Involvement Of Major Salivary Glands Research Articles

Accuracy of two-dimensional transverse wave elastography of salivary glands in diagnosis of Sjögren’s disease: a systematic review and meta-analysis

ObjectivesTwo-dimensional shear wave elastography (2D-SWE) is a non-invasive technique for the evaluation of Sjögren’s disease (SjD). This study investigated the diagnostic accuracy of 2D-SWE in assessing major salivary gland involvement...

AB1542 APPLICATION OF THE OMERACT ULTRASOUND SCORING SYSTEM FOR SALIVARY GLANDS IN PATIENTS WITH SUSPECTED SJÖGREN’S SYNDROME

BackgroundPrimary Sjogren’s Syndrome (pSS) is a connective tissue disease characterized by the presence of sicca syndrome. Confirmation of pSS requires immunological test and biopsy of the accessory salivary glands[1].Salivary gland ultrasonography (SGUS) allows the detection of structural changes of salivary glands and is proposed as a reliable tool to assess involvement of major salivary glands in patients with pSS. Recently, a new semi- quantitative score have been validated by experts: The OMERACT US score to assess salivary gland involvement in pSS [2].ObjectivesThe aim of our study was to describe salivary gland involvement in patient with suspected of pSS according to the OMERACT ultrasound scoring system for pSS.MethodsAll patients referred for a suspicion of pSS during the last period june 2022-2023 were enrolled. All underwent measurement of salivary flow, minor salivary gland biopsy, SSA antibody. SGUS was performed for all included patients. Both parotid glands and submandibular glands were exanimated using the scoring system 0-3 (OMERACT). Exact Fischer test was used to compare qualitative variables. Sensitivity, specificity and cutoffs were determined by ROC curve analysis.ResultsWe enrolled 73 patients with a suspected pSS. Women represented 80 % of the cohort. Median age was 59 years [Q1 52-Q3 65]. Thirty-four patients were diagnosed with primary SS (pSS) and all fulfilled the 2016 American College of Rheumatology (ACR)/EULAR classification criteria. Thirty-one (91%) patients with pSS had a focus score > 1 in the biopsy of minor salivary gland. Twenty-one (62%) of patients with pSS had a positive SSA antibody.Of the 73 patients, 26 (35%) patients had at least one gland with an ultrasound score of 2 among them, 21 patients were diagnosed with pSS. More patients with pSS compared with non-pSS had score ≥2 in at least one gland (21 pSS vs 5 non pSS) in at least one gland. Among the 26 patients with an ultrasonographic score ≥ 2, 14 patients had positive SSA antibody (p= 0.001). When using ROC analysis, we found that an ultrasound score cutoff ≥2 in at least one gland was associated with a sensitivity at 67 % and specificity at 88%, air under the curve (AUC) was 0.83 [IC95% 0.732-0.93].ConclusionMore patients with pSS compared with non-pSS had score ≥2 in at least one gland (21 pSS vs 5 non pSS) in at least one gland. An ultrasound cutoff > 1 is associated with a sensitivity at 67 % and Specificity at 88%. Our data supports what have been previously reported in several studies. Fana et al [3] reported in a cohort of 143 patients with suspected pSS that the best ultrasound cutoff value was ≥ 1 gland with a score ≥2 was associated to sensitivity at 72 % and specificity of 91%.Our data supports the importance of use of ultrasound as tool for diagnosing pSS. SGUS should be integrated as a criterion in ACR/EULAR score.

Can salivary gland ultrasonography replace salivary gland biopsy in the diagnosis of Sjögren's syndrome?

Ultrasound is a promising diagnostic method when it comes to assessing the involvement of major salivary glands in patients with primary Sjögren's syndrome (pSS). A matter of debate is whether ultrasound of the major salivary glands (SGUS) can replace a salivary gland biopsy in the diagnosis or classification of pSS. The intra- and inter-observer reliability of SGUS was found to be good, especially when focusing on hypoechogenic areas and homogeneity, and comparable to the reliability of histopathologic characteristics of salivary gland biopsies of pSS patients. However, replacing salivary gland biopsy by SGUS led to substantial decrease of the accuracy of the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria with clinical diagnosis as the gold standard. When SGUS was added as an additional item to the criteria, the accuracy of the criteria remained high, offering at the same time the clinicians a wider array of tools to assess patients. Combination of SGUS and anti-SSA antibodies was shown to be highly predictive of the classification of a patient suspected of pSS, making routine salivary gland biopsy debatable.

AN UNUSUAL PRESENTATION OF CLASSIC KAPOSI SARCOMA IN THE PAROTID GLAND – A CASE REPORT AND REVIEW OF THE LITERATURE

<h3>Introduction</h3> Kaposi sarcoma (KS) is an intermediate-type, locally aggressive, and rarely metastasizing vascular neoplasm associated with human herpes virus 8 (HHV-8) infection. There are 4 distinct clinico-epidermiologic subtypes with iatrogenic and AIDS-associated types related to immunosuppression. The classic subtype of KS, seen in immunocompetent men of typically Mediterranean/East European descent, is uncommon. KS is often multifocal and affects the skin, mucous membranes, lymph nodes, and visceral organs in decreasing order of frequency. Major salivary gland involvement is exceptionally rare, with fewer than 10 case reports identified following literature review. We describe an unusual presentation of classic KS arising in the parotid gland of an 83-year-old male patient. <h3>Case Details</h3> The patient presented with a 7-month history of a slow-growing lump in the left cheek. Ultrasound and magnetic resonance imaging revealed a 16-mm lesion within the superficial lobe of the parotid gland suggestive of a primary salivary gland neoplasm. Fine-needle aspiration was non-diagnostic; therefore, core biopsy was performed. This revealed a vascular lesion composed of tightly-packed capillaries and sieve-like spaces lined by endothelial cells, which demonstrated strong HHV-8 positivity by immunohistochemistry. HIV infection was excluded, and following clinical examination, imaging, and hematologic screening, no other soft tissue/cutaneous lesions or evidence of immunodeficiency were identified. Treatment with radiotherapy is planned following MDM discussion. <h3>Conclusions</h3> KS is rarely reported in the parotid gland with most classic types limited to the skin. Classic KS has the best prognosis and is often indolent, with either surgery or radiotherapy recommended for localized disease.

A Retrospective Cohort Study of Major Salivary Gland Tuberculosis: Our 13Year Experience.

The tuberculous infection of major salivary glands is rare because they are relatively resistant to tubercle bacilli. The aim of this study is to present our 13year experience on major salivary gland tuberculosis. The clinical, histopathological, microbiological and imaging records of 9 cases of major salivary gland tuberculosis were reviewed retrospectively. The cases diagnosed with either microbiological culture and/or polymerase chain reaction assay and/or Histopathological examination were included in the study. Descriptive statistics were used to summarize the data. There were 9 cases with tuberculous involvement of major salivary glands; parotid (n = 5), submandibular (n = 3), sublingual (n = 1). The most common local symptom was painless swelling present in 8 cases and constitutional symptoms were present in all the cases. The previous history of TB was present in 5 out of 9 cases. Surgical intervention was done in 2 cases of parotid TB for diagnostic purpose and therapeutic intervention was required in 1 patient. Patients responded well to medical therapy. Two patients with disseminated disease died. The major salivary gland is rare in both immunocompetent and immunocompromised patients. Diagnosis is difficult and requires high degree of suspicion especially in isolated cases. Medical management yields satisfactory results.

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.

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.

Ultrasound elastography in assessment of salivary glands involvement in primary Sjögren's syndrome

ObjectiveOur study aimed to assess the pathological sonoelastographic changes in the major salivary glands and to demonstrate the diagnostic effectiveness of Sonoelastography as an additional method to US in Sjögren's syndrome. MethodsFifty eight patients with primary Sjögren's syndrome (pSS) were selected according to the American-European Consensus Group Classification criteria. Twenty five healthy volunteers involved in this study. All patients were evaluated with B-mode and elastography by using Hitachi EUB 7500 digital ultrasound equipment. All subjects were female. The sonoelastography examination, which allowed us to assess the elasticity of the parenchyma, was performed and strain ratios were measured by comparing with the adjacent tissues. ResultsStatistically significant differences were found between the pSS and control groups for the elastographic scores and strain ratios (p < 0.001). The highest sensitivity and specificity levels were obtained when the strain ratio cut-off value was taken as 1.55 for the submandibular gland and 2.45 for the parotid gland (sensitivity and specificity were 83% and 88% respectively for the submandibular gland and 83% and 92% respectively for the parotid gland). However, no statistically significant differences were found between the disease duration and the elasticity scores or strain ratios in pSS group (p > 0.05). ConclusionUS examination is an efficient method to assess major salivary gland involvement in the diagnosis of pSS. Sonoelastography is a modality which can contribute to the diagnosis by improving specificity in the differential diagnosis of pSS. Strain ratio measurement, which is a semi-quantitative method, increases the diagnostic effectiveness by providing high sensitivity, specificity, and negative predictive values.

How to Distinguish Patients with pSS among Individuals with Dryness without Invasive Diagnostic Studies.

In the course of pSS, inflammatory cell infiltration consists mainly of lymphocytes infiltrating exocrine glands, which leads to their impaired function. The characteristic feature is generalized dryness. The aim of this study was to attempt to answer the question whether it is possible to distinguish between patients with pSS and individuals with dryness caused by other pathologies without applying invasive studies. The study included 68 patients with pSS and 43 healthy controls with dryness. FS ≥ 1 was observed in 90% of patients with pSS (with or without dryness), and only in 23% of the control group (only with xerostomia). In the pSS group, anaemia (p = 0.0085), lymphocytopenia (p = 0.0006), elevated ERS (p = 0.001), higher RF titer, and ANA antibodies were noted. Configuration of anti-SSA + SSB + Ro52 antibodies was characteristic for the pSS group. Considering the clinical symptoms, statistically significant differences were noted between pSS patients and the control group in frequency (p = 0.02) and severity (p = 0.042) of fatigue, lymphadenopathy, major salivary gland involvement, and photosensitivity to UV light. In conclusion, invasive methods are pivotal in pSS diagnosis in this salivary gland biopsy. Chronic fatigue syndrome is more common in pSS patients and can be subjective distinguishing factor in the group of people with dryness.

Wegener’s granulomatosis of the major salivary glands

Wegener's granulomatosis is one of the anti-cytoplasmic autoantibodies (ANCA)-associated vasculitides. Although it typically affects the lungs and kidneys, the head and neck are also involved in most cases but the site usually affected is the upper airway. However, there are 35 cases with well-documented clinicopathological data in which Wegener's granulomatosis manifested in the major salivary glands, most commonly the parotid. Twenty-four patients presented with salivary signs and symptoms, in eight of whom there was no other presenting manifestation. These signs and symptoms may mimic infection or neoplasia and laboratory investigations, including ANCA serology and histopathology, may be non-specific; thus, in 21 of the 35 patients (60%) there was a delay in diagnosis. Amongst the 21 were 11 of the 14 (78.6%) patients who presented with unilateral parotid disease and three of the five who died. Three other patients suffered permanent pulmonary, two renal and five facial nerve damage. This article reviews the literature on major salivary gland involvement in Wegener's granulomatosis, which should be considered in the differential diagnosis of major salivary gland disease particularly if commoner causes have been excluded. A detailed medical history, and persistently inconclusive laboratory tests, could provide the clues that enable prompt diagnosis.

Ultrasonography of major salivary glands could be an alternative tool to sialoscintigraphy in the American-European classification criteria for primary Sjogren's syndrome

To test the diagnostic accuracy of modified American-European classification criteria (AEC) for primary SS (pSS) by replacing sialoscintigraphy (sSC) with ultrasonography of the major salivary glands. One hundred and ninety subjects were evaluated for the diagnosis of pSS, including US of the salivary glands. We tested the diagnostic accuracy of the three different sets of five diagnostic criteria for pSS. Each set combined these four criteria (ocular symptoms, oral symptoms, Schirmer-I test and auto-SS-A antibody) and one of the following: US (US set), sSC (sSC set) or biopsy (Biopsy set). The area under the receiver operating characteristics curve (AUC-ROC) was used to evaluate the diagnostic accuracy of each set of criteria. Out of 190 subjects examined, 140 subjects fulfilled the AEC for the diagnosis of pSS, whereas 50 subjects were classified as non-pSS subjects. US score was positive in 129 (92%), sSC in 123 (88%) and biopsy in 93 (66%) of 140 pSS patients. Among 140 patients with pSS, 88 (63%) patients fulfilled the criteria of the US set, 85 (61%) patients of the sSC set and 71 (51%) patients of the Biopsy set. None of the subjects from the non-pSS group fulfilled any of the sets of criteria. Diagnostic accuracy of each of the three sets of criteria was high and similar [AUC-ROC (s.e.) for the US set was 0.99 (0.00), followed by the sSC set at 0.98 (0.00) and the Biopsy set at 0.97 (0.00)]. US finding of major salivary gland involvement could replace sSC in AEC for the diagnosis of pSS.

Primary Sjögren syndrome in a 2-year-old patient: role of the dentist in diagnosis and dental management with a 6-year follow-up

BACKGROUND. Primary Sjögren syndrome is a rare autoimmune disease, especially in children, mainly affecting girls (77%), and usually diagnosed around 10 years of age. Diagnosis during childhood is difficult, especially because of the diversity of the clinical presentation and difficulty obtaining reliable history data, accounting for a higher frequency of underdiagnosed cases. Differential conditions should be considered, especially the ones that promote xerostomia, such as diabetes, ectodermal dysplasia, rheumatoid arthritis, scleroderma, systemic lupus erythematosus, sarcoidosis, lymphoma, HIV and HTLV infection. Conditions associated with parotid enlargement should also be excluded, including juvenile recurrent parotitis (JRP), sialadenosis, sarcoidosis, lymphoma, infectious parotitis caused by streptococcal and staphylococcal infections, viral infections (paramyxovirus, Epstein-Barr virus, cytomegalovirus, and parvovirus), and diffuse infiltrative lymphocytosis syndrome (associated with HIV infection), and rare congenital conditions, such as polycystic parotid disease. CASE REPORT. A paediatric female patient was referred to our clinic for dental treatment complaining about dry mouth, oral discomfort, and dysphagia. The patient presented five of the required criteria to establish the diagnosis of pSS, including ocular symptoms, oral symptoms, evidence of keratoconjunctivitis sicca, focal sialadenitis confirmed by minor salivary gland biopsy, and evidence of major salivary gland involvement. Our patient did not have positive SS-A and SS-B autoantibodies. According to the literature, about 29% of individuals with pSS can present seronegativity for SS-A (anti-Ro) antibodies and about 33% can present seronegativity for SS-B (anti-La) antibodies. CONCLUSION. To the best of our knowledge, this is the youngest patient reported in the scientific English literature with pSS. Primary Sjögren syndrome has a wide clinical and immunologic spectrum and may progress with increased morbidity. Clinicians must be aware of the development of pSS in such an early age and exclude all possible differential findings to provide early diagnosis and treatment.

耳下腺腫脹を呈したウェゲナー肉芽腫例

A 65-year-old man admitted for a left parotid tumor and high fever unresponsive to peroral antibiotics was found in gallium scintigraphy to have “hot” spots in the left parotid gland and left maxillary sinus. The ineffectiveness of intravenous antibiotics necessitated open biopsy to histologically diagnose the man’s condition. Surgical parotid gland and maxillary sinus specimens showed necrotizing granulomas with giant cells. Elevated proteinase-3 antineutrophil cytoplasmic antibody (PR3-ANCA) comfirmed a clinical diagnosis of Wegener’s granulomatosis. Treatment with prednisolone and cyclophosphamide led to clinical improvement. Major salivary gland involvement is rarely a presenting symptom in Wegener’s granulomatosis and correctly diagnosing this condition may increase morbidity due to disease progression.

Rosai–Dorfman disease of the parotid and submandibular glands: salivary gland scintigraphy and oral findings in two siblings

Rosai-Dorfman disease (RDD) is an unusual clinical entity characterized by benign pseudolymphomatous proliferation with significant histiocytic infiltration. In the present paper, extranodal RDD of the major salivary glands causing salivary hypofunction and the results of salivary gland scintigraphy and ultrasound are presented in two siblings. Case 1: a 10-year-old boy with bilateral painless masses around the parotid and submandibular glands was referred. Ultrasound examination showed bilateral, well-defined, hypoechoic solid mass lesions within both parotid glands with minimal normal parenchyma in the upper poles. Both submandibular glands were markedly hypoechoic and heterogeneous. Mass lesions within the parotid glands appeared as cold lesions with regular contours on scintigraphy. Dynamic images showed normal uptake and normal response to secretion in the upper poles of the parotid glands, corresponding with ultrasonographically normal parenchyma. Both submandibular glands showed markedly diminished uptake and secretion. Case 2: a 9-year-old boy presented with mass lesions around the submandibular glands. Ultrasound examination showed normal parotid glands and markedly hypoechoic and heterogeneous submandibular glands. Salivary gland scintigraphy showed normal uptake and secretion of parotid glands with markedly diminished uptake and secretion in both submandibular glands. There were severe carious lesions in both patients due to salivary hypofunction. Treatments of the two patients' teeth were performed. Major salivary gland involvement of RDD is important for dentists as it may cause xerostomia and can mimic dental abscess. Functional evaluation of salivary glands with scintigraphy, besides radiological and pathological techniques, will help to explain whether salivary glands are affected or not and improve the diagnostic effectiveness.

Major salivary gland damage in allogeneic hematopoietic progenitor cell transplantation assessed by scintigraphic methods

Salivary gland dysfunction is a common sequela of hematopoietic progenitor cell transplantation (HPCT). The investigation of major salivary gland dysfunction with sodium pertechnetate scintigraphy is a non-invasive method that provides images of the parotid and submandibular glands. In this prospective trial, 20 HPCT patients were submitted to scintigraphic study with 99mTc-pertechenate and 67Ga in order to evaluate the major salivary glands early involvement following HPCT. Major salivary glands were evaluated prior to HCPT as well as at Days +30, +60 and +100 post transplant. Major salivary glands uptake and clearance of 99mTc-pertechenate results did not demonstrate any functional differences between pre- versus post transplant periods. Results of the 67Ga scan revealed inflammatory infiltration following HPCT, primarily in submandibular glands, suggest a persistent involvement of major salivary glands up to Day +100 after HPCT.

Sjögren's syndrome in progressive systemic sclerosis (scleroderma)

Sicca features of Sjögren's syndrome were investigated in 25 consecutive patients with progressive systemic sclerosis by means of clinical examination, Schirmer's tests, rose bengal staining tests, secretory parotid sialographies, scintillation scanning of salivary glands with 99-mTc pertechnetate, radionuclide salivary excretion studies and lip biopsies for study of minor salivary glands.All 25 patients had at least one abnormal test and all but 3 patients had more than two abnormal tests.Pathologic findings in minor salivary glands included both lymphocytic infiltration and duct cell proliferation characteristic of Sjögren's syndrome, as well as collagen infiltration and disruption attributable to scleroderma. However, the finding of lacrimal and major salivary gland involvement indicates that Sjögren's syndrome does occur in the majority of patients with progressive systemic sclerosis. Because Sjögren's syndrome coexists almost exclusively with autoimmune disorders, our findings support the contention that progressive systemic sclerosis is related to autoimmunity.