (IgG4-З), тогда как у 29 (91%) пациентов поражение нескольких органов протекало как системное заболевание, связанное с IgG4 (IgG4-СЗ). Результаты. Отмечено незначительное преобладание женщин с медианой дебюта заболевания в 42 года. Увеличение ПНЧСЖ (52,7%), поражение полости носа и околоносовых пазух (37,2%), увеличение слезных желез и псевдотумор глаз-ниц (31%) -наиболее частые клинические проявления в дебюте заболевания. При динамическом наблюдении наряду с поражением ПНЧСЖ (97%), ОУСЖ (72%), глазниц (72%), полости носа и околоносовых пазух (56%) у 1 / 3 больных выявля-ется генерализованная лимфаденопатия, нередко развиваются поражения легких, печени, поджелудочной железы, почек, и заболевание протекает в рамках IgG4-СЗ. Лабораторные проявления характеризуются умеренной эозинофилией и повышением уровня IgE в крови у 1 / 3 больных, умеренным повышением СОЭ и гипергаммаглобулинемией у 50%. Повыше-ние уровней IgG (84%) и особенно его подкласса IgG4 (86,4%) в крови служат показанием к дальнейшему подтверждению IgG4-З у больных с поражением больших СЖ. Для верификации диагноза с IgG4-З должны быть выполнены иммуногисто-химическое исследование с определением концентрации плазматических клеток (ПК), секретирующих IgG4, и определе-ние B-клеточной клональности в биоптатах. Заключение. Определение IgG4 в крови (>2 г/л) у больных со значительным увеличением больших СЖ позволяет пред-положить наличие IgG4-С. Малоинвазивные инцизионные биопсии ОУСЖ и ПНЧСЖ с последующим морфологическим и иммуноморфологическим исследованием необходимо проводить для корректной постановки диагноза. Обнаружение более 40% ПК, секретирующих IgG4, в ткани СЖ является обязательным для постановки диагноза IgG4-З. Ключевые слова: иммуноглобулин G 4-го подкласса (IgG4), cиалоаденит, связанный с IgG4, заболевания, связанные с IgG4, системные заболевания, связанные с IgG4, болезнь Микулича, опухоль Кюттнера, болезнь Шегрена.Aim. To provide the demographic, clinical, laboratory, radiological, morphological, and immunomorphological characteristics of IgG4-related sialoadenitis (IgG4-S), which allow the differential diagnosis with neuroendocrine, granulomatous, blood cancer lesions of the salivary gland (SG). Subjects and methods. In the period 2004 to 2014, IgG4-S was diagnosed in 32 (11%) out of 289 patients with significantly enlarged parotid and submandibular glands (PG and SMG). Only 4 (9%) patients had isolated IgG4-related disease (IgG4-D) whereas involveme...
Цель исследования-установить основные типы лимфом, дебютирующие с поражения больших слюнных желез (БСЖ), с которыми приходится сталкиваться в ревматологической практике и оценить эффективность малоинвазивных инцизионных биопсий БСЖ/слезных желез в диагностике различных заболеваний, протекающих с поражением околоушных, поднижнечелюстных, подъязычных и слезных желез.
BackgroundIt is assumed that in ophthalmological clinical practice lymphoproliferative disorders in patients with affection of different orbital organs and tissues constitute 25–55% and 21% of patients have IgG4-related ophthalmic disease [1,2].ObjectivesTo evaluate the most common conditions affecting the orbit in rheumatologic clinical practice.MethodsDuring 2004 – 2016 years 138 patients (male – 33, female - 105) with eyelid edema and/or tumefactive lesions in the orbit were examined in Nasonova Research Institute of Rheumatology. In all patients full clinical, ophthalmological, serological (rheumatoid factor, C-reactive protein, IgG, IgG4, IgM, IgA, ANA, anti-Ro/La, C3/C4 complement) examination was carried. In all cases diagnosis was verified pathomorphologicaly with immunohystochemical staining (anti-CD 138, CD 68, IgG, IgG4, κ-chain, λ-chain). Diagnosis was established on the basis of pathomorphological examination of different tissues: orbit – 79 patients, parotid salivary gland – 40, submandibular salivary gland – 14, nasal – 4, lymph nodes – 3 and other – 6. In 79 cases (54%) the diagnosis was established on the results of orbital biopsy.ResultsDifferent non-neoplastic diseases were diagnosed in 108 patients (78.5%) and 30 had different conditions of hematological spectrum, including malignant conditions in 25 (18.2%) patients (see table 1). Some patients at baseline had simultaneous involvement of the major salivary glands (23 patients with IgG4-related disease, 35 with sarcoidosis, 12 with non-Hodgkin lymphomas and 2 with AL-amyloidosis). The most rare conditions affecting the orbit were Cogan's syndrome, relapsing polychondritis, Erdheim-Chester disease, NK/T-cell nasal lymphoma and calcifying aponeurotic fibroma.ConclusionsIn rheumatologic practice in 78.5% of patients with orbital involvement different non-neoplastic conditions are diagnosed: IgG4-related ophthalmic disease (35.0%), granulematous lesions (29%). The most common hematological disorders in rheumatologic clinic are non-Hodgkin lymphomas (17.5%) and histiocytosis (3.5%).References Japanese study group of IgG4-related ophthalmic desease. A prevalence study 0f IgG4-related ophthalmic disease in Japan.JpnJOphthalmol.2013;57: 573–579.Shields JA et al. Survey of 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part I. Ophthalmology.2004;111: 997–1008.Goto H et al. Diagnostic criteria for IgG4-related ophthalmic disease. JpnJOphthalmol.2015;59:1–7. Disclosure of InterestNone declared
Макроглоссия -увеличение и утолщение язы-ка, возникающее в результате патологического про-цесса или как аномалия развития. Она может разви-ваться равномерно (диффузное увеличение языка) или частично захватывая корень, спинку или боко-вые поверхности языка. Язык не помещается во рту, *e-mail: kate.76@mail.ru выступает наружу, на нем определяются отпечатки зубов, возникает нарушение речи. В классификаци-онных критериях макроглоссии различают истин-ную (врожденная и приобретенная) и относитель-ную (при микрогнатии) [1,2]. Врожденная макро-глоссия чаще встречается в детском возрасте и явля- 4 отделение морфологии ФГБНУ «Научно исследовательский институт ревматологии им. в.А. Насоновой», Москва, россия; 5 отделение патологии слезного аппарата ФГБНУ «Нии глазных болезней», Москва, россия У 15% больных AL-амилоидозом присутствует уникальный симптомокомплекс, связанный с массивным увеличением под-нижнечелюстных слюнных желез, развитием диффузной макроглоссии с прикусыванием языка и изменением тембра голоса. Качественная дифференциальная диагностика на стоматологическом приеме позволяет спасти жизнь пациентам с тяжелыми системными поражениями. Предложена схема обследования пациентов, позволяющая быстро и четко осуще-ствить диагностику при минимальных временных и финансовых затратах. Ключевые слова: макроглоссия, увеличении подчелюстных слюнных желез, AL-амилоидоз, диагностика.The importance of macroglossia and the enlargement of the submandibular salivary glands for the diagnostics of AL-amyloidosis in the practical work of a dental practitioner. A case report and review of the literature AL-amyloidosis in 15% of the patients presenting with this condition is known to be associated with a unique symptom complex characterized by the enlargement of the submandibular salivary glands, the development of diffuse macroglossia with tongue biting and the alteration of the quality of the voice. The high-quality differential diagnostics of this condition during the patient's visit to the dentist's office provides a tool for life saving in the patients suffering from severe systemic lesions. The scheme is proposed for the examination of the patients presenting with the above pathology that allows to save the life of the patients with minimal time consumption and financial expenditures.
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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