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ID PMID Title PublicationDate abstract
1629834 Inclusion body myositis in association with vitamin B12 deficiency and Sjögren's syndrome 1992 Feb Autoimmune disorders are very rarely associated with inclusion body myositis (IBM). We describe a patient with IBM in association with Sjögren's syndrome (SS) and Vitamin B12 deficiency. B12 deficiency has not been reported in SS, and this deficiency may explain the neuropathic features of some patients with IBM.
1600845 Ocular surface lesions due to keratoconjunctivitis sicca: in primary Sjögren's syndrome. 1992 Keratoconjunctivitis sicca is usually part of Sjögren's syndrome (SS) which is systemic disease. Ocular surface lesions are ascribed either to the reduction, or to to the low quality of tears. To address this question, ocular surface lesions were evaluated in 20 SS patients by the rose bengal test (RBT). The results were compared to those obtained in 20 normal volunteers and collated with the quality (estimated by the break-up-time) and the quantity of tears (estimated by the shirmer's I test and the level of lacrimal lactoferrin, LF). The RBT scores did not correlate with the shirmer's test results, neither did they with the break-up times. This is consistent with the view that Schirmer's I Test and break-up-time cannot make any distinction between patients from controls. In contrast, the RBT scores correlated well with the levels lacrimal LF and paralleled to serological abnormalities.
8930579 Analysis of the concentration and output of whole salivary constituents in patients with S 1996 Aug In Sjögren's syndrome, salivary glands are affected, resulting in a diminished salivary flow. In the present study, the protein composition, sialic acid content and the amounts of calcium and phosphate of stimulated whole saliva from 43 patients with Sjögren's syndrome, were compared with those of control saliva samples from 17 healthy subjects. The absolute concentrations of albumin, cystatin C, cystatin S, total IgA and total protein, but not amylase, were increased significantly in both primary and secondary Sjögren's syndrome. The output/min of total protein, albumin, amylase, and IgA was, however, decreased in Sjögren patients. These results suggest that the diminished output of salivary defence factors, rather than their absolute concentrations, may be related to the oral health problems seen in Sjögren's syndrome patients.
7554556 Abnormal expression of apoptosis-related antigens, Fas and bcl-2, on circulating T-lymphoc 1995 May OBJECTIVE: To clarify the pathological role of the apoptosis-related molecules expressed on peripheral blood (PB) lymphocyte subsets in primary Sjögren's syndrome (SS). METHODS: The levels of apoptosis-regulating proteins, Fas and bcl-2, were determined in the PB lymphocyte subsets from 21 patients with SS and 14 healthy controls by 2-color flow cytometry. RESULTS: In the PB from SS patients, lymphocytopenia, especially CD4+ cell-lymphocytopenia, was prominent. As observed in previous studies, the percentages of CD4+ CD45RA+ cells were lower in the SS patients than in the controls, while activated (DR+) cells were increased in CD4+ cells from the patients. Fas+ cells were also increased in the patients' CD4+ cells and CD8+ cells, but not in their B cells or natural killer cells. Furthermore, we observed several positive correlations among the percentages of activated cells (DR+ cells or CD45RA-cells) and Fas+ cells recognized in the CD4+ and/or CD8+ cells from the patients. On the other hand, intra-cellular bcl-2 proteins measured as mean fluorescence intensity were significantly diminished in the CD4+ cells, CD8+ cells, CD19+ cells, CD45RO+ cells and Fas+ cells from 14 SS patients compared with 12 healthy controls. In addition, the numbers and/or percentages of CD4+ cells and Fas+ cells positively correlated with their expression of bcl-2 in SS patients. CONCLUSION: The abnormal balance between Fas and bcl-2 expression detected in the PB lymphocyte subsets from SS patients relates, at least partially, to the lymphocytopenia observed in the patients.
7517099 Sjögren's syndrome without mixed cryoglobulinemia is not associated with hepatitis C viru 1994 Jul OBJECTIVES: Hepatitis C virus infection has been associated with the formation of autoantibodies. Furthermore, several autoimmune and immune-complex mediated disorders have been proposed to be associated with hepatitis C virus infection. The best documented of these associations is between hepatitis C virus infection and essential mixed cryoglobulinemia. Essential mixed cryoglobulinemia is itself frequently associated with Sjögren's syndrome, and hepatitis C virus infection has been reported to be accompanied by lymphocytic sialadenitis and by xerophthalmia. We therefore searched for hepatitis C virus RNA and antibodies in 48 patients with SS-A/SS-B autoantibodies. METHODS: Patients with both SS-A and SS-B autoantibodies were included if stored serum samples and medical records were available. No patients had clinically apparent chronic liver disease, and no patients had cryoglobulinemia. Six patients (group 1) had abnormally high aminotransferase values on one or more occasion, a history of acute hepatitis, or blood product exposure through transfusion or intravenous drug use. In 18 cases (group 2) no liver disease could be established, but there was insufficient information to exclude it. The 24 remaining patients (group 3) had no risk factors for liver disease, no known history of acute or chronic liver disease, a normal physical examination for signs of chronic hepatitis, and repeatedly normal aminotransferase values. Antibody to hepatitis C virus was determined by RIBA-2 (Chiron Corp, Emeryville, CA). The samples were tested for hepatitis C virus RNA after polymerase chain reaction amplification. RESULTS: No patient had detectable antibody to hepatitis C virus; 47 were negative, and one sample from group 1 was indeterminate. No patients were positive for hepatitis C virus RNA. CONCLUSIONS: We conclude that our patients with Sjögren's syndrome, without cryoglobulinemia and with detectable SS-A/SS-B autoantibodies, do not have clinically apparent liver disease, hepatitis C viremia by polymerase chain reaction, or antibodies to hepatitis C virus by second-generation testing using RIBA-2.
8353984 Functional characterization of T cells bearing the gamma/delta T-cell receptor in patients 1993 May High percentages of gamma/delta+ T cells in the peripheral blood of a subgroup of patients with primary Sjögren's syndrome (SS) were found. This allowed us to purify and analyze them without their being previously expanded in vitro, and to investigate, therefore, the role of these cells in the pathological immune response which characterizes such systemic autoimmune disorders. The results showed poor proliferation of patient gamma/delta+ T cells in response to anti-CD3, due not to macrophage-dependent suppression but to defective interleukin 2 (IL-2) synthesis. Despite the defective proliferation patient gamma/delta+ cells, unlike those of the normal controls, provided a helper effect in inducing B cells to secrete immunoglobulins (Ig), particularly when they were preincubated with IL-2. The relative increase in a gamma/delta+ T cell subset which, although it secretes low levels of IL-2, is able to provide help for B-cell Ig synthesis, suggests that this T-cell subpopulation may be functional in vivo and may be involved in the pathological immune response encountered in pSS.
1395154 Oculocutaneous manifestations observed in multisystem disorders. 1992 Oct Patients with multisystem disorders frequently present with or eventually manifest cutaneous disease. Many of these patients will also manifest ocular changes. In these disorders, the dermatologist or ophthalmologist may act as primary provider or consultant. Thus, familiarity with these conditions will enable the specialist to provide better care for these patients. This article reviews the oculocutaneous manifestations of sarcoidosis, Behçet's disease, Reiter's syndrome, and Sjögren's syndrome.
1516595 Inheritance of monoclonal rheumatoid factor cross-reactive idiotypes in primary Sjögren's 1992 Jul A comparison of the presence of two idiotypes, one identified by a rabbit polyclonal antiidiotypic antibody, first found on a cryoprecipitable IgM chi rheumatoid factor (RF) from an SS patient (3rd SS) and the 17109 idiotype, identified by a monoclonal antibody was performed in 106 sera and eight minor salivary gland biopsies of Sjögren's syndrome (SS) patients and 125 sera from age-sex matched normal controls. Of 106 of SS patients' sera 36 had immunoglobulins positive for the 3rd SS idiotype. 17109 activity was more prevalent in SS patients positive for the polyclonal anti-idiotype 3rd SS, than those with negative idiotype (9/36 VS 2/70 chi 2 = 12.53 P less than 0.005). Cross inhibition studies, however, revealed that the polyclonal anti-idiotype binding was not inhibited by the 17109 moAb. 3rd SS and 17109 anti-idiotypes were reacted with immunoglobulins in the serum of 3.5% and 1.7% of normal human sera respectively. Immunohistologic studies demonstrated that 4/8 and 2/6 minor salivary gland biopsies had infiltrating plasma cells containing immunoglobulins bearing the 3rd SS and the 17109 idiotypes, respectively. The inheritance of both idiotypes was investigated in sera of 4 SS kindreds. In two kindreds with 3rd SS positive probands, the idiotype was detected in 3 first degree relatives of the same generation. 17109 activity was detected in the serum of a sister of the positive proband who had a high RF titer. These results suggest that the 17109 moAb recognizes a different epitope of that of the 3rd SS. The idiotypes of monoclonal RFs are not inherited and probably are produced by plasma cells infiltrating the labial minor salivary glands of SS patients.
1581472 Histopathological changes in exocrine glands of murine transplantation chimeras. II: Sjög 1992 Autoimmune reactions are evoked in hybrid mice after induction of a chronic graft-versus-host reaction by transfer of viable leucocytes from one of the parental strains to non-irradiated F1 recipients. We have previously demonstrated an SLE-like syndrome early in the reaction, with an additional Sjögren's syndrome-like glandular affection occurring later. In this study, we used Balb/c mice as donors and Balb/cxCBA/H-T6 F1 hybrids as recipients. We found serum autoantibodies characteristic of SLE after 9 weeks but not after 20 weeks. No clinical signs of disease were seen at any time. After prolonged studies (5 months), we found heavy inflammation in Harderian, salivary, and tear glands. All animals survived the entire length of the experiment without signs of renal failure. The pathological manifestations: lymphocytic infiltration of exocrine glands, and enlargement of lymph nodes, are similar to those seen in patients with Sjögren's syndrome. This murine transplantation chimera may be a useful experimental model for primary Sjögren's syndrome.
8164207 Minor salivary gland biopsies in patients investigated for primary Sjögren's syndrome. A 1993 Sep OBJECTIVE: To correlate presenting features and indication for biopsy with results in patients undergoing minor salivary gland biopsy for the diagnosis of primary Sjögren's syndrome (SS). METHODS: The charts of 187 patients undergoing minor salivary gland biopsy for primary SS over a 9-year period were reviewed. RESULTS: 76 patients had a focus score > 1, 111 had a focus score < or = 1. No difference between the 2 groups was noted in most features, including frequency of symptomatic dry eyes or mouth, or Schirmer test results. Patients with focus score > 1 had significant increases in frequency of salivary gland swelling (25 vs 9%), antinuclear antibodies > 1:100 (68 vs 32%), rheumatoid factor > 1:160 (63 vs 22%), anti-SSA (46 vs 9%), anti-SSB (32 vs 4%), or any serologic marker (87 vs 46%). Abnormal biopsies were more frequent in those biopsied for serologic abnormalities (53%) than for sicca symptoms (33%) or systemic illness (29%). CONCLUSION: Serologic markers are better predictors of results than clinical features in patients undergoing minor salivary gland biopsy for primary SS. The frequency of a positive biopsy is increased in patients in whom unexplained serologic markers are being evaluated.
8456268 [Suspected adult-onset Still's disease: unexpected manifestation of leprosy]. 1993 Mar 6 A 23-year old male from Sri Lanka was admitted to hospital with symmetrical inflammatory peripheral polyarthritis, fever of 39 degrees C and poly-lymphadenopathy. At first we suspected adult onset Still's disease. The histological findings from axillary lymph node biopsy strongly suggested the diagnosis of leprosy, for which we had had little evidence thus far. Typical skin lesions were absent, skin smears were negative and neurological symptoms only became obvious much later when fever and arthritis had subsided under anti-inflammatory treatment. At this time a right ulnar palsy developed, with atrophy of the interosseous muscles and thickening of the ulnar nerves at both medial epicondyles. Fite-stains of a sural nerve biopsy confirmed the diagnosis when mycobacteria were detected. Leprosy displays a clinico-pathological spectrum caused by variations in host resistance. A widely accepted classification is the five group system of Ridley and Jopling. At one extreme of this spectrum are patients with lepromatous or low resistance leprosy with numerous bacilli, and at the other those with high resistance or tuberculous leprosy where few or no bacilli are found. The numerous bacilli in the sural nerve biopsy classified the disease as lepromatous in our case. Of the various manifestations of the lepra reaction occurring in lepromatous leprosy, one is acute arthritis, but a more common one is erythema nodosum leprosum. Our patient's clinical presentation was interpreted to be a rheumatic manifestation of a type-2 reaction. This form of immunological response in leprosy is an immune complex syndrome and may mimic different rheumatic diseases.(ABSTRACT TRUNCATED AT 250 WORDS)
1440677 [The regional blood circulation in the parotid gland area in Sjögren's disease (syndrome) 1992 Jan Rheographic investigations of the parotid gland area in 103 patients with Sjogren's disease (syndrome) that developed in the presence of chronic nonspecific sialadenitis during its various stages and inflammation phases have demonstrated that the blood stream volumic rate directly depended on the inflammation phase and intensity of this phase. During remission the circulation in the salivary glands was close to normal.
8035388 Myositis in primary Sjögren's syndrome. Report of 3 cases. 1994 Apr OBJECTIVE: To describe the findings and course of myositis in primary Sjögren's syndrome (SS). METHODS: We studied myositis in SS when clinically indicated. Of 104 patients with SS, we identified 3 cases. In all, the diagnosis was made according to clinical data, biochemical, electromyographic and biopsy criteria. Other autoimmune diseases were excluded. RESULTS: We found a prevalence of 3% of myositis secondary to SS. There were no significant associations between myositis and other clinical or laboratory variables. CONCLUSION: Although rare, myositis must be considered a part of the spectrum of SS. In our experience, treatment with steroids and immunosuppressive drugs was successful.
8265827 Neurologic disease in Sjögren's syndrome: mononuclear inflammatory vasculopathy affecting 1993 Nov There is a growing appreciation that a subset of patients with primary Sjögren's syndrome (SS) also may develop a spectrum of central nervous system (CNS) complications. This article defines SS and its significance; discusses CNS, peripheral nervous system, and muscular complications of SS; identifies those areas in general or internal medicine in which SS patients with potential neurologic complications may present; describes those neurologic disorders that SS may mimic; places into perspective the controversy regarding the frequency and significance of CNS-SS; and extends our observations on the immunopathogenesis of neurologic complications in SS.
8474065 Digital subtraction sialography of the parotid glands in primary Sjögren's syndrome. 1993 Feb Digital subtraction sialography of the parotid glands was performed in 34 patients with primary Sjögren's syndrome (SS) and 78 patients suspected of having primary SS in whom the diagnosis could be excluded (NON-SS). The following sialographic features were found more frequently in the patients with primary SS than in the NON-SS patients: a sparse overall branching pattern of the ducts, progressive widening and irregularity of the duct walls, disappearance of a homogeneous parenchyma blush and the occurrence of peripheral acinar dilations. The most discriminating findings between the 2 patient groups were the presence of acinar dilations and the disappearance of the homogeneous parenchyma blush. The sensitivity and specificity of the presence of acinar dilatations were 79 and 95%, respectively. Both the sensitivity and specificity of the presence of an irregular or absent parenchyma blush were 91%. The concurrent finding of acinar dilatations and an irregular or absent parenchyma blush had a sensitivity of 77% and a specificity of 95%. We conclude that digital subtraction sialography is a useful adjunctive diagnostic procedure for primary SS.
8009146 [3 cases of rare peripheral neuropathies associated with primary Gougerot-Sjögren syndrom 1993 Several forms of peripheral neuropathy occur in Sjögren's syndrome (dryness of eyes, mouth and other mucous membranes). Symmetrical sensorimotor polyneuropathy occurs most frequently followed by sensory neuropathy. Pure sensory neuronopathy, trigeminal sensory neuropathy and autonomic neuropathy are also common. We report three rare forms of peripheral neuropathy related to Sjögren's syndrome and analyze their clinical course and response to therapy. There were a case of mononeuropathy multiplex (MM), a case of recurrent cranial polyneuropathy (RCP) and a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The patients were females, 70, 63 and 81 years old respectively. All of them fulfilled the criteria of Sjögren's syndrome, without clinical or serological abnormalities of systemic disease. Only one patient presented with positive antinuclear antibodies. In two patients the onset of neuropathy occurred before the sicca syndrome long. The sural biopsy of the patient with MM exhibited vasculitis of the small vessels and a mild sensory polyneuropathy was superimposed. The patient with RCP had 8 episodes of isolated third nerve palsy and 5 episodes of multiple cranial neuropathy. The patient with CIDP responded poorly to treatment while in the patients with MM and CRP the response to corticotherapy was good. In conclusion, Sjögren's syndrome must be considered in neuropathies of unknown cause not only when they are sensory, autonomic or trigeminal but also when they are recurrent suggesting an ischemic mechanism. Even a CIDP requires a search for Sjögren's syndrome before being considered idiopathic.
1313974 A quantitative index derived from 99mTc-pertechnetate scintigraphy to assist in the diagno 1992 Feb A safe and simple technique is reported by which primary Sjögren's syndrome can be detected with a relatively high specificity and sensitivity. The method of serial scintigraphy has been used with reasonable success; however, the application of the linearity index as described here produced superior results. In 71 patients investigated, a sensitivity of 87% and specificity of 93% were recorded and make this the method of choice for evaluating patients suspected of having primary Sjögren's syndrome.
8232677 [Coping with illness in myasthenia gravis]. 1993 Oct Within the past few years, the introduction of immunosuppressants in the treatment of myasthenia gravis, has turned this severe, chronic and life-threatening disease into a moderate illness with good chances of control or even remission. Of 44 patients whose coping behavior and course of disease we investigated, only seven experienced a significant change in severity of the disease within nine months. This consistency in the course of the disease was also reflected in the forms of coping with the disease: in contrast to the situation two decades ago, today's myasthenia gravis patients are no longer caught up in the dilemma between passive dependency and active resistance, most of them adopting an attitude of calm acceptance. According to the Berne Forms of Coping (BEFO) which we applied, this attitude is expressed as a pattern of passive cooperation, acceptance, distraction and relativization. This coping pattern remained largely intact even in relation to the severity of the disease and the retrospectively assessed course of the disease. We present two cases to illustrate the influence of coping behavior on the course of the disease. Comparison with two other patient groups (rheumatoid arthritis and hip osteo-arthrosis) showed that there is no disease specificity of coping behavior. It can be assumed that there is a basic pattern in coping with chronic diseases, the manifestation of which is dependent on the severity and prognosis of the disease. If depressive reactions to the disease are excluded, the prevalence of longer-term pre-existent psychiatric disorders among MG patients corresponds to the average for the general population, although there is a relatively high incidence of anxiety disorders.(ABSTRACT TRUNCATED AT 250 WORDS)
7943620 Systemic lupus erythematosus and systemic scleroderma in patients from the aboriginal peop 1994 Apr There is some information on the course of two main forms of large collagenoses-systemic lupus erythematosus (SLE) and systemic scleroderma (SSD) under the conditions of the North of the Asian part of Russia (Yakutia) in this paper. Seventy-nine cases (59 SLE patients and 20 SSD patients) belonging to different ethnic groups were studied. There were 47 patients of Yakutian nationality, among them SLE 38 patients and SSD nine patients. There were 32 patients (SLE-21, and SSD-11) migrant Europeans. It has been proved that the aboriginal people of the North are more subject to SLE and SSD diseases as compared to the migrants. It has also been proved that the greater spreading of the diseases, with the collagen metabolism disturbance in the first ethnic group, including Marfan's syndrome and rheumatoid arthritis may be explained by genetic peculiarities. Some ethnically stipulated differences in clinical manifestations of two large collagenosis were revealed. Thus, during SLE smaller frequency of the skin impairment in the Yakuts (due to natural hyperpigmentations) is connected with the considerable frequency of large joints impairment and more frequent course of SLE similar to rheumatoid variant with typical wrist deformation. One-third SLE and SSD patients of Yakut nationality reveal "overlap-syndrome", which are typical of other collagenoses, the so-called overlap-syndrome. Some industrial factors (dust of silicon dioxide, vibration, hydrocarbon compounds) are the main reason for the diseases among the newcomers migrants of Russian and Ukranian nationality. One case of silico-lupus was revealed.(ABSTRACT TRUNCATED AT 250 WORDS)
8544024 Bisphosphonate (pamidronate/APD) prevents arthritis-induced loss of fracture toughness in 1995 Nov Patients with rheumatoid and other inflammatory arthritis have an increased risk for fracture. This study was designed to determine the effect of experimental inflammatory arthritis on the material properties (fracture toughness and shear modulus) and structural properties (torque, angular deflection, and absorbed energy) of femoral diaphyseal bone tested in torsion to fracture, as well as the effect on these properties of APD (3-amino-1-hydroxypropylidene-1,1-bisphosphonate), a drug known to block osteoclast activity. Two dose levels were investigated. Experimental inflammatory arthritis was induced by intra-articular injection of carrageenan into the right tibiofemoral joint, given over 7 weeks, in three groups of animals. Simultaneously, daily subcutaneous injections of APD were given to three groups of rabbits. Five groups (12 animals each) were established: normal, arthritis, normal/high dose APD, arthritis/high dose APD, and arthritis/low dose APD. The diaphyses of each excised right femur were loaded to fracture in torsion at an angular deflection rate of 8 degrees/sec. In the arthritis group, the fracture toughness was 39% lower than in the normal group, and the structural properties all were reduced significantly. By contrast, the shear modulus was unaffected by arthritis. In this study, the higher dose level (0.3 mg/kg of body weight) of APD prevented loss of fracture toughness and maintained the structural properties in experimental inflammatory arthritis; the low dose was not effective.