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ID PMID Title PublicationDate abstract
9645417 Expression of the mucosal lymphocyte integrin alphaEbeta7 and its ligand E-cadherin in sal 1998 The expression of the mucosal lymphocyte integrin alphaEbeta7 and its ligand E-cadherin was analyzed in sialadenitic lesions of patients with Sjögren's syndrome in order to assess the potential interactions between the mucosal and the systemic immunological compartments. Immunohistochemical evaluation of the inflammatory infiltrates in the salivary gland tissues in Sjögren's syndrome patients revealed presence of alphaEbeta7-integrin-expressing lymphocytes, in 18 of 20 biopsies examined. The ligand for alphaEbeta7, E-cadherin was also found in 18 of 20 salivary gland biopsies analysed. These results suggest homing of lymphocytes derived from the gut epithelium through the blood stream to inflamed salivary gland tissue followed by in situ interaction with E-cadherin expressing cells, the ligand for alphaEbeta7 expressing T lymphocytes.
9191888 Association of Sjögren's syndrome with hereditary angioneurotic edema: report of a case. 1997 Jul A case of hereditary angioneurotic edema (HANE) associated with Sjögren's syndrome is presented. One of the members of a pedigree of HANE due to deficiency of C1 inhibitor (C1INH) had a positive titer for anti-SS-A and anti-SS-B antibodies in the serum, complaining of symptom of dry eyes and dry mouth. A lip biopsy revealed lymphocytic infiltration of minor salivary glands. The patient had renal tubular acidosis (RTA). Thus the patient was diagnosed as suffering from Sjögren's syndrome with RTA.
10833714 Transthoracic pacing in a very low birth weight infant with congenital complete atrioventr 2000 May We report our experience of pacemaker treatment in a premature infant of 830 g with congenital complete atrioventricular block due to maternal Sjögren's Syndrome. The infant was delivered by cesarean section at an estimated gestational age of 26 weeks because of fetal bradycardia, decreasing fetal movements, and hydrops. Immediate postnatal transesophageal ventricular pacing was not successful, whereas transthoracic pacing with self-adhesive patch electrodes adapted to body size resulted in an effective increase of the infant's heart rate until operative application of temporary epimyocardial pacing wires allowed external stimulation of the heart.
9598890 Oral candidiasis and Sjögren's syndrome. 1998 May OBJECTIVE: To determine the prevalence of oral candidiasis (OC) in patients with Sjögren's syndrome (SS), to identify oral signs and symptoms associated with OC, and to compare them with a healthy group. METHODS: Patients with primary SS, and with SS associated with autoimmune disease were identified according to established criteria. Each individual had: (1) a questionnaire, (2) an oral clinical evaluation, (3) a set of microbiological tests: tongue smear, culture of tongue smear, and saliva sample. Diagnosis of OC was established with 2 positive results and the presence of any sign or symptom. RESULTS: A total of 81 subjects were studied, 21 with primary SS, 29 secondary SS, and 31 healthy subjects. We diagnosed OC in 74% of patients with SS, which was statistically significant compared to the healthy group (23%) p < 0.001. Frequent symptoms associated with OC were increased sensitivity to spicy foods (58%) and unpleasant metallic taste (40%). Common signs of OC were erythematous lesion on the dorsum of the tongue (68%) and angular cheilitis (24%). CONCLUSION: Prevalence of OC was high in patients with SS. The frequency of signs and symptoms associated with OC may explain some of the clinical manifestations. If chronic erythematous OC is found in patients with SS an oral assessment at every appointment may help provide appropriate treatment.
10410277 Primary Sjögren's syndrome in children and adolescents: proposal for diagnostic criteria. 1999 May OBJECTIVE: Primary Sjögren's syndrome (pSS) in childhood is a rare disease. Diagnostic criteria are available for adult patients only. In order to establish diagnostic criteria for juvenile pSS an analysis of 7 girls and one boy suffering from pSS with early onset is reported. Due to the rarity of the disease, data on patients with pSS reported in the literature are included in the proposal for modified diagnostic criteria. METHODS: The diagnosis of pSS was established according to the criteria for adulthood pSS, duly modified, which include clinical symptoms and laboratory immunological evaluation. RESULTS: The average age of our patients at clinical onset was 13.5 years (range: 10-17 yrs.). Clinical signs included systemic (fever, fatigue) as well as local (parotitis, vulvovaginitis, conjunctivitis) symptoms. Paralysis due to hypokalemia linked to renal tubular acidosis and central nervous system (CNS) involvement was seen in one patient. Asymptomatic renal tubular acidosis was diagnosed in another 2 patients. Autoimmune hepatitis was present in 2 patients. All patients had laboratory abnormalities: hyperimmunoglobulinemia IgG, high titers of antinuclear antibodies (anti-SS-A and/or anti-SS-B) and elevated serum amylases. Sicca syndrome was never seen during childhood, although it developed later in 3 patients, after 7 to 10 years of follow-up. CONCLUSIONS: It has been stressed that the classical diagnostic criteria for adult Sjögren's syndrome, especially sicca syndrome, are not applicable to a pediatric onset of the disease. On the other hand, the presence of typical laboratory abnormalities can allow the diagnosis of these patients in the early stages. Both laboratory and clinical symptoms typical for childhood are included in our proposal for diagnostic criteria applicable to juvenile pSS. Life-threatening conditions such as hypokalemic paralysis, CNS involvement and hepatitis may also occur in children. Sicca syndrome tends to develop much later in pediatric patients.
9458209 Altered frequency of type 1 cytokine secreting cells in the peripheral blood of patients w 1998 Jan OBJECTIVE: An imbalance in immunoregulatory cytokines may contribute to the etiopathogenesis of Sjogren's syndrome (SS). We investigated systemic abnormalities in cytokine production in the peripheral blood in patients with SS. METHODS: ELISPOT assays were used to detect and enumerate cells spontaneously secreting interleukin 2 (IL-2), IL-6, IL-10, and interferon-gamma (IFN-gamma) in freshly isolated peripheral blood mononuclear cells from 20 patients with SS and 20 healthy controls. RESULTS: The number of cells spontaneously secreting type 1 cytokines IL-2 and IFN-gamma was decreased in the peripheral blood of patients with SS compared to controls. There was no change observed in the number of cells spontaneously secreting IL-6 and IL-10. Cells spontaneously secreting IL-4 were too rare in peripheral blood to evaluate, although cells capable of secreting IL-4 in response to phytohemagglutinin did not differ from controls. Patients with severe extraglandular symptoms (such as vasculitis) had a significantly lower frequency of IFN-gamma secreting cells in their peripheral blood than those without extraglandular involvement. CONCLUSION: These results suggest that decreased type 1 cytokine production may contribute to or reflect the pathogenesis of SS.
11480124 Benign salivary gland disease. 2001 Jul The majority of benign clinical problems that present affect the major salivary glands--parotid and submandibular. However, there are numerous minor salivary glands located in the mucosa of the head and neck which have the same predilection to the same diseases that affect the major glands but to a lesser frequency.
11407086 Fatigue and health profile in sicca syndrome of Sjögren's and non-Sjögren's syndrome ori 2001 May OBJECTIVE: To assess the health status and fatigue in sicca patients with or without Sjögren's syndrome (SS) and to test whether the immune-inflammatory activity or the extent of the disease predict fatigue in SS. METHODS: The Medical Outcomes Study Short-Form General Health Survey (MOS SF-36) was used in 1 degree SS (n = 90), 2 degrees SS (n = 24), non-SS patients with sicca symptoms (n = 15) and healthy population controls (n = 126). Laboratory values and clinical findings were used to predict fatigue in SS. RESULTS: 74% of the SS and 80% of the non-SS sicca patients felt themselves tired. Vitality score values were 40.2 +/- 20.3 in 1 degree SS, 42.1 +/- 20.6 in 2 degrees SS and 29.0 +/- 15.8 in non-SS. The health profiles were similar in 1 degree and 2 degrees SS, worse (p < 0.001) than in normal controls, but in most aspects better than in non-SS sicca patients. In SS neither hemoglobin, ESR nor CRP predicted fatigue. Surprisingly, high serum IgG (p < 0.05), antinuclear antibodies (ANA) (p < 0.01) and SS-A antibodies (p < 0.05) values correlated positively with vitality. The number of disease manifestations correlated negatively with vitality (p < 0.004). The total number of disease manifestations, and ANA and/or SS-A autoantibodies were the best predictors of fatigue, but explained it only to 17-57%. CONCLUSION: Patients with fatigue and perceived ill health but without fibromyalgia had sicca symptoms and low basal tear and salivary secretion rates, indicating that cortical events can lead to a SS-like sicca syndrome. Even in SS fatigue is only in part explained by clinical disease manifestations and laboratory tests assessing inflammation and autoimmunity. Fatigue in both SS and non-SS sicca syndrome more likely correlates to other features, such as neuroendocrine aspects of the disease.
10553980 Treatment of severe neutropenia due to Felty's syndrome or systemic lupus erythematosus wi 1999 Oct OBJECTIVES: To examine the efficacy and safety of recombinant human granulocyte colony-stimulating factor (rhG-CSF) and recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) for the treatment of severe neutropenia due to Felty's syndrome (FS) or systemic lupus erythematosus (SLE). METHODS: Eight patients with absolute neutrophil counts (ANC) below 1,000/microL attributable to FS (n = 4) or SLE (n = 4) were treated with rhG-CSF. The hematologic and clinical response as well as side effects were recorded. In addition, reports on the use of rhG-CSF/rhGM-CSF in FS and SLE retrieved from the English language literature were analyzed. RESULTS: RhG-CSF effectively corrected neutropenia due to FS and SLE in seven of the current eight patients. In 54 of 55 FS and SLE patients retrieved from the literature, G-CSF or GM-CSF, respectively, proved to be effective at elevating the neutrophil count, which was often associated with improvement of infectious complications. The neutrophil count often declined again when growth factor treatment was stopped but generally stabilized at a level that exceeded the pretreatment count. Side effects included rare cases of thrombocytopenia, arthralgias, and development of cutaneous leukocytoclastic vasculitis. Side effects were dose dependent and resolved when treatment was discontinued. One of our own patients and 17 previously reported patients continued to benefit from long-term administration of rhG-CSF over periods of more than 40 months. CONCLUSIONS: RhG-CSF is an effective and generally well-tolerated treatment for neutropenia due to FS or SLE. Exacerbation of the underlying rheumatic condition due to G-CSF appears to be rare if G-CSF is administered at the lowest dose effective at elevating the ANC above 1,000/microL.
10450101 Lymphoid lesions of the parotid. 1999 Sep Lymphoid lesions of the parotid gland are much less common than their epithelial counterparts, and thus cytologic experience on fine-needle aspiration (FNA) is limited. FNA of lymphoid lesions with ancillary aids (flow cytometry and immunophenotypic studies) can make a definitive diagnosis. All FNAs of the parotid gland performed at the Johns Hopkins Hospital in an 8-yr span (1990-1998) were reviewed retrospectively. In all, 391 cases were done, of which 76 cases of lymphoid lesions were identified. The relevant cytology, histology, and flow cytometry were analyzed. Of the 76 lymphoid lesions, results included reactive lesions (n = 35), lymphoepithelial cysts (n = 27), malignant lymphomas (n = 12), an atypical lymphoid population (n = 1), and Sjogren's disease (n = 1). We conclude that both reactive and malignant lymphoid lesions of the parotid can be diagnosed on FNA with adjunctive tests such as flow cytometry and immunophenotyping, obviating the need for surgery. Diagn. Cytopathol. 1999;21:170-173.
10192156 Relevance of complement fixing antinuclear antibodies. 1999 Feb BACKGROUND: Connective tissue diseases (CTDs) are a heterogeneous group of disorders defined by the association of a variety of clinical manifestations with immunologic and other laboratory findings. Overlap of syndromes and aberrant findings appear rather frequently. METHODS: Sera of eight antinuclear antibody (ANA) negative, cases of subacute cutaneous lupus erythematosus (SCLE) with antibodies to Ro (SS-A) and a ninth case with clinical and laboratory signs of Sjögren's syndrome and systemic lupus erythematosus (SLE) were tested for complement (C') fixing antinuclear antibodies (C-ANAs). The ninth case was examined in depth by direct immunofluorescence (DIF) and a two-step "C + DIF" test of biopsies for C' fixation to in vivo bound ANAs, as well as serum tests for C-ANA, ANA, and SCLE markers. RESULTS: Sera of five of the eight ANA negative, Ro(SS-A) positive SCLE cases had C-ANAs. The ninth case, a 50-year-old woman with clinical and laboratory signs of Sjögren's syndrome and SLE, gave a strong positive C + DIF reaction in the skin biopsy for in vivo bound ANAs that fix C', but negative ANAs and C-ANAs in routine serum tests; they revealed antimitochondrial antibodies. Serum tests on normal skin, however, revealed weak ANA and strong C-ANA reactions with in vitro fixed C'. CONCLUSIONS: ANA negative cases of SCLE or Sjögren's syndrome may have C-ANAs. A case with Sjögren's syndrome and signs of SLE had both in vivo and in vitro C' fixing ANAs. C-ANA tests can aid in the identification of such cases.
10052745 Pseudogout attack associated with chronic thyroiditis and Sjögren's syndrome. 1999 Jan A 66-year-old woman, diagnosed with chronic thyroiditis at age 63, presented with anorexia and fatigue. Therapy for the chronic thyroiditis consisted of levothyroxine sodium (100 microg/day). Her symptoms were attributed to the insufficient supply of levothyroxine sodium. Following a dosage increase to 150 microg/day, she suffered from an acute attack of pseudogout. Clinical features were complicated by Sjögren's syndrome, which appeared after treatment onset. Pseudogout was effectively treated by colchicine after administration of diclofenac sodium failed to alleviate the symptoms. Pseudogout is a recognized complication of thyroid replacement therapy, but association with Sjögren's syndrome has not been previously reported.
9870874 Murine cytomegalovirus induces a Sjögren's syndrome-like disease in C57Bl/6-lpr/lpr mice. 1998 Dec OBJECTIVE: To analyze Fas and tumor necrosis factor receptor I (TNFRI) apoptosis pathways in salivary gland inflammatory disease induced by murine cytomegalovirus (MCMV) infection. METHODS: Four different strains of mice (C57BI/6 [B6]-+/+, Fas-deficient B6-lpr/lpr, TNFRI-deficient B6-tnfr1(0/0), and B6-tnfr1(0/0)-lpr/lpr mice) were infected intraperitoneally with the Smith strain of MCMV (1 x 10(5) plaque-forming units). Viral load was determined by a plaque assay, inflammation and apoptosis by immunohistochemistry and staining with terminal dUTP nickend labeling, and autoantibodies by enzyme-linked immunosorbent assay. RESULTS: Infectious MCMV was not detectable by day 100. Although all MCMV-infected mice developed acute sialadenitis by day 28, a chronic (>100 days), severe salivary gland inflammation and anti-Ro and anti-La antibodies developed only in the B6-lpr/lpr mice. Apoptotic cells were detected during the acute, but not the chronic, phase of inflammation. CONCLUSION: Both Fas- and TNFRI-mediated apoptosis contribute to the clearance of MCMV-infected cells in the salivary glands. However, because Fas-mediated apoptosis is necessary for the down-modulation of the immune response, a defect in this process can lead to a postinfection, chronic inflammatory response that resembles Sjögren's syndrome.
9588738 Prevalence of the diffuse infiltrative lymphocytosis syndrome among human immunodeficiency 1998 May OBJECTIVE: To ascertain the prevalence of the diffuse infiltrative lymphocytosis syndrome (DILS) in human immunodeficiency virus type 1 (HIV-1)-positive outpatients. METHODS: The presence of sicca symptoms and visible salivary gland enlargement was determined by interview and physical examination, and the clinical stage was determined by chart review, in 523 HIV-positive patients. The diagnosis of DILS was established in those with parotid gland enlargement by minor salivary gland biopsy or radionuclide scintigraphy. RESULTS: Definite DILS was found in 15 (3%) of the 523 patients, and possible DILS in 18 (3.4%). The prevalence of definite DILS was significantly higher in African Americans (4.5%). Self-reported facial swelling and xerophthalmia that was not explained by the effects of medication were found more frequently in those with DILS than in the non-DILS patients. Patients with DILS had higher CD8 counts (mean +/- SD 1,456 +/- 813/mm3) compared with those without DILS (934 +/- 624/mm3; P < 1 x 10(-6)), and were less advanced in their HIV clinical stage (Centers for Disease Control and Prevention stages A or B in 76% compared with 60% of the non-DILS group; P = 0.01). CONCLUSION: DILS is an important problem in HIV-infected outpatients and tends to occur in patients whose clinical disease is at a less advanced stage. Patients with parotid gland enlargement accompanied by sicca symptoms should be screened for HIV infection.
9445528 [Implementation and indications for quantitative sialoscintigraphy]. 1997 Oct BACKGROUND: Since its introduction in 1965 salivary gland scintigraphy has been an established method to simultaneously investigate excretion function in major salivary glands. In order to elucidate parenchymal function of salivary glands, several authors described various quantitative and semiquantitative methods. However, no standardized protocol for quantitative salivary gland scintigraphy has been established so far. METHODS: Therefore, in this paper we report on a standardized and validated acquisition protocol for salivary gland scintigraphy using 99mTc-pertechnetate. RESULTS: A normal data base for both parenchymal and excretion function is given in detail. In addition, the diagnostic value of salivary gland scintigraphy is reviewed in various clinical settings, such as mild parenchymal damage in beginning Sjögren's syndrome, proof of functional obstruction in sialolithiasis with and without parenchymal damage, and parenchymal damage following radioiodine treatment. In a second part, applications of salivary gland scintigraphy in current clinical research are described, and radiation protection of salivary glands in rabbits and patients treated with high doses of I-131 are discussed. CONCLUSIONS: Salivary gland scintigraphy is a study that is easily performed and well tolerated by the patient. It yields quantitative parameters for parenchymal function and excretion fraction.
8990191 Sialadenitis histologically resembling Sjogren syndrome in mice transgenic for hepatitis C 1997 Jan 7 Hepatitis C virus (HCV), a major causative agent of non-A, non-B chronic hepatitis, is also suggested to be associated with extrahepatic manifestations such as mixed cryoglobulinemia and glomerulonephritis. Two independent lines of transgenic mice carrying the HCV envelope genes have been shown previously to express the HCV envelope proteins in organs, including the liver and salivary glands, which results in no pathological changes in the liver. Further analysis of these animals now has revealed that they develop an exocrinopathy involving the salivary and lachrymal glands. This pathology resembles Sjogren syndrome, which also is suggested to have a possible association with chronic hepatitis C. These observations suggest that HCV might be involved in the pathogenesis of sialadenitis in humans and that this transgenic mouse system would be a good animal model for the study of HCV infection.
11642520 Primary Sjögren's syndrome presenting as hypokalaemic periodic paralysis and respiratory 2001 We report a case of hypokalaemic, flaccid quadriparesis with sudden respiratory arrest in a 27-year-old Japanese woman in whom the discovery of distal renal tubular acidosis led to the diagnosis of primary Sjögren's syndrome (SS). Hypokalaemic periodic paralysis as the initial manifestation of primary SS is rare, but when it occurs it may precede symptoms of xerostomia and xerophthalmia. The diagnosis of primary SS should be considered in premenopausal women who present with rapidly progressive weakness and hypokalaemia. with or without the sicca complex.
11247557 Defective cellular trafficking of lacrimal gland aquaporin-5 in Sjögren's syndrome. 2001 Mar 3 Dry eyes and dry mouth are clinical hallmarks of Sjögren's syndrome. We assessed the distribution of aquaporin-5 (AQP5) in lacrimal gland biopsy samples. Healthy controls and patients with Mikulicz's disease or non-Sjögren's syndrome dry eye had the expected apical distribution of AQP5 in lacrimal acinar cells. By contrast, cytoplasmic AQP5 was seen in patients with Sjögren's syndrome. Sodium channel and sodium-potassium ATPase distributions were normal in all groups. These findings show a selective defect in lacrimal gland AQP5 trafficking in Sjögren's syndrome that might contribute to decreased lacrimation and dry eye in these patients.
11079585 Stimulated salivary pertechnetate clearance revisited: correlation with dynamic scintigrap 2000 Nov PURPOSE: The stimulated salivary clearance of Tc-99m pertechnetate (SSCP) introduced in 1985 by Blue and Jackson is revisited here in patients with known or possible salivary disorders and correlated with most commonly cited and up-to-date quantitative indices obtained from standard dynamic salivary scintigraphy. METHODS: An SSCP test was performed after dynamic salivary scintigraphy in 19 patients with oral or ocular dryness, among whom 7 had Sjögren's syndrome. Many scintigraphic indices were calculated: salivary to background ratio (S/B[t]), background-corrected uptake U[t]), maximal uptake (Umax), cumulative gland uptake (CGU), initial slope of the uptake curve (IS), stimulated excretion velocity (SEV), stimulated excretion fraction (SEF), and excreted activity (EA). RESULTS: The SSCP test was well tolerated, except in two patients in whom it had to be interrupted. Clearance values ranged from 5 to 40 ml/minute, with a clear-cut bimodal distribution centered around 15 to 20 ml/ minute. Six of the seven patients with Sjögren's syndrome had values less than 15 ml/minute. SSCP was closely correlated with all uptake indices (S/B, U, Umax, CGU, and IS) and uptake-related indices (EA, ISxSEF) (P < 0.01). A poor correlation was found with the excretion index SEV (P = 0.06) and none with SEF. CONCLUSIONS: SSCP is a quick and objective means to investigate the sicca syndrome that may be useful in most clinical situations. It reflects the parenchymatous salivary gland function and will provide a means to assess and predict salivary gland involvement. Dynamic salivary scintigraphy remains necessary in very early stages because of its high sensitivity rate and ability to locate the impaired gland, or in severe stages in which lemon juice could be deleterious.
10561713 Hepatitis C virus and oral disease: a critical review. 1999 Oct Hepatitis C virus (HCV) infection is widespread with an estimated 3% of the world population being infected. Acute infection is usually mild but chronicity develops in as many as 70% of patients, of whom at least 20% will eventually develop cirrhosis. A further 1-4% of cirrhotic individuals will develop hepatocellular carcinoma. Infection with HCV may have effects on various organs other than the liver. HCV has been causally associated with a remarkable array of extrahepatic manifestations, some of which remain unproven. This review discusses the evidence implicating HCV in the aetiology of two important oral conditions, namely Sjögren's syndrome and lichen planus.