Salivary Gland Involvement Research Articles

Bilateral Symmetrical Enlargement of the Cranial Nerves Extracranially; A Diagnostic Dilemma

Introduction IgG4-related disease (IgG4-RD) is increasingly being recognised as an entity affecting the head and neck region. Although most commonly seen with salivary gland or paranasal sinus involvement, IgG4-RD may also affect the cranial nerves.

Two-Dimensional Shear Wave Elastography in the Assessment of Salivary Gland Involvement in Primary Sjögren's Syndrome.

The aim of this study was to investigate the diagnostic performance of two-dimensional (2D) shear wave elastography (SWE) in the assessment of salivary gland involvement in primary Sjögren's syndrome (pSS). Fifty-three patients with pSS and 30 healthy volunteers were included. The echogenicity of all submandibular and parotid glands was evaluated with B-mode ultrasound, and their elasticity was assessed with 2D SWE. The mean and standard deviation of the shear wave speed and elasticity modes on 2D SWE were calculated. The mean shear wave speed and elasticity mode values for the submandibular and parotid glands were significantly higher in the patients with pSS (P < .05). The mean elasticity of the shear wave speed mode was best able to differentiate the parotid glands of patients with pSS from those of healthy volunteers at a cutoff value of 2.48 m/s, whereas the mean elasticity of the elasticity mode was best able to differentiate the submandibular glands of patients with pSS from those of healthy volunteers at a cutoff value of 21 kPa. Two-dimensional SWE is an effective technique for assessment of the parenchyma of the salivary glands in patients with pSS and predicts interstitial fibrosis and the severity of histologic damage.

Ultrasound salivary gland involvement in Sjogren's syndrome vs. other connective tissue diseases: is it autoantibody and gland dependent?

This study aims to investigate ultrasound (US) findings on salivary glands (SG) in patients with Sjögren syndrome (SS) vs. other connective tissue diseases (CTDs) and to assess the relationship of SGUS abnormalities with autoantibody profile in both groups. We enrolled 81 patients, 45 diagnosed with SS (39 with primary SS, 6 with secondary SS) and 36 diagnosed with other CTDs. All patients underwent a prospective evaluation of sicca symptoms, a Schirmer's test, and a B-mode US assessment of the parotid and submandibular glands, all blinded to the diagnosis. Each SG was semi-quantitatively scored 0-3; a grade ≥ 2 was considered pathological. SGUS involvement was classified as normal or pathological at the patient level and for each pair at the gland level. In addition, a total SGUS score of 0-12 and a parotid/submandibular score of 0-6 were calculated for each patient. Autoimmunity laboratory data were also obtained. All SGUS scores were higher in SS patients than in those with CTD (p < 0.001) and significantly more SS patients showed a pathological global (p < 0.001), parotid (p < 0.001), or submandibular (p = 0.001) US score compared with CTD patients. In SS patients, the presence of autoantibodies was significantly associated with pathological SGUS and higher scores, particularly at the parotid level, while in CTD patients, xerostomia and a pathological Schirmer's test were associated with pathological US and higher scores at the submandibular level (p < 0.05). SGUS showed a different grade of abnormality, site involvement, and associated autoantibody profile in SS patients as compared with other CTD. KEY POINTS: • Patients with SS and other CTDs showed different grades of SGUS abnormality. • Patients with SS and other CTDs showed different gland involvement and associated autoantibody profiles. • Anti-Ro60 and anti-Ro52 Ro60 positivity were associated with the severity of parotid involvement in SS patients.

Autoantigen-specific B cells and plasma cells are prominent in areas of fatty infiltration in salivary glands of patients with primary Sjögren’s syndrome

Salivary and lacrimal gland involvement is a characteristic feature of primary Sjögren’s syndrome (pSS), where tissue destruction is mediated by mononuclear cell infiltration, resulting in lacrimal and salivary gland impairment. We have previously shown distinct prevalence of adipose tissue replacement in the minor salivary gland tissue from pSS patients. The salivary gland microenvironment was further examined through microarray analysis, identifying signalling pathways that promoted adipose tissue development, inflammation, and lymphoma. As B cells may also contribute to disease progression, we now aimed to study the B cell pattern with regard to adipocyte development in pSS. Double immunohistochemical staining of paraffin-embedded salivary gland tissue from 22 pSS patients and 11 non-SS tissue controls was employed, using the characteristic pSS autoantigens Ro52 or Ro60, alongside CD27. Additional CD138/CD20 double staining was also performed to identify the plasma- and general B- cell pattern. Our results demonstrated CD27-positive Ro52 and Ro60 specific cells observed within and in close proximity to the adipose tissue. CD138-positive plasma cells were also seen in areas of adipose tissue replacement, while the CD20+ cells were located within focal infiltrates, forming distinct B cell zones. The quantification of CD138+ and CD20+ cells revealed elevated numbers of CD138+ cells in areas of fatty infiltration, and also interstitially, in the salivary glands of pSS patients when compared to non-SS controls. A significant increase (p < .01) in CD138+ cells close to areas of fatty infiltration, and interstitially, with increasing fatty infiltration and focus score was further observed in pSS patients. A correlation between the number of CD20+ B cell zones/mm2 of salivary gland tissue and focus score values was also witnessed in the patients (r2 = 0.6047, p < .001). In conclusion, autoantigen-specific B cells and plasma cells appear prominent in areas of fatty infiltration in salivary glands of pSS patients, where an increase in CD138+ plasma cells and CD20+ B cells, in relation to both fatty and focal infiltration, suggests their active involvement in promoting inflammation. Further studies are needed to assess whether these adipocytes are also a result of tissue repair.

1391 Too Much of a Good Thing: HIV and Immune Reconstitution in a Patient With IGG4-Related Pancreatitis

INTRODUCTION: Autoimmune pancreatitis accounts for 2% of cases of chronic pancreatitis. Autoimmune pancreatitis is differentiated into Type I (IgG4-related disease) and Type 2 (with normal IgG4 levels). Nearly 40% of patients with IgG4-related pancreatitis have involvement of salivary or lacrimal glands. CASE DESCRIPTION/METHODS: A 65 year-old-female with a history of HIV on anti-retroviral therapy (ART) (CD4 count of 588 cells/uL and undetectable viral load) and submandibular gland swelling presented with two weeks of epigastric discomfort and watery yellow diarrhea. The patient was noted to have elevated alkaline phosphatase to 802 U/L, aspartate aminotransferase to 284 U/L, alanine aminotransferase to 518, as well as total bilirubin of 2.8 mg/dL and direct bilirubin of 1.8 mg/dL. Amylase was 79 u/L and Lipase was 75 u/L. Four months prior to admission ART was changed from emtricitabine/tenofovir/raltegravir to bictegravir/emtricitabine/tenofovir; at that time CD4 count was 318 cells/uL. Liver tests were normal at that time. ART was held at the time of admission. Abdominal ultrasound demonstrated a diffusely enlarged, hypoechoic pancreas, consistent with pancreatitis. Magnetic resonance cholangiopancreatography showed abnormal morphology of the pancreas with loss of lobulation in the distal pancreatic body and tail, as well as irregularity of the pancreatic duct. Endoscopic ultrasound-guided biopsy with biliary stenting was performed. Prednisone was started empirically at 1 mg/kg/day for suspected IgG4 pancreatitis with rapid improvement in symptoms and liver tests. ART regimen was resumed simultaneously. Biopsy showed fibrosis with chronic inflammation, as well as the presence of IgG4 positive plasma cells. One month later, the patient's liver tests have returned to baseline. DISCUSSION: In this patient with a recent change in ART medications and newly elevated liver enzymes, drug-induced liver injury (DILI) can be considered initially. Imaging in this case showed diffuse edema and pancreatitis. Recent research suggests that patients with IgG4-related disease have over-activity of a specific class of cytotoxic CD4+ T cells driving the cytokine release that leads to its characteristic fibrosis. We suspect that this patient's flare of IgG4 pancreatitis may have been triggered by a recent change in her ART regimen. This allowed proliferation of all CD4+ T cell lines, leading to an response resembling the Immune Reconstitution Inflammatory Syndrome, resulting in the patient's clinical presentation.

Salivary gland involvement disparities in clinical characteristics of IgG4-related disease: a retrospective study of 428 patients.

IgG4-related disease (IgG4-RD) has recently been recognized as a fibro-inflammatory condition featuring tumefactive lesions in multiple organs, and the salivary gland is one of the most commonly involved sites. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-RD patients with salivary gland lesions (IgG4-RD SG+) and salivary-gland-free IgG4-RD (IgG4-RD SG-) in a large cohort. We carried out a retrospective review of the medical records of 428 cases of IgG4-RD diagnosed at Peking University People's Hospital between March 2006 and May 2018. Among 428 patients, 249 had salivary glands that were affected. IgG4-RD SG+ patients showed younger age at disease onset and diagnosis, and a longer interval between symptom onset and diagnosis. The IgG4-RD SG+ group involved more female patients, and allergic diseases were more common in this group. In terms of organ involvement, the IgG4-RD SG+ group were more frequently presented with lacrimal gland involvement, while lymph node, retroperitoneal fibrosis, pancreas, biliary system, kidney and aorta were more prominent in the IgG4-RD SG- group. In addition, the serum IgG4 level, IgG4/IgG ratio and IgE level were significantly higher in IgG4-RD SG+ patients. Patients with eosinophilia were more common in the IgG4-RD SG+ group, while elevated ESR, CRP and positive ANA were more common in the IgG4-RD SG- group. We have revealed demographic, clinical and laboratory differences between IgG4-RD SG+ and SG- patients, which indicated potential differences in pathogenesis and important implications for the diagnosis and management of these two phenotypes.

RElevance of UltraSonography for Assessing Salivary Gland Involvement in Systemic Sclerosis (SSc)

RElevance of UltraSonography for Assessing Salivary Gland Involvement in Systemic Sclerosis (SSc)

Prognostic markers of lymphoma development in primary Sjögren syndrome.

Sjögren syndrome is a systemic autoimmune disease that principally affects women between the fourth and sixth decades of life who present with sicca symptomatology caused by dryness of the main mucosal surfaces. The clinical spectrum of Sjögren syndrome extends from dryness to systemic involvement. Since 1978, Sjögren syndrome has been closely associated with an enhanced risk of lymphoma, one of the most severe complications a patient may develop. Primary Sjögren syndrome patients have a 10-44-fold greater risk of lymphoma than healthy individuals, higher than that reported for systemic lupus erythematosus and rheumatoid arthritis. The close link between lymphoma and Sjögren syndrome is clearly exemplified by the very specific type of lymphoma arising in Sjögren syndrome patients, mainly low-grade B-cell lymphomas (predominantly a marginal zone histological type) with primary extranodal involvement of the major salivary glands (overwhelmingly parotid), with a primordial role of cryoglobulinemic-related markers (both clinical and immunological). The most recent studies support a higher number of risk factors detected in an individual leads to a higher lymphoma risk. A close follow-up of high-risk groups with longitudinal assessments of all known risk factors, including cryoglobulin-related markers and EULAR Sjögren's syndrome disease activity index measurement in particular, is mandatory.

SYNCHRONOUS ORAL SALIVARY GLAND TUMORS: REPORT OF THREE NEW CASES AND REVIEW OF THE LITERATURE

Multiple synchronous or metachronous salivary gland tumors, benign or malignant, are rare yet more likely to occur in the major salivary glands compared to involvement of the minor salivary glands. In this poster we present three new cases of synchronous oral salivary gland tumors in minor salivary glands and review the previously reported cases. All three patients were female. Two of the patients aged 55 and 85, presented with submucosal nodules of the upper lip and left buccal mucosa, respectively. Histopathologically, both cases exhibited two separate encapsulated tumors identified as pleomorphic adenoma and canalicular adenoma presenting as a single nodule in the first case, but as two separated nodules in the second. The third patient was a 46-year old who presented with a grayish-blue, non-ulcerated, and painful nodule on the left soft palate. Histopathologic examination showed a nodule composed of two adjacent, yet separate tumors diagnosed as polymorphous adenocarcinoma demonstrating significant perineural invasion, and low-grade mucoepidermoid carcinoma. Conclusion Intraoral multiple synchronous salivary gland tumors are rare and unusual, with only a few cases reported in the literature. The diagnosis of such tumors would be significant from treatment, management, and prognostic standpoints. Cytogenetic studies might be useful in further clarification of these entities.

Value of Somatostatin Receptor Scintigraphy with 99mTc-HYNIC-TOC in Patients with Primary Sjögren Syndrome.

Objectives: Primary Sjögren syndrome (SS) is diagnosed based on the American European Consensus Group (AECG) criteria, but lacks specificity, not only in the involvement of salivary glands, but also in extra-glandular involvement. Whole-body somatostatin receptor scintigraphy with 99mTc-HYNIC-TOC scintigraphy could overcome these limitations. The aims of this study were to evaluate salivary gland uptake of 99mTc-HYNIC-TOC in untreated patients with de-novo diagnosis of SS as compared to control subjects and as compared to conventional sialoscintigraphy with 99mTcO4−. We also aimed to evaluate the involvement of joints. Methods: 99mTc-HYNIC-TOC was used with SS patients and uptake in joints and salivary glands was analyzed semi-quantitatively. Patients also underwent 99mTcO4 sialoscintigraphy. The control group that we analyzed consisted of 30 patients with neuroendocrine tumors. Results: Fifty-two females and 10 males fully met the AECG criteria for SS, and were included. A target background ratio (TBR) >1.18 in submandibular glands correctly classified 93% of the patients with SS in comparison to 27% for 99mTcO4 sialoscintigraphy. The area under the curve (ROC) analysis for TBR in submandibular glands was 0.95. In joints there was a huge variety in uptake. The median TBR was significantly higher in salivary glands in patients with SS compared to controls. Conclusions: 99mTc-HYNIC-TOC scintigraphy identified active inflammatory processes not only in the salivary glands, but, unexpectedly, also in many joints in patients with primary SS, contrary to popular belief. This technique provides an objective parameter to evaluate the inflammation burden in salivary glands and joints and could be used to evaluate response to treatment.

World Workshop on Oral Medicine VII: Immunobiologics for salivary gland disease in Sjögren's syndrome: A systematic review.

This systematic review evaluated the efficacy of immunobiologics for the management of oral disease in Sjögren's syndrome (SS). MEDLINE® , Embase, Scopus, and the Cochrane Library were searched for evidence on the use of immunobiologics for management of glandular disease in SS. Primary outcomes were xerostomia and salivary gland dysfunction, assessed via visual analogue scales, disease-specific scales for SS, measurement of salivary flow, ultrasound data, and quality of life measures. Seventeen studies (11 randomized controlled trials and 6 observational studies) met inclusion criteria. Rituximab showed efficacy in improving salivary gland function but not xerostomia. Abatacept showed promise in improving both xerostomia and salivary flow. Belimumab exhibited long-term improvement of salivary flow and subjective measures. The novel agent CFZ533 improved both disease activity and patient-reported indexes. There is strong evidence pointing to the efficacy of rituximab in the management of oral disease in SS. Future controlled trials may elucidate the efficacy of belimumab and abatacept. The new drug CFZ533 is a promising alternative for the management of SS and its salivary gland involvement. In considering these agents, the promise of efficacy must be balanced against the harmful effects associated with biologic agents.

Sjögren's syndrome with juvenile onset

The study included all consequently patients, who hospitalized to the pediatric department of V.A. Nasonova Scientific and Research Institute of Rheumatology from January 2013 to December 2018, which verified the diagnosis of the SS. The diagnosis of SS was established in 30 patients, among whom there were only 5 (16.7%) boys, the ratio of boys and girls was 1:5. According to the results of the examination, the following diagnoses were verified: 4 - primary SS, 9 - systemic lupus erythematosus with SS, 10 - juvenile rheumatoid arthritis with SS, 3 - mixed connective tissue disease, 3 - overlap syndrome, 1 - systemic sclerosis with SS. The median age of rheumatic disease onset was 10.4 (7.0; 13.75) years. The median of disease duration at the time of SS verification - 3.0 (0.85; 4.4) years. Recurrent parotitis were observed in 8 patients. 24 pts had isolated involvement of salivary glands, 6 - combined with lacrimal glands. Sicca syndrome was occurred in 8 patients. All patients had systemic manifestations: constitutional abnormalities - 50%, polyarthritis - 83.3%, lymphadenopathy - 73.3%, cutaneous involvement - 60%, pulmonary involvement - 23.3%. Of the hematological disorders, leuko/lymphopenia was more often recorded - in 30%, polyclonal hypergammaglobulinemia - in 26.7% of patients. ANA were detected in all cases, anti-Ro antibodies - 60%, a positive rheumatoid factor - 56.7% of patients. The most common combination of immunological disorders was the presence of ANA, RF and anti-Ro antibodies (40% of patients). The treatment for each patient was justified by the main manifestations and activity of rheumatic disease: 66.7% received nonsteroidal anti - inflammatory drugs, 80% - glucocorticoids, 46.7% - methotrexate, 20% - azathioprine, 43.3% - hydroxychloroquine, 10% - mycophenolatis mofetilum, 3.4% - cyclophosphamide. 66.7% of patients received the treatment of Biologics. Early diagnosis of SS in children with rheumatic diseases significantly affects to the choice of treatment and prognosis. In children the SS has no clinical manifestations for a long time. Such symptoms as a hypergammaglobulinemia, positive RF without persistant arthritis, nonspecific skin lesions, recurrent parotid swelling may help to diagnosis of SS.

Usefulness of 18 F-Labeled Fluorodeoxyglucose-Positron Emission Tomography for the Diagnosis of Lymphoma in Primary Sjögren's Syndrome.

The usefulness of positron emission tomography-computed tomography (PET-CT) with 18 F-labeled fluorodeoxyglucose (18 F-FDG) for the diagnosis of lymphoma in patients with primary Sjögren's syndrome (SS) is unclear, since the abnormalities it reveals may be due to systemic manifestations of SS. This study was undertaken to compare 18 F-FDG-PET-CT in patients with primary SS with lymphoma and those without lymphoma in order to identify patterns associated with lymphoma. A retrospective study was conducted in 2 centers and included patients who met the American College of Rheumatology/European League Against Rheumatism 2016 criteria for primary SS and had undergone PET-CT. Two independent readers who were blinded with regard to lymphoma diagnosis analyzed PET-CT scans. Abnormalities were compared between patients with and those without lymphoma. Of the 45 patients included, 15 had lymphoma. Compared to patients without lymphoma, the mean size (P = 0.048) and maximum standardized uptake value (SUVmax) (P = 0.001) of the parotid glands were higher in patients with lymphoma. FDG uptake in the lymph nodes was observed in 53.3% of the patients with lymphoma and 43.3% of the patients without lymphoma, with no difference in the number of sites, uptake pattern, or mean SUVmax. Focal pulmonary uptake (nodules or condensations) was observed in 5 of the patients with lymphoma (33.3%) but only 1 patient without lymphoma (3.3%) (P = 0.01). Having an SUVmax in the parotid gland of ≥4.7 and/or the presence of focal pulmonary lesions was highly suggestive of lymphoma (sensitivity 80%, specificity 83.3%). Some systemic manifestations of primary SS (lymphadenopathy, pulmonary involvement, and salivary gland involvement) can be visualized by PET-CT. Involvement of the lymph nodes and parotid glands is commonly observed with a similar frequency in SS patients with and those without lymphoma. An SUVmax in the parotid glands of ≥4.7 and/or the presence of focal lung lesions are associated with the diagnosis of lymphoma.

Primary hematologic malignancies with the onset of involvement of the major salivary glands in rheumatologic practice

Objective : to identify the main types of lymphoma with the onset of involvement of the major salivary glands (MSGs), which are encountered in rheumatologic practice, and to evaluate the efficiency of minimally invasive incisional biopsies of the MSGs/lacrimal glands in the diagnosis of different diseases affecting the parotid, submandibular, sublingual, and lacrimal glands. Patients and methods . A total of339patients (291 women and 48 men) aged 18 to 78 years (median age, 55 years) with MSG involvement were examined at the V.A. Nasonova Research Institute of Rheumatology in 2004 to 2017. Minimally invasive incisional biopsies of the parotid, submandibular salivary (SMSG), and lacrimal glands were carried out to verify the diagnosis. Results and discussion . Different variants of hematological malignancies with MSG involvement were diagnosed in 187 (55%) patients; nonneoplastic diseases were in 152 (45%) patients. Primary tumors with the onset of MSG involvement were detected in 52 (15.3%) patients. Hematologic malignancies were diagnosed in 32 (61.5%) cases and 20 (38.5%) patients had various benign neoplastic or infectious diseases with MSG involvement. The prevailing hematologic malignances with MSG involvement were non-Hodgkin lymphomas (NHL), indolent MALT-lymphomas in 12 (23%) patients, follicular lymphomas in 6 (11.5%), and AL-amyloidosis affecting the SMSG in 9 (17.3%). The diagnosis was morphologically verified using minimally invasive incisional biopsy of the salivary/lacrimal glands; in all cases, the biopsy was informative for full morphopathological examination and diagnosis verification. Conclusion . Minimally invasive incisional biopsy of the MSG/lacrimal glands should be actively used in dentistry, rheumatology and blood oncology hospitals for verification of nosological diagnoses when the MSG and lacrimal glands are involved. A sufficient number of tissues obtained using these techniques allows one to abandon severe surgical interventions (partial and complete parotidectomy, SMSG removal, and orbitotomy) currently used for diagnostic purposes in various types of hospitals.

Major salivary gland enlargement in IgG4-related disease is associated with multiorgan involvement and higher basal disease activity

Objective: To evaluate if major salivary gland enlargement in patients with IgG4-related disease (IgG4-RD) is associated with systemic involvement.Methods: We included 47 patients with diagnosis of IgG4-RD. We retrospectively collected demographics, organ involvement, disease activity and damage assessed by the IgG4-RD Responder Index (IgG4-RD RI) and treatment.Results: 25 patients (53%) were men, mean age 50.8 years and median disease duration 27 months. Most frequently anatomic sites affected were lymph nodes 55%, pancreas 51% and lacrimal glands 43%. We observed major salivary gland involvement in 22 (46.8%) patients. When we compared patients with (n = 22) vs. without (n = 25) salivary gland enlargement, the first group had a higher number of affected organs, a higher prevalence of lacrimal glands, lymph nodes, and lung involvement, rheumatoid factor positivity, azathioprine and prednisone use, as well as a higher baseline IgG4-RD RI and a longer delay in diagnosis. At logistic regression analysis we found an association of major salivary gland enlargement with the basal IgG4-RD RI (OR 1.37, 95%CI 1.09–1.61, p = .001) and with lacrimal gland involvement (OR 34.7, 95%CI 4.6–258, p = .001).Conclusion: Our study highlights the systemic nature of IgG4-RD. Patients with major salivary gland enlargement should be routinely screened for multi-organ disease.

Adverse reactions of systemic drugs in the oral cavity

Adverse reactions of systemic drugs in the oral cavity

Study of Reduced Salivary Secretion and Increased Caries Susceptibility in Rheumatoid Arthritis

It is a chronic inflammatory musculoskeletal disease with extra-articular manifestations including involvement of exocrine lacrimal and salivary glands. The aim of this study was to assess the reduced salivary secretion and calculate and compare DMF index in rheumatoid arthritis patients. The salivary output was determined in unstimulated (resting) whole saliva and caries status in all individuals was determined by using the DMF index in 20 rheumatoid arthritis patients and 20 healthy control subjects. Compared with healthy control groups, rheumatoid arthritis patients had significantly decreased output of saliva and higher DMF index than in the control group. The results indicate that xerostomia in patients with rheumatoid arthritis may be indicative of increased disease activity and impacts negatively on oral health leading to increased caries susceptibility and should receive increased stomatological attention.

Diagnostic accuracy of salivary gland ultrasonography with different scoring systems in Sj\xf6gren\u2019s syndrome: a systematic review and meta-analysis

Noninvasive objective salivary gland ultrasonography (SGU) had been widely used to evaluate major salivary gland involvement in primary Sjögren’s syndrome (pSS) and treatment responses. However, the evaluation score, diagnostic sensitivity, and diagnostic specificity significantly varied among clinical studies. We conducted this meta-analysis to assess the diagnostic accuracy of different SGU scoring systems using the American-European Consensus Group criteria. Of the 1301 articles retrieved from six databases, 24 met the criteria for quality assessment and 14 for meta-analyses. The pooled sensitivities were 75% (0–4) with I2 = 92.0%, 84% (0–16) with I2 = 63.6%, and 75% (0–48) with I2 = 90.9%; the pooled specificities were 93% (0–4) with I2 = 71.5%, 88% (0–16) with I2 = 65.4%, and 95% (0–48) with I2 = 83.9%; the pooled diagnostic odds ratios were 71.26 (0–4) with I2 = 0%, 46.3 (0–16) with I2 = 73.8%, and 66.07 (0–48) I2 = 0%; the areas under the SROC curves were 0.95 (0–4), 0.93 (0–16), and 0.94 (0–48). These results indicated that the 0–4 scoring system has a higher specificity and a less heterogeneity than other systems, and could be used as a universal SGU diagnostic standard.

Histopathology of salivary glands.

Salivary gland (SG) biopsy is a technique broadly applied for the diagnosis of primary Sjögren's syndrome (pSS), lymphoma accompanying SS, sarcoidosis, amyloidosis, and IgG4-related disease The most peculiar feature of pSS on biopsy is focal lymphocytic sialadenitis. In the past, several histological scores have been reported in the literature to describe glandular involvement during pSS. However, the variability among centres in reporting glandular scores is one of the rationales behind the development of standardised consensus guidance. SGs as well as lacrimal glands are involved in up to 50% of patients with IgG4-related disease with 3 histopathological hallmarks such as dense lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. SGs can be also affected by amyloidosis with MSG biopsy being more sensitive than that of rectal mucosa or subcutaneous fat. SG involvement is a rare manifestation during sarcoidosis, and the presence of non-caseating granulomas needs to be differentiated from granulomas of other etiology. This review article provides an overview of normal and pathological SGs in the context of rheumatic diseases, identifying key elements in the tissue as diagnostic and prognostic biomarkers, useful in the current clinical practice.

IgG4-Related Disease Mimicking Extranodal Lymphoma of the Hard Palatal Mucosa: A Case Report and Review of the Literature

Background: Immunoglobulin G4 (IgG4)–related disease is a rare fibroinflammatory disease first recognized as a systemic condition in 2003. The disorder is characterized by multiple tumor-like swellings, a lymphoplasmacytic infiltrate with numerous with IgG4-positive plasma cells, and fibrosis. Organs commonly involved include the pancreas, lungs, hepatobiliary tract, lymph nodes, and major salivary glands; involvement of the minor salivary glands of the mucosa of the hard palate has rarely been reported. We describe here a patient with IgG4-related disease presenting with palatal swelling mimicking extranodal non-Hodgkin lymphoma.