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ID PMID Title PublicationDate abstract
10810665 [Annular erythema in Sjögren syndrome. A variant of cutaneous lupus erythematosus?]. 2000 Apr Annular erythema has been recognised as a cutaneous manifestation of Sjögren's syndrome in the Asian literature and has been assumed to represent a distinct clinical entity. Since there are common pathophysiologic mechanisms, mainly the presence of anti-Ro/SSA or anti-La/SSB antibodies, it is difficult to separate the annular erythema from subacute cutaneous lupus erythematosus. Histological examination may reveal dermal mucin deposition resembling lupus erythematosus tumidus. We present a Chinese patient with widespread annular erythema, keratoconjunctivitis sicca, and anti-Ro/SSA antibodies. Clinical, histopathological, and immunogenetic findings are discussed reviewing the current literature, and the differences between annular erythema associated with Sjögren's syndrome and cutaneous lupus erythematosus are emphasized.
10399235 [Comparative sonographic, x-ray and morphological studies of the salivary glands in Sjögr 1999 AIM: To define diagnostic value of parotid gland (PG) sonography in Sjogren's syndrome (SS) as compared to sialography and morphological changes of labial salivary glands (LSG). MATERIALS AND METHODS: Examination of 50 females with primary (20 patients) and secondary (30 patients) SS consisted of clinical, ultrasonic, x-ray and morphological investigations. RESULTS: Sonographically, PG in SS is characterized by nonhomogeneity of parenchymal picture detected in 75% of patients with primary and 50% of patients with secondary SS. Moderate and severe nonhomogeneity of PG parenchyma was seen in the stage of marked manifestations of chronic parenchymatous parotitis. CONCLUSION: Relationship between PG sonographic image in SS and morphological changes in LSG need further study. PG sonography may help in combined examination of SS patients.
9932962 Improvement of myelopathy in Sjögren's syndrome with chlorambucil and prednisone therapy. 1999 Jan 15 The authors present a patient with Sjögren's syndrome with a fluctuating and then progressive myelopathic syndrome, and optic nerve involvement. Treatment with chlorambucil and prednisone improved the patient's function from being wheelchair bound to walking unaided. Spinal MRI showed multiple, extensive intraparenchymal areas of abnormal T2-weighted signal intensity, gadolinium enhancement, and cord swelling, which also improved during the period of treatment.
9797560 Salivary and serum hyaluronic acid concentrations in patients with Sjögren's syndrome. 1998 Aug OBJECTIVE: To evaluate salivary hyaluronic acid (HA) concentration in patients with primary Sjögren's syndrome (SS). METHODS: Salivary and serum HA concentrations were evaluated using a radiometric assay. Thirty nine patients with SS served as the study group and their results were compared with 19 patients having clinical symptoms and signs of dry mouth and with 10 normal controls. RESULTS: Salivary HA concentrations were significantly increased (p < 0.05) in the 39 patients with SS compared with the 19 patients with dry mouth and the 10 normal controls (240.7 (38.5) v 99.8 (14.6) and 91.3 (7.9) ng/ml, respectively) (mean (SEM)). No significant differences were noted in the serum HA concentrations between the three groups (42 (3.9) v 36.3 (4.1) and 32 (4.3) ng/ml, respectively) (mean (SEM)). No correlation could be found between salivary HA concentrations and the focus score of lip biopsies, nor between salivary HA concentrations and erythrocyte sedimentation rate or other serological tests. CONCLUSION: Increased salivary HA concentrations can serve as a marker of local inflammation and may be of value in the diagnosis of SS.
11669164 Prevalence and characteristics of Sjögren's syndrome or Sicca syndrome in chronic hepatit 2001 Oct OBJECTIVE: To describe the prevalence and clinical and laboratory characteristics of sicca syndrome and Sjögren's syndrome (SS) in chronic hepatitis C virus (HCV) infection. METHODS: Forty-five consecutive HCV infected patients referred for liver biopsy were enrolled in a prospective study. Subjective and objective criteria of xerophthalmia or xerostomia were systematically investigated and the patients classified according to 3 sets of criteria (European, Manthorpe, and Fox criteria) for the diagnosis of SS. RESULTS: Sicca syndrome was present in 28 (62%) patients; all had oral dryness and 14 had both oral and ocular dryness. Twenty-four (53%) patients had SS by the European criteria, 25 (56%) by Manthorpe criteria, and 4 (8%) by Fox criteria. Salivary gland biopsy was positive for SS (grade III or IV by Chishom classification) in 21 samples (47%); 9 samples (21%) were classified grade 0, and 15 (32%) grade I or II. No patient had anti-SSA or anti-SSB antibodies. The presence of SS or sicca syndrome was associated with older age and liver disease activity according to the METAVIR scoring system, but not with the presence of other extrahepatic manifestations or with HCV genotype. A high METAVIR activity score was only statistically associated with primary SS. CONCLUSION: HCV infection appears to account for a subgroup of patients with sicca syndrome in which half the cases meet the definition for SS according to European and Manthorpe criteria. This subgroup is characterized by the constant finding of xerostomia, the absence of classical systemic manifestations observed in primary SS, and the absence of anti-SSA or anti-SSB antibodies. Such characteristics delineate a distinctive, virus associated entity that differs from primary SS.
10714099 Systemic mononuclear inflammatory vasculopathy associated with Sjögren's syndrome in a pa 2000 Jan We report a 46-year-old woman with primary biliary cirrhosis (PBC) presenting with Sjögren's syndrome and systemic mononuclear inflammatory vasculopathy. Biopsy specimens of sural nerve showed findings consistent with vasculitic neuropathy. Perivascular inflammatory mononuclear cell infiltration was observed on muscle biopsy specimen. The findings of abdominal computed tomography and brain magnetic resonance imaging were suggestive of vasculitis. Clinical manifestations and radiologic findings were improved after high dose prednisolone therapy.
9714980 Primary Sjögren's syndrome followed by chronic myelogenous leukemia: a case report with a 1998 Jul We describe a female patient with primary Sjögren's syndrome who subsequently developed chronic myelogenous leukemia. The patient received a bone marrow transplant (BMT) from a genotypically HLA-identical brother after pre-transplant preparation with busulfan and cyclophosphamide. Eighteen weeks later, cutaneous and mucosal chronic graft-versus-host disease developed, followed by death 11 months after transplantation. Anti-Ro/SS-A antibodies disappeared within 6 months post-BMT, but antinuclear antibodies remained positive through the post-transplant course.
9709762 Meibomian gland dysfunction in patients with Sjögren syndrome. 1998 Aug OBJECTIVE: Changes in the ocular surface of patients with Sjögren syndrome (SS) often are more severe than those in patients with dry eye without SS. This study was conducted to investigate the possible involvement of meibomian gland dysfunction in SS-related ocular surface abnormalities. DESIGN: A nonrandomized, prospective, clinical study. PARTICIPANTS: Twenty-seven eyes of 27 consecutive patients with SS (SS group) were studied. Twenty-nine eyes of age- and gender-matched non-SS patients with aqueous tear deficiency (non-SS group) were examined as control subjects. INTERVENTION: Changes in the ocular surface, tear function, and meibomian gland were examined. MAIN OUTCOME MEASURES: Tear evaporation rate, meibomian gland expression, and meibography were measured. RESULTS: Fluorescein and rose bengal staining scores were significantly higher in the SS group than in the non-SS group (P = 0.0001). Evaporation of tears was increased significantly in the SS group compared with the non-SS group. There were no significant differences in the rate of tear production between the SS and non-SS groups. Meibography showed that 11 (57.9%) of 19 eyes in the SS group had gland dropout (i.e., histologic destruction of meibomian glands) in more than half of the tarsus. The incidence was significantly higher than that in the non-SS group (5 [18.5%] of 27 eyes; P = 0.005). CONCLUSIONS: The results of this study indicate that destruction of meibomian glands and an increase in tear evaporation often are associated with changes in the ocular surface in patients with SS. Severe ocular surface changes in patients with SS may be attributed, in part, to the meibomian gland dysfunction.
9855167 Elevated tear interleukin-6 levels in patients with Sjögren syndrome. 1998 Dec OBJECTIVE: The purpose of the study was to investigate interleukin-6 (IL-6) levels in the tear fluid and sera of patients with Sjögren syndrome (SS). PARTICIPANTS: Twelve patients with primary SS and 12 normal control subjects participated. INTERVENTION: Tear fluid and sera were obtained from the study and the control groups. Evaluation of tear fluid and sera IL-6 levels was done by using a quantitative enzyme-linked immunosorbent assay kit. All assays were carried out blindly with respect to diagnosis. MAIN OUTCOME MEASURES: Tear fluid IL-6 levels were measured. RESULTS: The mean concentration (+/- standard error) of IL-6 in the tears of patients with SS was elevated significantly compared to that of normal control subjects (88.6+/-16.2 vs. 42.1+/-10.6 pg/ml; P < 0.05). No significant differences were noted in the serum IL-6 levels between the two groups. A significant correlation (r = 0.742, P = 0.006) was found between tear fluid IL-6 levels and the focus score of lip biopsy specimens in patients with SS. CONCLUSION: Tear fluid IL-6 levels may serve as an important marker for tear gland involvement in SS.
9835893 [Idiopathic autonomic neuropathy. An important disease in differential diagnosis]. 1998 Nov 6 HISTORY AND CLINICAL FINDINGS: Two weeks after a mild viral infection a previously healthy 22-year-old woman developed ileus, a severe abnormality of orthostasis, sicca (Sjögren's) syndrome, bilateral miosis and generalized hyphidrosis. Pelvic endoscopy and laparotomy failed to clarify the cause of the mechanical ileus. INVESTIGATIONS: Neurological examination revealed an isolated abnormality of the autonomic system, involving both sympathetic and parasympathetic components. Schellong's, Schirmer's and the ninhydrin tests were markedly abnormal. There was no heart rate variation on breathing and a post-Valsalva hypotensive blood pressure overshoot. Further tests failed to find a cause of the neuropathy. DIAGNOSIS, TREATMENT AND COURSE: The diagnosis of idiopathic panautonomic neuropathy (pandysautonomia) was made. The ileus and hypotension were treated symptomatically with neostigmine, cisapride, midodrine and, initially, with enemas, nasogastric tube feeding and parenteral fluids. The patient was free of symptoms at follow-up examination a year later. CONCLUSIONS: Idiopathic autonomic neuropathy should be considered in the differential diagnosis of functional abnormalities of the sympathetic and/or parasympathetic nervous system, especially in previously healthy young people, in the presence of orthostatic, unexplained gastrointestinal and hidrotic symptoms.
9542204 [Sjogren's syndrome: a new diagnostic approximation]. 1998 Feb At presentation the history of a 51 years old woman with xerostomia and dry keratoconjunctivitis sicca (KCS) with a development of ten months. Investigations revealed the presence in serum of antibodies against cytoplasmic antigens SS-A (Ac anti-Ro/SS-A), antinuclear antibodies (ANAS), and rheumatoid factor (RF). The test with Rose of Bengal was positive, and in the salivary gammagraphy made with pertecnate-99 m Tc it was observed a decreased of the captation and excretion of the designer for the salivary glands. The histopathology and immunohistochemical study of the minor salivary glands showed the presence of a focal lymphocytic sialadenitis (fls) and a predominance of lymphocytes CD4+. It was diagnosed a primary Sjögren a Syndrome (SSP) and the patient was treated with salivary substitutes and artificial tears. We analyse the current diagnostic criteria of the group of studies of the European Community for the SS. We emphasize the importance of the histologics and immunohistochemical study that with the rest of the complementary test will let us to distinguish not only the different forms of presentation of the illness, but also those of all the patients with pathologies which are very prevalent in our environment nowadays, such as the infections by virus C of the hepatitis (VCH) and the human immunodeficiency (VIH).
9249624 Early asymmetric neuropathy in primary Sjögren's syndrome. 1997 Jun We report three female patients, 43, 47, and 50 years old, with a rare asymmetric form of clinically pure sensory neuropathy associated with primary Sjögren's syndrome. In all three patients glandular involvement was accompanied by peripheral nerve disease. Sensory conduction studies showed completely normal results in two of three patients. Yet assessment of thermal-specific thresholds and thermal pain thresholds, combined with autonomic function tests (sympathetic skin response and R-R interval variation) supported the clinical suspicion of peripheral nerve disorder. Sjögren's syndrome must be considered in asymmetric sensory neuropathies of unknown cause.
9073014 Sicca syndrome and silicoproteinosis in a dental technician. 1997 Mar Secondary sicca syndrome has been described in silicotic and exposed nonpneumoconiotic individuals. Systemic autoimmune pathogenic mechanisms have been thought to be responsible. Salivary gland mineral deposition has not so far been implicated. We describe the case of a dental technician who sand-blasted dental prostheses and developed silicoproteinosis and sicca syndrome. Immunological studies were negative. Scanning electron microscopy study and energy-dispersive X-ray analysis were carried out in the patient's lung and salivary gland and in two salivary glands from nonexposed individuals diagnosed with sicca syndrome. High peaks of silicon and aluminium were detected in the patient's lung and salivary gland. Silicon/sulphur and aluminium/sulphur ratios were higher in the patient's glands (0.612 and 0.402, respectively) than in the two control salivary glands (0.193 and 0 and 0.127 and 0, respectively). We suggest that, in this case, sicca syndrome could have been caused by inorganic deposition.
9868312 [A case of primary Sjögren's syndrome with CNS disease mimicking chronic progressive mult 1998 Jul We report a 40-year-old woman with primary Sjögren's syndrome (SjS) with slowly progressive CNS disease. At age 38, she noticed spasticity and very gradual onset of monoparesis in the left leg. She hardly walked by herself at age 40. On admission, neurological examination revealed mild slurred speech, vertical nystagmus, spasticity in the four extremities, spastic monoparesis of the left leg, exaggerated jaw jerk, hyperreflexia in all limbs except right biceps and brachioradialis reflex, and positive bilateral Hoffmann reflexes and Babinski signs. Laboratory examinations disclosed positive anti-nuclear antibody (speckled type) and anti-SS-A/Ro antibody (64x). CSF examination revealed cell count 8/mm3, protein 42 mg/dl, 4 bands of oligoclonal band and the elevation of IgG index. MR-imaging presented multiple plaque-like lesions in white matter of cerebrum and brainstem, which did not show gadolinium enhancement. Additionally she complained of dry eyes. Lacrimal and salivary secretion tests showed hyposecretion of tears and hyposialosis. The biopsied specimen of labial minor salivary gland revealed the destruction of the ducts and periductal lymphocytic infiltrations. The diagnosis of primary SjS was confirmed. We herein report a rare case of primary SjS with CNS disease mimicking chronic progressive multiple sclerosis (MS), and discuss a difficulty in differentiating CNS disease of SjS from MS.
11550985 Etanercept in the treatment of macrophage activation syndrome. 2001 Sep Macrophage activation syndrome (MAS), a recognized complication of systemic juvenile rheumatoid arthritis (sJRA), has been associated with significant morbidity and mortality. Dysregulation of macrophage-lymphocyte interactions leading to uncontrolled proliferation of highly activated macrophages and massive release of proinflammatory cytokines including tumor necrosis factor-alpha (TNF-alpha) appears to be central to the pathogenesis of this syndrome. Until now the mainstay of therapy has been corticosteroids and cyclosporin A. We describe a patient with MAS and sJRA successfully treated with the anti-TNF agent etanercept. The outcome in this patient suggests etanercept might be an effective therapeutic agent in MAS.
10493687 Health related quality of life in a population sample with arthritis. 1999 Sep OBJECTIVE: To determine the prevalence and health related quality of life of a community sample of people with arthritis and compare it with persons with other chronic diseases and the healthy population in South Australia. METHODS: A representative population survey by trained interviewers in autumn 1995 using a multi-stage, systematic, clustered area sample of 4200 urban and country households. There were 3001 (73.6%) respondents aged 15 or over. Subjects were asked, "Have you ever been told by a doctor that you have arthritis?" and "What type?", in addition to the Medical Outcome Survey Short Form-36 (SF-36) health status survey. RESULTS: Medically confirmed arthritis was self-reported in 666 (22.1%) as osteoarthritis (OA) (8.6%), rheumatoid arthritis (RA) (4.0%), and other, or unspecified arthritis (9.6%). People with arthritis were more likely to be female, aged, and of lower socioeonomic status. SF-36 scores were compared to nonarthritic subjects and adjusted for differences in age, sex, and occupational status. Scores were significantly lower for respondents with arthritis, compared with the rest of the population across all subscales of the SF-36 (p<0.05). This was most marked in the subscales measuring physical function and pain. CONCLUSION: Self-reported arthritis is common in the South Australian population, particularly in those aged over 65 years. Arthritis has a major impact on the health related quality of life in the community setting.
10434542 [Saliva production in patients with diffuse lung disease]. 1999 Jun We explored the potential involvement of Sjögren's syndrome as a cause of diffuse lung disease. A prospective clinical study was performed with measurements of saliva production made using the Saxon test. Sixty-seven diffuse lung disease patients who did not exhibit xerosis were examined. The group included 43 patients with sarcoidosis, 11 with interstitial pneumonia, 3 with primary pulmonary lymphoma (PPL), 2 with idiopathic BOOP, 2 with chronic eosinophilic pneumonia, and 6 with other diseases. Decreased saliva production was detected in 11 (16.4%), and Sjögren's syndrome was diagnosed in 4 (6.0%). Lung lesions displayed by the group with Sjögren's syndrome included PPL, bronchiolitis, sarcoidosis, and interstitial pneumonia. We concluded that in patients with diffuse lung diseases, it is always important to discriminate between those with sicca syndrome and Sjögren's syndrome. In our study, the Saxon test proved highly effective as a screening procedure for this purpose.
9598889 Oral manifestations in patients with Sjögren's syndrome. 1998 May OBJECTIVE: To investigate oral signs and symptoms in patients with Sjögren's syndrome (SS), and to compare results with a group of healthy individuals. METHODS. Each individual completed a questionnaire designed to investigate his/her perception of oral health and dryness, as well as a list of signs and symptoms associated with xerostomia. An oral clinical evaluation was performed. RESULTS: A total of 81 subjects were studied, 21 with primary SS, 29 with secondary SS, and 31 healthy individuals. Patients with SS considered that their oral health was poor. Oral dryness was perceived as intense. Symptoms most commonly expressed were: sensitivity to acids (68%), difficulty eating dry foods (66%), and sensitivity to spicy foods (58%). Dryness of the lips (76%) and the tongue (68%) were also among the most frequent complaints. Oral findings of patients with SS included cervical or atypical caries in 83%, fissured erythematous tongue in 70%, and oral candidiasis in 74%. Significant differences were found when results obtained from patients with SS were compared with the healthy group. CONCLUSION: The oral health of this group of patients with SS was deficient. Adequate management of dry mouth was not achieved, mainly for the modifiable components such as dental decay and oral candidiasis. Therefore. treatment and prevention plans are mandatory. These measures might help to reduce the discomfort associated with oral dryness and to reduce dental loss.
9918688 Rheumatoid factor B cell tolerance via autonomous Fas/FasL-independent apoptosis. 1999 Jan 10 Normal individuals do not express the high-affinity autoantibodies specific for self-IgG (rheumatoid factors, RF) that are commonly seen in rheumatoid arthritis patients. Studies of transgenic mice expressing a human IgM rheumatoid factor have shown that one mechanism by which higher affinity RF B cells are tolerized to IgG is through abortive RF B cell activation followed by deletion in the absence of T cell help. We show that RF B cell deletion occurs through an intrinsic apoptotic mechanism that is independent of the Fas/FasL pathway and does not involve active killing by T cells, as it occurs in RAG-1-deficient RF transgenic mice to the same extent as in the parental RF transgenic line.
10778601 Cutaneous vasculitis in a patient with primary Sjogren's syndrome. 1999 Jul Sjogren's syndrome (SS) is an uncommonly described disorder from India. In this report we describe a patient with SS and who developed cutaneous vasculitis.