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ID PMID Title PublicationDate abstract
8398208 [Primary Sjogren syndrome with involvement of the nervous system. Description of 4 cases]. 1993 Aug Four patients, 2 males and 2 females, with primary Sjögren's syndrome (PSS) and neurologic involvement are described. The first patient presented a progressive axonal polyneuropathy due to endo-epineural vasculitis. The second patient developed an acute and focal lymphocytic meningoencephalitis with increased IgG synthesis and excellent response to corticosteroids. The remaining 2 patients had previously been diagnosed as having definite multiple sclerosis (MS), but after a standardized study both fulfilled PSS diagnostic criteria. These cases illustrate the polymorphism of the neurological manifestations in PSS. Pathogenetic relationships between PSS and MS are briefly reviewed.
8344013 Hemiparkinsonism in a patient with primary Sjögren's syndrome. A case report and a review 1993 Jun A 55-year-old woman presented with hemiparkinsonism on the left side. Clinical features suggested primary Sjögren's syndrome. Eye tests and a salivary gland biopsy confirmed this diagnosis. Magnetic resonance imaging of the brain showed distinct linear lesions of increased intensity on the T2-weighted images in the right striatum and globus pallidum. Treatment with prednisone and azathioprine did not give any improvement of the neurological symptoms. To date, hemiparkinsonism in association with primary Sjögren's syndrome has not been reported.
1435804 [Transient, prolonged, combined sicca syndrome of the eyes and mouth. Occurrence along wit 1992 Aug A girl of eight years fell ill with signs of common cold and bronchitis. Already on the second day of illness, before any treatment was started, she showed symptoms of a sicca-syndrome, beginning in the eyes, later the mouth. Ophthalmologic examination showed keratitis filiformis, histology of the salivary glands in the mucosa of the mouth was normal. Local substitution therapy led to subjective improvement. The disorder of secretion persisted for four months only, then rapid restitutio ad integrum of tears and saliva production occurred. Pathogenetically a parainfectious immunologic mechanism seems probable. During the observed period there were significant changes of antibody titers against RS-virus and streptococci, but it is not possible to identify the cause of the illness really. We conclude that a sicca-syndrome with normal histology of mouth-mucosa-glands is not necessarily prognostically infavorable.
1605151 Optic neuropathy and central nervous system disease associated with primary Sjögren's syn 1992 Jun Three cases of optic neuropathy associated with primary Sjögren's syndrome are reported. All three patients had clinical manifestations of primary Sjögren's syndrome, although two of the patients did not report sicca symptoms at initial examination. Two patients had focal neurologic signs in addition to optic neuropathy. The differentiation of this syndrome of optic neuropathy, focal neurologic signs, and Sjögren's syndrome from multiple sclerosis and antiphospholipid antibody syndrome is important for reasons of treatment and prognosis. This diagnostic differentiation was facilitated by positive tests for xerophthalmia and findings of positive minor salivary gland biopsy. High titers of antinuclear antibody, anti-SSA(Ro), and anti-SSB(La), and the absence of antiphospholipid antibodies provided additional help in the differential diagnosis. In 5 years of observation, none of the patients developed symptoms of multiple sclerosis or additional connective tissue disorders.
8014941 Spanish version of the Health Assessment Questionnaire: reliability, validity and transcul 1993 Dec OBJECTIVE: To study the feasibility, reliability, and validity, of a Spanish European version of the Health Assessment Questionnaire (SHAQ). METHODS: A total of 170 patients with rheumatoid arthritis (RA) from 11 public general hospitals in Spain were included. We studied (a) the feasibility of the SHAQ by finding the proportion of patients able to self-administer it and the time they take to do so; (b) the test-retest reliability of the SHAQ when completed twice, with an interval of one week, calculating the Pearson correlation coefficient (r) between the first and second SHAQ scores; (c) the criterion validity of the SHAQ by comparing the clinician's assessment of the patient's activities (observed disability) with the scores noted by the patient in the questionnaire (referred disability); (d) cross sectional construct validity comparing the scores of the SHAQ with the other tests used in the assessment of RA; and (e) the longitudinal construct validity of the SHAQ comparing the difference between the initial and final SHAQ scores with the patient rating of improvement or worsening after a 3-month followup. RESULTS: The SHAQ was self-administered by 63% of patients, the rest needed the presence of an interviewer. The time taken to complete the questionnaire was 6.4 min. Test-retest reliability (r = 0.89), criterion validity (r = 0.87), cross sectional construct validity, and longitudinal construct validity were satisfactory and similar to other HAQ versions used in different countries. CONCLUSION: The SHAQ is a reliable, valid tool, but for use in Spain an interviewer may be necessary.
7774091 Juvenile chronic arthritis in Costa Rica. A pilot referral study. 1995 Jan In order to adequately care for patients with chronic disorders and to properly allocate resources, the epidemiology of the underlying disease must be know. Proper population based studies involve substantial planning and educational programs, however. To prepare for such a study of pediatric rheumatic disorders, we performed a referral-based pilot study. During an eleven-month period pediatricians all over Costa Rica were asked to refer to us all new cases of possible rheumatic disorders among children less than 16 years of age, using the EULAR criteria for juvenile chronic arthritis. The children were evaluated at the National Children's Hospital. An annual incidence of 5.9 cases of all types of pediatric rheumatic diseases per 100,000 was found. Incidences of 5.4 per 100,000 children below 16 years of age and 6.1 for children below 12 years of age were observed for juvenile chronic arthritis (JCA). 77% of the JCA cases were of pauciarticular onset, and 23% were of polyarticular onset. No cases of systemic JCA were diagnosed. The female to male ratio was 3:2. Antinuclear antibodies were positive in 13% of the JCA cases, and IgM rheumatoid factor was found in 15% of the children. Chronic iritis was noted in only 2 cases; both were girls and both were ANA positive. The incidence found was low compared to population-based studies, but in the same range as hospital-based investigations.
8674240 Expression of selectins (CD62 E,L,P) and cellular adhesion molecules in primary Sjögren's 1996 Jul Adhesion molecules are important signal transmitters of the immune system and may mediate the homing of leukocytes to sites of inflammation. The aim of this work was to examine the presence of selecting and cellular adhesion molecules on epithelial and endothelial cells in labial salivary glands (LSG) in Sjögren's syndrome (SS). LSG biopsies were obtained from patients with primary SS (n = 31) and normal subjects (n = 21). Cryostat sections were examined with indirect immunoperoxidase. Epithelial cells in LSG from both patients and controls expressed LFA-3 (CD58) and Hermes I (CD44). A significantly increased number of acinar and ductal epithelial cells in LSG from patients expressed class I MHC (74%, as mean percentage of ductal epithelial cells) (P < 0.05), HLA-DR (58%) (P < 0.0001), and HLA-DQ (11%) (P < 0.001). To a lesser extent limited ICAM-1 (CD54) epithelial expression (6%) was noted only in a few biopsies from patients but none of the controls. Epithelial cells did not express any of the selectins CD62 E, L, and P and sometimes they expressed sialyl Le(x) (a ligand for selectins). Although the number of endothelial structures expressing ICAM-1 (CD54), HLA-DR, HLA-DQ, and class I MHC (per surface area) was increased in patients (P < 0.05), this may be due to the total increase of number of endothelial structures (P < 0.05) (Von Willebrand factor +ve) as part of the chronic inflammatory process. A smaller proportion of endothelial structures expressed E-selectin (CD62 E) (32%) and to a lesser extent VCAM-1 (CD106) (approximately 7%) as detectable only in some LSG from patients. P-selectin (CD62 P) was demonstrated on about one-third of endothelial structures in LSG from patients. Infiltrating mononuclear cells expressed CD11a (68%), CD18 (73%), CD11b (13%), CD11c (21%), CD58 (13%), CD4 (44%), CD8 (17%), CD62L (L-selectin) (18%), CD49d (38%), CD49e (15%), CD2 (56%), and CD44 (77%). The relatively reduced number of CD62 L +ve lymphocytes may be due to shedding of that molecule after activation. Sialyl Le(x) was not detectable on infiltrating lymphocytes. Although infiltrating mononuclear cells were activated, as evidenced by their expression of HLA-DR (72%) and ICAM-1 (55%), they did not express IL-2Ralpha (CD25, confirmed by two antibodies 2A3 and ACT1) or IL-2Rbeta (CD122), except rarely (< or = 1%). In some biopsies, CD106 and CD11c were localized on lymphocytes at the central areas of periductal lymphoid follicles with the appearance of dendritic cells. We conclude that adhesion molecules probably play a major role in the pathogenesis of SS. The pattern of expression of these molecules demonstrates a regulated altered activation in the glands associated with this disease. The glands may be subject to specific regulatory factors, in addition to proinflammatory cytokines.
8621782 Common T cell receptor clonotype in lacrimal glands and labial salivary glands from patien 1996 Apr 15 Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration into lacrimal and salivary glands leading to symptomatic dry eyes and mouth. Immunohistological studies have clarified that the majority of infiltrating lymphocytes around the lacrimal glands and labial salivary glands are CD4 positive alphabeta T cells. To analyze the pathogenesis of T cells infiltrating into lacrimal and labial salivary glands, we examined T cell clonotype of these cells in both glands from four SS patients using PCR-single-strand conformation polymorphism (SSCP) and a sequencing method. SSCP analysis showed that some infiltrating T cells in both glands expand clonally, suggesting that the cells proliferate by antigen-driven stimulation. Intriguingly, six to sixteen identical T cell receptor (TCR) Vbeta genes were commonly found in lacrimal glands and labial salivary glands from individual patients. This indicates that some T cells infiltrating into both glands recognize the shared epitopes on autoantigens. Moreover, highly conserved amino acid sequence motifs were found in the TCR CDR3 region bearing the same TCR Vbeta family gene from four SS patients, supporting the notion that the shared epitopes on antigens are limited. In conclusion, these findings suggest that some autoreactive T cells infiltrating into the lips and eyes recognized restricted epitopes of a common autoantigen in patients with SS.
8837816 Multiple cerebral arterial occlusions in a young patient with Sjögren's syndrome: case re 1996 Mar We describe a case of a young patient with multiple occlusions of major cerebral arteries and Sjögren's syndrome. This 17-year-old female patient experienced repeated transient ischemic attacks of right hemiparesis, speech disturbance, and unconsciousness. Angiography revealed progressive occlusion of the bilateral carotid and vertebral arteries. Examinations, including a serological test, a rose bengal test, Ga scintigraphy, and a biopsy of the parotid gland, indicated Sjögren's syndrome. The patient was successfully managed with bypass surgery. Patients with Sjögren's syndrome may experience progressive occlusion of the major cerebral arteries resembling that of moyamoya disease.
8870313 Undifferentiated systemic connective tissue disease-"overlap' in Zimbabwe. 1996 Jun OBJECTIVE: To define the clinical characteristics of the overlapping, undifferentiated systemic connective tissue disease in one of the tropical African countries. DESIGN: A descriptive retrospective study of records of patients registered on a special rheumatology clinic between 1989 and 1994. SETTING: Harare Central Hospital Rheumatology Clinic, Zimbabwe. SUBJECTS: All registered patients with systemic connective tissue diseases. MAIN OUTCOME MEASURES: Numbers of patients, laboratory and clinical features of undifferentiated overlapping connective tissue disease. RESULTS: The distribution for those who satisfied internationally accepted criteria for classification/diagnosis were: 48(52.8pc) rheumatoid arthritis (RA), 17(18.7pc) systemic lupus erythematosus (SLE), 10(11pc) juvenile chronic arthritis (JCA), four (4.4pc) polymyositis/dermatomyositis (PMD) and 11 (12.1pc) overlapping connective tissue disease (overlap) and one unclassified disease. No pure progressive systemic (PSS) was registered. Among the overlap patients, five had SLE-RA, three SLE-PSS and one each for SLE-RA-PSS, RA-PSS and SLE-PDM overlapping clinical features respectively. In two patients with SLE-PSS, the criteria of each of the individual connective tissue disease were satisfied. Similarly, in one patient with SLE-RA and another with RA-PSS features, the criteria of individual disease were also satisfied. CONCLUSION: In undifferentiated overlapping connective tissue diseases-overlaps occur in tropical Africa, the SLE-RA overlapping features predominate and are moderately severe diseases.
8604725 Nonsteroidal anti-inflammatory drugs: practical and theoretical considerations in their se 1996 Feb 26 Nonsteroidal anti-inflammatory drugs (NSAIDs) are prescribed frequently for patients with painful musculoskeletal conditions: Each year, physicians write approximately 60 million NSAID prescriptions. Because of the magnitude of patient exposure, gastrointestinal and other side effects of NSAIDs are a significant clinical concern. The mechanism of action of NSAIDs is inhibition of cyclooxygenase with secondary inhibition of proinflammatory prostaglandins. This mechanism also accounts for gastrointestinal toxic side effects of NSAIDs. Two forms of cyclooxygenase, cox-1 and cox-2, appear to be differentially inhibited by NSAIDs. Because cox-1 is responsible for maintaining normal physiologic function in gastric mucosa and other tissue, "ideal" NSAIDs would suppress only cox-2. The design of future NSAIDs-related peptic ulceration is characterized by its location in the gastric antrum, asymptomatic nature, and ability to develop through both topical and systemic effects of NSAIDs. Major risk factors for patients with rheumatoid arthritis include age >60 years, magnitude of disability, concomitant use of corticosteroids, larger doses/longer duration of NSAID treatment, and a history of peptic ulcer disease. A prophylactic strategy includes the identification of high-risk patients and, if NSAIDs must be used, the addition of misoprostol.
7995164 Crohn's disease in the Chinese population. An experience from Hong Kong. 1994 Dec PURPOSE: Crohn's disease was extremely rare among Chinese. We reviewed all cases diagnosed as having Crohn's disease during a five-year period. METHODS: A diagnosis of Crohn's disease was made only if all of the following criteria were fulfilled: 1) clinical symptom(s) and sign(s) compatible with chronic inflammatory bowel disease; 2) exclusion of intestinal infection by repeated stool cultures; 3) macroscopic features of small and/or large intestinal inflammation with skip lesion, stricture, and fistula formation; 4) histologic features of Crohn's disease, i.e., focal lymphoid aggregate, focal cryptitis, and granuloma formation; 5) clinical response to conventional therapy for inflammatory bowel disease. RESULTS: Fifteen ethnic Chinese patients were diagnosed as having Crohn's disease in this period. All patients had colitis, whereas small intestine inflammation was documented in only 47 percent of patients. Extraintestinal manifestations were uncommon except for arthropathy: ankylosing spondylitis (2), sacroiliitis (1), juvenile rheumatoid arthritis (1), and colitic arthritis (1). The majority of our patients responded to medical therapy. Surgery was undertaken in 33 percent of patients. CONCLUSION: Although there is a general increased incidence of Crohn's disease in the Western world, we too are beginning to see more cases in the Far East. Nevertheless, gastrointestinal infection with bacteria and/or parasites should still be carefully excluded in these countries.
8016417 Histocompatibility typing in the rheumatic diseases. Diagnostic and prognostic implication 1994 May Associations of HLA antigens with many of the rheumatic diseases have been established over the last two decades. Although these discoveries provide potential new insights into disease pathogenesis, the clinical utility of HLA typing has been limited. The major exception is that of HLA-B27 in the spondyloarthropathies, where clinical uses of HLA-B27 testing has permitted identification of a large spectrum of disease that was previously misdiagnosed and misclassified. HLA-B27 remains potentially useful in the diagnosis of atypical spondyloarthropathies because of its high frequency in patients with these diseases (yielding good sensitivity) and its relatively low frequencies in most normal populations (yielding good specificity). Its predictive value in individual cases, however, depends on the quality of the physician's assessment of the likelihood of a spondyloarthropathy. In patients with juvenile-onset arthritis, typing for HLA-B27, as well as several HLA-class II alleles (DR5, DR8, DP2, and DP3), may prove to be useful in diagnosis and classification; however, additional studies are necessary. HLA oligotyping of DNA in patients with early rheumatoid arthritis to determine homozygosity versus heterozygosity for the DRB1 susceptibility sequence promises a potential new parameter for predicting clinical disease severity, and thus the possible early initiation of more aggressive therapies. Additional studies are necessary, however, to determine the validity of this approach. Finally, the future diagnosis, prevention, and treatments of these diseases may depend on the identification and manipulation of specific immune responses mediated by HLA molecules, thus making HLA typing for clinical purposes routine.
7510100 The role of nitric oxide in parasitic diseases. 1993 Dec Murine macrophages express high levels of nitric oxide synthase and produce large amounts of nitric oxide (NO) when stimulated with certain cytokines in the presence of a trace amount of lipopolysaccharide (LPS). The stimulatory cytokines include interleukin-1 (IL-1), interferon-gamma (IFN-gamma), tumour necrosis factor-alpha (TNF-alpha) and migration inhibitory factor. Activated macrophages are highly effective killers of intra- and extra-cellular pathogens. However, as excessive NO can lead to immunopathology (diabetes, graft-v.-host disease, EAE, liver cirrhosis, rheumatoid arthritis), NO production is necessarily under tight regulation. A number of cytokines, including IL-4, IL-10 and transforming growth factor-beta, can down regulate the induction of NO synthase in macrophages. In addition, macrophages exposed to LPS alone and then stimulated with a mix of IFN-gamma and LPS express significantly lower levels of NO synthase than cells stimulated without pre-exposure to LPS. Furthermore, NO can reduce the activity of NO synthase by feedback inhibition, and also inhibit the production of IFN-gamma by Th1 cells (thus turning off its own synthesis from upstream). The regulatory pathways involve tyrosine kinase and protein kinase C.
8489544 Susceptibility to relapsing polychondritis is associated with HLA-DR4. 1993 May OBJECTIVE: To determine the frequency of HLA class II antigens in Caucasian central European patients with relapsing polychondritis (RP). METHODS: HLA class I, DR, and DQ specificities were identified in 41 patients with RP, and the frequencies were compared with those in 204 healthy, unrelated control subjects. HLA typing was performed using the standard complement-dependent microcytotoxicity assay. HLA-DR genotyping of 12 DR4-positive RP patients and 57 controls was performed by allele-specific oligonucleotide probing after amplification of genomic DNA by polymerase chain reaction. RESULTS: A significant increase in DR4 antigen frequency was found in the patients (56.1%) as compared with that in healthy controls (25.5%) (Pcorr < 0.001). Genotyping of DR4-positive patients and controls revealed no predominance of any DR4 subtype. CONCLUSION: There are important clinical similarities and overlaps between RP and rheumatoid arthritis (RA). In RA, the association with DR4 has been well established. Our findings show that although there is a DR4 association with RP, the situation is sufficiently distinct from that of RA to imply considerable differences in pathogenesis of the two conditions.
7683882 Labial salivary gland biopsy is a reliable test for the diagnosis of primary and secondary 1993 May OBJECTIVE: Reports of the detection of amyloidosis by labial salivary gland (LSG) biopsy have been mostly anecdotal. The aim of this study was to assess the value of this method in the diagnosis of amyloidosis. METHODS: LSG biopsy tissues were studied with a combination method using Congo red stain and immunohistologic characterization using an antibody directed against the serum amyloid P (SAP) component. Electron microscopy was performed in all cases. In a prospective study, we evaluated 30 patients with biopsy-proven AA or AL amyloidosis. We compared these patients with a control group of 29 age-matched patients without clinical or biologic evidence of amyloid disease (14 had rheumatoid arthritis and 15 had plasma cell dyscrasia). RESULTS: In 26 of the 30 patients with known systemic amyloidosis, amyloid deposits were identified on LSG biopsy (sensitivity of 86%). In 1 of the remaining patients, amyloid deposits were identified on LSG biopsy and systemic amyloidosis was confirmed by abdominal fat biopsy and 123I-labeled SAP scintigraphy. CONCLUSION: This study emphasizes the high sensitivity of LSG biopsy in the diagnosis of amyloidosis, even in the absence of oral symptoms.
7652465 Neutrophil gelatinase levels in plasma and synovial fluid of patients with rheumatic disea 1995 To examine the clinical significance of neutrophil gelatinase in rheumatic diseases, plasma and synovial fluid (SF) gelatinase levels were determined in 62 patients with rheumatoid arthritis (RA), 12 patients with ankylosing spondylitis (AS), 18 patients with osteoarthritis (OA) and 17 healthy controls. The gelatinase level was measured by enzyme-linked immunoassay (ELISA). The assay had a sensitivity of 1 ng/ml and a working range of 5-25 ng/ml. Gelatinase levels were significantly higher in the plasma of patients with RA and of patients with RA complicated by amyloidosis or vasculitis as compared to those of healthy controls. Moreover, the mean value of gelatinase in the plasma of patients with RA complicated by vasculitis was found to be significantly higher than that of RA patients without vasculitis. A significant increase in gelatinase concentration was also observed in the plasma of AS patients but not in the plasma of patients with OA. The concentration of gelatinase in the RA SF samples was much higher (18-fold) than the level of the enzyme in the plasma of RA patients. There was also a higher concentration of gelatinase (four-fold) in OA SF compared with OA plasma. The results suggested that circulating gelatinase may reflect some degree of neutrophil activation in patients with inflammatory arthritis, especially in those with RA complicated by vasculitis. However, the results did not allow a differentiation between chronic and acute inflammation.
7839154 Amyloidosis: prognosis and treatment. 1994 Oct The objective of this study was to review (1) the factors that have been linked to prediction of clinical outcome and survival in amyloidosis and (2) the available studies on the therapy for localized and systemic forms of amyloidosis. We made a retrospective review of the relevant literature on treatment and prognosis in localized and systemic amyloidosis dating back to 1975. The most important prognostic factors in amyloidosis are the presence of congestive heart failure, beta 2-microglobulin, and whether peripheral neuropathy dominates the presentation. The presence of a monoclonal light chain in serum or urine, multiple myeloma, and hepatic involvement are also important adverse factors. Colchicine is beneficial in treating familial Mediterranean fever and may play a role in managing secondary amyloidosis in inflammatory bowel disease. Chlorambucil is particularly useful in juvenile rheumatoid arthritis with amyloidosis. Dimethyl sulfoxide provides benefit in bladder and lichen amyloidosis. A trial of alkylating agent-based chemotherapy is reasonable in symptomatic primary systemic amyloidosis. Advances have been made in the treatment of amyloidosis and include chemotherapy, dialysis, transplantation, and improved supportive care. Definite disease regressions with long-term survival (> 10 years) are seen. Unfortunately, alternatives still need to be developed: Of 859 patients with primary systemic amyloidosis seen at the Mayo Clinic from 1982 to 1992, the median survival was 2.1 years.
8849382 Sensitivity and specificity of anti-Jo-1 antibodies in autoimmune diseases with myositis. 1996 Feb OBJECTIVE: To determine the sensitivity and specificity of anti-Jo-1 in systemic sclerosis (SSc) patients with and without myositis. METHODS: Immunoblots on HeLa nuclei were used to screen sera from 554 consecutive connective tissue disease patients. Those who had 45-55-kd bands, all patients with polymyositis/dermatomyositis (PM/DM), and a random selection of SSc, Raynaud's disease, systemic lupus erythematosus, and rheumatoid arthritis patients were also studied by anti-Jo-1 enzyme-linked immunosorbent assay and by immunoblots on rabbit pooled aminoacyl-transfer RNA synthetase. RESULTS: Anti-Jo-1 was present only in 8 of the 40 PM/DM patients. CONCLUSION: Anti-Jo-1 is specific for PM/DM.
8011155 Oral tolerance: a biologically relevant pathway to generate peripheral tolerance against e 1994 OT is a relevant biological pathway for generating peripheral tolerance against both self and external antigens with minimal side effects (fig. 3). This route might, therefore, contain promising potential for the treatment of autoimmune and allergic diseases in the human (fig. 3). Thus, oral administration of autoantigens suppresses experimental autoimmune diseases (EAE, EAU, AA, collagen-induced arthritis, NOD diabetes) in a disease- and antigen-specific manner, and oral administration of alloantigens has led to increase of allograft survival. OT might be important in treatment of immune complex diseases and food allergies. OT is mediated by T lymphocytes using at least two nonmutually exclusive mechanisms: suppression and anergy. Suppression can be adoptively transferred by CD8+ T lymphocytes which act by releasing TGF-beta and IL-4 following antigen-specific triggering. Antigen-driven tissue-directed suppression occurs following oral administration of an antigen from the target organ, even if it is not the disease-inducing antigen (bystander suppression). Thus, synthetic peptides can induce OT, and tolerogenic epitopes of antigen may be different from the autoreactive epitope. Due to the promising results in animal models, OT is being tested in clinical trials in multiple sclerosis, rheumatoid arthritis and uveitis [193, 194].