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ID PMID Title PublicationDate abstract
18938274 Unstimulated whole saliva flow rate in relation to sicca symptoms in Hungary. 2008 Jul OBJECTIVE: To assess the prevalence of xerostomia and the related oral and extraoral dryness symptoms in Hungary, to evaluate the association of those symptoms with the unstimulated whole saliva (UWS) flow rate, and to find correlation between the level of UWS flow rate and the oral health status of the questioned patients. SUBJECTS AND METHODS: A total of 600 patients between the age of 18 and 92 years, 265 male, 335 female, were selected in accordance with the current regional age and residence distribution scheme of the Hungarian Statistical Office. A questionnaire was designed to determine the subjective presence or absence of the sicca symptoms. UWS flow rate and the dental and periodontal status were determined. RESULTS: The percentages of subjective symptoms in the questioned subjects were oral dryness, 34%; reduced salivation, 11%; mucous saliva, 15%; dysphagia, 13%; glossopyrosis, 7%; dysphonia, 31%; dysgeusia, 9%; nasal dryness, 32%; ocular dryness, 21%; itching, 40% and xeroderma, 60%. Vaginal dryness was 14%, vaginal itching was 16% in the interviewed women. The grade of xerostomia, dysphagia, tiredness, and additionally the gingival bleeding index showed a negative correlation with the UWS flow rate. After all decayed, missing and filled teeth (DMF-T) mean values, gingival bleeding index and plaque index were significantly higher in hyposalivators, compared with those who had normal flow rates CONCLUSION: This cross sectional study, representative of the Hungarian population, clearly shows that one-third of the adult population suffers from xerostomia. The clinical severity of the xerostomia demonstrated a strong relationship with the lower levels of UWS flow rate. Reduced levels of UWS flow rate in this study were also shown to be associated with dysphagia, fatigue, and increased DMF-T numbers. The data show that oral dryness, its associated desiccation symptoms and its clinical manifestations are significant health problems in Hungary.
18808771 Bilateral optic neuropathy revealing Sjögren's syndrome. 2008 Dec INTRODUCTION: The central nervous system involvement has been reported in 20% of cases of primary Sjogrën's syndrome (SS), a chronic autoimmune disease characterized by a disorder of the exocrine glands secondary to progressive lymphocyte infiltration. Classically described neurological manifestations include sensorimotor deficits, aseptic meningitis or meningoencephalitis, multiple sclerosis-like syndromes and myeolopathies. OBSERVATION: We report here the case of a 53-year-old woman who exhibited rapidly progressive visual loss, disclosing bilateral optic neuropathy, as an uncommon initial symptom of primary SS. Examination of CSF revealed associated aseptic meningitis. Because of the lack of efficacy of the first treatment by intravenous corticosteroids, monthly intravenous cyclophosphamide was quickly introduced. After six months, significant visual recovery was observed. CONCLUSION: Optic neuropathies have been rarely reported as the initial symptom revealing primary Sjogrën syndrome, and bilateral simultaneous lesions remain exceptional.
18709689 Salivary resistin reflects local inflammation in Sjögren's syndrome. 2008 Oct OBJECTIVE: To assess the role of resistin in primary Sjögren's syndrome (pSS) and its relation to local inflammation. METHODS: Blood and saliva were collected from 37 patients with pSS (duration of symptoms 12.6+/-1 yrs) and 32 healthy controls. Expression of resistin in salivary glands was visualized immunohistologically, and levels of resistin were detected by ELISA. Levels of resistin were evaluated at baseline and following oral dehydroepiandrosterone (DHEA) treatment (50 mg/day). The effect of DHEA treatment on the secretion of resistin was assessed in vitro in human leukocytes after challenge with insulin and lipopolysaccharide. RESULTS: Levels of resistin in saliva were significantly higher in patients with pSS than in controls, while circulating levels of resistin were similar in both groups. Resistin was expressed in the epithelial cells of striated ducts and in the lymphocytic foci. Resistin levels in saliva were related to the intensity of inflammation in the minor salivary glands of pSS patients. No changes of the levels of resistin in blood or saliva were observed during DHEA treatment. Exposure of naive leukocytes to DHEA in vitro induced significant expression of resistin compared to nonstimulated peripheral blood mononuclear cells (p=0.031). CONCLUSION: We showed that levels of resistin are upregulated locally in the salivary glands of patients with pSS; and that the levels of resistin correspond to the intensity of lymphocytic inflammation in patients with pSS. We suggest that resistin is expressed in the salivary glands of patients with pSS and may be a driving factor of local inflammation.
18704825 Sjogren's syndrome complicated with IgA nephropathy and leukocytoclastic vasculitis. 2008 We report a case of primary Sjogren's syndrome (SS) with cutaneous leukocytoclastic vasculitis and IgA nephropathy. The accurate diagnosis of SS was established based on objective signs and symptoms of ocular and oral dryness, a characteristic appearance of a biopsy sample from a minor salivary gland, and the presence of anti-SS-A autoantibody. A second autoimmune disorder was not present, so the diagnosis of primary SS was established. A histologic finding of skin biopsy of purpuric lesion was typical for leukocytoclastic vasculitis. Renal biopsy was performed for nephrotic range proteinuria. The pathologic finding of renal biopsy was IgA glomerulonephritis with crescent formation. The patient was treated with small doses of glucocorticoids and maintenance hemodialysis. Leukocytoclastic vasculitis is one of the most characteristic extraglandular manifestations of SS. However, IgA nephropathy associated with SS and leukocytoclastic vasculitis is a rare finding. SS patients with glomerulonephritis present a more diverse outcome, even requiring hemodialysis. Therefore, renal biopsy is warranted in SS with glomerulonephritis and systemic vasculitis.
18329915 Sonographic diagnosis of Sjögren syndrome: evaluation of parotid gland vascularity as a d 2008 Oct OBJECTIVE: To evaluate the usefulness of the vascularity in parotid glands in sonographic diagnosis for Sjögren syndrome. STUDY DESIGN: Sonographic images of 72 cases of previously suspected Sjögren syndrome (including 43 actual cases) were analyzed retrospectively for the abnormal vascularity in the parotid gland parenchyma. The relationships between the vascularity and the results of sialographic, serologic, and histopathologic examinations were analyzed. We also compared the diagnostic accuracy of B-mode only with that of B-mode plus Doppler-mode. RESULTS: Sjögren-positive cases showed significantly higher vascularity. As the grade of vascularity became higher, the rate of the Sjögren-negative cases became lower. The highest mean vascular score could be observed both in the initial stage and in the cavitary-destructive stage in the sialographic grades. Sensitivity and accuracy were markedly improved with vascular information. CONCLUSION: By using vascular information, sonographic diagnosis for Sjögren syndrome can be improved.
17523077 The role of conjunctival epithelial cell xanthine oxidoreductase/xanthine oxidase in oxida 2007 Sep Previous papers examined lipid peroxidase levels and myeloperoxidase activity as products of oxidative and inflammatory reactions in the tear fluid of patients suffering from dry eye. The aim of the present paper was to investigate whether the enzymes xanthine oxidoreductase/xanthine oxidase known to generate reactive oxygen species contribute to oxidative reactions on the ocular surface. Xanthine oxidoreductase/xanthine oxidase were examined immunohistochemically as well as histochemically in conjunctival epithelial cells of patients suffering from dry eye. Patients with verified autoimmune dry eye (Sjögren's syndrome) participated in our study; normal eyes served as controls. Conjunctival epithelial cells were obtained by the method of impression cytology using Millicell membranes. The results revealed a pronounced expression, as well as activity of xanthine oxidoreductase/xanthine oxidase in the conjunctival epithelium of dry eye. It is suggested that reactive oxygen species which are generated by this enzymatic system, contribute to oxidative reactions on the eye surface of patients with ocular manifestations of autoimmune disease (Sjögren's syndrome).
16362447 Seronegative Sjögren syndrome with asymptomatic autoimmune sclerosing pancreatitis. 2007 Jan We report two elderly patients with seronegative Sjögren syndrome who showed benign swelling of the pancreas on computed tomography. Immunostaining of the biopsied lip tissue or serum examination confirmed an increase in production of IgG4, leading to a diagnosis of autoimmune sclerosing pancreatitis (ASP) as a cause of the asymptomatic swelling of the pancreas. Sicca symptoms and ASP spontaneously improved in one patient, and the other responded well to oral prednisolone. Seronegative Sjögren syndrome and ASP can concurrently occur as a clinical manifestation of the IgG4-related systemic disorder, particularly in elderly subjects, and, in such a case, corticosteroid may be a potent therapeutic option.
17992596 The future of biologic agents in the treatment of Sjögren's syndrome. 2007 Jun The gain in knowledge regarding the cellular mechanisms of T and B lymphocyte activity in the pathogenesis of Sjögren's syndrome (SS) and the current availability of various biological agents (anti-TNF-alpha, IFN- alpha, anti-CD20, and anti-CD22) have resulted in new strategies for therapeutic intervention. In SS, various phase I and II studies have been performed to evaluate these new strategies. Currently, B cell-directed therapies seem to be more promising than T cell-related therapies. However, large, randomized, placebo-controlled clinical trials are needed to confirm the promising results of these early studies. When performing these trials, special attention has to be paid to prevent the occasional occurrence of the severe side effects.
18043395 IgG4-positive pulmonary disease. 2007 Nov We report herein high-resolution computed tomography findings from a patient with IgG4-related pulmonary disease for the first time. The 61-year-old male patient complained of low-grade fever, dry mouth, and night sweats. He was diagnosed as having autoimmune pancreatitis, Sjögren syndrome, and elevated serum IgG4. High-resolution computed tomography of the lungs showed dense alveolar consolidation and air bronchograms in bilateral perihilar regions. IgG4-positive lymphoplasmacytes were detected in pulmonary lesions by immunostaining of biopsy samples. IgG4-related pulmonary disease can be associated with various radiologic findings.
16697772 Characterization of peripheral natural killer cells in primary Sjögren's syndrome: impair 2006 May The aim of this study was to compare the number of peripheral blood natural killer (NK) cells, NK cell activity, expression of NK cell activating receptors, and serum cytokine levels in patients with primary Sjögren's syndrome (SS) vs normal controls. The authors found that NK cell number, NK cell killing activity, and the expression of activating receptors CD2 and NKG2D were significantly decreased, and the expression of NKp46, as well as the percentage of apoptotic NK cells, were significantly increased in primary SS patients compared with healthy controls. NK cell killing activity on a per-cell basis was similar in primary SS patients and healthy controls. Moreover, the levels of IL-18 and TNF-alpha, cytokines that have been shown to promote NK cell death, were significantly increased in sera from patients with primary SS compared with controls. These data suggest that reduced NK cell numbers, probably a result of apoptotic death, may contribute to impaired NK cell activity in patients with primary SS.
16512445 [MALT-lymphomas in Sjogren's disease]. 2006 AIM: To develop algorithm of early diagnosis of extranodal lymphoma arising in patients with Sjorgen's disease (SD). MATERIAL AND METHODS: SD diagnosis was made in 457 patients treated in Rheumatology Institute clinic in 1999-2004, 38 (8.3%) females aged 19-82 had lymphoproliferative diseases. MALT-lymphomas were diagnosed in 15 (42.2%) patients. All the patients have undergone morphological, immunomorphological investigations of the salivary glands, postoperative material was analysed in some patients. In addition, the following investigations were made: ultrasonography of the salivary glands, lymph nodes, viscera; scintigraphy; trephine biopsy of the bone marrow; myelograms; CT of the chest, abdomena and brain; tests for monoclonal immunoglobulins in the serum and light chains in urine; biopsy of the parotid gland. Clinical, morphological and immunophenotypical characteristics of MALT-lymphomas were assessed by WHO classification. Lymphoma stages were classified according to Ann Arbor. RESULTS: Parotid glands were affected with MALT-lymphoma most frequently. Predominant were extranodal lymphomas of the parotid submandibular, minor salivary glands of the lip and lacrimal glands of stage I E-II E. Extranodal lymphoma with nodal lesion of stage IV occurred less frequently. Untreated long existing MALT-lymphomas of the parotid glands may transform into B-large cell lymphomas deteriorating SD prognosis. The presence of long-term (> 12 months) massive enlargement of parotid/submandibular salivary and lacrimal glands, massive infiltration, monoclonal immunoglobulins in blood serum and their light chains in the urine predict development of MALT-lymphoma in SD. CONCLUSION: In SD, MALT-lymphomas develop primarily in target organs--salivary and lacrimal glands. SD patients with persistent enlargement of the parotid glands need biopsy for early detection of malignant lymphoproliferation.
18537651 Treatment of central nervous system involvement associated with primary Sjögren's syndrom 2008 Sjögren's syndrome (SS) is a chronic autoimmune disease that mainly affects the exocrine glands and usually presents with sicca symptoms of the main mucosal surfaces. The prevalence and the type of central nervous system (CNS) tissue damage caused by SS are debatable. The wide spectrum of CNS manifestations, different classification criteria used and unclear inclusion or exclusion criteria pose some difficulty reviewing these studies. Careful examination of the SS patients and to be aware of neurological findings which may be associated with suspicious CNS involvement is highly important. Central nervous system may also hypothetically have a role in the pathophysiology of SS. The wide spectrum of CNS involvement includes focal (sensorial and motor deficits, brain stem, cerebellar lesions, seizure, migraine etc.) or non-focal (encephalomyelitis, aseptic meningitis, neuropsychiatric dysfunctions), spinal cord (myelopathy, transverse myelitis, motor neuron disease etc.) findings or multiple sclerosis-like illness and optic neuritis. Evolving imaging techniques such as single photon emission computed tomography (SPECT), magnetic resonance spectroscopy or magnetization transfer imaging are promising for better understanding the nature of CNS involvement in SS. Treatments usually comprise symptomatic approach in milder cases however, pulse cyclophosphamide and steroids or other immunosuppressants (chlorambucil or azathioprine) are required in cases with progressive symptoms leading to neurological impairment. Anti-TNF agents (infliximab and etanercept) and B cell targeted therapies (rituximab and epratuzumab) are used in primary SS however their efficacy on CNS manifestation is still unclear. Randomized, multicenter studies are warranted to confirm the efficacy of treatment regimes which were reported to be effective in anecdotal reports or in small uncontrolled series. This article reviews the clinical approach to current therapy of CNS involvement in patients with primary SS.
18368937 [Diagnostic validity of dynamic salivary gland scintigraphy with ascorbic acid stimulation 2008 Jan BACKGROUND/AIM: Beside many actual groups of classification criteria, uniform classification criteria for Sjögren's syndrome (SS) are still missing. The ophtalmic component of SS is well defined. Criteria for classifying its oral component remain controversial. The fifth item of the European Union and the United States of America (EU-US) revised diagnostic classification criteria in 2002, is an objective evidence of xerostomia, diagnosed by one of the tests: unstimulated whole sialometry (UWS), parotid sialography, and dynamic salivary gland scintigraphy (DSGS). The aim of this study was to evaluate senstitivity, specificity, positive and negative predictive value and accuracy of DSGS with ascorbic acid stimulation in detecting xerostomia in SS patients and to compare DSGS findings with UWS values. METHODS: Tests DSGS and UWS were done in 20 patients with SS and in 10 of the control subjects. The findings of DSGS were graded from 1 to 4 scintigraphie (SCT) grade 1--normal finding; SCT grade 2--moderate function damage; SCT grade 3--serious function damage, SCT grade 4--very serious function damage. UWS measured 1.5 hour after the breakfast lasted 15 minutes. UWS bellow 2.5 ml/15min min. considered pathological. RESULTS: All SS patients had pathological SCT findings. Comparing SCT grade between the patients and the control group, high statistical significance was found (p < 0.001). The estimated sensitivity of DSGS was 100%, specificity 80%, positive predictive value 91%, negative predictive value 100% and accuracy 93%. The calculated sensitivity of UWS was 75%. Salivary function damage detected by scintigraphy was in positive correlation with UWS findings. CONCLUSION: DSGS is a diagnostic test with high sensitivity, specificity, accuracy and positive and negative predictive values in detecting salivary function damage in SS patients. DSGS and UWS are very sensitive diagnostic tests for objective evidence of xerostomia, and have to be ones of the earliest investigations which shoud be performed in subjects suspected of SS. Test DSGS is more sensitive, and seems to better reflect symptoms of dry mouth than UWS.
17642049 [Sensory ganglionopathy as a manifestation of celiac disease]. 2007 Jul 16 INTRODUCTION: The neurological manifestations of celiac disease (CD) may be caused by the disease itself, by associated autoimmune diseases or by complications from the tumours that may develop in the long term. We report a case of sensory ganglionopathy associated to CD. CASE REPORT: A 59-year-old female with chronic diarrhoea and loss of weight, who visited because of a clinical picture of gait disorders that progressed to the point where she was barely able to walk. Having been diagnosed with CD, finding a sensory ganglionopathy with dysautonomia (an atypical manifestation of this disease) led to a diagnosis of associated Sjogren's syndrome (SS). CONCLUSIONS: The neurological manifestations of CD are very varied, but in the presence of a sensory ganglionopathy, a neurological picture that is atypical in this disease, it becomes necessary to suspect SS, which is an infrequent but well established association. Likewise, all patients with SS must be screened for CD, which (albeit subclinically) can be complicated in the long term by the development of tumours. The differential diagnosis of the neurological manifestations of CD and of sensory ganglionopathy, as well as the association between celiac disease and SS, is also discussed.
17337148 Ultrasonographic changes of major salivary glands in primary Sjögren's syndrome. Evaluati 2007 Sep The aim of our study was to determine the reproducibility of a new semi-quantitative scoring system based on ultrasonographic (US) evaluation of structural changes of salivary glands in primary Sjögren's syndrome (SS). US evaluation of parotid and submandibular glands was performed in 28 SS patients and 29 control subjects independently by two blinded observers. Echogenicity, delineation of glandular borders and sonographic structure (homogeneity, hypoechoic areas, hyperechoic foci) of salivary glands were semi-quantitatively assessed and the final US score calculated. Inter-observer variability was determined by Cohen's test. A high degree of inter-observer agreement was found regarding the final US score (0.90) and in the assessment of glandular homogeneity (0.90), echogenicity (0.88) and hypoechoic areas (0.88). This study showed good reproducibility of the US evaluation of salivary glands using our novel scoring system. This may have important implications on the diagnostic algorithm in patients with SS.
18431095 Secondary cutaneous nodular AA amyloidosis in a patient with primary Sjögren syndrome and 2008 Feb We describe a 62-year-old female with primary Sjögren syndrome and myopathy, severe osteoporosis, and vertebral fractures that were attributed to celiac disease. A year after the diagnosis, she developed a skin nodule on the extensor surface of her right elbow, which was due to an amyloid deposit of AA type. Amyloidosis, although relatively common in some chronic inflammatory diseases, has been uncommon in Sjögren syndrome or celiac disease. Visceral amyloid was not found in this patient.
18310733 Salivary dysfunction and quality of life in Sjögren syndrome: a critical oral-systemic co 2008 Mar BACKGROUND: The importance of oral health to systemic health and quality of life (QOL) is gaining attention. Although several studies have examined generic (general) QOL in Sjögren syndrome (SS), little information exists on the effect of oral health on QOL and relationships among self-reported oral health, systemic health and objective clinical measures of health. The authors conducted this study to characterize these relationships in a sample of patients with SS. METHODS: Thirty-nine patients with a diagnosis of SS ascertained by means of the 2002 American-European Consensus criteria completed both the Oral Health Impact Profile (OHIP-14) and the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36) QOL questionnaires. OHIP-14 measures pain; functional limitation; and psychological, emotional and social disability associated with the mouth. SF-36 measures physical and emotional health and the ability to perform usual activities. Additional measures included the number of self-reported autoimmune symptoms and an index of disease damage. Statistical analysis was performed by using hierarchical regression analysis. RESULTS: Both generic and oral health-related QOL were poor in these patients. Specifically, the findings indicated that salivary flow rate was correlated significantly with both Disease Damage Index and OHIP-14 ratings, the number of autoimmune symptoms was correlated significantly with both oral and generic QOL, and oral health accounted for a significant percentage of variance in SF-36 domains of general health and social function. CONCLUSIONS: Oral health appears to have an independent influence on general QOL in patients with SS. These findings underscore the importance of proactive dental management of the oral manifestations of SS. CLINICAL IMPLICATIONS: Dentists and physicians must work collaboratively to maintain oral health and quality of life for patients with Sjögren syndrome. The dentist should address patients' concerns of xerostomia and hyposalivation in an aggressive manner.
17341301 CTLA-4 +49A/G and CT60 gene polymorphisms in primary Sjögren syndrome. 2007 CTLA-4 encodes cytotoxic T lymphocyte-associated antigen-4, a cell-surface molecule providing a negative signal for T-cell activation. CTLA-4 gene polymorphisms have been widely studied in connection with genetic susceptibility to various autoimmune diseases, but studies have led to contradictory results in different populations. This case-control study sought to investigate whether CTLA-4 CT60 and/or +49A/G polymorphisms were involved in the genetic predisposition to primary Sjögren syndrome (pSS). We analysed CTLA-4 CT60 and +49A/G polymorphisms in a first cohort of 142 patients with pSS (cohort 1) and 241 controls, all of Caucasian origin. A replication study was performed on a second cohort of 139 patients with pSS (cohort 2). In cohort 1, the CTLA-4 +49A/G*A allele was found on 73% of chromosomes in patients with pSS, compared with 66% in controls (p = 0.036; odds ratio (OR) 1.41, 95% confidence interval (CI) 1.02 to 1.95). No difference in CTLA-4 CT60 allelic or genotypic distribution was observed between patients (n = 142) and controls (n = 241). In the replication cohort, the CTLA-4 +49A/G*A allele was found on 62% of chromosomes in patients with pSS, compared with 66% in controls (p = 0.30; OR 0.85, 95% CI 0.63 to 1.16). Thus, the CTLA-4 +49A/G*A allele excess among patients from cohort 1 was counterbalanced by its under-representation in cohort 2. When the results from the patients in both cohorts were pooled (n = 281), there was no difference in CTLA-4 +49A/G allelic or genotypic distribution in comparison with controls. Our results demonstrate a lack of association between CTLA-4 CT60 or +49A/G polymorphisms and pSS. Premature conclusions might have been made if a replication study had not been performed. These results illustrate the importance of case-control studies performed on a large number of patients. In fact, sampling bias may account for some contradictory results previously reported for CTLA-4 association studies in autoimmune diseases.
16886810 [A case of primary Sjögren' s syndrome with pathological findings resembling multicentric 2006 Jul A 49-year-old woman with primary Sjögren's syndrome a few years previously was admitted to our hospital complaining of tongue and skin eruptions, swelling of the face and neck and for examination of liver injury and hypereosinophilia. A blood test revealed leukocytosis with eosinophilia, mild liver injury, polyclonal hypergammaglobulinemia, and positive results for anti-nuclear antibody, anti-SS-A antibody and anti-SS-B antibody. Chest CT scan showed multiple nodular opacities with cavities in peripheral regions of both lungs. Biopsy specimens from the right lower lobe obtained by video-assisted thoracoscopy revealed marked infiltration of plasma cells and lymphocytes in alveolar lumina, lymph follicles with germinal centers in other areas of the pulmonary parenchyma, and lymphocytes infiltrate in alveolar wall adjacent bronchi and bronchioles. The histological diagnosis was pulmonary involvement of multicentric Castleman's disease. This was a rare case of Sjögren's syndrome accompanied by pathological findings of multicentric Castleman' s disease showed multiple nodular opacities in chest CT scans.
16831312 Sjogren's syndrome in a celiac patient: searching for environmental triggers. 2006 Apr In the last few years many studies have shown the potential role of different triggers in the pathogenesis of several autoimmune diseases. In particular, in Sjogren's syndrome the presence of a genetic background is considered determining, but environmental factors have recently been described as triggers or precipitators. In this report, we describe the case of a young woman affected by celiac disease in which an Ascaris lumbricoides infestation and estrogen therapy could have played a role in the development of Sjogren's syndrome.