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Patients with Sjögren syndrome (SS) experience difficulties in wearing conventional dentures. After removal of all teeth, the oral rehabilitation is challenging and time consuming using conventional treatment protocols. Although implant-retained overdentures are beneficial for this specific patient group, the average total oral rehabilitation time (TORT) usually takes at least 9 months and needs to be reduced to increase patients' quality of life (QoL). In this paper, we report on a new treatment concept for immediate implant-based oral rehabilitation in a 77-year-old patient with partial edentulous SS. Because of persistent pain, discomfort, and retention problems with the conventional prosthetic devices, full clearance of the remaining mandibular dentition and immediate oral rehabilitation with an implant-retained overdenture were suggested. The treatment protocol included virtual surgical planning (VSP), combining a guided bone reduction of the mandibular alveolar process, immediate dental implant placement, and restoration using a prefabricated bar and placement of the overdenture. This method demands the use of ionizing 3D imaging optionally combined with an optical dental scan or a conventional impression. Furthermore, one needs to gain experience using VSP software. This novel treatment concept for immediate implant-based oral rehabilitation using VSP proved to be feasible and safe in a patient with SS, resulting in a significantly reduced TORT and improved QoL. Further research is needed to what extent this treatment concept could be beneficial to other patient groups, such as patients with head and neck cancer.

Objectives/hypothesis: This study is a systematic review of the literature which seeks to estimate the expected treatment outcomes of a patient with Sjogren's syndrome (SS) undergoing therapeutic sialendoscopy., Study Design: Systematic Review., Methods: PubMed, Scopus, and Cochrane library databases were used to search for studies published as of August 2020 regarding the treatment outcomes of SS with sialendoscopy. The key search terms included "Sjogren's syndrome" and "sialendoscopy." Only studies in the English language involving more than one human patient were included. PRISMA guidelines were followed in study inclusion and data extraction. The primary outcome assessed was improvement in patient symptoms., Results: Six studies met criteria and were analyzed in this review, including 125 patients undergoing sialendoscopy of parotid and/or submandibular glands as well as 25 controls. Of these patients, 90% were female with an age range of 18 to 79 years. There was significant diversity in outcome reporting tools. The outcomes of symptom improvement were pooled qualitatively based on improvement noted in each study. Outcomes were defined as partial improvement if the measured outcomes improved and complete improvement if measured outcomes resolved entirely. Despite the limited number of studies on this topic, this meta-analysis suggests that a similar study of therapeutic sialendoscopy could expect to provide at least temporary improvement of symptoms 90% to 99% of the time., Conclusions: This review provides support for the application of sialendoscopy in the treatment of SS salivary disease. Larger studies with consistent outcome reporting tools and control groups are needed to validate these results and provide a consistent therapy protocol. Laryngoscope, 131:1474-1481, 2021., (© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

Primary Sjögren's syndrome (pSS) is a progressive systemic autoimmune disease characterized by lymphocytic infiltrates in exocrine glands, leading to the injury of salivary and lachrymal glands. Mesenchymal stem cells (MSCs) have been demonstrated to exert great potential in the treatment of various autoimmune diseases. Although MSCs have provide an effective therapeutic approach for SS treatment, the underlying mechanisms are still elusive. Our previous study has shown the reduced suppressive capacity of myeloid-derived suppressor cells (MDSCs) advanced the progression of experimental Sjögren's syndrome (ESS). In this study, we found that BM-MSCs significantly enhanced the suppressive function of MDSCs with high levels of Arginase and NO, decreased the levels of CD40, CD80, CD86, and MHC-II expression on MDSCs, thus attenuating the disease progression in ESS mice. Furthermore, the enhanced suppressive function of MDSCs was mediated by BM-MSC-secreted TGF-β, and the therapeutic effect of BM-MSCs in inhibiting ESS was almost abolished after silencing TGF-β in BM-MSCs. Taken together, our results demonstrated that BM-MSCs alleviated the ESS progression by up-regulating the immunosuppressive effect of MDSCs through TGF-β/Smad pathway, offering a novel mechanism for MSCs in the treatment of pSS., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2020 Tian, Hong, Zhu, Zhou, Zhang, Shen, Guo, Zhang, Ai, Zhao, Rui, Xu and Wang.)

Between August 2009 and May 2016, 74 patients underwent sialoendoscopic surgery. 32 patients had parotid gland disease and 9 patients had intermittent swelling of the parotid gland and sialoliths were not detected with CT imaging. 4 patients were diagnosed with idiopathic Stensen's duct stenosis. Sialendoscopy directly confirmed Stensen's duct stenosis in 2 patients. However, the sialendoscope was unable to be inserted in the other 2 patients, who had stenosis of the orifice of the Stensen's duct. Balloon expansion of the duct was performed in these 2 patients and a steroid drug was injected into the duct in one patient. Complete remission was archived in one patient treated with sialendoscopy. Three patients had sialolithiasis. Microsialoliths and/or white floating matter was observed and removed using sialendoscopy. All patients experienced complete remission. In cases of Sjögren syndrome and recurrent parotitis, sialendoscopic surgery was performed, but the symptoms showed no improvement. For patients with microsialoliths, sialendoscopy may be most useful for diagnosis and treatment when the sialoliths are not detected with CT imaging. At present, sialendoscopic surgery have limitation in the treatment of Stensen's duct stenosis and may similarly have limitation in the treatment of Sjögren's syndrome and recurrent parotitis., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objectives/hypothesis: To characterize clinical, imaging, and sialendoscopy findings in patients with chronic parotitis and multiple parotid calcifications., Study Design: Retrospective review., Methods: Clinical history, radiographic images and reports, lab tests, and operative reports were reviewed for adult patients with chronic parotitis and multiple parotid calcifications who underwent parotid sialendoscopy., Results: Thirteen of 133 (10%) patients undergoing parotid sialendoscopy for chronic sialadenitis had more than one calcification in the region of the parotid gland. Seven patients (54%) were diagnosed with immune-mediated disease from autoimmune parotitis (positive Sjögren's antibodies or antinuclear antibodies) or human immunodeficiency virus (HIV) disease. The six patients (46%) who did not have an immune-mediated disorder had most calcifications located anterior or along the masseter muscle. Eight of 13 patients (61%) had at least one calculus found in the parotid duct on sialendoscopy. Four patients (38%) had multiple punctate calcifications within the parotid gland, all of whom had either autoimmune parotitis or HIV. None of the proximal or punctate parotid calcifications posterior to the masseter were visualized on sialendoscopy., Conclusions: Chronic parotitis in conjunction with multiple parotid calcifications is uncommon and was identified in 10% of our cohort. We contrast two classifications of parotid calcifications: 1) intraductal stones that cause recurrent duct obstruction and are often located within the main parotid duct along or anterior to the masseter and 2) punctate intraparenchymal parotid gland calcifications that are not visualized on sialendoscopy and may represent underlying inflammatory disease., Level of Evidence: 4 Laryngoscope, 127:1565-1570, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Purpose: Recent literature suggests that sialendoscopy of the major salivary glands could alleviate symptoms of Sjögren syndrome (SS) and restore salivary function. The aim of this study was to evaluate the authors' experience of sialendoscopy of the salivary glands in patients with SS., Materials and Methods: In this retrospective case series study, the surgical data of patients with SS who had undergone sialendoscopy at the VU University Medical Center (Amsterdam, The Netherlands) from November 2014 through April 2015 were used. Outcome measurements were successful entry to the salivary gland and completion of sialendoscopy as planned. Furthermore, pre- and postoperative complications were scored. Descriptive analysis of the data was performed., Results: Surgical data of sialoendoscopic procedures in 26 patients with SS (24 women and 2 men; mean age, 57 yr; range, 27 to 72 yr) were analyzed. Sialendoscopy was successfully performed in 78 of 104 salivary glands (75%; 50 parotid and 28 submandibular glands) in the 26 patients. Sialendoscopy failed in 26 of the 104 sialoendoscopic procedures (25%; 2 parotid and 24 submandibular glands). In 16 salivary glands, the ductal orifice could not be identified. In 7 salivary glands, sialendoscopy could not be performed because of partial or complete stenosis of the salivary duct. In 3 salivary glands, sialendoscopy was not performed because of a ductal perforation. Three patients developed a postoperative infection., Conclusion: The overall rate of complications was limited and the sialoendoscopic complications in patients with SS could be regarded as minor. Most complications were seen for sialendoscopy of the submandibular glands in this specific patient category. Careful preoperative selection of patients and salivary glands could contribute to a lower rate of complications and more predictable results., (Copyright © 2016. Published by Elsevier Inc.)

Autoimmune diseases of major salivary glands include Sjögren's syndrome and a complex of disorders classified as immunoglobulin G4-related diseases. These pathologies are characterised by an autoimmune reaction mediated by T-helper lymphocytes that targets the ducts of exocrine glands in Sjögren's syndrome and glandular parenchyma in immunoglobulin G4-related diseases. Immunoglobulin G4-related diseases represent recently introduced multi-organ diseases that also involve the salivary glands. However, the morbid conditions once known as Mikulicz's disease and Kuttner's tumour were recently considered as two variants of immunoglobulin G4-related diseases affecting the major salivary glands ( immunoglobulin G4-related sialadenitis). This review briefly summarises the pathogenesis and clinical features of autoimmune diseases of the major salivary glands, focusing on the diagnostic and therapeutic role of sialendoscopy., (© Copyright by Società Italiana di Otorinolaringologia e Chirurgia Cervico-Facciale, Rome, Italy.)

Purpose: To describe a case of keratoconjunctivitis sicca treated with punctal plug placement with the first reported complication of subsequent plug erosion through the canaliculus and extrusion through the lid., Methods: Punctal plugs were placed in a patient with Sjogren syndrome for treatment of keratoconjunctivitis sicca. More than 12 years later, the plug was noted to have eroded through the canaliculus and extruded through the conjunctiva and lid., Results: The extruded plug was removed, and punctal cautery was performed on 2 other puncta with improved symptom control., Conclusions: Plug extrusion through the conjunctiva and lid is a possible consequence of punctal plug placement.

Background: Limited evidence is available for applying dental implants in Sjögren's syndrome (SS) patients., Purpose: This study aims to retrospectively assess clinical outcome of implant therapy in a cohort of well-classified patients with SS., Materials and Methods: All SS patients attending the University Medical Center Groningen for follow-up (n = 406) were asked whether they had implants. In SS patients with implants peri-implant health and implant survival was recorded and compared with data from matched healthy controls. Patients' symptoms, health-related quality of life, oral functioning, and satisfaction were assessed using validated questionnaires., Results: Of the responding SS patients (n = 335), 21% was provided with implants. Of these 69 SS patients, 50 SS patients were willing to join our study. In SS patients, peri-implant health was reasonably good with minor marginal bone loss and a peri-implantitis prevalence of 14%, comparable with healthy controls. Implant survival was 97% (median follow-up 46 months) [IQR 26;73], and overall patients' satisfaction was high. Oral functioning correlated negatively with dryness, patients' satisfaction, and chewing ability in SS patients., Conclusions: Implant therapy is common in our cohort of SS patients. In spite of shortcomings of our retrospective analysis, implants in SS patients seem to perform comparable with implants in healthy patients., (© 2015 Wiley Periodicals, Inc.)

Rumpel-Leede phenomenon is a rare clinical sign involving the appearance of purpura after application of a tourniquet or in noninvasive arterial blood pressure monitoring. This sign has been most commonly associated with hypertensive and diabetic microvascular fragility and thrombocytopenia. We describe a case of Rumpel-Leede phenomenon in an otherwise healthy patient with positive laboratory markers for Sjögren disease, a previously undescribed relationship. We aim to inform physicians of this potential complication in patients with Sjögren disease and suggest special consideration be given to patients with autoimmune diseases with secondary vascular or dermal manifestations.

To reveal dental implants survival rates in patients with oral mucosal diseases: oral lichen planus (OLP), Sjögren's syndrome (SjS), epidermolysis bullosa (EB) and systemic sclerosis (SSc). A systematic literature search using PubMed/Medline and Embase databases, utilising MeSH and search term combinations identified publications on clinical use implant-prosthetic rehabilitation in patients with OLP, SjS, EB, SSc reporting on study design, number, gender and age of patients, follow-up period exceeding 12 months, implant survival rate, published in English between 1980 and May 2015. After a mean observation period (mOP) of 53·9 months (standard deviation [SD] ±18·3), 191 implants in 57 patients with OLP showed a survival rate (SR) of 95·3% (SD ±21·2). For 17 patients with SjS (121 implants, mOP 48·6 ± 28·7 months), 28 patients with EB (165 implants, mOP 38·3 ± 16·9 months) and five patients with SSc (38 implants, mOP 38·3 ± 16·9 months), the respective SR was 91·7 ± 5·97% (SjS), 98·5 ± 2·7% (EB) and 97·4 ± 4·8% (SSc). Heterogeneity of data structure and quality of reporting outcomes did not allow for further comparative data analysis. For implant-prosthetic rehabilitation of patients suffering from OLP, SjS, EB and SSc, no evidence-based treatment guidelines are presently available. However, no strict contraindication for the placement of implants seems to be justified in patients with OLP, SjS, EB nor SSc. Implant survival rates are comparable to those of patients without oral mucosal diseases. Treatment guidelines as for dental implantation in patients with healthy oral mucosa should be followed., (© 2015 John Wiley & Sons Ltd.)

Background: Sjögren syndrome is a chronic autoimmune disorder of the exocrine glands associated with cardiovascular events. We aimed to evaluate postoperative complications in patients with Sjögren syndrome undergoing noncardiac surgery. Specifically, we tested the primary hypothesis that patients with Sjögren syndrome have a greater risk of postoperative cardiovascular complications than those without the disease. Our secondary hypotheses were that patients with Sjögren syndrome are at greater risk of thromboembolic complications, microcirculatory complications, and mortality., Methods: We obtained censuses of 2009 to 2010 inpatient hospital discharges across 7 states. Sjögren syndrome was identified by the present-on-admission diagnosis code 710.2. Each Sjögren n syndrome discharge was propensity matched to 4 control discharges. A generalized linear model was used to compare matched Sjögren syndrome patients and controls on risk of in-hospital cardiovascular complications, thromboembolic complications, microcirculatory complications, and mortality., Results: Among 5.5 million qualifying discharges, our final matched sample contained 22,785 matched discharges, including 4557 with Sjögren syndrome. Sixty-six (1.45%) of the matched discharges with Sjögren syndrome and 213 (1.17%) of the matched controls had associated in-hospital cardiovascular complications. The adjusted odds ratio (99% confidence interval) was estimated at 1.14 (0.79-1.64), which was not statistically significant (P = 0.35). There were no significant differences in the odds of in-hospital thromboembolic complications (1.12 [0.82-1.53]; P = 0.36), in the odds of in-hospital microcirculatory complications (0.98 [0.77-1.26]; P = 0.86), or in the odds of in-hospital mortality (1.11 [0.76-1.61]; P = 0.49)., Conclusions: The presence of Sjögren syndrome does not place patients at an increased risk for postoperative complications or in-hospital mortality.

Purpose: To evaluate the effect of lid debridement-scaling (LDS) on dry eye signs and symptoms in subjects with Sjögren syndrome (SS)., Methods: This prospective randomized controlled study enrolled 14 female subjects with SS. Seven subjects were randomized into the treatment group where they were selected to receive LDS; the remainder did not receive LDS and served as control subjects. Lid debridement-scaling was conducted using a stainless steel golf club spud (Hilco Wilson Ophthalmics, Plainville, MA) on both the upper and lower eyelids of both eyes. Outcome variables were assessed before LDS and again 1 month later. The outcome variables were the Ocular Surface Disease Index (OSDI), Symptom Assessment iN Dry Eye (SANDE) visual analog scores, ocular staining (SICCA OSS [Sjögren's International Collaborative Clinical Alliance Ocular Staining Score]), fluorescein tear breakup time (FLBUT), meibomian gland score (MGS), meibomian gland yielding liquid secretions (MGYLS) score, and line of Marx's (LOM) position., Results: Thirteen subjects completed the study. Data from only the right eye were analyzed. For the control group (n = 6; mean [± SD] age, 62.3 [± 11.6] years), the pre-LDS, post-LDS, and significance level (pre-LDS mean [± SD] vs. post-LDS mean [± SD]; p value) were as follows: OSDI (58.3 [± 22.1] vs. 48.3 [± 29.0]; p = 0.051), SANDE (77.4 [± 22.1] vs. 89.6 [± 32.6]; p = 0.20), SICCA OSS (7.0 [± 4.5] vs. 8.2 [± 3.5]; p = 0.25), MGS (1.3 [± 1.5] vs. 1.0 [± 0.9]; p = 0.75), MGYLS (0.3 [± 0.5] vs. 0.0 [± 0.0]; p = 0.50), FLBUT (2.99 [± 1.54] vs. 2.85 [± 1.79]; p = 0.63), and LOM (2.0 [± 0.0] vs. 2.0 [± 0.0]; p = n/a). For the treatment group (n = 7; mean [± SD] age, 58.0 [± 8.1] years), the pre-LDS, post-LDS, and significance level were as follows: OSDI (63.2 [± 13.3] vs. 46.9 [± 19.4]; p = 0.04), SANDE (72.6 [± 17.1] vs. 77.0 [± 28.0]; p = 0.54), SICCA OSS (6.6 [± 2.9] vs. 5.0 [± 3.9]; p = 0.02), MGS (1.0 [± 1.2] vs. 3.1 [± 1.7]; p = 0.01), MGYLS (0.0 [± 0.0] vs. 0.6 [± 1.0]; p = 0.50), FLBUT (3.13 [± 0.81] vs. 3.45 [± 1.03]; p = 0.53), and LOM (0.9 [± 0.9] vs. 1.0 [± 1.0]; p = 1.00)., Conclusions: This pilot study showed that LDS improved symptoms, ocular staining, and meibomian gland function for the group that received LDS. This indicates that LDS can aid in the management of SS dry eye.

Background: Renal involvement is rare in primary Sjögren syndrome (PSS). In this study, we examined renal biopsy findings in patients with PSS and correlated them with their clinical and renal findings., Methods: Twenty-five patients with PSS who underwent renal biopsies from two renal units in Scotland between 1978 and 2013 were identified from renal biopsy database. We examined the renal morphologic, clinical and renal findings at the time of renal biopsy, renal and patient outcomes., Results: The diagnosis of PSS preceded renal biopsy in 18/25 patients. In this group, the median duration of the disease was 5.5 years. Significant proteinuria, combined microscopic haematuria and proteinuria and reduced renal excretory function were found in 76, 56 and 84% of patients, respectively. The 3-year actuarial patient survival was significantly lower in patients with glomerulonephritis as compared with tubulointerstitial nephritis (66 versus 100%, P = 0.02). There was no difference in 3-year actuarial renal survival between these two groups (92 versus 92%, P = 1.0)., Conclusions: Renal biopsy is rare in PSS and often reveals diverse pathological findings. Glomerulonephritis, as compared with tubulointerstitial nephritis, is associated with higher early mortality. Further studies are needed to evaluate the utility of renal biopsy and its impact on disease management., (© The Author 2015. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.)

Background: IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS), so-called Mikulicz's disease, is characterized by elevated serum IgG4 and infiltration of IgG4-positive plasma cells in glandular tissues. Recently, several studies reported both malignant lymphoma developed on the background of IgG4-associated conditions and IgG4-producing malignant lymphoma (non-IgG4-related disease)., Case Presentation: We report on the case of a 70-year-old man who was strongly suspected IgG4-DS because of high serum IgG4 concentration (215 mg/dl) and bilateral swelling of parotid and submandibular glands. Biopsies of cervical lymph node and a portion of submandibular gland were performed. These histopathological findings subsequently confirmed a diagnosis of marginal zone B cell lymphoma., Conclusion: Differential diagnosis of IgG4-DS is necessary from other disorders, including Sjögren's syndrome, sarcoidosis, Castleman's disease, Wegener's granulomatosis, lymphoma, and cancer. We suggest that biopsy of swollen lesions is important for a definitive diagnosis of IgG4-DS and discuss the mechanism of development in this case.

Sjögren's syndrome, a chronic and progressive autoimmune disorder mainly characterized by xerophthalmia, xerostomia, and parotid enlargement, is primarily managed medically, but some patients will require surgical management. Patients with Sjögren's syndrome have an increased risk of non-Hodgkin lymphoma. Superficial parotidectomy is indicated for diagnostic purposes and can be therapeutic in limited circumstances. Surgical indications for parotidectomy in Sjögren's syndrome include recurrent parotitis refractory to medical management; salivary gland malignancy; and severe, refractory pain. Surgical complications include transient or permanent facial nerve injury, post-operative pain, persistent inflammation of remnant parotid tissue, Frey syndrome, and facial scarring., (Copyright © 2014 Elsevier Inc. All rights reserved.)

The pancreas and salivary glands have similar anatomical structures and physiological functions producing bicarbonate-rich fluid containing digestive enzymes and other components to be delivered into the gut. Despite these similarities, the two organs are also different in numerous respects, especially regarding the inflammatory diseases affecting them. This article will summarize the pathophysiology and current and potential pharmacological treatments of chronic inflammatory diseases such as chronic pancreatitis, autoimmune pancreatitis, Sjögren's syndrome and irradiation-induced salivary gland atrophy. Despite the differences, in both organs the inflammatory process is accompanied by epithelial tissue destruction and fibrosis. Both in pancreatic and in salivary research, an important task is to stop or even reverse this process. The utilization of stem/progenitor cell populations previously identified in these organs and the application of mesenchymal stem cells are very promising for such regenerative purposes. In addition, gene therapy and tissue engineering research progressively advance and have already yielded clinically beneficial preliminary results for salivary gland diseases. For the hard-to-access, hard-to-regenerate pancreas these developments may also offer new solutions, especially since salivary and pancreatic progenitors are very similar in characteristics and may be mutually useful to regenerate the respective other organ as well. These novel developments could be of great significance and may bring new hope for patients since currently used therapeutic protocols in salivary and in pancreatic chronic inflammatory diseases offer primarily symptomatic treatments and limited beneficial outcome.

An 83-year-old woman with Sjögren syndrome underwent insertion of large punctal plugs in the right upper and lower puncti. Nine months later, she presented with upper canaliculitis, although the plugs were not found in the puncti. Dacryoendoscopic examination revealed punctal plug migration, granulation, and a large amount of debris in the upper canaliculus. The plug was situated parallel to the canaliculus, occluding the intracanalicular space. The lower canaliculus also showed plug migration, but little granulation and debris were present. The plug was situated perpendicular to the intracanalicular space, securing a space around the plug. The migrated plugs were pushed directly toward the nasal cavity by the dacryoendoscope. Curettage was performed in the upper canaliculi. Bicanalicular silicone intubation was performed, and removal was performed 3 months later. The patient remained symptom-free for 6 months postoperatively.

Primary lymphomas of the salivary glands are rare. It is estimated that they constitute no more than 5% of all lymphomas in different locations. The most common subtype developing in parotid glands is marginal zone B-cell mucosa associated lymphoid tissue type lymphoma (MALT) that belongs to a group of low-grade tumours. There are many factors associated with the incidence of that proliferative process: environmental and infectious agents as well as immune deficiency states. We describe a case of primary non-Hodgkin's lymphoma of the parotid gland arising in the background of previously undiagnosed and untreated Sjögren's syndrome in a 52-year-old woman. The article concerns a short review of the literature regarding etiology, symptoms, treatment and survival prognosis in that rare disease as well. MALT lymphomas should always be considered in the differential diagnosis of the tumors and swelling of the parotid gland area. A special, regular monitoring should include all patients with Sjögren's syndrome as those with the proven greater risk of developing that proliferative disease. The role of the laryngologist in the case of MALT-type lymphoma of the parotid gland should focus on a diagnosis and possible tumor cytoreduction with maximal saving of the facial nerve. The essential treatment of this pathology is one of the oncologists and haematologists., (Copyright © 2012 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by Elsevier Urban & Partner Sp. z.o.o. All rights reserved.)

Objective: The relationship of cryoglobulinaemia with lymphoproliferation of mucosa-associated lymphoid tissue (MALT) as risk factors for lymphoma evolution in SS remains to be clarified. The different biologic background of SS-related cryoglobulinaemia as compared with cryoglobulinaemia linked to HCV infection was clarified by different clinical and biologic approaches., Methods: B-cell clonal expansion was analysed in the bone marrow of 27 consecutive cases with primary SS and mixed cryoglobulinaemia, HCV unrelated, in comparison with 55 HCV-related patients with cryoglobulinaemic vasculitis (CV) without SS. The results were related to the possible occurrence and localization of B-cell lymphoma in the single case. Secondly, the prevalence of mixed cryoglobulinaemia was investigated in 41 unselected patients with primary SS showing either parotid myoepithelial sialadenitis (MESA) or a frank B-cell non-Hodgkin's lymphoma. Thirdly, the levels of serum cryoglobulins and RF were followed in one patient with primary SS, CV and parotid B-cell lymphoma of MALT after bilateral subtotal parotidectomy., Results: A polyclonal pattern of B expansion in the bone marrow was significantly more frequent in SS-related (19/27 cases) than in HCV-related cryoglobulinaemia (19/55) (P = 0.003). Cryoglobulins were positive in a fraction of patients with SS and malignant lymphoma or with parotid MESA (13/18 and 7/23, respectively), whereas MALT involvement by the lymphoproliferative disorder was the rule. Finally, the levels of serum cryoglobulins and RF markedly decreased in the SS patient undergoing bilateral subtotal parotidectomy., Conclusion: Lymphoproliferation of MALT appears as the biologic background of cryoglobulinaemia in SS, differently from HCV-related cryoglobulinaemia.

Aims: To evaluate the clinical outcomes following Moscow Eye Microsurgery Complex in Russia keratoprosthesis (MICOF KPro) implantation in end-stage autoimmune dry eyes., Methods: A retrospective observational case series. Fourteen patients with end-stage autoimmune dry eyes underwent MICOF KPro surgery by one surgeon (YH) in the Chinese PLA General Hospital between 2000 and 2009. Retention of the prosthesis and vision were recorded and postoperative complications were analysed., Results: Preoperative diagnosis included Stevens-Johnson syndrome (n=7), ocular cicatricial pemphigoid (n=4) and Sjogren's syndrome (n=3). The mean follow-up period was 3.9 years (range 10 months-7.8 years). After surgery, 13 eyes (93%) showed a visual acuity of 20/200 or better and 6 eyes (43%) achieved excellent vision of 20/40 or better. At last visit, 69% of the eyes (9/13) maintained a visual acuity of at least 20/200. The first case retained a visual acuity of 20/80 7.8 years after the operation. The most common complication was stromal melting that threatened anatomical success. Seven of the first eight patients showed tissue melting, and four of them had leakage. All of these were repaired successfully. The latter six cases received prophylactic autologous auricular cartilage implantation to reinforce the anterior surface of recipient's cornea at either stage 1 or 2. Other causes of vision loss included pre-existing glaucoma (n=6), sterile vitritis (n=5), cylinder loosening (n=1) and retroprosthetic membrane (n=4)., Conclusion: MICOF KPro provided useful vision for the end stage of autoimmune dry eyes in our study. Anatomical stability of KPro was achieved in all cases using repair and reinforcing surgery.

Purpose: To analyze the influence of thermal partial punctal occlusion on the ocular surface of dry eye related to Sjögren syndrome., Material and Methods: Thirty-seven eyes of 19 patients (3 male and 16 female; 49.11 ± 14.33 years old) with keratoconjunctivitis sicca were enrolled in this study. Superior and inferior partial occlusion were performed in both eyes under topical anesthesia using thermal cautery with a sterile tip to obtain lacrimal punctum smaller than 0.5 mm. Schirmer I, break-up-time, diameter of lacrimal puncta, corneal fluorescein, and rose Bengal staining scores were analyzed before and after 24 weeks and after 24 months of the procedure. All measurements were performed under controlled climate., Results: The average lacrimal punctum diameter before the procedure was 0.65 ± 0.134 mm. All lacrimal puncta were successfully reduced to less than 0.5 mm after 4 weeks of the procedure. The average Schirmer I test values improved statistically after 24 weeks and maintained stable after 24 months. Average break-up-time, rose Bengal, and fluorescein staining score values improved statistically after 24 weeks and improved even more after 24 months. Average Schirmer I test, break-up-time, rose Bengal, and fluorescein staining scores showed significant improvement (p < 0.0001) after 24 months of partial thermal punctal occlusion., Conclusion: Our study showed that reducing the punctum diameter to 0.5 mm can improve vital staining scores, break-up-time, and Schirmer I test in dry eye related to Sjögren syndrome.

Purpose: To measure the sparkle of the human eye evaluated by the intensity of corneal light reflection in normal subjects and dry eye patients to investigate whether ocular surface wetness has an impact on the sparkle of the eye., Design: Prospective case-control study., Methods: We examined a consecutive series of eight dry eye patients with Sjögren syndrome (SS, 15 eyes), as well as eight normal subjects (16 eyes). The specular corneal surface light reflection was quantitatively measured with an ophthalmic slit-lamp microscope and image capturing system under fixed conditions of light source, incident angle, and detector sensitivity. The intensity of images from subjects' corneal light reflection was quantified with image analysis software along with the measurement of grade of self-reported brilliancy of the eye, corneal fluorescein staining score, tear film break-up time, and Schirmer test value. The intensity of corneal light reflection was also compared before and after dry eye treatment., Results: The mean intensity of corneal light reflection was significantly lower in dry eye patients (125.0 ± 40.1) than normal subjects (167.6 ± 36.6, P=.004). Grade of self-reported brilliancy of the eye, corneal fluorescein staining scores, tear film break-up time, and Schirmer test values showed good correlation to the intensity of corneal light reflection. After punctal plug treatment, the intensity of corneal light reflection significantly increased from 125.0 ± 40.1 to 167.2 ± 45.0 (P<.0001)., Conclusions: The intensity of corneal light reflection representing the sparkle of the eye was significantly more intense in normal subjects compared to dry eye patients, and was increased after punctal plug treatment. The intensity of corneal light reflection appeared to correlate well with tear film stability, volume, and ocular surface desiccation. We showed that tears contributed not only to ocular surface wetness but also to the extent of the light reflection from the eye., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Sjogren syndrome is a chronic autoimmune disease witch affects mostly lachrymal and salivary glands. The exocrinopathy can be encountered alone (primary Sjogren syndrome) or in association with other autoimmune disorders (secondary Sjogren syndrome). Visual prognosis of the patients with secondary Sjogren syndrome depends on the early diagnosis, applied therapy follow-up controls along with an effective collaboration between ophthalmologist and rheumatologist. We present therapeutic options in secondary Sjogren syndrome: hygiene and protective measures, medical nonspecific substitution treatment, treatment to stimulate tear secretion, autoimmune disease-specific medical treatment and surgery.

A 52-year-old woman with Sjögren's syndrome presented with multiple cerebral aneurysms, which resulted in recurrent subarachnoid hemorrhage (SAH) manifesting as severe headache. Computed tomography (CT) showed SAH. Cerebral angiography revealed aneurysms of the anterior communicating artery and the left middle cerebral artery. The necks of the aneurysms were successfully clipped. Postoperative course was uneventful and she was discharged without neurological deficit. Thirteen years later, she suddenly lost consciousness and was brought to our hospital. CT demonstrated recurrence of SAH. Cerebral angiography revealed cerebral aneurysms of the right internal carotid artery and the right middle cerebral artery. The clipped aneurysms were stable. The internal carotid artery aneurysm was successfully treated with endovascular coil embolization. However, she regained no neurological function and died. Patients with Sjögren's syndrome may develop cerebral aneurysm complicated with SAH.

We report on a 48-year old female patient suffering from recurrent suppurative sialo-adenitis. Resections of both parotid and the left submandibular glands had to be performed due to abscesses. The woman had suffered from recurrent swelling of the salivary glands as well as xerostomia and xerophthalmia for years. Sjögren's syndrome was diagnosed with a delay of 17 years. The current case implicates recurrent bacterial suppurative sialo-adenitis caused by longstanding Sjögren's syndrome. In every case rheumatic disorders have to be considered in the differential diagnosis of recurrent suppurative parotitis. A detailed anamnesis, ultrasound, a differential hemogram, testing for rheumatoid factor and anti-nuclear antibodies (ANA) as well as SS-A and SS-B ENAs can give early information years before Sjögren's syndrome becomes clinically apparent.

Pulmonary and thymic lymphoid hyperplasia with characteristic clinicoradiological manifestations were seen in a 71-year-old woman who was diagnosed with primary Sjögren's syndrome. A hazy opacity 12 mm in diameter and an anterior mediastinal nodule 20 mm in diameter were incidentally detected on computed tomography. Thoracoscopic biopsy revealed lymphoid hyperplasia in the lung accompanying thymic lymphoid hyperplasia. Immunohistochemically, the pulmonary lesion was considered to be nodular lymphoid hyperplasia (NLH), not lymphoma. A previously undescribed finding in the NLH was the hazy opacity containing an air bronchiologram, not a solid nodule. This finding might suggest a common causal relation for NLH, thymic hyperplasia, and primary Sjögren's syndrome.

Silicone punctal plugs are effective and relatively safe method of managing keratoconjunctivitis sicca. We present a case where a silicone punctal plug migrated to cause aseptic necrosis of the surrounding tissue leading to a lid ulcer.

Purpose: The study presents a case of bilateral lacrimal gland hypertrophy with secondary glaucoma due to the increased episcleral venous pressure., Method: The case was investigated by means of clinical methods associated with ultrasound (A and B-scan, Doppler-ultrasound) and magnetic resonance imaging techniques., Results: The clinical data revealed proptosis, episcleral congestion, and elevated intraocular pressure. An abnormal Schirmer test and xerophthalmia were also present. Ultrasound examination identifies enlarged masses in the bilateral lacrimal fossa, supra-temporal to the globe, mostly on the left side, having a cystic structure. The Doppler ultrasound investigation revealed the vascularisation, and the magnetic resonance imaging completed the dates offered by the ultrasound methods. According to the clinical aspect and the possible visual impairment produced by the secondary glaucoma, the mass lesion was removed on the left side by neurosurgical approach. The histopathological exam confirmed the destruction of the lacrimal gland, and, furthermore, the immunohistochemical study revealed the aspect of Sjögren syndrome lesions., Conclusions: Sonography is able to provide noninvasively much of the information needed by the clinician. Ultrasound techniques (A-scan, B-scan and color Doppler) and magnetic resonance imaging allow tracking and discrimination of the orbital diseases, such as lacrimal gland lesions. Associated with the clinical features, these methods offer the basis of a correct diagnosis and a proper therapy for lacrimal gland pathology.

Purpose: To compare noninvasive tear meniscus height (NI-TMH) using a tear interference device in normal subjects and dry eye patients with Sjögren syndrome (SS), and to investigate the applicability of this new method before and after the punctal occlusion procedure., Design: Prospective case control study., Methods: Tear meniscus was visualized noninvasively using a tear interference device (Tearscope plus, Keeler, Windsor, United Kingdom). Tear interference image was captured with digital video camera (SP-321, JFC Sales Plan Co, Tokyo, Japan) attached to the slit-lamp. Lower lid margin NI-TMH was measured using image analysis software. NI-TMH of 28 eyes from 17 normal subjects and 46 eyes from 27 aqueous tear deficiency (ATD) dry eye patients with SS were compared. The change of NI-TMH three weeks after the successful punctal occlusion was examined in 11 eyes of eight dry eye subjects., Results: Tear meniscus was well visualized with the tear interference device in all cases. Lower lid margin NI-TMH was 0.22 +/- 0.065 mm in normal subjects, and 0.13 +/- 0.042 mm in SS subjects, respectively (P < .0001). After the punctal occlusion, lower lid margin NI-TMH increased significantly from 0.12 +/- 0.026 mm to 0.42 +/- 0.21 mm (P = .001)., Conclusions: NI-TMH was substantially lower in SS subjects and also significantly improved after punctal occlusion. This method is expected to be helpful in the diagnosis and in the evaluation of the efficacy of punctal occlusion in ATD dry eyes such as SS.

We report the first case of Sjögren's syndrome causing gross lacrimal gland swelling and secondary hypoglobus and uncharacteristically absent sicca syndrome. Histopathological diagnosis of the excision biopsy of the orbital lobe of the gland confirmed a mixed T and B cell infiltrate of the gland with no lymphomatous transformation. The condition remains quiescent following complete excision.

The presence of multiple calculi in the major salivary glands is an uncommon finding. Sjögren's syndrome is a chronic autoimmune disease characterized by lymphocyte-mediated destruction of the exocrine glands. The case is presented of a 49-year-old female with Sjogren's syndrome found to have bilateral multiple sialolithiasis in the parenchyma of the parotid glands. The patient presented with a right sided painful inflamed swelling of the parotid region. Even though she had been diagnosed with primary Sjögren's syndrome 3 years prior to admission, she did not report any previous episode of sialadenitis. Full blood count showed leukocytosis (white blood cells = 14,900/10(6) L) with neutrophilia (75%). Radiological assessment included ultrasound and computed tomography scan of the parotids which demonstrated intra-parenchymal multiple calculi of both parotid glands and obstruction of the right Stensen's duct. The patient was treated with intravenous antibiotics and anti-inflammatory drugs. On the second day of hospitalisation, she reported spontaneous extrusion of a calculus during massage of the gland, with immediate relief of symptoms. In patients with Sjögren's syndrome and radiological findings of calculi in the major salivary glands, close observation is mandatory for better control of recurrent sialadenitis and early recognition of mucosa-associated lymphoid tissue lymphomas.

Background: This study was designed to examine if patients undergoing the same operative procedure (blepharochalasis operation) have different subjective recognition of dry eye problems depending n whether they had he operation for cosmetic or medical reasons., Patients and Method: The study included 32 patients (25 women, 7 men, mean age 57.8 +/- 12.1 years), who underwent blepharochalasis operation between 1/2005 and 5/2005. In 17 patients (group 1) the operation was done for cosmetic reasons, in 15 patients we found a medical indication (group 2). We evaluated the subjective discomfort from the lid problems as well as the satisfaction with the operation in general and pre- or postoperative recognition of dry eye problems (all done by scales). Additionally we measured tear secretion by Schirmer's test and break-up time before and after operation., Results: The subjective recognition of a dry eye was stronger in group 1 than in group 2 before and after operation (p < 0.005), but objective data were nearly identically (BUT, Schirmer's test)., Conclusion: When operated due to cosmetic reasons the stronger recognition of sicca problems (while objectively lacking) is may be caused by a stronger fixation on the lid disease itself. It seems necessary to document objective data for that reason.

Salivary gland mucosa-associated lymphoid tissue (MALT) lymphomas (SGML) are rare, as are data concerning their behaviour. We analysed clinical features at presentation, particularly the association with Sjoegren syndrome (SS) and hepatitis C virus (HCV) infection, and outcome in 33 cases of SGML diagnosed between March 1985 and April 2003. There were five males and 28 females, with a median age of 61 years. At presentation, 12/33 (36%) had multiple salivary glands or mucosal involvement and four had bone marrow infiltration. Ann Arbor stage was IE in 15 (46%), IIE in four (12%) and IV in 14 patients (42%). Fifteen patients had a history of SS (46%), two of other autoimmune diseases, seven of HCV infection. No case had both SS and HCV. Of the 29 treated patients, 17 received surgery or local radiotherapy; 69% achieved complete remission. Histological transformation occurred in four (12%). Five patients died (three of lymphoma, two of unrelated causes). The 5 year-overall survival (OS), cause-specific survival and progression-free survival was 85 +/- 8%, 94 +/- 6% and 65 +/- 10% respectively. Overall, the disease course was indolent, despite the advanced stage at diagnosis, and local therapy often appeared to be adequate. The only prognostic factors influencing OS were histological transformation and age. The close association of SGML with either autoimmune diseases or HCV infection in our series (73%) confirms their possible role in the pathogenesis of these lymphomas.

Purpose: This was a prospective case-control study conducted to evaluate the effectiveness of the Tear Stability Analysis System (TSAS) for the assessment of patients with dry eye., Methods: The TSAS can take 10 consecutive corneal topograms at one per second for 10 seconds. Examinations using the TSAS were conducted in 26 eyes of 26 healthy control subjects and in 27 eyes of 27 patients with dry eye. Examinations were also conducted in 14 eyes of 14 patients before and after the insertion of punctum plugs. Surface regularity and asymmetry indices (SRI, SAI), as well as new tear stability regularity and asymmetry indices (TSRI, TSAI), derived from SRI and SAI, were analyzed., Results: The mean SRI and SAI in dry eyes were significantly greater than in control eyes (P < 0.05). The time-wise change of SRI and SAI was significantly different between dry eyes and control eyes (P < 0.05). TSRI and TSAI in dry eyes were also significantly greater than in control eyes. Punctum plug insertion was associated with a significant decrease in SRI and SAI (P < 0.05)., Conclusions: TSAS was effective in objectively assessing the tear stability in patients with dry eye. This system may be useful in noninvasive diagnosis of dry eye and evaluation of treatment effects.

Objective: To compare the efficacy, retention rates, and complications of two types of silicone lacrimal punctal plugs in patients with or without Sjögren syndrome., Methods: We studied 36 patients with keratoconjunctivitis sicca (KCS) including 17 cases with Sjögren syndrome (SS) and 19 without SS. The fluorescein and rose bengal staining scores and the Schirmer values with and without nasal stimulation were evaluated before and after insertion of the Eagle Plugs and the punctal plugs (FCI Punctal Plugs). The retention rates and complications of these plugs were also investigated., Results: The staining scores were significantly improved after the insertion of the plugs, but the Schirmer values did not increase significantly in either SS or non-SS patients. A spontaneous loss of the plugs was observed in 29% of all plugs within 1 month after insertion. The Eagle Plugs were lost more frequently, and plugs in the upper punctum were lost more often for the Eagle Plugs. There was one case of granulomatous proliferation and two cases of punctal infection with the FCI Punctal Plugs., Conclusions: Both types of punctal plugs led to an improvement of the fluorescein and rose bengal staining scores in eyes with KCS. The difference in the retention rate and complications between the two types of plugs was probably caused by the differences in the material and the design of the plugs. Close monitoring is necessary to check for loss of plugs and to prevent complications.

A 61-year-old woman, who had been diagnosed as having Sjögren's syndrome, developed an anterior mediastinal mass. She was diagnosed with Sjögren's syndrome with thymoma, preoperatively. Extended thymectomy was performed. Macroscopically, the mediastinal mass showed thick-walled multiloculated cavities filled with turbid yellow fluid. Microscopically, the cyst lining was continuous with thymic lobules in the wall with inflammatory process, cholesterol granuloma formation, and prominent lymph follicular hyperplasia. She was diagnosed with multilocular thymic cysts associated with Sjögren's syndrome.

Sjögren's syndrome is an important autoimmune disease in the head and neck. Patients have an increased arrival risk of up to 6% per year for developing B-cell lymphomas, including mucosa-associated lymphoid tissue (MALT) lymphomas. The following case report shows this relation and the difficulty of differentiating clinically recurrent swelling of the parotid gland in Sjögren's syndrome from malignant lymphoma. A 64-year-old woman had a 2-year history of indolent, recurrent swelling of both parotid glands. Blood examination showed elevated ESR and a hypergammaglobulinemia. Immunosuppressive therapy produced no improvement. Two years after the diagnosis of Sjögren's syndrome, swelling of the left parotid gland persisted. Superficial parotidectomy of the left side was performed and histopathological examination revealed a MALT-related lymphoma. Subsequent parotidectomy of the right side also showed infiltration of the gland by a MALT lymphoma. Postoperative radiation therapy was given. During the follow-up period no recurrence or systemic disease was detected. Patients with Sjögren's syndrome should be examined regularly by the otolaryngologist. If a lymphoma cannot be ruled out, open biopsy must be considered for histological diagnosis. Prognostic factors for developing a lymphoma are possibly a high ESR and hypergammaglobulinemia. Further prognostic factors have to be evaluated.

Implant-supported prostheses offer a solution to the problems experienced by edentulous patients with Sjögren's syndrome. These patients often find it very difficult, if not impossible, to wear conventional complete dentures. Three clinical reports provide an insight into some of the difficulties involved in treating patients afflicted with this complex multifactorial disease using the Brånemark system.

We describe a case of a young patient with multiple occlusions of major cerebral arteries and Sjögren's syndrome. This 17-year-old female patient experienced repeated transient ischemic attacks of right hemiparesis, speech disturbance, and unconsciousness. Angiography revealed progressive occlusion of the bilateral carotid and vertebral arteries. Examinations, including a serological test, a rose bengal test, Ga scintigraphy, and a biopsy of the parotid gland, indicated Sjögren's syndrome. The patient was successfully managed with bypass surgery. Patients with Sjögren's syndrome may experience progressive occlusion of the major cerebral arteries resembling that of moyamoya disease.

We report the case of a fetus diagnosed at 24 weeks' gestation with complete heart block associated with maternal collagen vascular disease and Sjögren's antibody. Serial fetal echocardiograms noted increased echogenicity along the tricuspid and mitral valves without insufficiency. Postnatally, severe tricuspid insufficiency occurred following placement of an endocardial pacing lead, and severe mitral insufficiency occurred acutely at 2.5 months of age. Both valves were noted at surgery to have fibrotic and calcific chordae tendineae that had ruptured from their papillary muscles. An inflammatory reaction within the fetal heart related to transplacental passage of Sjögren's antibody was likely responsible.

After a delay of 7 to 21 years, bilateral parotidectomy with complete preservation of the facial nerves and myoplasty of the post-operative cavities for enlarged parotid glands due to Sjögren's syndrome was found to have been beneficial in 10 patients. Excellent results were obtained both in terms of aesthetic and psychic outcome, but also in terms of greatly improved general health. Patients' psychoaffective, familial, professional and social lifestyle had returned to normal.

One hundred sixty patients with Sjogren's syndrome have been evaluated and managed at Scripps Clinic. Objective diagnosis has relied heavily on rose-bengal vital staining and corneal slit lamp examination to establish the presence of KCS and lip biopsy. The role of the head and neck surgeon in evaluating the patient with "dry mouth" is discussed. Usually Sjogren's syndrome is managed nonsurgically, but problems of abscess, recurrent infection, disfigurement, and malignant transformation may result in the need for total parotidectomy with nerve preservation. Radiation for Sjogren's syndrome is rarely, if ever, indicated. The etiology of Sjogren's syndrome may be closely tied to the homogeneous genetic background of its patients and the presence of a chronic immunogenic stimulus well recognized in the secondary form but less clear in the primary.