Background:In the international scientific community there has been a long discussion about the role of ultrasound and ultrasound scores for assessing the structure of the salivary glands in Sjögren’s syndrome. Stomatological examination according to The American College of Rheumatology (ACR) 2012 classification criteria of primary Sjögren’s syndrome (pSS) includes only labial salivary gland biopsy (focus score - FS) and the ACR with European League Against Rheumatism (ACR/EULAR) 2016 criteria include FS and unstimulated saliva flow test (SFT).Objectives:to compare the diagnostic value of ultrasonography salivary glands (SGUS) with other methods (sialography, stimulated SFT, FS).Methods:From 2016 to 2020, we examined 94 patients (93 women, 1 man) with the mean age 48.6 ± 14.3 years (minimum 18, maximum 78) with newly diagnosed pSS according to Russian criteria 2001, 86% of patients fulfilled ACR 2012 criteria and 87% - the ACR/EULAR 2016 criteria. All patients underwent classical examination (stomatological, ophthalmological, immunological), and SGUS was performed using GE LOGIQ 9 on the two parotid and two submandibular glands. Ultrasound images were evaluated with the OMERACT SS severity scoring system from grades 0 to 3 (1). Statistical analyses (Spearman coefficient p <0.05) were performed using STATISTICA version 12.Results:All patients in SGUS had inhomogeneity structure from mild to diffuse with hypoechoic areas from single to multiple. 4 patients had no signs of sialectasia on sialography, however, in ultrasound we found SGUS grade 2. In 5 patients with SGUS grade 0 we determined punctate sialectasia. Detailed characteristics of patients are presented in Table 1.Table 1.ParametersN%Oral dryness7883,0 Enlargement of parotid salivary glands3234,0Recurrent parotitis1920,2Retention pain3133,0RF positive >2UNL (>30 IU/ml)5154,2anti-SSA (anti-Ro) positive (>25 IU/ml)8186,1anti-SSB (anti-La) positive (>25 IU/ml)4851,0 ANA ≥ 1:32094100,0SGUS 088,5SGUS 111,0SGUS 22021,2SGUS 36569,1Stimulated SFT < 2,5ml/5 min5962,8Sialectasia on parotid sialography9094,7Punctate sialectasia1212,6 Globular sialectasia6366,3Cavitary sialectasia1010,5 Distructive sialectasia66,3FS ≥ 1foci/4 mm28388,2Correlation was calculated for each SGUS (0-3) with clinical and classical methods of examination of the salivary glands. Pronounced changes in SGUS 3 correlated with recurrent parotitis r = 0.22 (p = 0.03), enlargement of parotid salivary glands r = 0.23 (p = 0.02) and cavitary sialectasia r = 0.23 (p = 0.02). Histological changes FS ≥ 1 correlated with all grades on ultrasound (SGUS 0 r = -0.24 (p = 0.01), SGUS 1 r = 0.41 (p = 0.000027), SGUS 2 r = - 0.21 (p = 0.03), SGUS 3 r = 0.40 (p = 0.00006)). The punctate sialectasia correlated with SGUS 1 r = -0.28 (p = 0.005) and SGUS 0 r = 0.45 (p = 0.000004). Stimulated SFT didn’t correlate with ultrasound changes.Conclusion: :Probably, in everyday clinical practice, SGUS can be used as an additional method for salivary gland assessment, given the availability and safety of the method. The most convenient system for assessing the salivary glands, at present, is the OMERACT SS severity scoring system, however, now we don’t have a score for the complete differentiation in pSS and lymphoproliferative complications.References: :[1]Jousse-Joulin, Sandrine et al. “Video clipassessment of a salivary gland ultrasound scoring system in Sjögren’s syndrome using consensual definitions: an OMERACT ultrasound working group reliability exercise.” Annals of therheumatic diseases vol. 78,7 (2019): 967-973. doi:10.1136/annrheumdis-2019-215024Disclosure of Interests:None declared
Background:Felty’s syndrome (FS) is a rare subtype of seropositive rheumatoid arthritis (RA) and is characterized by neutropenia and splenomegaly. Some researchers suggest that FS and T-cell large granular leukemia (T-LGLL) may have common pathogenetic relationships [1].Objectives:to characterize the clinical and laboratory manifestations of FS, to evaluate the frequency of T-LGLL and Sjogren’s syndrome (SS) in this group of patients with RA, neutropenia and splenomegaly (pts).Methods:We observed 18 pts with ACCP-positive (100%) and RF-positive (94.5%) RA diagnosed according to ACR 2010 criteria, who also had neutropenia and hepatosplenomegaly. All 18 pts underwent T-LGLL diagnostics using blood smears and phenotyping of peripheral lymphocytes for the presence of granular lymphocytes, determination of T-cell clonality using the rearrangement of the γ-chain of the T-cell receptor of lymphocytes by PCR, histological/immunohistochemical study of bone marrow biopsy specimens for the presence of LGL invasion studies, as well as the study of 4 spleens after splenectomy. SS was diagnosed in 8 out of 18 pts (44.5%) according ACR 2016 criteria.Results:Twelve (66.6%) of 18 pts with RA, neutropenia and splenomegaly were diagnosed with T-LGLL, the patients were divided in 2 groups: FS (6 pts) and RA+T-LGL (12 pts). Pts with FS debuted with arthritis of small hand joints, extremely rarely with extra-articular manifestations, mainly at a young age (36.5±3.9 years), and developed neutropenia after 10 years of RA. Pts with T-LGLL debuted at a younger age (39,5±4,5 and 51,5 + 7,8 years, respectively), had a longer course of RA before the development of neutropenia (14.3±3.3 and 5±1.5 years, respectively, p=0.03), and more often had extra-articular manifestations at the onset of the disease. RA activity did not differ between groups and in most cases was characterized by a mild course of articular syndrome. Though the course of RA+T-LGLL group was characterized by low (50%) and moderate (33%) DAS28-CRP activity and active synovitis in only 41.5% of pts, severe joint deformities (stage III and IV) developed in 58.5% of pts. Pts with T-LGLL showed a higher incidence of hepatomegaly (75% and 16.5%, respectively, p=0.02) and more severe neutropenia (p=0.02). The development of severe leukopenia (<1x109) and massive hepatosplenomegaly was observed only in pts with T-LGLL, which required splenectomy in 4 cases. SS was more often detected in the FS group than in the RA+T-LGLL group (83.5% and 25%, respectively, p = 0.02).Conclusion:Clinical and laboratory manifestations of FS and T-LGLL are extremely close, therefore, pts who are diagnosed with FS should be examined to exclude T-LGLL.References:[1]Liu, Xin, and Thomas P Loughran Jr. “The spectrum of large granular lymphocyte leukemia and Felty’s syndrome.” Current opinion in hematology vol. 18,4 (2011): 254-9. doi:10.1097/MOH.0b013e32834760fb.Disclosure of Interests:None declared
Background:B-cells play a pivotal role in primary Sjogren’s syndrome (SS) pathogenesis. Recent studies have shown disturbances in the peripheral B cell populations in primary SS.Objectives:To examine В-cell subsets in peripheral blood of SS patients (pts) and to analyze the association between B-cell subsets and SS activity.Methods:Twenty active SS pts (19F/1M): median age 42 years (range) (32-54); disease duration 3 (2-10) years; ESSDAI score ≥5 in 6 pts, <5 in 14 pts), were included. SS was diagnosed based on the ACR-EULAR 2016 criteria. Twenty healthy donors composed the control group. CD19+B cells, memory B-cells (CD19+CD27+), non-switched memory B-cells (CD19+IgD+CD27+), switched memory B-cells (CD19+IgD-CD27+), naïve (CD19+IgD+CD27-), double negative (CD19+IgD-CD27-), transitional (CD19+IgD+CD10+CD38+) B-cells, plasmablasts (CD19+СD38+++IgD-CD27+), and plasma cells (CD19+СD38+) were analyzed by multicolor flow cytometry using cytometer Navios (Beckman Coulter, USA).The nonparametric Mann-Whitney test, the unpaired Student’s t test for group comparison, and the Pearson`s x2 criterion were used for statistical analysis. Data were shown as median (Me) with an interquartile range of 25 - 75 percentile. The differences were considered statistically significant when p <0.05. Statistica 10 for Windows (StatSoft Inc., USA) package was used for statistical data processing.Results:The percentages/absolute numbers of plasmablasts, plasma cells and transitional B-cells in SS were significantly higher than in healthy donors, р<0.01 for all cases (Table 1).Table 1.B-cell subsets in patients with SS, SS and MALT lymphoma and healthy donors.B-cell subsetsSSHDPt 1FN%Abs. cells/μl%Abs. cells/μl%Abs. cells/μlB lymphocyte12,8 (7,9 – 19,7)151 (95-112)8,5 (7,2-11,0)200 (100-200)4,531**plasma cells6,9 (4,2-10)12 (6-22)0,1 (0,05-0,1)0,1 (0-0,2)5,713*plasmablasts0,88 (0.37-1.8)2 (1-3)0,1 (0,1-0,2)0,2 (0,1-0,4)26,612*/**transitional B-cells5.7 (0.9-17)8 (4-32)0,1 (0-0,1)0,1 (0-0,3)00*switched cells9.1 (5.9-20.7)18 (11-29)12,8 (9,3-17,0)20 (10-40)5216**non-switched memory B-cells4.13 (2.7-5.4)7 (3-15)7,4 (3,7-11,1)10 (5-20)165**SS Sjogren’s patients; HD healthy donors, Pt 1 patient with SS and MALT lymphoma* SS and HD groups compared, р<0.01** Pt 1 and SS group comparedOne of the patients (Pt 1) was diagnosed with MALT lymphoma of the parotid salivary gland; she had a distinct B-lymphocyte subpopulations distribution, which was different from the rest of the SS group. She had the lowest percentage / absolute B lymphocyte count of all patients with SS, the highest percentage of switched cells and non-switched memory B-cells, and the highest percentages/absolute numbers of plasmablasts. For this reason, the patient was excluded from the subsequent analysis (Table 1).Patients with activity of SS by ESSDAI ≥ 5 had a significantly higher percentage of B cells (p=0.007), and significantly higher absolute B lymphocyte count of naïve cells (p=0.04) and plasmablasts (p=0.048).Conclusion:Immunophenotyping showed disturbed homeostasis of the B-cells subpopulations in our SS cohort. A significant increase in plasmablasts in SS, as well as a positive correlation of the level of plasmablasts with the SS activity and presence of lymphoma could suggest the important role of these cells in the pathogenesis of SS.Disclosure of Interests:None declared.
Background:IgG4-related disease (IgG4-RD) is a systemic immunomediated fibroinflammatory condition that can affect almost any organ in the body. This is the reason for dramatic variety of clinical symptoms and complexity of diagnostics. 2011 Comprehensive diagnostic criteria (CDC) for IgG4-RD are used to establish the diagnosis for all lesions (except autoimmune pancreatitis type 1). In 2019 the new ACR/EULAR classification criteria for IgG4-RD were proposed to facilitate the formation of more homogenous groups of patients primarily for clinical trials inclusion purpose. They also provide a framework for clinicians considering diagnosis of IgG4-RD.Objectives:To evaluate 2019 ACR/EULAR classification criteria for IgG4-RD in Russian cohort of patients with IgG4-RD.Methods:59 patient with IgG4-RD according to CDC with biopsy proven diagnosis were included.Results:The mean number of affected organs was 2.1; 31 patients (52,5%) were women. Majority of patients had sialoadenitis (25 patients) and/or orbital disease (31 patients), 9 had retroperitoneal fibrosis (RPF). Other affected organs were lungs, pancreas, lymph nodes, paranasal sinuses, thyroid and low urinary tract. Twenty five (25) patients (42,4%) had definite, 14 (23,3%) probable and 20 (34,3%) possible diagnosis of IgG4-RD. Twenty three (23) patients (39%) didn’t fulfill the 2019 ACR/EULAR classification criteria for IgG4-RD. Among them were the majority of patients with RPF (7 patients) who were lacking other organ involvement and IgG4 hypersecretion either in the tissue or serum. The majority of excluded cases were due to inadequate pathomorphological evaluation (lacking of the exact number and percentage (if >40%) of IgG4+ cells), lacking of multi-organ involvement or different patterns of involvement, e.g. in case of lungs involvement.Conclusion:The new 2019ACR/EULAR classification criteria for IgG4-RD are very useful in evaluation of typical organ involvement and systemic course of IgG4-RD. It is essential to adjust Russian pathomorphologists’s approach to cell counting and percentage determination for IgG4-RD cases to get suitable protocols.Disclosure of Interests:None declared
BackgroundApart from anti-Ro and/or anti-La positive primary Sjögren’s syndrome (pSS), there is a unique subtype of pSS with anticentromere antibodies (ACA). At the present time salivary gland ultrasonography (SGUS) is widely used to assess the structure of the salivary glands in pSS, but there are few publications about SGUS changes in ACA-positive patients [1].ObjectivesTo investigate SGUS changes in ACA (ACA+) positive pSS patients and compare with ACA-negative (ACA–).MethodsWe examined 141 patients with pSS, including 103 ACA– patients (pSS-ACA–) with the mean age 49,8 ± 14,1 years and 38 ACA+ positive (pSS-ACA+) with the mean age 59,1 ± 10,2 years. All patients underwent standard examination for the diagnosis of pSS (stomatological, ophthalmological, immunological), and SGUS performed using GE LOGIQ 9 of two parotid and two submandibular glands. Ultrasound images were evaluated with the OMERACT SGUS scoring system (SGUS SS) from grades 0 to 3 [2]. Statistical analyses (chi-squared test, p <0.05) were performed using STATISTICA version 12.ResultsCharacteristics of patients with pSS-ACA+ and pSS-ACA– are presented in Table 1.Table 1.ParametersACA–ACA+N%N%Oral dryness8683,53694,7Ocular dryness7673,83078,9Enlargement of parotid salivary glands3634,9718,4Recurrent parotitis2322,3760,5ANA ≥1:32010310038100Stimulated saliva flow test < 2.5 ml/5 min6563,13078,9Sialectasia on parotid sialography10310038100Stimulated Schirmer’s test <10 mm/5 min6765,02668,4Tear breakup time <10 seconds5957,32052,6Focus score ≥ 1foci/4 mm29491,312/1392,3MALT-lymphomas87,7513,1Characteristics of SGUS SS in pSS-ACA+ and pSS-ACA– are shown in Figure 1.Figure 1.We did not find significant differences when comparing SGUS SS in patients with pSS-ACA+ and pSS-ACA–.ConclusionWe did not find significant differences in SGUS and SGUS SS in patients with pSS-ACA+ and pSS-ACA–.ReferencesFURS-2022-003[1]Min HK, Kim SH, Park Y, et al. Ultrasonographic characteristics of major salivary glands in anti-centromere antibody-positive primary Sjögren’s syndrome. PLoS One. 2021;16(11):e0259519. Published 2021 Nov 3. doi:10.1371/journal.pone.0259519[2]Jousse-Joulin, Sandrine et al. “Video clip assessment of a salivary gland ultrasound scoring system in Sjögren’s syndrome using consensual definitions: an OMERACT ultrasound working group reliability exercise.” Annals of the rheumatic diseases vol. 78,7 (2019): 967-973. https://doi:10.1136/annrheumdis-2019-215024Disclosure of InterestsNone declared
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