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Sjögren's Syndrome (sjögren + syndrome)
Kinds of Sjögren's Syndrome Terms modified by Sjögren's Syndrome Selected AbstractsIs there progressive cognitive dysfunction in Sjögren Syndrome?ACTA NEUROLOGICA SCANDINAVICA, Issue 3 2010A preliminary study Martínez S, Cáceres C, Mataró M, Escudero D, Latorre P, Dávalos A. Is there progressive cognitive dysfunction in Sjögren Syndrome? A preliminary study. Acta Neurol Scand: 122: 182,188. © 2010 The Authors Journal compilation © 2010 Blackwell Munksgaard. Objective,,, The aim of this study was to determine the progression of cognitive dysfunction in primary Sjögren Syndrome (SS). Methods,,, Twelve subjects with SS were compared with ten subjects with migraine and ten healthy controls on neuropsychological, mood and fatigue tests at baseline and 8 years later. Results,,, At follow-up, SS subjects performed below subjects with migraine on the Continuous Performance Test (CPT) but did not differ on other tasks. Compared with controls, both clinical groups obtained lower scores on simple reaction time, patients with SS obtained lower scores on the Wisconsin Card Sorting Test (WCST) and patients with migraine performed below controls on the Benton's Judgment of Line Orientation Test (JOLO). Clinical groups did not differ on cognitive changes over time, except that migraine subjects improved on verbal fluency. Compared with baseline, both SS and migraine patients were more impaired on simple reaction time, Trail Making Test part B, Stroop and JOLO. However, they showed higher scores on verbal and visual memory, WCST and CPT reaction time. SS also showed higher levels of depression and fatigue than migraine and controls, with no significant changes over time. Discussion,,, Preliminary evidence indicates some cognitive deficits in both SS and migraine following a pattern of fronto-subcortical dysfunction without a significant cognitive decline over time. [source] Preferential recognition of the phosphorylated major linear B-cell epitope of La/SSB 349,368aa by anti-La/SSB autoantibodies from patients with systemic autoimmune diseasesCLINICAL & EXPERIMENTAL IMMUNOLOGY, Issue 3 2006A. G. Terzoglou Summary Sera from patients with primary Sjögren Syndrome (pSS) or Systemic Lupus Erythematosus (SLE) often contain autoantibodies directed against La/SSB. The sequence 349,368aa represents the major B-cell epitope of La/SSB, also it contains, at position 366, a serine aminoacid residue which constitutes the main phosphorylation site of the protein. In this study we investigated the differential recognition of the 349,368aa epitope and its phosphorylated form by antibodies found in sera from patients with systemic autoimmune diseases. Peptides corresponding to the sequence of the unphosphorylated (pep349,368aa) and the phosphorylated form (pep349,368aaPh) of the La/SSB epitope 349,368aa, as well as to a truncated form spanning the sequence 349,364aa and lacking the phosphorylation site (pep349,364aa), were synthesized. Sera from 53 patients with pSS and SLE with anti-La/SSB specificity, 30 patients with pSS and SLE without anti-La/SSB antibodies, 25 patients with rheumatoid arthritis and 32 healthy individuals were investigated by ELISA experiments. Autoantibodies to pep349,368aaPh were detected in sera of anti-La/SSB positive patients with a higher prevalence compared to the pep349,368aa (66%versus 45%). Pep349,368aaPh inhibited the antibody binding almost completely (92%), while pep349,368aa inhibited the binding only partially (45%). Anti-La/SSB antibodies presented a higher relative avidity for the phosphorylated than the unphosphorylated peptide. Immunoadsorbent experiments using the truncated peptide pep349,364aa indicated that the flowthrough showed a selective specificity for pep349,368aaPh, while the eluted antibodies reacted with both peptide analogues of the La/SSB epitope. These data suggest that sera from pSS and SLE patients with anti-La/SSB reactivity possess autoantibodies that bind more frequently and with a higher avidity to the phosphorylated major B-cell epitope of the molecule. [source] The heat shock protein 70 molecular chaperone network in the pancreatic endoplasmic reticulum , a quantitative approachFEBS JOURNAL, Issue 19 2007Andreas Weitzmann Traditionally, the canine pancreatic endoplasmic reticulum (ER) has been the workhorse for cell-free studies on protein transport into the mammalian ER. These studies have revealed multiple roles for the major ER-luminal heat shock protein (Hsp) 70, IgG heavy chain-binding protein (BiP), at least one of which also involves the second ER-luminal Hsp70, glucose-regulated protein (Grp) 170. In addition, at least one of these BiP activities depends on Hsp40. Up to now, five Hsp40s and two nucleotide exchange factors, Sil1 and Grp170, have been identified in the ER of different mammalian cell types. Here we quantified the various proteins of this chaperone network in canine pancreatic rough microsomes. We also characterized the various purified proteins with respect to their affinities for BiP and their effect on the ATPase activity of BiP. The results identify Grp170 as the major nucleotide exchange factor for BiP, and the resident ER-membrane proteins ER-resident J-domain protein 1 plus ER-resident J-domain protein 2/Sec63 as prime candidates for cochaperones of BiP in protein transport in the pancreatic ER. Thus, these data represent a comprehensive analysis of the BiP chaperone network that was recently linked to two human inherited diseases, polycystic liver disease and Marinesco,Sjögren syndrome. [source] Evaluation of a new automated enzyme fluoroimmunoassay using recombinant plasmid dsDNA for the detection of anti-dsDNA antibodies in SLEJOURNAL OF CLINICAL LABORATORY ANALYSIS, Issue 5 2002D. Villalta Abstract ELISA methods to detect anti-double-stranded DNA (anti-dsDNA) antibodies are highly sensitive, but are less specific for the diagnosis of SLE than the immunofluorescence test on Crithidia luciliae (CLIFT) and the Farr assay because they also detect low-avidity antibodies. This study evaluated the specificity, sensitivity, positive predictive value (PPV), and negative predictive value (NPV) of a new automated fluoroimmunoassay (EliA dsDNA; Pharmacia, Freiburg, Germany). We compared the results with those obtained using a commercial CLIFT and an in-house anti-dsDNA IgG ELISA method, and verified its putative ability to detect only high-avidity anti-dsDNA antibodies. Sera from 100 SLE patients and 120 controls were studied. The control group included 20 healthy donors, 70 patients with other rheumatic diseases (32 systemic sclerosis (SSc); 18 primary Sjögren syndrome (pSS), 20 rheumatoid arthritis (RA)), and 30 patients with various infectious diseases (ID). Anti-dsDNA avidity was estimated using an ELISA method based upon the law of mass action, and a simplified Scatchard plot analysis for data elaboration; the apparent affinity constant (Kaa) was calculated and expressed as arbitrary units (L/U). Sensitivity, specificity, PPV, and NPV for SLE were 64%, 95.8%, 93.8% and 72.7%, respectively, for the EliA anti-dsDNA assay; 55%, 99.2%, 98.5%, and 68.8%, respectively, for the CLIFT; and 64%, 93.3%, 90.6%, and 72.3%, respectively, for the in-house ELISA. Although EliA anti-dsDNA was positive mainly in SLE patients with high- (Kaa>80 L/U) and intermediate- (Kaa 30,80 L/U) avidity antibodies (45.3% and 49.9%, respectively), it was also positive in five (7.8%) SLE patients with low-avidity anti-dsDNA antibodies, and five controls (three SSc, one pSS, and one ID) (mean Kaa = 16.4 ± 9.04 L/U). In conclusion, EliA anti-dsDNA assay showed a higher sensitivity than the CLIFT, and a good specificity and PPV for SLE. Its putative ability to detect only high-avidity anti-dsDNA antibodies remains questionable. J. Clin. Lab. Anal. 16:227,232, 2002. © 2002 Wiley-Liss, Inc. [source] Primary Gougerot,Sjögren syndrome: a dermatological approachJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 3 2006A-M Roguedas Abstract Gougerot,Sjögren syndrome (GSS) is a chronic heterogeneous non-organ-specific autoimmune disease, encompassing a wide spectrum of clinical manifestations. It is characterized by a lymphocytic infiltration of the exocrine glands, also called epitheliitis, resulting in xerostomia and keratoconjunctivitis sicca. The skin can also be involved; for example, xerosis is a consequence of epitheliitis. Dermatological consequences of polyclonal reactivity are vasculitis and manifestations of B-cell proliferation vary from plasma cell infiltrates to B-cell lymphoma. [source] Clinicopathological features of pure mica pneumoconiosis associated with Sjögren syndromeAMERICAN JOURNAL OF INDUSTRIAL MEDICINE, Issue 3 2004FCCP, Hideo Kobayashi MD Abstract Background There are few reports on the clinical, radiologic, and pathologic features of pure mica pneumoconiosis. Methods A case of definite pure mica pneumoconiosis in a rubber factory worker is reported with the clinicopathological findings. Results Chest HRCT demonstrated subpleural and peribronchovascular interstitial thickening. The characteristic histologic features were pulmonary fibrosis accompanied by prominent histiocytic granulomas containing giant cells. Conclusions Pure mica pneumoconiosis can be identified by specific radiologic and pathologic findings. Am. J. Ind. Med. 45:246,250, 2004. © 2004 Wiley-Liss, Inc. [source] Health-related quality of life in patients with Primary Sjögren's Syndrome and Xerostomia: a comparative studyGERODONTOLOGY, Issue 1 2002Jocelyne Rostron Abstract Objective: To compare the health status of groups of Primary Sjögren's and Xerostomia patients, using the Medical Outcomes Short Form 36 (SF-36). The SF-36 is a generic measure, divided into eight domains, used in the assessment of health-related quality of life. Patients and methods: The SF-36 was given to 2 groups: Group 1 comprised 43 patients diagnosed with Primary Sjögren's Syndrome (1SS) and an unstimulated whole salivary flow rate (UFR) of <0.1 ml/min). Group 2 (n = 40) reported Xerosiomia but had an UFR >0.2 ml/min. Sub groups of patients in Groups 1 and 2 were compared with community normative data, for the SF-36 Results: There were trends to suggest lower SF36 scores for 1SS patients but there were no significant differences between the mean domain scores of Groups 1 and 2. 1SS and Xerostomia patients registered lower mean scores across all 8 domains, compared with normative community data. Conclusion: The SF-36 was unable to detect significant differences between subjects with 1SS and Xerostomia but a larger sample size is required to confirm these findings. The results of this limited study suggest that a disease-specific measure is required to assess the impact 1SS on health-related Quality of life (QOL). [source] Clinical and microbiological studies of periodontal disease in Sjögren's syndrome patientsJOURNAL OF CLINICAL PERIODONTOLOGY, Issue 2 2002B. Kuru Abstract Background: Little is known about the periodontal status of patients with Sjögren's Syndrome (SS), a chronic inflammatory autoimmune disease characterized by xerophthalmia and xerostomia. The aim of the present study was to evaluate whether the periodontal status of SS patients, in terms of clinical and microbiological parameters, differs from systemically healthy age- and gender-matched controls. Methods: 8 primary SS and 10 secondary SS patients were examined in comparison with 11 control subjects. All patients were diagnosed by the European Community Criteria. Control subjects were systemically healthy and not undergoing periodontal treatment. The comparison of clinical status was made in terms of mean periodontal parameters (plaque index, gingival index, gingival recession, probing pocket depth, probing attachment level and bleeding on probing) as well as the frequency distribution of probing pocket depth and probing attachment level measurements. Microbiological assays of the subgingival dental plaque samples were carried out by both a chairside enzyme test (Periocheck®) for the detection of peptidase activity (PA) and a polymerase chain reaction (PCR) analysis for 9 selected periodontal micro-organisms (Actinobacillus actinomycetemcomitans, Fusobacterium nucleatum, Prevotella intermedia, Treponema denticola, Porphyromonas gingivalis, Eikenella corrodens, Campylobacter rectus, Bacteroides forsythus, Streptococcus oralis). Results: The occurrence, severity and extent of periodontal lesions were not significantly different between the 3 patient groups for all periodontal parameters examined. No significant differences in the sub-gingival plaque samples from control, primary or secondary SS patients for the PA test, frequency or type of periodontal micro-organisms observed. Conclusion: No significant differences could be detected in either clinical or microbiological parameters of primary or secondary SS patients compared with that of control subjects. The results of the present study thus support the notion that the periodontal status of patients with SS do not differ from systemically healthy age- and gender-matched controls. Zusammenfassung Hintergrund: Es ist wenig über den parodontalen Status von Patienten mit Sjögren Syndrom (SS) bekannt, einer chronischen entzündlichen Autoimmunerkrankung, die durch Xerophtalmie und Xerostomie charakterisiert ist. Das Ziel der vorliegenden Studie war zu überprüfen, ob der parodontale Status der SS-Patienten bi Berücksichtigung der klinischen und mikrobiologischen Parameter von demjenigen bei systemisch gesunden alters- und geschlechtspassenden Kontrollen abweicht. Methoden: 8 primäre SS und 10 sekundäre SS Patienten wurden mit 11 Kontrollpersonen vergleichend untersucht. Alle Patienten waren durch Kriterien der EU diagnostiziert. Die Kontrollpersonen waren systemisch gesund und erhielten keine parodontale Behandlung. Der Vergleich des klinischen Status wurde auf der Basis von mittleren parodontalen Parametern (Plaque-Index, Gingivaindex, gingivale Rezession, Sondierungstiefe, Stützgewebeniveau, Provokationsblutung) sowie der Verteilungsmuster der Sondierungstiefe und des Stützgewebeniveaus vorgenommen. Mikrobiologische Assay's von subgingivalen Plaqueproben wurden sowohl mit einem chairside Enzymtest (Periocheck®) für die Feststellung der Peptidaseaktivität (PA) und einer Polymerasekettenreaktion (PCR) für 9 selektierte parodontale Mikroorganismen (Actinobacillus actinomycetemcomitans, Fusobacterium nucleatum, Prevotella intermedia, Treponema denticola, Porphyromonas gingivalis, Eikenella corrodens, Campylobacter rectus, Bacteroides forsythus, Streptococcus oralis) durchgeführt. Ergebnisse: Das Vorkommen, die Schwere und die Ausdehnung von parodontalen Läsionen unterschied sich nicht signifikant zwischen den 3 Patientengruppen für alle geprüften parodontalen Parameter. Es gab auch keine signifikanten Differenzen in den subgingivalen Plaqueproben von den Kontrollen, den primären oder sekundären SS Patienten für die PA Teste und Frequenz oder Art von beobachteten parodontalen Mikroorganismen. Schlussfolgerung: Es konnten keine signifikanten Differenzen sowohl bei den klinischen oder mikrobiologischen Parametern von primären oder sekundären SS Patienten im Vergleich mit Kontrollpersonen entdeck werden. Die Ergebnisse der vorliegenden Studie unterstützen die Ansicht, dass sich der parodontale Status von Patienten mit SS nicht von demjenigen gesunder alters- und geschlechtspassender Kontrollen unterscheidet. Résumé Origine: On en sait peu sur l'état parodontal des patients atteints du syndrome de Sjögren (SS), une maladie chronique autoimmune inflammatoire caractérisée par une xérophtalmie et une xérostomie. Le but de cette étude était d'évaluer si l'état parodontal des patients SS, en terme de paramètres cliniques et microbiologiques était différent de sujets contrôles en bonne santé générale du même âge et du méme sexe. Méthodes: 8 patients atteints de SS primaires et 10 de SS secondaires furent examinés et comparés avec des sujets contrôles. Tous les patients étaient diagnostiqués selon les critères de la communauté européenne. Les sujets contrôles étaient en bonne santé générale et ne suivaient pas de traitement parodontal. La comparaison des états parodontaux fut réalisée pour les paramètres cliniques moyens (indice de plaque, gingival, récession gingivale, profondeur de poche au sondage, niveau d'attache et saignement au sondage) et aussi pour la frèquence de distribution des mesures des profondeurs de poche au sondage et des niveaux d'attache. Les tests microbiologiques des échantillons de plaque sous-gingivale ont été réalisés à la fois par un test enzymatique au fauteuil (Periocheck®) pour la détection de l'activité peptidase (PA) et par réaction de polymérase en chaine (PCR) pour 9 micro-organismes parodontaux sélectionnés (Actinobacillus actinomycetemcomitans, Fusobacterium nucleatum, Prevotella intermedia, Treponema denticola, Porphyromonas gingivalis, Eikenella corrodens, Campylobacter rectus, Bacteroides forsythus, Streptococcus oralis). Résultats: La survenue, la sévérité et l'étendue de la maladie parodontale n'étaient pas significativement différente entre les 3 groupes de patients pour tous les paramètres parodontaux examinés. Aucune différence significative ne fut observée entre les échantillons de plaque sous-gingivale des contrôles et ceux des patients atteints de SS primaire et secondaire, pour PA, la frèquence ou le type de micro-organismes. Conclusions: Aucune différence significative ne put être détectée, ni pour les paramètres cliniques, ni pour les paramètres microbiologiques des patients atteints de SS primaire ou secondaire lorsque l'on comparait avec les sujets contrôles. Les résultats de cette étude corroborent ainsi l'idée suivant laquelle l'état parodontal des patients atteints de SS ne différe pas de celui des sujets en bonne santé du même âge et du même sexe. [source] Systemic increase in type,I interferon activity in Sjögren's syndrome: A putative role for plasmacytoid dendritic cellsEUROPEAN JOURNAL OF IMMUNOLOGY, Issue 7 2008Manon Abstract In the salivary glands of primary Sjögren's syndrome (pSjS) patients, type,I IFN activity is increased, but systemic levels of type,I IFN proteins are rarely detected. This study focused on the systemic activity of type,I IFN in pSjS, as well as the role of peripheral plasmacytoid dendritic cells (pDC). Monocytes obtained from pSjS patients showed an increased expression of 40,genes. Twenty-three of these genes (58%), including IFI27, IFITM1, IFIT3 and IFI44, were inducible by type,I IFN. pSjS serum had an enhanced capability of inducing IFI27, IFITM1, IFIT3 and IFI44 in the monocytic cell line THP-1, likely due to the action of IFN-,. This effect could be inhibited by blocking the type,I IFN receptor, supporting a high type,I IFN bioactivity in pSjS serum. In addition, circulatory pDC showed increased expression of CD40. This expression was correlated to the expression level of the type,I IFN-regulated genes IFI27 and IFITM1 in monocytes of the same individual. This study indicates that the increased type,I IFN activity observed in pSjS patients is not only a local but also a systemic phenomenon and points to pDC as a possible source of this activity. [source] Development of nephritis but not sialadenitis in autoimmune-prone BAFF transgenic mice lacking marginal zone B cellsEUROPEAN JOURNAL OF IMMUNOLOGY, Issue 9 2006Carrie Abstract B cell-activating factor belonging to the TNF family (BAFF) is a B cell survival factor required for B cell maturation. BAFF transgenic (Tg) mice develop autoimmune disorders characterized by autoantibody production, which leads to nephritis and salivary gland destruction (sialadenitis), features reminiscent of systemic lupus erythematosus and Sjögren's syndrome (SS), respectively. Disease in BAFF Tg mice correlates with the expansion of the marginal zone (MZ) B cell compartment and the abnormal presence of MZ-like B cells in the blood, LN and inflamed salivary glands, suggesting a role for these cells in BAFF-induced autoimmunity. Lymphotoxin-, (LT,)-deficient mice show disrupted splenic architecture, lack MZ B cells and some peripheral LN, and are unable to mount T cell-dependent immune responses. BAFF Tg mice lacking LT, (LT,,-BTg) retained these defects, yet still developed nephritis associated with the presence of B-1 B cells in the kidneys. However, in contrast to old BAFF Tg mice, aging LT,,-BTg mice no longer developed sialadenitis. Thus, autoimmune disorders in BAFF Tg mice are possibly events coordinated by MZ and B-1 B cells at separate anatomical sites. [source] Functional estrogen receptors alpha and beta are expressed in normal human salivary gland epithelium and apparently mediate immunomodulatory effectsEUROPEAN JOURNAL OF ORAL SCIENCES, Issue 5 2009Maria Tsinti Salivary gland epithelial cells (SGECs) have been shown to participate in immunological responses and have been implicated in the pathogenesis of Sjögren's syndrome (SS). Experimental evidence from animal models indicates that estrogen deficiency may also participate in SS pathogenesis. However, the expression and functionality of the estrogen receptors alpha (ER,) and beta (ER,) in normal human salivary epithelium is unknown. To investigate these points, formalin-fixed, paraffin-embedded specimens and cultured non-neoplastic SGEC lines derived from nine minor salivary gland (MSG) biopsies with normal histology were studied. Immunohistochemical analyses detected the epithelial expression of ER,, ER,1, and ER,2 protein isoforms both in MSG tissues and in cultured SGECs. Such epithelial expression was verified by immunoblotting of various ER proteins in cellular extracts of cultured SGECs (full-length-ER,, ER,-,3, ER,1-long, ER,1-short, and ER,2-long isoforms). Estrogens did not induce growth or apoptosis in cultured SGECs. However, similarly to other cellular systems, treatment of cultured SGECs with estrogens (17,-estradiol and the ER,- and ER,-selective agonists propylpyrazole-triol and diarylpropiolnitrile, respectively) inhibited the interferon-,-inducible expression of intercellular adhesion molecule-1. This finding corroborated the functionality of ER expressed by SGEC. Our results suggest that salivary epithelium expresses constitutively functional ER, and ER, proteins that apparently mediate immunomodulatory effects. [source] Salivary gland parameters and clinical data related to the underlying disorder in patients with persisting xerostomiaEUROPEAN JOURNAL OF ORAL SCIENCES, Issue 2 2007Ianthe Van Den Berg This study assessed salivary gland parameters and clinical data in patients referred to our clinic because of persisting xerostomia of unknown origin, in order to facilitate early diagnosis and recognition of the underlying disorder. Most patients were referred for diagnostic analysis of a possible Sjögren's syndrome (SS). A complete diagnostic work-up was available in all patients (n = 176), including data on salivary gland function, saliva composition, sialography, salivary gland swelling, pattern of complaints, general health, and medication. Patients were diagnosed with SS (n = 62), sialosis (n = 45), sodium retention syndrome (n = 30), or medication-induced xerostomia (n = 9). In 30 patients no disease related to salivary gland pathology was found. Unstimulated whole salivary flow was decreased in all patients, except in patients with sodium retention syndrome and in patients without salivary gland pathology. Submandibular/sublingual salivary flow was lowest in SS patients. SS and sialosis patients had increased salivary potassium concentrations, whereas only SS patients had increased sodium concentrations. About half of the sialosis patients mainly complained of persistent parotid gland swelling. Xerostomia-inducing medication was used by most patients. It was concluded that gland-specific sialometry and sialochemistry is useful in discriminating between the various disorders causing persisting xerostomia. [source] The health status burden of people with fibromyalgia: a review of studies that assessed health status with the SF-36 or the SF-12INTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 1 2008D. L. Hoffman Summary Objective:, The current review describes how the health status profile of people with fibromyalgia (FM) compares to that of people in the general population and patients with other health conditions. Methods:, A review of 37 studies of FM that measured health status with the 36-item Medical Outcomes Study Short-Form Health Survey (SF-36) or the 12-item Short-Form Health Survey (SF-12). Results:, Studies performed worldwide showed that FM groups were significantly more impaired than people in the general population on all eight health status domains assessed. These domains include physical functioning, role functioning difficulties caused by physical problems, bodily pain, general health, vitality (energy vs. fatigue), social functioning, role functioning difficulties caused by emotional problems and mental health. FM groups had mental health summary scores that fell 1 standard deviation (SD) below the general population mean, and physical health summary scores that fell 2 SD below the general population mean. FM groups also had a poorer overall health status compared to those with other specific pain conditions. FM groups had similar or significantly lower (poorer) physical and mental health status scores compared to those with rheumatoid arthritis, osteoarthritis, osteoporosis, systemic lupus erythematosus, myofacial pain syndrome, primary Sjögren's syndrome and others. FM groups scored significantly lower than the pain condition groups mentioned above on domains of bodily pain and vitality. Health status impairments in pain and vitality are consistent with core features of FM. Conclusions:, People with FM had an overall health status burden that was greater in magnitude compared to people with other specific pain conditions that are widely accepted as impairing. [source] Livedoid vasculopathy and hypercoagulability in a patient with primary Sjögren's syndromeINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 4 2007Raquel Cardoso MD Background, A 31-year-old woman presented with a 5-year history of painful ulcerations, palpable purpura, porcelain-white atrophic scars of the malleolar region and dorsal aspect of the feet, livedo reticularis on the limbs, arthralgia, xerophthalmia, and xerostomia. Methods, Skin biopsy revealed vessel wall hyalinization and thrombosis of the microvasculature with a very scarce dermal inflammatory infiltrate. Biopsy of the oral mucosa showed mononuclear infiltration of an intralobular duct of a salivary gland. Results, Laboratory studies, including autoantibodies and inflammation markers, were normal, except for a positive rheumatoid factor. Coagulation screening revealed C677T methylenetetrahydrofolate reductase (MTHFR) mutation, with a normal serum homocysteine. The patient was treated with oral methylprednisolone (32 mg/day with progressive reduction) and enoxaparin (20 mg/day subcutaneously), with complete ulcer healing within 4 months. Conclusion, Livedoid vasculitis or vasculopathy has not been referred to previously in association with Sjögren's syndrome, but may be associated with other autoimmune disorders and anomalies of coagulation, namely factor V Leiden mutation, protein C deficiency, and MTHFR mutation, associated or not with hyperhomocysteinemia, a condition that seems to confer an increased risk of recurrent arterial and venous thrombosis. We stress the importance of anticoagulant therapy for ulcer healing and for the prevention of other thrombotic events. [source] Breast lymphoma in Sjögren's syndrome complicated by acute monocular blindnessINTERNATIONAL JOURNAL OF RHEUMATIC DISEASES, Issue 2 2010Helmar F. SOLDEVILLA Abstract A 69-year-old hypertensive woman presented with eye and mouth dryness, bilateral parotid gland enlargement, associated with anasarca and proteinuria. Family history was notable for malignancies including breast, nasopharyngeal and colon cancers. Physical exam disclosed hypertension, bilaterally enlarged, firm, non-tender parotid glands, fine bibasilar crackles and bipedal edema. Anti Ro/Sjögren's syndrome antigen A antibody was positive, with negative tests for anti La/Sjögren's syndrome antigen B and anti-nuclear antibody (ANA). Chest radiographs showed basal infiltrates. Sjögren's syndrome associated with glomerulonephritis and interstitial lung disease was diagnosed, and she received pulse methylprednisololone followed by oral prednisone with dramatic improvement. Two months later, while on prednisone 5 mg/day, she returned to the clinic with an enlarging fixed non-tender right breast mass. She underwent modified radical mastectomy of the right breast, and pathologic report revealed diffuse, small cell, non-Hodgkin's lymphoma of the breast; axillary lymph nodes were negative for tumor. She opted for alternative therapy and did not return to the clinic until 7 months later when she developed sudden monocular blindness in the right eye with no other systemic manifestations. Magnetic resonance imaging (MRI) revealed swelling and enhancement of intracanalicular and pre-chiasmatic segments of the right optic nerve and right side of the optic chiasm. Considerations were Devic's disease versus metastases. She received pulse methylprednisolone therapy (1 g/day for 3 days) with partial recovery of vision. She is scheduled for lymphoma chemotherapy to include rituximab. [source] Association of Sjögren's syndrome and rosacea: a diagnostic challengeINTERNATIONAL JOURNAL OF RHEUMATIC DISEASES, Issue 2 2007Leopoldo Luiz Dos SANTOS-NETO Abstract Both Sjögren's syndrome and rosacea present clinical manifestations that include ocular involvement. We report a case of a 45-year-old woman with a history of persistent erythematous malar rash, associated with conjunctival hyperemia, xerophthalmia and blefaritis. The patient filled the current classification criteria proposed for Sjögren's syndrome and those for rosacea. The coexistence of these diseases has not been previously described in the literature. Both diseases have similar symptoms and different treatment approaches. We believe that it is important for clinicians to identify this association in order to provide better care for the patient. [source] Sonographic appearances of malignant lymphoma of the salivary glandsJOURNAL OF CLINICAL ULTRASOUND, Issue 9 2001Mayumi Yasumoto MD Abstract Purpose We undertook this retrospective study to describe the sonographic findings in patients with malignant lymphoma of the major salivary glands. Methods We reviewed the sonograms and medical records of 7 patients with histologically proven lymphoma of the parotid (3 patients) or submandibular glands (4 patients). Results Primary lymphoma was found in 1 parotid gland and 2 submandibular glands. The remaining 4 cases were secondary lymphomas. One patient had been diagnosed with Sjögren's syndrome and had been followed up with sonography. In parotid glands, both parenchymal and intraparotid nodal lymphomas were found. All submandibular gland tumors were parenchymal. Intraparotid nodal involvement appeared as multiple small nodules with relatively smooth margins, whereas the parenchymal parotid and submandibular gland lymphomas were larger (25 to 45 mm in longitudinal diameter) and showed various degrees of margin irregularity. All tumors were hypoechoic relative to the normal parenchyma. The primary parotid lymphoma and intraparotid nodal lymphomas had a homogeneous echotexture; the secondary parotid lymphomas and submandibular gland lymphomas were heterogeneous. One submandibular gland lymphoma showed intratumoral echogenic stripes. Neither calcification nor cystic degeneration was observed within the lesions. Conclusions Lymphomas of the salivary glands present a variety of sonographic appearances, ranging from multiple small, hypoechoic nodules to an irregularly shaped heterogeneous mass without cystic areas or calcifications. © 2001 John Wiley & Sons, Inc. J Clin Ultrasound 29:491,498, 2001. [source] Hepatitis C virus and lichen planusJOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, Issue 10 2004YUMIKO NAGAO Abstract Hepatitis C virus (HCV) is an important factor in the development of chronic liver disease and hepatocellular carcinoma. In recent years it has become known that HCV induces various extrahepatic manifestations including mixed cryoglobulinemia, membranoproliferative glomerulonephritis, Sjögren's syndrome, autoimmune thyroiditis, malignant lymphoma, porphyria cutanea tarda and lichen planus. Although the mechanisms of extrahepatic manifestations remain unclear, it is known that interferon (IFN) therapy and coadministration of IFN with ribavirin are effective in promoting the disappearance or alleviation of such extrahepatic lesions, which have tended to be overlooked. The present review focuses on lichen planus, one of the major extrahepatic manifestations. © 2004 Blackwell Publishing Asia Pty Ltd [source] Incidence of Sjögren's syndrome in Japanese patients with hepatitis C virus infectionJOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, Issue 3 2003YUMIKO NAGAO Abstract Background and Aim: Hepatitis viruses induce not only chronic liver diseases but also the impairment of other organs and tissues as extrahepatic manifestations. In particular, hepatitis C virus (HCV) is involved in various extrahepatic manifestations. The purpose of the present study was to evaluate Sjögren's syndrome (SS) and lichen planus (LP) involvement, which are various extrahepatic manifestations in patients with liver diseases related to hepatitis B virus (HBV) or HCV. Methods: We examined a total of 110 Japanese patients with chronic liver disease: 29 with HBV infections and 81 HCV infections. Results: The prevalence of SS according to European and Japanese criteria in patients with chronic HCV infection was significantly higher than in patients with chronic HBV infection (European criteria: 25.9 vs 3.4%; P < 0.05, Japanese criteria: 21.0 vs 3.4%; P = 0.05). Lichen planus was observed in one (3.4%) of 29 patients with chronic HBV infection, and in 11 (13.6%) of 81 patients with chronic HCV infection. Simultaneously combined LP and SS occurred in 8.6% (seven of 81) of patients with HCV infection, but in none with HBV infection. Conclusions: Clinicians should routinely follow the HCV-infected patients, paying sufficient attention to the presence of SS and LP, and they should also carefully monitor their prognosis. [source] Follicular dendritic cells confirm lymphoid organization in the minor salivary glands of primary Sjögren's syndromeJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 9 2008Malin V. Jonsson Background:, Sjögren's syndrome (SS) is an autoimmune chronic inflammatory disorder affecting the salivary and lacrimal glands. The aim of this study was to explore immunophenotypic features of chronic inflammatory reactions in the minor salivary glands in patients with primary SS (pSS). Methods:, Formalin-fixed, paraffin-embedded labial minor salivary gland tissue sections from randomly selected patients with pSS (n = 60) were investigated for the expression of CD21, CD23, CD35 and IgD by immunohistochemistry. Results:, Based on the distribution and staining pattern of CD21, CD23, CD35 and IgD in lymphoid aggregates, several stages of chronic inflammatory reactions were observed. In 12/60 (20%) patients, lymphoid infiltrates with germinal centre (GC)-like features such as extensive networks of CD21-, CD23- and CD35-positive cells were observed in the minor salivary gland tissue. Smaller networks and,/or focal infiltrates with scattered CD21+, CD23+ and CD35+ cells were observed in the remaining 48/60 (80,%) cases. When dividing patients according to the presence (GC+) or the absence (GC,) of GC in the minor salivary glands, the mean focus score was significantly higher in the GC+ patients (P < 0.05). Double staining of the minor salivary glands revealed focal infiltrates with follicular dentritic cell networks and B cells resembling normal GCs in tonsillar tissue. Conclusion:, A particular cellular profile was demonstrated in a sub-group of patients with pSS and could be linked to serological aberrations. These findings warrant further prospective studies. [source] Coexistence of Sjögren's syndrome and sarcoidosis: a report of five casesJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 6 2007M. J. Mansour Background:, Sjögren's syndrome (SS) and sarcoidosis are diseases that can affect the salivary glands and result in the loss of salivary gland function. Most of the criteria used for the diagnosis of SS exclude sarcoidosis before establishing the diagnosis of SS. However, several reports have suggested the coexistence of both SS and sarcoidosis in the same patient. Objective:, The purpose of this study was to present five cases that support a true coexistence of sarcoidosis and SS. Methods:, Clinical and laboratory findings of patients with evidence of having both SS and sarcoidosis were reviewed. The diagnosis of SS was based on the European community criteria; the diagnosis of sarcoidosis was based on the presence of serological, radiographic and/or histopathologic findings that are consistent with sarcoidosis. Results:, All patients fulfilled the criteria for the diagnosis of both diseases. Conclusion:, Our findings appear to support a true coexistence of sarcoidosis with SS. Therefore, it is reasonable to suggest removing the exclusion of sarcoidosis from the diagnostic criteria for SS. [source] Comparison of salivary calmodulin binding proteins in Sjögren's syndrome and healthy individualsJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 3 2007Fanasy P. Deming Background:, Reduction in salivary secretion is the hallmark of Sjögren's syndrome (SS). Calmodulin (CaM) and calmodulin binding proteins (CaMBPs) play a key role in the secretory process of saliva. Recent studies have suggested that SS-B, an autoantibody associated with SS, is a CaMBP. This finding suggests that CaMBP may contribute to the loss of saliva in SS. To better understand the role(s) of these proteins in SS, the purpose of this study was to compare salivary CaMBPs in Sjögren's patients and controls. Methods:, Saliva samples were collected from 20 patients and 20 age-, race-, and gender-matched controls. CaM overlay was used to identify CaMBPs in saliva of patients and controls. Results:, Higher number of salivary CaMBPs was observed among patients than controls. Conclusions:, The increased number of salivary CaMBPs in SS may suggest a potential role for these proteins in the pathogenesis of the disease. [source] Innervation pattern and Ca2+ signalling in labial salivary glands of healthy individuals and patients with primary Sjögren's syndrome (pSS)JOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 3 2000Anne Marie Pedersen Abstract: We have characterised the innervation pattern and intracellular Ca2+ -signalling in labial salivary glands (LSG) of 16 patients with primary Sjögren's syndrome (pSS) and 27 healthy controls. Numerous immunoreactive nerve fibers (IRF) containing vasoactive intestinal peptide (VIP) and pituitary adenylate cyclase activating peptide (PACAP) were found around acini, ducts and blood vessels. Substance P (SP)-, neuropeptide Y-, tyrosine hydroxylase- and nitric oxide synthase-IRF were mainly surrounding ducts and blood vessels. The majority of pSS patients had inflamed LSG and the presence of focal lymphocytic infiltrates (FI) were more frequent and pronounced as compared with healthy controls. In areas with normal or diffusely inflamed LSG tissue, pSS patients demonstrated the same distribution of IRF as healthy controls with similar histology. However, IRF were absent in central areas of FI both in pSS and age-matched healthy controls. Although all pSS patients had hyposalivation, stimulation with acetylcholine, norepinephrine, phenylephrine, isoproterenol, VIP, PACAP, SP, adenosine 5,-triphosphate and uridine 5,-triphosphate induced the same increase in the intracellular free Ca2+ concentration in LSG acini from both pSS patients and healthy controls, indicating the presence of functional receptor systems in vitro. [source] A histopathological study of lymphoepithelial island formation in labial salivary glands in patients with primary Sjögren's syndromeJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 3 2000Yukiko Yamamura Abstract: The proliferative status of lymphoepithelial islands in the labial salivary glands of primary Sjögren's syndrome (pSS) patients was investigated by counting the number of argyrophilic nucleolar organizer regions (AgNORs) in epithelial cells constituting the islands. The islands were classified into four groups and evaluated in terms of total area and three discrete zones of the islands. In each pSS group, the mean AgNOR number per total island epithelial cell nucleus was significantly higher than in control ductal epithelial cells. The zonal AgNOR number fluctuated during the process of island formation but became more uniform as the islands developed. Furthermore, statistically significant trends among the four pSS groups were observed in the ratio of T lymphocytes, B lymphocytes and plasma cells surrounding the islands. The results indicated that the islands are highly proliferative once island formation begins and that zonal island cell proliferation may be associated with the inflammatory cells. [source] Antimitochondrial antibodies in patients with chronic hepatitis C virus infection: description of 18 cases and review of the literatureJOURNAL OF VIRAL HEPATITIS, Issue 6 2005M. Ramos-Casals Summary., To describe the clinical and immunologic patterns of disease expression of patients with chronic hepatitis C virus (HCV) infection and positive antimitochondrial antibodies (AMA). We investigated the presence of AMA in 237 consecutive HCV patients with extrahepatic manifestations from an International Registry. AMA were detected by indirect immunofluorescence in triple rat tissue (liver, stomach and kidney), aceton-fixed criosections and FITC-conjugated rabbit anti-human immunoglobulins. We found positive AMA in 18 (8%) out of 237 HCV patients. All patients were female with a mean age at protocol inclusion of 65.8 years (ranging from 37 to 87 years). Twelve (67%) patients fulfilled classification criteria for systemic autoimmune diseases (SAD), including Sjögren's syndrome (n = 7), systemic sclerosis (n = 3) and systemic lupus erythematosus (n = 2). Fourteen (78%) of the HCV-AMA patients presented at least one of the highly suggestive characteristics of primary biliary cirrhosis (PBC): 9 (50%) had a specific M2 pattern, 6 (33%) had more than twice normal levels of alkaline phosphatase, 5 (28%) had raised IgM levels and 4 (22%) a histological pattern compatible with PBC. Five (28%) patients developed neoplasia after detection of AMA. Seven (39%) patients died, due to neoplasia (n = 4), cirrhotic complications (n = 2) and hepatopulmonary syndrome (n = 1). We describe a subset of HCV patients with positive AMA who presented a broad spectrum of clinical features, including liver, autoimmune and neoplasic manifestations. Two-thirds of these patients presented an associated SAD, mainly Sjögren's syndrome or systemic sclerosis, together with a high frequency of multiple autoantibodies and an increased prevalence of cirrhosis and neoplasia. [source] Subacute inflammatory polyradiculopathy associated with Sjögren's syndromeMUSCLE AND NERVE, Issue 6 2009Andrea Rigamonti MD Abstract Peripheral nervous system involvement is common in Sjögren's syndrome (SS); however, polyradiculopathy has been reported rarely in association with SS, and predominantly chronic forms have been described. We describe a patient with clinical, cerebrospinal fluid, neurophysiological, and neuroradiological evidence of subacute inflammatory polyradiculopathy in whom Sjögren's syndrome was diagnosed after the onset of neurological symptoms. Our case suggests that SS should be included in the differential diagnosis of subacute inflammatory polyradiculopathy. Muscle Nerve, 2009 [source] Peripheral neuropathy in an outpatient cohort of patients with Sjögren's syndromeMUSCLE AND NERVE, Issue 5 2006Glenn Lopate MD Abstract Peripheral neuropathy is common in patients with Sjögren's syndrome (SS), but its precise prevalence is unknown. Most prior studies were conducted at neurology or rheumatology specialty clinics and likely selected for a more severely affected population. We evaluated 22 SS patients and 10 controls for evidence of neuropathy in an outpatient setting at a regional meeting of the Sjögren's Syndrome Foundation. We performed neurological examinations and nerve conduction studies (NCSs) and measured serum antinuclear antibody (ANA) and SS-A and SS-B antibody levels. Participants filled out a questionnaire pertaining to symptoms, diagnosis, and treatment. We found that signs and symptoms related to small axons were more common in patients with SS than in controls. Complaints of painful distal paresthesias in the feet were noted in 59% of patients but in only 10% of controls, and of abnormal sweating in 41% and 0%, respectively. Examination revealed decreased pinprick sensation in 64% of patients with SS, but in only 30% of controls. Overall, 45% of the patients but none of the controls were thought to have an isolated small-fiber neuropathy. Large-fiber dysfunction (as measured by testing vibration, deep tendon reflexes, and NCSs) was similar between the two groups. We conclude that small-fiber neuropathy is common in patients with SS. Muscle Nerve 2006 [source] Immunotherapy of idiopathic inflammatory neuropathiesMUSCLE AND NERVE, Issue 3 2003Peter D. Donofrio MD Abstract Evaluation of peripheral neuropathy is a common reason for referral to a neurologist. Recent advances in immunology have identified an inflammatory component in many neuropathies and have led to treatment trials using agents that attenuate this response. This article reviews the clinical presentation and treatment of the most common subacute inflammatory neuropathies, Guillain,Barré syndrome (GBS) and Fisher syndrome, and describes the lack of response to corticosteroids and the efficacy of treatment with plasma exchange and intravenous immunoglobulin (IVIG). Chronic inflammatory demyelinating polyneuropathy, although sharing some clinical, electrodiagnostic, and pathologic similarities to GBS, improves after treatment with plasma exchange and IVIG and numerous immunomodulatory agents. Controlled trials in multifocal motor neuropathy have shown benefit after treatment with IVIG and cyclophosphamide. Also discussed is the treatment of less common inflammatory neuropathies whose pathophysiology involves monoclonal proteins or antibodies directed against myelin-associated glycoprotein or sulfatide. Little treatment data exist to direct the clinician to proper management of rare inflammatory neuropathies resulting from osteosclerotic myeloma; POEMS syndrome; vasculitis; Sjögren's syndrome; and neoplasia (paraneoplastic neuropathy). Muscle Nerve 28: 273,292, 2003 [source] Measuring fatigue among women with Sjögren's syndrome or rheumatoid arthritis: A comparison of the Profile of Fatigue (ProF) and the Multidimensional Fatigue Inventory (MFI)MUSCULOSKELETAL CARE, Issue 1 2008C. E. Goodchild BSc MSc Abstract Background:,Fatigue is common in both Sjögren's syndrome (SS) and rheumatoid arthritis (RA) and can restrict functioning. Aims:,We tested the convergent validity of the Profile of Fatigue (ProF) using the Multidimensional Fatigue Inventory (MFI) in SS and RA. Methods:,The 16-item ProF and the 20-item MFI were completed by 82 White-British women aged 35,79 years (mean 60.4 years). Thirty-four had been diagnosed with SS for a mean of 7.0 years and 48 had been diagnosed with RA for a mean of 14.5 years. The ProF measures four somatic facets of fatigue and two mental facets; the MFI contains one mental and four somatic facets. The structures of the items from both measures were tested by principal component factor analysis using varimax rotation. Results:,No significant differences in fatigue were found between the women with SS or RA. Five factors explained a total of 76% of the variance of the MFI; six factors explained 94% of the variance of the ProF. Mental fatigue items from both questionnaires loaded onto separate factors from somatic fatigue items; the two original facets of mental fatigue in the ProF were replicated. The four somatic fatigue facets of the ProF were generally replicated but the somatic facets of the MFI did not replicate as clearly. Equivalent facets correlated well between the two questionnaires (r , 0.65). Conclusions:,Both the ProF and the MFI distinguish between somatic and mental fatigue in SS and RA but the ProF appears better at resolving somatic facets of fatigue. Copyright © 2007 John Wiley & Sons, Ltd. [source] Sjögren's syndrome sufferers have increased oral yeast levels despite regular dental careORAL DISEASES, Issue 2 2008KCM Leung Aim:, To investigate the prevalence and quantity of oral yeasts and their association with oral candidiasis in Sjögren's syndrome (SS) patients receiving regular dental care. Materials and methods:, Yeasts in oral rinse and full-mouth supra-gingival plaque samples from 25 primary SS, 27 secondary SS and 29 control subjects were selectively cultured. All yeasts except single-species isolates were genotyped using pulsed field gel electrophoresis (PFGE). Results:, Ten (19%) SS sufferers had symptomless candidiasis. SS subjects had a higher prevalence (73%vs 7%) and quantity of yeasts than controls in both oral rinse and plaque samples (P < 0.05). The prevalence of yeasts in plaque was associated with candidiasis regardless of denture wearing (P , 0.04). Candida albicans was the predominant yeast isolated. PFGE showed 20 (66% of total) C. albicans isolate pairs, i.e. C. albicans species isolated from plaque and oral rinse samples of the same individual, were of closely related genetic clonal types (P < 0.01). Conclusions:, Despite effective oral hygiene, more SS subjects than controls had detectable levels of oral yeasts and their presence in supra-gingival plaque was associated with candidiasis. Candida albicans colonized supra-gingival biofilm even in well-maintained SS individuals, posing a challenge to the control of oral candidiasis. [source] | |||||||||||||||||||||||||||||||||||||