Extraglandular Involvement Research Articles

Association of focus score and extraglandular involvement in Sjögren's syndrome: A study on antinuclear antibodies and minor salivary gland pathology.

This study aimed to investigate the relationship between patients with and without extraglandular involvement in Sjögren's syndrome (SS) by analyzing ANA (antinuclear antibody) and ENA (extractable nuclear antigen) results and minor salivary gland pathology findings. A total of 265 (245 females; 20 males; mean age: 50.4±12.4; range, 19 to 79 years) patients diagnosed with SS were included in the retrospective cohort study between March 1, 2011, and December 1, 2021. Detailed documentation was performed, capturing demographic characteristics, clinical information, laboratory findings, medication usage, and manifestations of the syndrome. The patients were divided into two groups, with (78 females; 8 males; mean age: 52.7±11.5; range, 22 to 78 years or without (167 females; 12 males; mean age: 49.3±12.8; range, 19 to 79 years extraglandular involvement. The mean follow-up duration was 63.1±31.9 months. Extraganular involvement, including joint involvement, lung involvement, central nervous system involvement, hematological involvement, hepatitis, and lymphoma, was observed in 32.5% of the patients. Patients with extraglandular involvement required multiple medications, while those with only glandular involvement predominantly used hydroxychloroquine. The mean duration from SS diagnosis to extraglandular involvement was 15.2±27.8 months. The comparison between patients with and without extraglandular involvement revealed a significant association between higher focus scores (FS) and extraglandular manifestations. However, no significant differences were observed in terms of ANA positivity, ANA titers, or ENA positivity. Regression analysis indicated that age and FS were linked to systemic involvement. This study highlights the importance of FS as a predictive indicator for extraglandular manifestations in SS. Advanced age was found to be associated with an increased likelihood of extraglandular involvement. Assessing FS and age can aid in predicting extraglandular manifestations.

Rare presentations of Sjogren syndrome

Sjögren’s syndrome is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands. It most commonly presenting with sicca symptoms. Sicca refers to dryness most often involving the eyes and mouth due to inflammation and resultant pathology of the lacrimal and salivary glands. Up to one-half of affected individuals also develop extra-glandular involvement in organs such as the joints, skin, lungs, gastrointestinal (GI) tract, nervous system, and kidneys. This condition is frequently associated with other autoimmune disorders including rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) - called the secondary Sjogrens syndrome. Evaluation of a patient with suspected Sjogren syndrome should include an evaluation of oral and ocular dryness and function. In addition to the history, this may include the performance of a Schirmer test, slit-lamp exam with vital dye staining, salivary flow rate, and/or nuclear scintigraphic evaluation of the salivary glandular function. Assessment of autoantibodies (ANA, RF, SS-A, and SS-B) should also be performed. Of these, SS-A is probably the most sensitive and specific antibody for Sjogren's but alone is not diagnostic since it may be present in other autoimmune disorders and may be absent in up to a third of Sjogren cases. The most specific single test is a minor salivary gland (lip) biopsy which will demonstrate focal lymphocytic sialadenitis (FLS). Therapies are directed toward replacing moisture at affected glandular sites and suppressing the autoimmune response locally as well as systemically. This activity reviews the evaluation and management of Sjogren syndrome and explains the different rare presentations in which it can present.

Beyond sicca: high prevalence and predictors of baseline and worsening systemic involvement in patients with Sjögren's disease.

Systemic extraglandular involvement in SS has been reported in one-third of patients but may be more frequent. We aimed to evaluate systemic disease prevalence at baseline and throughout follow-up and find its predictors. We conducted a retrospective cohort study including SS patients followed in a tertiary centre. The cumulative EULAR SS disease activity index (ESSDAI) was calculated by adding each domain's maximum score throughout follow-up. We identified independent predictors of systemic involvement (ESSDAI ≥1 at baseline and/or follow-up) through logistic regression modelling. A survival analysis was conducted to identify predictors of new/worsening ESSDAI domains. A total of 216 patients were included, most of whom had systemic involvement (86%), frequently at diagnosis (76%). Biological (53%) and articular ESSDAI domains (44%) were most commonly involved, but all were affected at least once. Around half of the patients with baseline systemic disease developed an additional/worsening domain throughout follow-up. Although most patients had low disease activity at baseline, 60% eventually reached moderately active disease. Younger age at diagnosis [odds ratio (OR) 0.95 (95% CI 0.91, 0.99)], a positive minor salivary gland biopsy [OR 4.08 (95% CI 1.40, 11.86)] and RF [OR 4.67 (95% CI 1.52, 14.33)] were independent predictors of systemic involvement. Patients with baseline constitutional involvement [hazard ratio (HR) 2.23 (95% CI 1.13, 4.40)] and RF [HR 1.89 (95% CI 1.20, 3.00)] were more likely to develop new/worsening systemic disease activity. Systemic involvement is seen in most SS patients. Younger and RF and salivary gland biopsy-positive patients are at higher risk of systemic disease. Around half of patients with systemic involvement experienced aggravated disease over time, especially those with constitutional involvement or RF.

Sjögren's disease: From pathophysiology to treatment

Sjögren's disease (SjD) is a systemic autoimmune disorder characterized by a triad of key symptoms affecting almost all patients (salivary and lacrimal dryness, pain and fatigue) and extra-glandular systemic involvement affecting one to two-thirds of patients. Over the past decade, knowledge of the epidemiology, classification criteria, assessment of systemic activity and symptoms presented by patients has grown. In addition, advances in understanding the pathophysiology of SjD have enabled a more targeted therapeutic approach. Current management of SjD is based on EULAR treatment guidelines. But since these recommendations, new drugs targeting specific pathophysiological pathways of the disease, and essentially B lymphocyte activation, have shown efficacy in phase 2 trials. In this review, we will summarize the available evidence on systemic therapies, including: 1. advances in outcome assessment, 2. current evidence on targeted disease-modifying therapies and biologic drugs targeting primarily B lymphocytes, 3. an overview of promising drugs being tested in ongoing trials.

Risk factors for cardiovascular disease in primary Sjögren's syndrome (pSS): a 20-year follow-up study.

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterized by a chronic grade of inflammation. Cardiovascular events represent the major causes of morbidity and mortality in patients with inflammatory rheumatic diseases; however, the significance and prevalence of cardiovascular disease in patients with pSS remain unclear. To determine the clinical significance of cardiovascular disease in pSS and analyze the risk of cardiovascular disease according to glandular/extraglandular involvement and positivity to anti-Ro/SSA and/or anti-La/SSB autoantibodies. A retrospective study including patients diagnosed with pSS fulfilling the 2016 ACR/EULAR classification criteria was followed and evaluated in our outpatient clinic between 2000 and 2022. The prevalence of cardiovascular risk factors with pSS was evaluated, and a possible association with clinical and immunological characteristics, the treatments received, and the impact on cardiovascular disease were determined. Univariate and multivariate regression analyses were performed in an attempt to determine potential risk factors associated with cardiovascular involvement. A total of 102 pSS patients were included. Eighty-two percent were female, with a mean age of 65±24 years and a disease duration of 12.5 ±6 years. Thirty-six patients (36%) had at least one cardiovascular risk factor. Arterial hypertension was diagnosed in 60 (59%) patients, dyslipidemia in 28 (27%), diabetes in 15 (15%), obesity in 22 (22%), and hyperuricemia in 19 (18%). History of arrhythmia was found in 25 (25%), conduction defects in 10 (10%), arterial peripheral vascular disease in 7 (7%), venous thrombosis in 10 (10%), coronary artery disease in 24 (24%), and cerebrovascular disease in 22 (22%) of patients. Patients with extraglandular involvement had a higher prevalence of arterial hypertension (p=0.04), dyslipidemia (p=0.003), LDL mean values (p=0.038), hyperuricemia (p=0.03), and coronary artery disease (p=0.01) after adjusting for age, sex, disease duration, and the significant variables in the univariate analysis. Patients with Ro/SSA and La/SSB autoantibodies had a substantially higher risk of hyperuricemia (p=0.01), arrhythmia (p=0.01), coronary artery disease (p=0.02), cerebrovascular disease (p=0.02), and venous thrombosis (p =0.03). In the multivariate logistic regression analysis, higher odds of cardiovascular risk factors were associated with extraglandular involvement (p=0.02), treatment with corticosteroids (p=0.02), ESSDAI>13 (p=0.02), inflammatory markers including ESR levels (p 0.007), and serologic markers such as low C3 levels (p=0.03) and hypergammaglobulinemia (p=0.02). Extraglandular involvement was associated with a higher prevalence of arterial hypertension, dyslipidemia, hyperuricemia, and coronary artery disease. Anti-Ro/SSA and anti-La/SSB seropositivity was associated with a higher prevalence of cardiac rhythm abnormalities, hyperuricemia, venous thrombosis, coronary artery disease, and cerebrovascular disease. Raised inflammatory markers, disease activity measured by ESSDAI, extraglandular involvement, serologic markers including hypergammaglobulinemia and low C3, and treatment with corticosteroids were associated with a higher risk for cardiovascular comorbidities. Key Points • Patients with pSS are vulnerable to cardiovascular risk factors. There is an interconnection between extraglandular involvement, disease activity, inflammatory markers, and cardiovascular risk comorbidities. • Anti-Ro/SSA and anti-La/SSB seropositivity was associated with a higher frequency of cardiac conduction abnormalities, coronary artery disease, venous thrombosis, and stroke. • Hypergammaglobulinemia, elevated ESR, and low C3 are associated with a higher prevalence of cardiovascular comorbidities. • Valid risk stratification tools to help with prevention and consensus on the management of CVDs in pSS patients are warranted.

Cardiac Manifestations of Sjogren's Syndrome: A Review of Literature.

Sjogren's syndrome (SS) is a chronic inflammatory disorder of the exocrine glands. It is characterized by a lymphocytic infiltrate in the lacrimal and salivary glands causing keratoconjunctivitis sicca and xerostomia. Extra-glandular involvement may be present in about one-third of patients with primary Sjogren's syndrome (pSS).The most commonly affected organs are the thyroid, lungs, gastrointestinal tract, kidneys, skin, and nervous system.Cardiac manifestations of Sjogren's syndrome are rare and not well-described in the current literature. Most of the evidence is present in the form of case reports and small case series.However, recent studies have shown that patients with Sjogren's syndrome (SS) seem to have a greater overall risk of cardiovascular (CV) events. Although not conventionally considered a feature of the disease, cardiac manifestations can lead to increased morbidity and mortality in this patient population.In this review article, we study the association between cardiac diseases and primary Sjogren's syndrome.

#4323 A RARE CASE OF MEMBRANOUS NEPHROPATHY AS THE INITIAL PRESENTATION OF SJöGREN SYNDROME

Abstract Introduction Primary Sjögren's syndrome (pSS) is an autoimmune disorder characterized by lymphocytic infiltrates in exocrine glands, most commonly manifesting as dry eyes and mouth. Even though extra glandular involvement can occur in Sjögren, renal involvement is rare. It affects less than 10% of the patient population, with tubulointerstitial nephritis being the usual presentation. Glomerular involvement and, in particular, membranous nephropathy are not commonly encountered. We describe a rare case of membranous nephropathy as the initial presentation of Primary Sjögren's syndrome. Case history A 21-year-old female with a history of sickle cell trait presented to our emergency department with one week history of anasarca. Vital signs were unremarkable, and the physical exam was significant for a 2+ pitting edema of bilateral upper and lower extremities. Initial investigation revealed acute kidney injury (Creatinine 2.9 mg/dl from normal baseline), nephrotic range proteinuria of 6 grams on 24-hour urine collection, and hypoalbuminemia (serum albumin 1.5 mg/dl). Immunological workup showed positive ANA (speckled), strongly positive anti-SSA, borderline elevated anti-SSB, negative anti-PLA2R, negative cryoglobulins, negative anti-dsDNA, and negative anti-Smith antibody (Figure 1). Renal biopsy (Figure 2) revealed membranous nephropathy with acute tubular necrosis (ATN). PLA2R staining was negative. We attributed acute kidney injury to ATN and proteinuria to membranous nephropathy. Negative serologies and renal biopsy findings ruled out systemic Lupus. The patient was therefore diagnosed with membranous nephropathy secondary to pSS. The patient was started on Mycophenolate mofetil and steroid taper with an improvement of proteinuria and anasarca and currently follows at our clinic. Discussion Glomerular involvement is rare, and when involved, it is membranoproliferative glomerulonephritis (MPGN) associated with B-cell activation and positive cryoglobulins. Membranous nephropathy is rare with primary Sjögren syndrome. In our patient, a negative anti-PLA2R goes against primary membranous nephropathy. A negative anti-dsDNA and anti-Smith and biopsy findings essentially rule out Lupus nephritis, leaving Sjögren induced secondary membranous nephropathy as the most likely diagnosis. A literature search revealed few described cases of membranous nephropathy as the presenting feature of pSS with later development of sicca symptoms and progression of renal disease to MPGN. Interestingly, our patient had no sicca symptoms at the presentation time. Occult salivary gland involvement has been described in some of these patients; however, our patient deferred salivary gland biopsy. The management of renal disease in pSS has yet to be well described because of its rarity. Rituximab has shown the most benefit in patients with pSS who have positive cryoglobulins and associated MPGN. Steroids and mycophenolate mofetil (MMF) are other possible agents that can be tried. Given that experience, we started on steroids and MMF with an excellent response to treatment indicated by improvement in proteinuria. Conclusion This case adds to the limited available literature on membranous nephropathy as the initial manifestation of pSS. Although the European Alliance of Associations for Rheumatology (EULAR) criteria strongly emphasizes sicca symptoms and glandular involvement in pSS, the possibility of a small subset of pSS patients who present with early renal involvement with latent glandular findings needs to be recognized.

The Spectrum of Extraglandular Manifestations in Primary Sjögren's Syndrome.

Extraglandular manifestations (EGMs) in primary Sjogren's syndrome (pSS) represent the clinical expression of the systemic involvement in this disease. EGMs are characterized by a wide heterogeneity; virtually any organ or system can be affected, with various degrees of dysfunction. The existing gaps of knowledge in this complex domain of extraglandular extension in pSS need to be overcome in order to increase the diagnostic accuracy of EGMs in pSS. The timely identification of EGMs, as early as from subclinical stages, can be facilitated using highly specific biomarkers, thus preventing decompensated disease and severe complications. To date, there is no general consensus on the diagnostic criteria for the wide range of extraglandular involvement in pSS, which associates important underdiagnosing of EGMs, subsequent undertreatment and progression to severe organ dysfunction in these patients. This review article presents the most recent basic and clinical science research conducted to investigate pathogenic mechanisms leading to EGMs in pSS patients. In addition, it presents the current diagnostic and treatment recommendations and the trends for future therapeutic strategies based on personalized treatment, as well as the latest research in the field of diagnostic and prognostic biomarkers for extraglandular involvement in pSS.

Clinical and Serological Profile of Sjögren’s Syndrome Patients in a Tertiary Care Center in Kashmir, North India

Abstract Background: Sjögren’s syndrome (SS) is a chronic autoimmune illness characterized by lymphocyte infiltration and exocrine gland inflammation. SS has been considered a rare disorder in India, and data on its clinical manifestations and serology are lacking from North India. Aim: We aimed to study clinical manifestations, organ involvement, and autoantibodies in SS patients from Jammu and Kashmir. Methods: This was a single-center observational prospective study. A total of 50 patients satisfying the American-European Consensus Group criteria were included in the study. Data regarding subjective and objective evidence of disease were compiled. Extraglandular manifestations in the form of musculoskeletal, renal, pulmonary, and neurological were also recorded. Results: Of the 50 patients, 29 were primary SS and 21 were secondary SS. Female preponderance was observed with a female-to-male ratio of 24: 1. Xerostomia was the most common symptom present in all of the patients. The most common extraglandular involvement was musculoskeletal followed by renal and pulmonary. Conclusions: Early onset of the disease by a decade is a surprising observation in this study and rest of the Indian literature compared to western literature.

Salivary gland ultrasound in clinical practice: What is its real usefulness?

BackgroundSalivary gland ultrasound (SGU) provides information about structural gland abnormalities that can be graded and used for primary Sjögren's syndrome (pSS) diagnosis. Its potential role as a prognostic marker for detecting patients at high risk of lymphoma and extra-glandular manifestations is still under evaluation. We aim to assess the usefulness of SGU for SS diagnosis in routine clinical practice and its relationship with extra-glandular involvement and lymphoma risk in pSS patients. MethodsWe designed a retrospective observational single-center study. Data was collected using the electronic health records of patients referred to an ultrasound outpatient clinic for evaluation over a 4-year period. Data extraction included demographics, comorbidities, clinical data, laboratory tests, SGU results, salivary gland (SG) biopsy, and scintigraphy results. Comparisons were made between patients with and without pathological SGU. The external criterion for comparison was the fulfillment of the 2016 ACR/EULAR pSS criteria. ResultsA total of 179 SGU assessments were included from this 4-year period. Twenty-four cases (13.4%) were pathological. The most frequently diagnosed conditions prior to SGU-detected pathologies were pSS (9.7%), rheumatoid arthritis (RA) (13.1%), and systemic lupus (4.6%). One hundred and two patients (57%) had no previous diagnosis (sicca syndrome work-up); of these, 47 patients (46.1%) were ANA positive and 25 (24.5%) anti-SSA positive. In this study, the sensitivity and specificity of SGU for SS diagnosis were 48% and 98% respectively, with a positive predictive value of 95%. There were statistically significant relationships between a pathological SGU and the presence of recurrent parotitis (p=.0083), positive anti-SSB antibodies (p=.0083), and a positive sialography (p=.0351). ConclusionsSGU shows high global specificity but low sensitivity for pSS diagnosis in routine care. Pathological SGU findings are associated with positive autoantibodies (ANA and anti-SSB) and recurrent parotitis.

Renal failure and pleural effusion; a diagnostic challenge

Sjögren’s syndrome is a chronic inflammatory disorder mostly involving the exocrine glands. Extraglandular disease may occur in up to one quarter of patients. Kidney involvement is rare, more often manifested by tubular dysfunction secondary to tubulointerstitial nephritis. Primary glomerular disease is uncommon. The authors present the case of a 73-year-old woman with xerostomia and positivity for anti-Ro and anti-La antibodies admitted for acute kidney injury and exudative pleural effusion. Biopsy of salivary glands was compatible with Sjögren’s syndrome. Extraglandular involvement was also confirmed by renal and pleural deposition of AA-amyloid. The patient was started on prednisolone followed by azathioprine with rapid improvement of lung disease. However, due to progressing renal disease and clinical deterioration, prognosis was guarded and the patient died. We describe a case of secondary amyloidosis with systemic involvement and infrequent clinical manifestations, briefly reviewing the key aspects of Sjögren’s syndrome and AA-amyloidosis.

Sjogren’s Syndrome: An Ocular Overview

Sjogren’s syndrome (SS) is a chronic systemic autoimmune disorder which predominantly affects the salivary and lacrimal glands (exocrine glands) resulting in presentation of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia), collectively known as sicca symptoms. Although ren SS primarily affects glands, in some cases there is extra-glandular involvement indicating an autoimmune response can occur in other organs and tissues. SS is categorised into primary, when symptoms occur in isolation, or secondary, when symptoms occur in conjunction with another autoimmune disorder such as rheumatoid arthritis or systemic lupus erythematosus.

Multiple Cutaneous Features Associated with Primary Sjogren’s Syndrome

Sjogren’s syndrome is a systemic autoimmune disorder that mainly affects exocrine glands, although the clinical manifestations include both exocrine gland involvement and extraglandular disease features. In addition to dryness, musculoskeletal pain and fatigue are the hallmarks of this disease and constitute the classic symptom triad presented by the vast majority of patients. The authors present the case of a 59-year-old woman with achalasia, sicca syndrome, subcutaneous nodules affecting the knees, purpuric plaques on the palms and vitiligo. Serological tests were consistent with Sjögren′s syndrome, therefore the diagnosis of primary Sjögren’s syndrome with extensive cutaneous manifestations as extraglandular organ involvement was made.

Severe extra-glandular involvement and pleural effusions complicating primary Sjogren’s syndrome: a case report

BackgroundSjogren’s syndrome, an autoimmune disease of the exocrine glands, results in keratoconjunctivitis sicca, xerostomia, and dental caries. It is often overlooked, considered by clinicians to be a benign disease. However, it can cause life-threatening extra-glandular complications that affect multiple organ systems.Case presentationHere we present a 78-year-old Caucasian woman with a history of primary Sjogren’s syndrome (pSS) whose symptoms of keratoconjunctivitis sicca were managed managed conservatively. She was evaluated for sub-acute shortness of breath. Imaging showed severe bronchiectasis with features of lymphocytic interstitial pneumonia. She also had exudative bilateral pleural effusions and skin ulcers, likely vasculitic in origin. The workup was significant for anti-Ro antibody, pancytopenia, hypocomplementia, cryoglobulinemia and monoclonal gammopathy, all of which reflect disease severity. Although there was no evidence of malignancy, she developed B-cell non-Hodgkin lymphoma during follow-up.ConclusionsPrimary Sjogren’s syndrome can result in severe multi-organ disease. Pleural effusions are a rare complication of pSS, with only ten cases reported in the literature over the last 30 years, and tend to respond well to steroids. Prognostic biomarkers for disease severity include hypocomplementia, cryoglobulinemia, monoclonal gammopathy, and hypergammaglobulinemia. In this report we review the literature and the management of the disease.

Gougerot Sjögren Syndrome and Medular Aplasia: A Case Report and Review of the Literature

Introduction: Gougerot-Sjögren's syndrome (GSS) is a non-organ specific auto-immune epithelitis that affects essentially exocrine glands characterized by an inflammation of the epithelium, in particular salivary and lacrimal epithelium, leading to oral (xerostomia) and ocular (xerophthalmia) dryness, with the possibility of extra-glandular involvement, which reflects its systemic nature. Its prevalence is estimated between 0.01 and 0.09%. Its association with medullary aplasia (MA) is rare; the latter is of various origins, in particular drug-related, linked to methotrexate. Objective: To present an observation that seems to us remarkable by the exceptional association of primary GougerotSjögren's syndrome (PGS) and bone marrow aplasia in an octogenarian woman (80 years old). Observation: This was an octogenarian woman followed in rheumatology since 1993 for a pGSS based on joint involvement made of persistent bilateral asymmetric dry polyarthritis without deformity associated with ocular and oral dryness, accessory salivary gland biopsy (ASGB) at Chisholm and Masson grade 4 by ACR/EULAR 2016 7-point classification criteria, she was on 15mg methotrexate/semaine, 400mg hydroxychloroquine/day for 23years. She presented at entry with fever (T° 39.5°C), the pallor of integuments and conjunctivae, ecchymosis versus hepatomegaly, splenomegaly and bone pain were absent. Biology finds: Pancytopenia made of aregenerative anemia (Hemoglobin at 7.2g/dl, reticulocyte rate at 5934/mm3), leukopenia at 270/mm3, neutropenia at 70/mm3, lymphopenia at 150/mm3 and Thrombopenia at 9000/mm3. The medullogram concluded that the patient was suffering from bone marrow aplasia with poor cellularity, even deserted, but the renal, hepatic, cardiac and infectious investigations were normal. Thus, bone marrow aplasia in a patient followed for primary GSS under methotrexate for 23 years was retained. The treatment was hospitalization, transfusion of 1200ml of platelet concentrate, tramadol 50mg x 3/d in mini-infusion, Fleming (amoxicillin + clavulanic acid) 1g x2/d and Rovamycin 3 million x 2/d, unfortunately, the continuation was marked by her death at D4 of hospitalization. Conclusion: Medullar aplasia and pGSS are an exceptional association in elderly subjects requiring early diagnosis and management to improve the vital prognosis.

PRIMARY SJÖGREN SYNDROME AND PULMONARY INVOLVEMENT: UTILITY OF SALIVARY GLAND BIOPSY IN DIAGNOSIS

PRIMARY SJÖGREN SYNDROME AND PULMONARY INVOLVEMENT: UTILITY OF SALIVARY GLAND BIOPSY IN DIAGNOSIS

USE OF INHALED CORTICOSTERIODS/LONG-ACTING BETA AGONIST (ICS-LABA) IN PATIENTS WITH BRONCHIOLITIS CAUSED BY SJÖGREN SYNDROME DECREASES NEED FOR ORAL STEROIDS

USE OF INHALED CORTICOSTERIODS/LONG-ACTING BETA AGONIST (ICS-LABA) IN PATIENTS WITH BRONCHIOLITIS CAUSED BY SJÖGREN SYNDROME DECREASES NEED FOR ORAL STEROIDS

POS0790 18F-FLUORODEOXYGLUCOSE (FDG) PET-CT IMAGING OF SALIVARY GLANDS IN PRIMARY SJÖGREN’S SYNDROME AND ITS CORRELATION WITH ULTRASONOGRAPHIC SCORES AND SALIVARY FLOW RATE COMPARED TO HEALTHY CONTROLS

BackgroundThe use of salivary gland imaging modalities in patients with primary Sjögren’s syndrome (pSS) has been increasing recently. The contribution of each imaging method in terms of diagnosis or disease activity differs from each other. Although ultrasound and MRI are the most commonly used imaging modalities, the role of PET-CT for diagnosing pSS and determining glandular and extraglandular involvement has largely been neglected.ObjectivesThis study aimed to compare the sizes and metabolic activities of the major salivary glands in patients with pSS and healthy controls (HC). Correlation of the 18F-FDG PET-CT uptake characteristics with ultrasound scores and salivary flow rates of the patients and HC was also determined.Methods22 patients with pSS and 10 age/sex-matched HC were included in the study. The sizes and FDG uptakes of the parotid and submandibular glands of pSS patients and HC were assessed by PET-CT. The maximum standardized uptake value (SUVmax) was evaluated for FDG uptakes, and each patient’s liver uptake and salivary gland uptake ratio were calculated. In addition, correlations of gland sizes and FDG uptakes in PET-CT with OMERACT and Hocevar ultrasound scores, stimulated and unstimulated SFR, ESSPRI dryness scores and disease durations of pSS patients were calculated by Spearman test.ResultsThe mean age (SD) of the patients was 58.6 years (10.5) versus 58.6 years (19.1) of HC; the mean (SD) disease duration was 8.96 (8.77) years. ANA was positive in all patients, anti-SSA positivity was present in 82.6%, and 30.4% of patients experienced ≥1 parotid swelling episode.Compared to HCs, the mean size of both submandibular glands (p=0.006 for left and p=0.032 for right) and SUVmax of the left submandibular gland (p=0.044) were significantly smaller in patients with pSS. In pSS patients, both right and left parotid sizes were smaller and SUVmax uptake was greater than in HC; these differences however did not reach statistical significance.When the PET-CT involvement characteristics of the patients were compared with the salivary gland ultrasonography scores, there was a statistically significant negative correlation between the left parotid gland size in PET-CT and the ultrasonographic inhomogeneity of Hocevar score and OMERACT score. There was a statistically significant negative correlation between right parotid gland size measured on PET-CT with ultrasonographic inhomogeneity, hyperechoic foci, parenchymal echogenicity, Hocevar total score, and OMERACT score. No statistically significant correlation was found between SUVmax scores detected by PET-CT and ultrasound scores in both parotid glands and submandibular glands.A statistically significant positive correlation was found between the total gland size measured in PET-CT and the unstimulated salivary flow rate (p=0.038, r=0.604). There was a negative correlation between total gland size and ESSPRI dryness scores and symptom duration, which did not reach statistical significance.ConclusionPET-CT SUVmax measurements do not provide sufficient information for pSS-related involvement of the major salivary glands. Secondly, size measurement of the parotid glands by PET-CT is associated with OMERACT ultrasound scores, and also the sizes of both submandibular and parotid glands are smaller than HC.Table 1.Correlations of gland sizes and ultrasonographic scoresGlandOMERACT ScoreParenchymalHomogeneityHypoechoic AreasHiperechoicVisibility of Gland BorderParenchymal InhomogenicityTotalSize (PET/CT)EchogenityFociR-Parotisr-.699*-.717*-.704*-0,598-.656*-0,368-.758**-.645*p0,0170,0130,0160,0520,0280,2660,0070,032L-Parotisr-.699*-.717*-.704*-0,598-.656*-0,368-.758**-0,482p0,0170,0130,0160,0520,0280,2660,0070,134R-Submr-0,0110.118-0,011-0,2700,203-0,0060,2470.024p0,9720.7140,9720,3970,5260,9860,4390.942L-Submr0,2450,3060,2450,0710,3270,1180,3060,435p0,4430,3330,4430,8260,3000,7140,3330,158Disclosure of InterestsNone declared

Indications and risk factors for hospitalization in patients with primary Sjögren syndrome: experience from a tertiary center in Turkey.

ObjectiveIn this study, it was aimed to reveal the hospitalization reasons for patients diagnosed with primary Sjögren syndrome (pSS) and potentially associated factors in a tertiary health center.MethodOne hundred and sixty-three pSS patients who regularly attended their follow-ups between January 2010 and May 2021 were included in the study. These patients’ reasons for hospitalization, duration of hospitalization, and numbers of presenting to the hospital were recorded. The demographic, clinical and serological characteristics of the hospitalized and non-hospitalized patients were compared.ResultsHospitalization occurred in 22.7% of the patients, and the total number of hospitalizations was 79. The hospitalization incidence density rate was 6.21 per 100 patient-years. The most frequently encountered reason for hospitalizations was pSS-related organ involvement (44.3%). Infections (17.7%), malignancy (16.5%), endocrine, and various other reasons were the other indications for hospitalization. While male sex (p = 0.005), the presence of extra-glandular involvement (p < 0.001), and interstitial lung disease (p = 0.001) were more common in the hospitalized patients, anti-nuclear antibody positivity was less frequent (p = 0.032). The usage rate of hydroxychloroquine (p = 0.022) was lower in the hospitalized patients, whereas the use of glucocorticoids (p < 0.001) and azathioprine (p = 0.005) was more frequent. The multivariable analyses revealed a relationship between extra-glandular involvement (OR: 4.57 [1.05–19.84], p = 0.043), glucocorticoid use (OR: 3.23 [1.13–9.21], p = 0.028) and hospitalization.ConclusionpSS-related system involvement and infection accounted for the majority of hospitalizations of the pSS patients. The presence of extra-glandular involvement and glucocorticoid use were found to be associated with hospitalization.Key Points • pSS-related system involvement and infection accounted for the majority of hospitalizations of pSS patients.• The presence of extra-glandular involvement was found to be associated with hospitalization.

Discordant Predictions of Extraglandular Involvement in Primary Sjögren's Syndrome According to the Anti-SSA/Ro60 Antibodies Detection Assay in a Cohort Study.

Electrophoresis-derived techniques for anti-SSA/Ro60 KDa (anti-SSA) antibodies detection have been progressively replaced by methods using non-native antigens. We aimed to compare the patients’ phenotypes and the occurrence of extraglandular manifestations in primary Sjögren’s syndrome according to the method used to detect anti-SSA antibodies. Sera from patients with a diagnosis of pSS according to ACR/EULAR 2016 criteria between 2008 and 2017 were tested for anti-SSA antibodies using methods with non-native antigens (magnetic bead multiplex assay; line immunoassays) and one with native antigens (counterimmunoelectrophoresis (CIE)). The population was split into three groups according to anti-SSA antibodies status: absence (SSA−), presence in any method except for CIE (SSA+CIE−), and presence in CIE (SSA+CIE+). The patients in the SSA+CIE+ group (n = 70, 42.7%) were ten years younger and presented more immunological activity compared with both the SSA− (n = 80, 48.8%) and SSA+CIE− groups (n = 14, 8.5%). The SSA− and SSA+CIE− groups were poorly distinct. The presence of anti-SSA antibodies solely in CIE was significantly associated with the occurrence of extraglandular manifestations of pSS (HR = 4.45 (2.35–8.42)). Contrary to CIE, methods using non-native antigens to detect anti-SSA antibodies were unable to predict the occurrence of systemic expression of pSS.