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.
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.
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.
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