Positive Labial Salivary Gland Biopsy Research Articles

Aksiyel Spondiloartrit, Sistemik Lupus Eritematozus, Sjögren Sendromu ve Sekonder Antifosfolipid Sendromunun Birlikte Görüldüğü Olgu Sunumu

Hereby we report a 55-year-old female patient having the association of non-radiographic axial spondyloarthritis (nr-AxSpA), systemic lupus erythematosus (SLE), secondary antiphospholipid antibody syndrome (APS), and Sjögren's Syndrome (SjS). Diagnosis of nr-AxSpA was made based upon the presence inflammatory low-back pain, human leukocyte antigen B27 positivity, and presence of sacroiliitis only in magnetic resonance imaging (MRI). SLE was diagnosed with butterfly-shaped rash on her cheeks, inflammatory arthritis, photosensitivity, erythema involving dorsal inter-joint area of hand fingers, alopecia together with antinuclear antibody (ANA) and anti-dsDNA positivity, low serum complement levels, leucopenia and thrombocytopenia. Additional presence of sicca symptoms, low Schirmer I test, anti SSA/Ro and anti-SSB/La positivity, supported by positive labial salivary gland biopsy led to the diagnosis of SjS. Furthermore, this patient also had miscarriage at 16th week and cerebral vascular disease at 33 years. Besides, IgG and IgM anticardiolipin antibodies were found to be positive twice. Therefore, she was also diagnosed as secondary APS. She fulfilled the relevant classification criteria for AxSpA, SLE, SjS and APS. To our knowledge, this is the first case report showing the association of these four diseases, with different genetic, etiopathogenetic and clinical systemic inflammatory diseases.

Ultrasonography predicts the results of labial salivary gland biopsy in patients with suspected Sjögren's syndrome: a matrix risk model.

Objective:Although a positive result of labial salivary gland biopsy (LSGB) is critical for the diagnosis of Sjögren’s syndrome, rheumatologists prefer assessing the non-invasive objective items and hope to learn the predicted probability of positive LSGB before referring patients with suspected Sjögren’s syndrome to receive biopsy. This study aimed to explore the predictive value of combined B-mode ultrasonography (US) and shear-wave elastography (SWE) examination on LSGB results.Methods:A derivation cohort and later a validation cohort of patients with suspected Sjögren’s syndrome were recruited. All participants received clinical assessments, B-mode US and SWE examination on bilateral parotid and submandibular glands before LSGB. Positive LSGB was defined by a focus score ⩾1 per 4 mm2 of glandular tissue.Results:In the derivation cohort of 91 participants, either the total US scores or the total SWE values of four glands significantly distinguished patients with positive LSGB from those with negative results (area under the curve (AUC) = 0.956, 0.825, both p < 0.001). The positive predictive value (PPV) was 100% in patients with total US scores ⩾9 or with total SWE values ⩾33 kPa. The negative predictive value (NPV) was 100% in patients with total US scores <5, but 68% in patients with total SWE values <27 kPa. A matrix risk model was derived based on the combination of total US scores and total SWE values. Patients can be stratified into high, moderate, and low risk of positive LSGB. In the validation cohort of 52 participants, the PPV was 94% in the high-risk subpopulation and the NPV was 93% in the low-risk subpopulation.Conclusion:A novel matrix risk model based on the combined B-mode US and SWE examination can help rheumatologists to make a shared decision with suspected Sjögren’s syndrome patients on whether the invasive procedure of LSGB should be performed.

Primary Sjogren Syndrome: Clinical Features and Management

Primary Sjögren syndrome (SS) is a chronic inflammatory disease affecting the lacrimal and salivary glands, resulting in dry eyes and dry mouth. It may also lead to systemic manifestations, including fatigue, arthritis, lung involvement, kidney disease, neuropathy, and vasculitis, and predisposes to B cell lymphoma. The diagnosis of primary SS is based on a composite of clinical, serologic, and pathologic features, namely, objective evidence of dry eyes and dry mouth and either a positive test for anti-SSA (Ro) antibodies or a positive labial salivary gland biopsy. Primary SS may be confused with other conditions affecting the lacrimal and salivary glands, such as chronic hepatitis C virus infection, sarcoidosis, and a new entity, IgG4-related disease. The management of patients with primary SS is largely symptomatic and consists of tear supplementation, regular dental hygiene, and liberal use of moisturizers. Cyclosporine 0.05% ophthalmic emulsion and lifitegrast 5% ophthalmic solution are approved agents for the treatment of dry eyes. Two oral secretagogues, pilocarpine and cevimeline, have been shown in trials to significantly reduce the symptoms of dry eyes and dry mouth. Although hydroxychloroquine may reduce fatigue and joint pain, the results of a randomized controlled trial failed to confirm its efficacy in this clinical setting. More serious organ system involvement may be managed with corticosteroids and other immunomodulatory drugs. Although no disease-modifying drugs are currently approved for the treatment of primary SS, a robust pipeline of promising therapies for this disease is currently in development. This review contains 3 figures, 5 tables, and 69 references. Key words: anti-Ro/SSA antibodies, B cell lymphoma, classification criteria, EULAR Sjögren’s syndrome Patient Reported Index (ESSPRI), EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI), hydroxychloroquine, keratoconjunctivitis sicca, rituximab, Sjögren syndrome

Association of Dry Eye Tests With Extraocular Signs Among 3514 Participants in the Sjögren's Syndrome International Registry

To identify a screening strategy for dry eye patients with a high likelihood of having Sjogren syndrome (SS) through the evaluation of the association of ocular surface tests with the extraocular signs used for the diagnosis of SS. Multicenter cross-sectional study. The Sjogren's International Clinical Collaborative Alliance (SICCA) registry enrolled 3514 participants with SS or possible SS from 9 international academic sites. Ocular surface evaluation included Schirmer I testing, tear breakup time (TBUT), and staining of the cornea (0-6 points) and conjunctiva (0-6 points). Multivariate logistic regression analysis was performed to identify predictive factors for (1) histopathologic changes on labial salivary gland (LSG) biopsies (positive= focus score of ≥1 focus/4mm2) and (2) positive anti-SSA/B serology. The adjusted odds of having a positive LSG biopsy were significantly higher among those with an abnormal Schirmer I test (adjusted OR= 1.26, 95% CI 1.05-1.51, P= .014) and positive conjunctival staining(for each additional unit of staining 1.46; 95% CI 1.39-1.53, P < .001) or corneal staining (for each additional unit of staining 1.14; 95% CI 1.08-1.21, P< .001). The odds of having a positive serology were significantly higher among those with an abnormal Schirmer I test (adjusted OR= 1.3; 95% CI 1.09-1.54, P= .004) and conjunctival staining (adjusted OR=1.51; 95% CI 1.43-1.58, P < .001). In addition to corneal staining, which was associated with a higher likelihood of having a positive LSG biopsy, conjunctival staining and abnormal Schirmer I testing are of critical importance to include when screening dry eye patients for possible SS, as they were associated with a higher likelihood of having a positive LSG biopsy and serology.

THU0008 A Negative High-Resolution Salivary Gland Ultrasound is Highly Predictive of Negative Labial Gland Biopsy in Patients with SICCA Symptoms

BackgroundSjögren's syndrome (SS) is a chronic autoimmune disease that affects salivary and lacrimal glands and results in xerostomia and xerophtalmia. Current criteria require the presence of either anti-Ro/La antibodies (ENA)...

The role of a labial salivary gland biopsy in the diagnostic procedure for Sjögren's syndrome; a study of 94 cases.

Objectives: The purpose of the present study is to examine the role of the outcome of the labial salivary gland biopsy (LSGB) in the diagnostic procedure of patients suspected of suffering from Sjögren’s syndrome (SS). Material and Methods: In a retrospective study the result of histopathological assessment of 94 consecutively taken labial salivary gland biopsies has been examined. For the diagnosis of SS the American-European Consensus Group classification (AECG, 2002) have been used. The outcome of the assessment has been discussed in relation to a recently reported classification provided by the American College of Rheumatology (ACR, 2012). Results: In the 94 LSGBs support for a diagnosis of SS has been encountered in 24 out of 26 patients with SS. In the 68 patients with a negative diagnosis of SS only six positive LSGBs were observed. The sensitivity of the labial biopsy amounted 0.92; the specificity was 0.91, while the positive predictive value and the negative predictive value amounted 0.80 and 0.97 respectively. LSGBs taken by or on the request of the departments of Rheumatology or Internal Medicine had a significant higher yield compared to LSGBs taken in other clinical departments. Conclusions: The LSGB may play a role in the diagnostic procedure of Sjögren’s syndrome when using either the AECG classification or the ACR classification. A LSGB should preferably taken after counseling for the possible presence of SS by a department of Rheumatology or Internal Medicine since the yield of such biopsies is much higher than in patients who have not been counseled by these departments prior to the taking of a LSGB. When using the ACR classification, a positive serologic result and a positive ocular test make the taking of a LSGB redundant. Only in case of a negative serologic outcome or a negative result of the ocular test a LSGB is indicated. Since both the serologic test and the ocular test carry hardly any morbidity, these tests should, indeed, be performed first before considering to take a LSGB. Key words:Labial salivary gland biopsy, Sjögren’s syndrome.

Ocular sarcoidosis: when should labial salivary gland biopsy be performed?

To assess the usefulness of a labial salivary gland biopsy (LSGB) in subsets of patients with uveitis. A retrospective study of 115 consecutive patients with uveitis for whom a LSGB had been done because of suspected ocular sarcoidosis (n = 86) or unexplained uveitis (n = 29). Eighty-six patients had a suspicion of ocular sarcoidosis because of ocular features (n = 67), an elevated angiotensin converting enzyme (ACE) (n = 29), or because of CT findings (n = 32) suggestive of sarcoidosis. The biopsy results were analyzed together with their ophthalmological features and the results of other relevant examinations, such as the serum levels of ACE and a chest radiography or a CT scan. Six of the 115 patients (5.2 %) with uveitis had sarcoid granulomas on the LSGB. At the end of the study, 32 patients had proven sarcoidois while 22 patients were considered as having either indeterminate or presumed sarcoidosis, according to the criteria of Abad et al. A raised ACE (p = 0.016) and a compatible radiology (p = 0.033) were related to a positive LSGB test, but not to the features of uveitis. Granulomas were only found in the LSGB of the patients with an elevated ACE or compatible CT scans. In this study, the LSGB sensitivity (18.75 %) in the patients with proven sarcoidosis appears to be lower than in other reports. Our results suggest that this investigation is a possible method of tissue diagnosis in patients with raised ACE and/or CT scan pattern compatible with sarcoidosis, and should not be performed in patients with unexplained uveitis or because of their ophthalmological features.

Ocular sarcoidosis: when should labial salivary gland biopsy be performed ?

AbstractPurpose To assess the usefulness of a labial salivary gland biopsy (LSGB) in subsets of patients with uveitis.Methods A retrospective study of 116 consecutive patients with uveitis for whom a LSGB had been done because of suspected ocular sarcoidosis (n=87) or unexplained uveitis (n=29). Eighty six patients had a suspicion of ocular sarcoidosis because of ocular features (n=67), an elevated angiotensin converting enzyme (ACE) (n=30) or because of CT findings (n=33) suggestive of sarcoidosis. The biopsy results were analyzed together with their ophthalmological features and the results of other relevant examinations, such as the serum levels of ACE and a chest radiography or a CT scan.Results Six of the 116 patients (5.2%) with uveitis had sarcoid granulomas on the LSGB. At the end of the study, 32 patients had proven sarcoidois while 23 patients were considered as having either indeterminate or presumed sarcoidosis, according to the criteria of Abad et al. A raised ACE (p=0.033) and a compatible radiology (p=0.019) were related to a positive LSGB test but not to the features of uveitis. Granulomas were only found in the LSGB of the patients with an elevated ACE or compatible CT scans.Conclusion In this study, the LSGB sensitivity (18.75%) in the patients with proven sarcoidosis appears to be lower than in other reports. Our results suggest that this investigation should be limited to the patients with a raised ACE and/or CT scans pattern compatible with sarcoidosis and should not be performed in patients with unexplained uveitis or because of ophthalmological features.

Diagnosing, managing, and preventing salivary gland disorders.

Salivary function provides host protection, assists in the initiation of food and fluid intake, and enables communication through speech. Without adequate salivary output, oral and pharyngeal health declines along with a person’s quality of life. The complaint of a dry mouth (xerostomia) and the objective finding of salivary dysfunction are common occurrences in older individuals, producing transient and permanent oral and systemic problems. Salivary dysfunction, however, is not a normal consequence of growing older, and is due to systemic diseases, medications, and head and neck radiotherapy. Diagnosis of salivary disorders begins with a careful medical history, head, and neck examination. While complaints of xerostomia may be indicative of a salivary gland disorder, salivary diseases can present without symptoms. Therefore, routine examination of salivary function must be part of any head, neck, and oral examination. Therapies are designed to prevent the development of oral and pharyngeal sequelae of salivary hypofunction. Current xerostomia-based treatments include replacement therapies and gustatory, masticatory, and pharmacological stimulants. Healthcare professionals can play a vital role in identifying patients at risk for developing salivary dysfunction, and should provide appropriate preventative and interventive techniques that will help preserve a person’s health, function, and quality of life.

Sjogren's syndrome in Israel: Primary versus secondary disease

Sixty Israeli patients, 30 with primary Sjögren's syndrome (SS) and 30 with rheumatoid arthritis (RA) and secondary SS, were evaluated. The Schirmer-1 test and a positive labial salivary gland biopsy were found to be the most helpful tools in assessing the diagnosis of SS. Extraglandular features such as Raynaud's phenomenon, lymphadenopathy and CNS involvement as well as parotid gland enlargement (p < 0.05) were more common in primary SS. Antinuclear antibodies, especially anti-Ro (SSA) and anti-La (SSB) were also more common in primary SS (p < 0.05). Our results are in accord with those of many European centers, despite the different genetic background.

Amylose localisée ou systémique ? Intérêt et limites de la scintigraphie au composant amyloïde P marqué à l'iode 123, place de la biopsie de glandes salivaires accessoires

We evaluated the value of serum amyloid P component scintigraphy (123I-SAP) and labial salivary gland biopsy in four patients with apparently localized amyloidosis. One patient had pharyngeal amyloidosis, the second laryngeal amyloidosis, the third retro-ocular amyloidosis and the fourth had carpal tunnel syndrome. Three patients (no 2, no 3, no 4) have abnormal whole body retention of 123I-SAP and positive labial salivary gland biopsy for amyloidosis. Only the first patient had effectively localized amyloidosis and local treatment was sufficient. Melphalan was given to the patients no 2 and no 3, colchicine was given to the patient no 4. Labial salivary gland biopsy and 123I-SAP scintigraphy may give positive argument for the diagnosis of systemic amyloidosis in patients with apparently localized amyloidosis.

Prevalence of primary Sjögren's syndrome in patients with multiple sclerosis

Sixty-four consecutive patients with clinically or laboratory-supported definite multiple sclerosis (MS) were evaluated prospectively for evidence of primary Sjögren's syndrome (SS). This diagnosis was established when a patient had objective keratoconjunctivitis sicca, xerostomia, or both together with positive labial salivary gland biopsy. We found 2 patients (3.1%) with clinical evidence of primary SS. Whether this association is fortuitous or whether there is pathogenetic linkage between MS and primary SS remains to be established.

Clinical implications of the presence of anti-Ro(SSA) antibodies in patients with rheumatoid arthritis

The clinical and serologic findings in 25 patients with rheumatoid arthritis (RA) of more than 5 years' duration, who had antibodies to Ro(SSA) cellular antigen, were analyzed and compared with those in 50 anti-Ro(SSA) negative RA patients with similar disease duration. Ninety-six percent of the anti-Ro(SSA) positive patients were female, compared to 74% in the negative group. The majority of antibody positive patients had symmetric erosive synovitis, similar to that observed in the negative group. Extraarticular manifestations were similar in both groups, except for features of Sjögren's syndrome (keratoconjunctivitis sicca and positive labial salivary gland biopsy), which were significantly more common in patients with anti-Ro(SSA) antibodies. Anemia of chronic disease was also more common in these patients, as were rheumatoid factor (RF) and antinuclear antibodies (ANAs). No patient in either group had antibodies to ds-DNA or Sm and none developed clinical features suggestive of systemic lupus erythematosus (SLE). D-penicillamine, given in the majority of patients in both groups, produced adverse reactions in 72% of the anti-Ro(SSA) positive patients, but only in 27% of the negative ones. Thus, anti-Ro(SSA) antibodies seem to characterize a distinct group of RA patients who are almost exclusively female, express more activated B-cell function, have a high prevalence of Sjögren's features and commonly develop side effects to D-penicillamine.