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ID PMID Title PublicationDate abstract
9132312 A case of Sjögren's syndrome associated with Sweet's syndrome. 1997 Jan We report a case of Sjögren's syndrome whose clinical course had been indolent until the patient presented with Sweet's syndrome (acute febrile neutrophilic dermatosis). This patient showed renal failure and renal tubular acidosis. Sweet's syndrome resolved within 3 weeks without corticosteroid therapy. Renal biopsy findings were consistent with interstitial nephritis. His renal manifestations responded to corticosteroid therapy and the renal function remained stable during 6 years follow-up without recurrence of Sweet's syndrome. Although close association of both syndromes is already known, in our case Sjögren's syndrome may have been exacerbated by occurrence of Sweet's syndrome.
9627951 [Concomitant dermatitis herpetiformis Duhring, arthritis and Sjögren syndrome in a patien 1998 Apr We report on a 26 year old woman with dermatitis herpetiformis Duhring, diagnosed at 10 years of age, who developed arthritis, symptoms of celiac disease, and Sjögren's syndrome 15 years later. Clinical symptoms, biopsies of duodenal mucosa and salivary glands as well as serological findings established the diagnoses. A coincidence of these pathologies has not been reported before. Gluten-free diet alleviated severity of clinical symptoms very quickly indicating the basic pathology of celiac enteropathy in the immunological disorders.
11732722 The occurrence of various collagen diseases in one family: a sister with ISSc, PBC, APS, a 2001 Oct We encountered siblings who had collagen diseases and related symptoms. Case 1 was a 53-year-old woman who had limited cutaneous systemic sclerosis (ISSc) associated with primary biliary cirrhosis (PBC), antiphospholipid antibody syndrome (APS), and subclinical Sjögren's syndrome (SS). Case 2 was a 48-year-old man, her younger brother, with systemic lupus erythematosus (SLE) that developed at 32 years of age. Investigation of their family revealed that their mother had Raynaud's phenomenon, arthritis, and subclinical Sjögren's syndrome, and that another younger brother of Cases 1 and 2 had Raynaud's phenomenon and general fatigue. HLA analysis revealed that the sister and brother had some identical HLA antigens in common, including A2, A33 (19), B67, B44 (12), Cw7, DR2, DR6, DR52, and DQ1. The sister, brother and their mother had common HLA antigens including A2, B67, Cw7, DR2, and DQ1. Although Cases 1 and 2 shared the same HLA system, they presented different phenotypes of collagen disease.
10709169 Clinical evaluation of a commercially available oral moisturizer in relieving signs and sy 2000 Feb A major complication of irradiation therapy for head and neck cancer is salivary gland dysfunction and xerostomia. The purpose of this clinical investigation was to evaluate the effects of a commercially available oral moisturizer (Optimoist) on salivary flow rate, symptoms of xerostomia, oral pH, oral microflora, and swallowing in postirradiation head and neck cancer patients (XRT) and patients with Sjögren's syndrome (SS). Subjects who were post-XRT and subjects with SS (n = 24; mean age = 54.1) discontinued their use of any salivary substitute or moisturizer for 2 weeks prior to entering the study. Baseline whole unstimulated saliva was collected for 5 minutes using a standard sialometric technique. Candida albicans and Lactobacillus cultures were performed using kits from Orion Diagnostica, Inc., and a pH analysis was performed on the salivary sample using a Markson (model 00663) pH meter. Swallowing was assessed by clinical measures by videofluoroscopic techniques. Several subjective assessments were performed to evaluate symptoms of xerostomia. Subjects were instructed in the use of a daily diary and to use only the provided article ad libitum for a period of 2 weeks. After the 2-week period, the results indicated significant subjective and objective improvements in signs and symptoms of xerostomia. Whole unstimulated salivary flow rate improved from (mean +/- SEM) 0.1150 +/- 0.02 to 0.2373 +/- 0.09 mL/min. Salivary pH did not change. Global subjective improvement in xerostomia improved in 58% of the subjects. Candida colonization decreased in 43% of the subjects. There was no change in Lactobacilli colonization. Swallowing objectively improved in 75% of subjects. These results indicate significant improvement in both signs of hyposalivation and symptoms of xerostomia with the use of Optimoist in postirradiation head and neck cancer patients and patients with SS.
10406404 The use of oral pilocarpine in xerostomia and Sjögren's syndrome. 1999 Jun OBJECTIVES: To analyze the role of oral pilocarpine in the treatment of xerostomia of Sjogren's syndrome (SS). METHODS: The medical literature was reviewed for all studies using oral pilocarpine to treat xerostomia caused by SS or radiotherapy registered in the MedLine Silver Platter database from 1966 to 1998. RESULTS: All the studies identified excluded elderly individuals with cardiac or pulmonary disease. Patients with postradiation xerostomia and incomplete resection of the salivary glands were more likely to benefit from oral pilocarpine when there was sufficient residual glandular function than patients with radical surgery for head and neck cancer (HNC). However, patients with SS and other inflammatory disorders seemed to benefit from oral pilocarpine, when compared with patients with postradiation xerostomia. The optimal dose of oral pilocarpine, which was less likely to cause side effects, was 5 mg four times daily. A recent multi-center study in SS patients suggests that oral pilocarpine is effective and safe for long-term administration. Although some studies did not show evidence for increased salivary gland secretion rate as measured by sialometry, symptoms improved, perhaps because of increased secretion from the minor salivary glands or better conditioning of the oral mucosa. CONCLUSIONS: Oral pilocarpine is likely to benefit patients with SS by reducing the symptoms of xerostomia, even if the salivary gland secretion rate does not increase. Further controlled studies are needed in patients with SS and should include elderly patients with cardiovascular disease treated with moderate doses of oral pilocarpine.
10225819 HTLV-I associated Sjögren's syndrome is aetiologically distinct from anti-centromere anti 1999 May OBJECTIVE: To investigate whether Sjögren's syndrome (SS) with anti-HTLV-I antibodies is aetiopathologically distinguishable from SS without these antibodies, the study compared prevalence of autoantibodies in serum samples of SS patients with or without anti-HTLV-I antibodies. METHODS: The test group included 135 patients with primary SS and 97 patients with secondary SS. Serum samples of the patients were examined for the presence of anti-nuclear antibodies (ANA), anti-SS-A/Ro antibodies, anti-SS-B/La antibodies, anti-centromere antibodies (ACA), and anti-HTLV-I antibodies. RESULTS: Anti-HTLV-I antibodies were detected in 25.0% of primary SS patients and in 29.2% of secondary SS patients. There were no significant differences in the mean age, sex, values of asparate aminotransferase, alanine aminotransferase, alkaline phosphatase, serum complements and IgG between HTLV-I seropositive and seronegative SS patients. The rheumatoid factor, ANA, anti-SS-A/Ro, and anti-SS-B/La antibodies in serum samples of SS patients were detected in 60.0%, 84.0%, 51.9%, and 12.0%, respectively. There was no significant difference in the prevalence of these antibodies between HTLV-I seropositive and seronegative SS patients. Using the indirect immunofluorescence test, 14.2% showed a discrete speckled staining pattern. All serum samples contained significant amounts of ACA determined by enzyme linked immunosorbent assay. These antibodies were detected in only 4% of HTLV-I seropositive SS patients but were present in 19.9% of HTLV-I seronegative SS patients. Furthermore, the prevalences of anti-SS-A/Ro and anti-SS-B/La antibodies in serum samples of ACA positive patients were significantly lower than those in ACA negative SS patients. CONCLUSION: These results suggest that SS patients with anti-SS-A/Ro or anti-SS-B/La antibodies, or both, might be aetiopathologically distinct from SS patients with ACA. HTLV-I might be involved in the pathogenesis of SS in a subset of patients with anti-SS-A/Ro or anti-SS-B/La antibodies, or both, but not SS patients with ACA.
10491219 Femoral neck length and hip fracture risk. 1999 Oct To determine whether there are differences in femoral skeletal geometry in fracture-prone subjects when size, positioning diagnosis, and age are controlled, we compared femoral measurements made from the uninvolved hip on 119 plane anteroposterior pelvis radiographs of women without fracture to those of the contralateral hip in a group of 43 female patients with hip fractures (neck, 23; intertrochanteric, 20). The hip was imaged in a standardized position of rotation and adduction. Race, age, and musculoskeletal diagnosis were known. Subjects were grouped by diagnosis of the opposite hip condition (rheumatoid arthritis, osteoarthritis, and normal) and compared. Measurements were also analyzed as ratios to head diameter (HD), neck diameter (ND), and pelvic width. Femoral neck length (NL) was measured from skeletal preparations and imaged in controlled positions of abduction and external rotation. No differences were found between the neck and intertrochanteric fracture groups. The differences between the fracture group and the controls were a thinner femoral cortex (measured at a point one head radius below the lesser trochanter) a larger femoral head, and a larger femoral ND in the fracture group (p < 0.025). The difference in cortical thickness was still significant when scaled by size, but the ratio of HD to ND was equivalent in fractures and controls. No difference in femoral NL could be demonstrated. The experimental measurements showed that apparent NL is significantly position sensitive and this may explain previously reported differences in fracture-prone groups.
11511800 A case of cholestatic autoimmune hepatitis and acute liver failure: an unusual hepatic man 2001 Aug Although hepatomegaly is reported to occur occasionally in patients with mixed connective tissue disease (MCTD) or Sjögren's syndrome (SS), autoimmune liver diseases such as primary biliary cirrhosis, sclerosing cholangitis, and autoimmune hepatitis in association with MCTD or SS have rarely been described. We report a case of severe cholestatic autoimmune hepatitis presenting with acute liver failure in a 40-yr-old female patient suffering from MCTD and SS. The diagnosis of MCTD and SS was made at the age of 38. The patient presented severe jaundice and elevation of conjugated bilirubin. The patient denied alcohol and drug use and had no evidence of viral hepatitis. On the 8th day of her hospitalization, the patient developed grade III hepatic encephalopathy. She was diagnosed as autoimmune hepatitis presenting with acute liver failure based on clinical features, positive FANA and anti-smooth muscle antibodies, negative anti-mitochondrial antibodies, high titers of serum globulin, liver biopsy findings, and a good response to corticosteroid therapy, The patient was managed with prednisolone and the clinical symptoms, liver function test results, and liver biopsy findings showed much improvement after steroid therapy.
10714160 [Comparison of primary and secondary Sjögren's syndrome]. 2000 Feb PURPOSE: We studied the difference in severity between primary and secondary Sjögren's syndrome (SS). SUBJECTS AND METHODS: Two groups of patients (all females, mean age: 58 years), 31 with primary SS and 18 with secondary SS were studied. We performed the following dry eye tests: fluorescein score and Rose Bengal staining, grading of tear lipid layer interference patterns, measurement of fluorescein break up time, cotton thread test, and Schirmer-I test. Auto antibodies were also investigated. RESULTS: There was no significant difference between primary and secondary SS with respect to any dry eye tests or auto antibodies. In primary SS, however, the presence of anti SS-A antibody was significantly correlated with Rose Bengal scores (p = 0.044). CONCLUSION: The severity of SS is independent of the primary or secondary type. In primary SS, the presence of anti SS-A antibody may be correlated with the severity.
9364427 Subclinical multisystemic autoimmunity presenting as a progressive myelopathy. 1997 Autoimmunity can manifest clinically in many ways; however, despite the various efforts to classify autoimmune disorders into specific disease entities, the borders between these disorders remain, in many cases, unclear. In this report we describe a young woman with subclinical Sjögren's syndrome and biliary cirrhosis, who presents clinically with symptoms exclusively from the central nervous system. This neurological syndrome is consistent with a progressive myelopathy. Although the patient has a serologically and histologically confirmed multisystemic autoimmune disorder, she fulfills none of the classification criteria for the diagnosis of a specific connective tissue disease.
11769416 [Detection of B-lymphocyte clonality in samples of salivary gland tissue in patients with 2001 Sep Intensive lymphoplasmocytic infiltration with atrophy of glandular tissue structures is the dominant patohistological feature found in exocrine glands of patients with Sjögren syndrome (SS). The infiltrates consist of T and B lymphocyte clusters that make the structures resembling germinal centers, and numerous plasmocytes that are secreting imunoglobulines locally, including autoantibodies. By applying the polymerase chain reaction (PCR) in our study we have shown the existence of dominant B cell clone in salivary glands samples of 4 out of 6 patients with SS, in the absence of clinical, routine laboratory, and patohistological signs of the lymphoma. B lymphocyte clones were detected upon the amplification of gene segment that encoded variable heavy chain immunoglobulin CDR3 region. Finding of single, dominant B lymphocyte clone could be of predictive significance, because these patients are predisposed to non-Hodgkin lymphoma (NHL) for which there is an assumption that it originates out of salivary glands from one of the clusters of proliferating B lymphocytes.
10348356 Salivary and serum soluble interleukin-2 receptor in primary Sjögren's syndrome. 1999 Apr Salivary and serum concentrations of soluble interleukin-2 receptor (sIL-2R) were studied in a group of patients with Sjögren's syndrome and a group suffering from dry mouth. Salivary sIL-2R levels was significantly higher (57.9+/-15.1 vs 16.7+/-4.7 pg/ml) (p < 0.05) in the group of 26 patients with Sjögren's syndrome than in the dry-mouth group. Both the salivary and the serum sIL-2R of normal controls were below the level of detection. No significantly statistical differences were noted between the concentrations of serum sIL-2R in either abnormal groups. No correlations were found between salivary or serum sIL-2R and the erythrocyte sedimentation rate, C-reactive protein, the presence of various autoantibodies or the focus score from lip biopsies in the group of patients with Sjögren's syndrome. The results show that, although the salivary sIL-2R does not actually reflect the extent of inflammation, it might have an important role in the diagnosis of Sjögren's syndrome.
11710718 Immunoglobulin Vlambda light chain gene usage in patients with Sjögren's syndrome. 2001 Nov OBJECTIVE: To determine whether patients with Sjögren's syndrome (SS) have abnormalities in Ig Vlambda and Jlambda gene usage, differences in somatic hypermutation, defects in selection, or indications for perturbations of B cell maturation. METHODS: Individual peripheral B cells from SS patients were analyzed for their Vlambda gene usage by single-cell polymerase chain reaction amplification of genomic DNA and compared with those from normal controls. RESULTS: Molecular differences from controls in Vlambda-Jlambda recombination were identified that were reflected by findings in the nonproductive Vlambda repertoire of the patients, including enhanced rearrangement of Vlambda10A and Jlambda2/3 gene segments. In addition, a number of abnormalities in the productive repertoire were identified, indicating disordered selection. A greater usage of 4 Vlambda genes (2A2, 2B2, 2C, and 7A), representing 56% of all productive Vlambda rearrangements, was observed, suggesting positive selection of these genes. Overutilization of Jlambda2/3 and underutilization of Jlambda7 in both nonproductive and productive Vlambda rearrangements of SS patients compared with controls suggested decreased receptor editing in SS. The mutational frequency did not differ from that in controls, and positive selection of mutations into the productive V gene repertoire was found, similar to that in controls, although mutational targeting toward RGYW/WRCY motifs, typically found in controls, was not found in SS patients. CONCLUSION: Disturbed regulation of B cell maturation with abnormal selection, defects in editing Ig receptors, and abnormal mutational targeting may contribute to the emergence of autoimmunity in SS.
11406525 Concomitant diagnosis of primary Sjögren's syndrome and systemic AL amyloidosis. 2001 Jul A 48 year old woman was referred to hospital for buccal discomfort. Physical examination showed a macroglossia and features of xerostomia. She was diagnosed as having primary Sjögren's syndrome according to the criteria proposed by the European Community study group in 1993. Furthermore, a lower lip salivary gland biopsy showed amyloid deposits that were also seen in the stomach and in the bone marrow. Echocardiography was consistent with cardiac amyloidosis. Serum immunofixation identified a monoclonal IgGlambda. As far as is known, this is the first report of systemic primary amyloidosis associated with Sjögren's syndrome. The relation between these two disorders is discussed.
9887430 [Immunohistochemical study of inflammatory infiltrates in minor salivary glands in Sjögre 1998 Nov 28 BACKGROUND: A study of the phenotype, activation and adhesive cells factors and cytokines in minor salivary glands in patients with primary Sjögren's syndrome (pSS), secondary Sjögren's syndrome (sSS) and autoimmune diseases (AD) without Sjögren's syndrome. PATIENTS AND METHODS: We have studied the minor salivary glands in 30 patients with pSS, 30 patients with sSS, 19 patients with AD without SS and 18 controls, using immunohistochemical techniques to analyze the molecular expression of CD3, CD4, CD8, CD20, CD25, CD14, CD56, CD11a, CDw50 (ICAM-3), HLA-DR, IL-1, TNF-alpha and IFN-gamma in lymphocytic infiltration and epithelial cells. RESULTS: Phenotype features were similar in patients with pSS and sSS, except that CD20+ lymphocyte expression was significantly higher in the sSS group (p = 0.023). The patients affected by AD without SS had activated lymphocytes in minor salivary glands in a similar manner to patients affected by pSS and sSS. No significant differences were found in HLA-DR expression in epithelial cells. We found unusual CD25 expression in epithelial cells in patients with SS but not in patients with AD without SS. The differences between pSS and sSS are related to SS theoretical time development and to immunosuppressive treatments. CONCLUSIONS: The immunohistochemical pattern of minor salivary glands is similar in patients with pSS and sSS. Patients with AD are likely to develop immunological changes in minor salivary glands attributable to activated lymphocytes.
11246934 Use of orally administered anhydrous crystalline maltose for relief of dry mouth. 2001 Feb OBJECTIVES: To examine the safety and efficacy of anhydrous crystalline maltose (ACM) for treatment of dry mouth. DESIGN: ACM was delivered orally as a 200-mg lozenge given three times daily over a 12-week (study Alpha) or 24-week (study Omega) period to a total of 22 and 97 subjects, respectively. All participants had prominent complaints of persistent dry mouth associated with primary Sjögren's syndrome. Patients were examined every 4 weeks in study Alpha and every 6 weeks in study Omega. SETTINGS: Patients were seen in outpatient clinics at a total of 33 sites within the United States. OUTCOME MEASURES: Unstimulated whole saliva output, a measure of basal salivary gland function, was determined at each visit. Symptoms associated with oral and ocular dryness were assessed at the same time with the use of 100-mm visual analog scales. Safety was assessed by physical examination and laboratory studies. RESULTS: During these clinical trials, a majority of subjects demonstrated an increase in unstimulated whole saliva output and the treatment exhibited an excellent safety profile. The ACM treatment in study Omega led to significant improvement in several subjective measures of oral and ocular comfort. CONCLUSIONS: In these two studies, ACM lozenges administered three times daily for 12 or 24 weeks improved salivary output and decreased complaints of dry mouth and eyes. Side effects were minimal, and treatment was without significant adverse events. This safe and simple intervention may provide clinical benefit to individuals with distressing dry mouth symptoms.
10981655 Central pontine myelinolysis--a rare manifestation of CNS Sjogren's syndrome. 2000 Central nervous involvement in Sjogren's syndrome (CNS-SS) is not uncommon and has a variety of manifestations. We describe a 47-year-old woman with Sjogren's syndrome who presented with distal renal tubular acidosis with severe hypokalemia and hypokalemic myopathy. She developed progressive obtundation after years of stable disease. ANA, anti-Ro antibodies were positive. Brain MRI showed a cleft in the mid pons which was hypointense on T1 and hyperintense on T2 which was considered to be classical of central pontine myelinolysis. Serial MRI showed initial enlargement of the lesion which persisted despite successful immunosuppressive therapy with pulse methylprednisolone, pulse cyclophosphamide, plasmapheresis and IVIG.
9585336 Role of the masseter reflex in the assessment of subacute sensory neuropathy. 1998 Jun In 3 patients with a severe pure sensory neuropathy of subacute onset, the masseter reflex remained normal despite absent blink reflex responses and absent stretch reflexes in the extremities. In 20 patients with primary disorders of peripheral nerve axons or myelin, the masseter reflex was abnormal. This study suggests that a normal masseter reflex in patients presenting with a pure sensory neuropathy favors a polyganglionopathy rather than a primary axonal sensory neuropathy, particularly if the blink reflex is abnormal.
9267154 [Sjögren's syndrome with presenting as aseptic meningoencephaloradiculopathy in an elderl 1997 May A 76-year-old woman was found to have acute aseptic meningoencephalitis with meningial irritation, disturbance of consciousness, elevation of cell counts in cerebrospinal fluid, and swelling of a right temporal-lobe lesion on a CT scan of the head. Muscle weakness in the lower extremities and urinary dysfunction developed and progressed gradually. The protein content of cerebrospinal fluid was high, and the distal latencies of F waves were prolonged, which suggested that the inflammation extended to the nerve roots. Sjögren's syndrome was diagnosed on the basis of atrophy of the labial salivary glands; invasions of lymphocytes and plasma cells to the intercellular space; and elevation of the titers of serum antinuclear antibody, anti-SS-A antibody, and anti-SS-B antibody. The patient had no xerosis. Aseptic meningoencephalitis was the first manifestation of Sjögren's syndrome. In recent years, several cases in which Sjögren's syndrome was associated with aseptic meningitis have been reported. However, we know of no previous report of such a case in a patient of this age. Aseptic meningoencephalitis can be the first manifestation of Sjögren's syndrome.
9256307 Severe reversible cardiomyopathy associated with systemic vasculitis in primary Sjögren's 1997 A 40y old woman with primary Sjögren's syndrome developed elevated purpura, peripheral neuropathy, muscular tenderness, abdominal pain, heart failure, and convulsive spells. The hallmarks of this disease were high titers of anti-Ro antibodies and low complement levels in the serum, leukocytoclastic small vessel vasculitis in the cutaneous biopsy specimen, and a life threatening clinical course. Echocardiography revealed left ventricular hypokinesis with low ejection fraction, which is unlike the more common features of cardiomyopathy complicating Sjögren's syndrome. The rapidly deteriorating heart failure and other systemic complications remitted on pulse corticosteroid and cyclophosphamide therapy. The pathogenesis of heart failure, which appeared concurrently with vasculitis and was reversed on immunosuppressive therapy, is explained in the context of the systemic disease. Leukocytoclastic vasculitis might be at the origin of this rare variant of acute, severe but reversible cardiomyopathy in pSS.