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ID PMID Title PublicationDate abstract
1690557 HC-IgA antibodies of different specificities are normally present in serum: quantitation b 1990 Mar Enzyme-linked immunosorbent assays (ELISA) were developed for direct measurement of protein HC-IgA complexes (HC-IgA) in serum with antibody specificity for rabbit IgG (rheumatoid factor (RF) activity), lipopolysaccharide from Yersinia enterocolitica serotype O:3 (Y3) and tetanus toxoid (TT). About 80% of patients with rheumatoid arthritis had increased concentrations of HC-IgA-RF. The values were correlated with the concentrations of IgA-RF and IgM-RF. HC-IgA anti-Y3 was measured in 45 sera with anti-Y3 antibodies of IgM, IgG and IgA class. The HC-IgA anti-Y3 levels were correlated with those of anti-Y3 of IgG and IgM class, but not of IgA class. For HC-IgA anti-Y3, the closest correlation was that with the specific IgM antibody concentration, rs = 0.63 (p less than 0.001). In 25 normal sera, significant correlations were observed between HC-IgA anti-TT and specific antibodies of IgG and IgA class, but not of IgM class. In 107 sera containing IgA M-components, the total concentration of HC-IgA correlated poorly with both protein HC and with IgA concentrations. It was concluded that specific HC-IgA antibodies are normal constituents of serum, and that their concentrations are not directly related to the serum content of specific IgA antibodies.
3286523 Molecular interactions between human IgG, IgM rheumatoid factor and streptococcal IgG Fc r 1988 Group A streptococci type M15 were previously shown to bind both human IgG via the Fc component and a purified monoclonal IgM kappa rheumatoid factor (IgM RF). Using 125I-labelled IgG and 125I-labelled IgM RF, the present study gave association constants of 2.2 x 10(7) and 2.9 x 10(8) M-1, respectively. The binding of 125I-IgG to the streptococci was inhibited by unlabelled IgG, IgG Fc and fragment D of staphylococcal protein A but not by the IgM RF or F(ab')2 of anti-idiotype antibodies to RF (anti-Id RF). Inversely, unlabelled IgM RF and anti-Id RF inhibited the binding of 125I-IgM RF markedly and unlabelled human IgG and IgG Fc only slightly or moderately, respectively. Thus, group A streptococci type M15 showed different binding sites for IgG Fc and the antibody combining sites of a human monoclonal RF. The findings were still more complex on a background of previous reports showing that streptococcal IgG Fc receptors and RFs bind to the same amino acids on the Fc molecule. This complex pattern may play a role in the pathogenesis of rheumatoid arthritis.
3221041 Development of thyroid disease in patients with primary and secondary Sjögren's syndrome. 1988 Oct Autoimmune thyroid disease associated to Sjögren syndrome (SS) may be defined by serological and functional abnormalities. We studied the prevalence of serum anti-thyroid autoantibodies and the development of thyroid functional defects by a follow-up investigation in patients with primary (pSS) and secondary SS (sSS). In keeping with previous literature data, our findings confirmed an increased prevalence of thyroid autoimmune phenomena in the whole series of patients with SS. In addition, evidence was provided for a greater incidence of serological and functional thyroid abnormalities in pSS when compared to sSS. Follow-up study also showed that the rate of thyroid autoimmune disease development was higher in pSS than in sSS. The appearance of circulating anti-thyroid antibodies was particularly frequent in patients with preexisting antibodies against extractable nuclear antigens (anti-ENA). These data indicate the need for a careful monitoring of thyroid function in SS patients, with particular regard to those with pSS and positive anti-ENA tests.
3056790 Polyneuropathy in Sjögren's syndrome. A case of prevalently autonomic neuropathy with ton 1988 Jul A 38-year-old woman suffering from primary Sjögren's syndrome displayed a neurologic picture consisting of left tonic pupil, generalized tendon areflexia and left-sided hypohidrosis. Electrophysiological and pathological studies suggested a mild degree of peripheral nerve involvement. Moderate loss of large myelinated fibres and obliteration of small endoneurial vessels were seen in the sural nerve biopsy. Sympathetic skin response was absent and alterations of unmyelinated nerve fibres were found. A prevalently autonomic neuropathy with tonic pupils may represent a characteristic picture in the spectrum of peripheral nerve involvement in Sjögren's syndrome. Other similar cases reported in the literature are reviewed.
3078788 [The immunologic processes of the etiopathogenesis of Sjögren's syndrome]. 1988 Jan In this study 32 Sjögren's syndrome cases--16 primary and 16 secondary--have been followed-up for 3 years in Hacettepe University Medicine Faculty Ophthalmology Department. The serum levels of rheumatoid factor and complement have been determined and autoantibodies have been looked for by the indirect immunofluorescence technique. The immunological processes that play a role in the pathogenesis of Sjögren's syndrome have been discussed with regard to the results of the study.
3498492 High levels of in vitro IgM rheumatoid factor synthesis correlate with HLA-DR4 in normal i 1987 Aug The association between the HLA-DR4 histocompatibility antigen and in vitro synthesis of IgM and IgM rheumatoid factor (IgM-RF) by blood mononuclear cells was investigated in 35 normal subjects. In vitro cultures of T and B cells were stimulated with pokeweed mitogen, and the secreted IgM-RF, tetanus antibody, and total IgM protein were measured by solid-phase radioimmunoassay. In cultures containing unseparated T cells, IgM-RF production in the DR4+ and DR4- subgroups was not significantly different. However, depletion of OKT8+ cells containing T suppressor cells resulted in significantly higher IgM and IgM-RF synthesis in the DR4+ subgroup. Moreover, 6 of the 8 highest levels of IgM-RF were produced by DR4+ individuals, while only 7 of the remaining 27 individuals were DR4+ (P = 0.035). Ratios of secreted IgM-RF:IgM indicated that there was a relative enrichment for IgM-RF- specific B cell precursors in DR4+ high responders, although total numbers of circulating B cells were not increased. High responder B cells had high levels of responsiveness when mixed with T helper cells from low responders, whereas low responder B cells consistently produced low responses, even when cocultured with T helper cells from high responder donors. The data suggest that a subset of normal individuals has a pokeweed mitogen-responsive lymphocyte population that contains a B cell subpopulation specific for IgM-RF synthesis and that this condition is associated with HLA-DR4. In certain individuals who have the DR4 type, there may be a component of a susceptible genetic background, upon which other factors act to induce IgM-RF synthesis and which may produce clinical manifestations of rheumatoid arthritis.
2290166 Dapsone is an effective therapy for the skin lesions of subacute cutaneous lupus erythemat 1990 Sep The deficiency of second component of complement (C2d) is the most common hereditary complement deficiency. Patients with C2 deficiency are frequently associated with an auto-immune disease process, in particular, systemic lupus erythematosus (LE)-like syndrome and/or vasculitic syndrome or bacterial infections. C2d has been associated with the LE subset of subacute cutaneous LE (SCLE), the presence of anti-Ro (SSA) antibodies, and the human leukocyte antigen (HLA) types A10, B18, DR2. We describe the clinical, serologic and immunogenetic data in a patient with manifestations of Sjögren's syndrome who developed urticarial vasculitis and photosensitive annular SCLE which were effectively treated with oral dapsone. Our case illustrates the dynamic nature of LE.
2371975 Thrombotic thrombocytopenic purpura as a complicating factor in a case of polymyositis and 1990 Aug A 62-year-old woman was admitted for evaluation of muscular weakness, skin pigmentation, dry mouth, and interstitial pneumonia. During the course of her stay, adult respiratory distress syndrome, hemolytic anemia, renal failure, neurologic dysfunction, and thrombocytopenia appeared. A clinical diagnosis of thrombotic thrombocytopenic purpura (TTP) accompanied by polymyositis and Sjögren's syndrome was made. She died two weeks after the beginning of plasmapheresis, and an autopsy was performed. Immunohistochemistry disclosed deposits of IgM, fibrinogen, and C1q in glomeruli and arterioles and deposits of C3 in small arteries. von Willebrand's factor antigen, which promotes the adhesion of platelets to the subendothelium, was positive in onion-peeled arteries of the kidney and the spleen. These results suggest that immune complexes may have triggered a sequence of events from vascular endothelial injury to TTP.
2256441 Neuropathy and myopathy in primary Sjögren's syndrome: neurophysiological, immunological 1990 Aug Seventeen consecutive patients with primary Sjögren's syndrome (PSS) received neurophysiological examination, analysis of antibodies against peripheral nerve-myelin (PNM) and muscle biopsy, to show the prevalence and characteristics of peripheral neuropathy (PN) and myopathy; 3 PSS cases showed a clinical mild sensorimotor polyneuropathy, 1 of them had been treated with cytostatic drugs; 1 had mononeuropathia multiplex; and 1 clinical carpal tunnel syndrome. In these 5 patients neurophysiological investigation verified the clinical diagnosis of peripheral nerve involvement; 2 with PN had serum-antibodies against PNM; 1 of IgM-, and 1 of IgA-isotype. Muscle biopsies were taken from 15 PSS patients; 11 showed inflammatory myositis or inflammatory perivascular infiltrates and 3 showed signs of denervation. A combination of inflammation and morphological signs of myopathy, compatible with the biopsy diagnosis of polymyositis, was seen in 4, 1 of whom showed clinical signs of polymyositis. We conclude that the peripheral nervous and muscular systems are often involved in PSS, but commonly with relatively mild symptoms and laboratory findings. The common findings of inflammatory myopathy indicate an immune reaction in muscles in addition to other autoimmune manifestations of the disease.
2575320 A prospective immunofluorescence study of immune deposits in the skin of primary Sjögren' 1989 There are conflicting opinions concerning the epidermal immunofluorescence pattern in primary Sjögren's syndrome. In a prospective study of 12 patients we found a characteristic pattern of epidermal nuclear/cytoplasmic IgG deposits in 8 (67%). This pattern was associated with the presence of antibodies against SSA/Ro and SSB/La in the serum and was also found in 2 out of 5 LE patients with monospecific antibodies against SSA/Ro. There is a resemblance to the pattern of dust-like particles described in the diseased skin of patients with subacute cutaneous LE. In one patient with primary Sjögren's syndrome, IgG deposits were confined to epidermal cell nuclei (in vivo ANA). Instead of antibodies against SSA/Ro or SSB/La, this particular patient had nRNP-antibodies. From this study, we conclude that the epidermal IgG deposits in primary Sjögren's syndrome may represent antibody binding to the sites within epidermal cells where the respective antigens are located.
3418646 Sjögren's syndrome in progressive systemic sclerosis. 1988 Jun Forty-four sequential, unselected patients with progressive systemic sclerosis (PSS) were prospectively evaluated for evidence of coexistent Sjögren's syndrome (SS). This diagnosis was established when a patient with focal lymphocytic infiltration in the labial salivary gland (LSG) biopsy, scoring greater than or equal to 2+ in Tarpley's scale, had keratoconjunctivitis sicca (KCS) (positive rose bengal test) and/or xerostomia (subjective xerostomia and decreased parotid flow rate). Ten patients had an LSG biopsy score of greater than or equal to 2+, 3 a 1+ score, 17 had mild to moderate fibrosis only and 14 had normal tissue. Nine of the 10 patients with a greater than or equal to 2+ score had SS, according to applied criteria, suggesting a 20.5% prevalence of SS in our population with PSS. On the other hand, pure fibrosis in the biopsy was felt to be secondary to PSS. Parotid gland enlargement was present in 44.4% of the patients with SS, but was extremely uncommon in the fibrosis and normal tissue groups. Subjective xerophthalmia and xerostomia, although elicited by specific questionnaire in the majority of the patients with SS, did not constitute major complaints. Serious internal manifestations, with the exception of esophageal and pulmonary involvement, were unusual in all groups. Anti-Ro (SSA) antibodies were detected in 33.3% of the patients with SS and 11.8% of those with fibrosis. Our study suggests that SS in scleroderma is relatively common and, although lacking prominent exocrine gland symptomatology, resembles primary SS in some clinical and serologic respects.
1757949 Immunohistological characterization of lymph nodes in two cases of adult onset Still's dis 1991 Sep Although lymphadenopathy occurs in 60% of patients with adult Still's disease, its histopathological pattern has only recently been described. We report on the immunohistological study of a lymph node biopsy and the clinical course of 2 patients with adult Still's disease. The lymph node histology was characterized by a diffuse paracortical expansion with a high content of immunoblasts and plump endothelium venules. Immunohistochemistry using the alkaline phosphatase, antialkaline phosphatase technique showed the mixed B and T cell nature both of proliferating cells and immunoblasts. When antiheavy and light chain reagents were applied, the B cell component became polytypic. Our report stresses the relevance of immunohistological investigation in the diagnostic approach to Still's disease in which histological findings, if taken alone, might be misleading.
2060160 Autoimmune thyroiditis and primary Sjögren's syndrome: clinical and laboratory evidence o 1991 Mar The prevalence of primary Sjögren's syndrome (primary SS) among patients with autoimmune thyroiditis (AT), and that of AT among patients with primary SS were studied prospectively. Of 63 patients with AT, one had precipitating antibodies against SS-A/Ro antigen together with objectively verified primary SS, and none had precipitating anti-SS-B/La antibodies; in contrast 17/63 (27%) had above normal values of anti-SS-B/La antibodies, as tested with ELISA. Of 19 AT patients tested objectively for xerostomia and keratoconjunctivitis sicca, six (32%) had keratoconjunctivitis sicca together with xerostomia and four (21%) had autoimmune sialadenitis. The prevalence of AT in patients with primary SS (n = 28) was 18%; of the 28 patients, 64% had an enlarged or abnormal thyroid gland on palpation and four of them (14%) had cytology verified AT. Ten (36%) had anti-thyroglobulin and/or antimicrosomal autoantibodies. Based upon the present investigation we conclude that the prevalence of primary SS is ten times higher among patients with AT, and that of AT is nine times higher among patients with primary SS, compared with the general population.
2107806 Dissociation of immune responses to the SS-A (Ro) 52-kd and 60-kd polypeptides in systemic 1990 Mar The cellular RNA particle SS-A (Ro) is a target of autoimmune response in many patients with Sjögren's syndrome (SS) and systemic lupus erythematosus (SLE). Recent immunologic, biochemical, and DNA cloning studies have shown that the SS-A particle consists of at least 2 polypeptide components, of 52 kd and 60 kd. Immunodiffusion analysis of 60 sera from patients with primary SS revealed 47 (78%) to be SS-A precipitin positive. Western blotting studies of the sera showed 3 groups of reactivities: 22 (47%) possessed autoantibodies against both the 60-kd and the 52-kd polypeptides, 19 (40%) reacted only with the 52-kd protein, and 6 (13%) were nonreactive in Western blots although positive in immunodiffusion. Fifty-one of 90 SLE sera (57%) were SS-A precipitin positive by immunodiffusion. In Western blots, 24 (47%) possessed antibodies against both the 60-kd and the 52-kd antigens, while 9 (18%) reacted only with the 60-kd protein. Eighteen (35%) were nonreactive by Western blot, although positive by immunodiffusion. Antibody to the 52-kd antigen without concomitant antibody to the 60-kd antigen was seen only in patients with primary SS, whereas antibody to the 60-kd antigen without concomitant antibody to the 52-kd antigen was seen only in SLE patients. Although antibodies to SS-A are detected in both SS and SLE, our findings show that there is dissociation of immune responses to the 2 component antigens of the particle, which may be evidence of different events initiating the autoimmune process in these diseases.
2796402 Pulmonary lymphoma in Sjögren's syndrome. 1989 Aug Sjögren's syndrome (SS) is an immunologic disease characterized by progressive destruction of the exocrine glands that causes mucosal and conjunctival dryness. In addition to the common pulmonary complications of diffuse interstitial lung disease, airways obstruction, desiccation of the upper respiratory tract, localized parenchymal nodules, recurrent tracheobronchitis, bronchiectasis, interstitial pneumonitis, and pleural effusion, patients with SS have a high risk of developing non-Hodgkin's lymphoma that may affect the lungs. Among 50 patients with SS and associated lymphoma, 10 had pulmonary involvement by lymphoma. The mean age of these 10 patients was 59.7 years, and 8 were women. The mean duration of SS was 7.2 years, and the mean interval between the onset of SS and lymphoma was 5.4 years. Cough and slowly progressive dyspnea were the most common pulmonary symptoms, and chest roentgenographic findings varied. Lung biopsies revealed a spectrum of low-to high-grade lymphomas, and high-grade lymphomas were associated with increased mortality. Of the 10 patients, 4 died from 8 to 48 months after lymphoma was diagnosed. We conclude that pulmonary involvement is common in patients with lymphoma associated with SS; thus, lymphoma should be considered in the differential diagnosis of pulmonary lesions in patients with SS.
1369638 Patient selection criteria for electrostimulation of salivary production in the treatment 1990 Electrostimulation has been introduced as a technique for increasing salivary output in the treatment of patients with xerostomia (dry mouth) secondary to Sjogren's syndrome. The procedure uses an electrostimulation device (salivation electrostimulator) to increase salivary production from existing glandular tissue. The device delivers a low-voltage electrical stimulus to the mouth via a probe. Patients with residual salivary tissue in the oral and pharyngeal regions who demonstrate a decrease in the flow rate of saliva are potential candidates for this procedure. It is estimated that more than one million people in the United States, predominantly middle-aged and elderly women, suffer from Sjogren's syndrome. Patients with chronic xerostomia complain of a continual feeling of oral dryness and have difficulty eating dry foods. These patients are susceptible to increased caries, oral pain, infection, and have difficulty speaking, chewing, and swallowing. The approach to the treatment of xerostomia in Sjogren's patients is usually determined by the level of severity of the symptoms. Appropriate management of patients with xerostomia requires that those patients whose salivary flow can be increased by means of sialagogues be distinguished from those patients whose salivary flow is either unaffected or insufficiently stimulated. To alleviate some of the complications due to salivary dysfunction in those patients who respond to stimuli, pharmacologic sialagogues as well as sialagogues that include sugarless gums, mints and candies are prescribed in order to increase salivary flow. Recently, electrostimulation via a hand-held stimulus probe has been introduced as a method of treatment in xerostomia secondary to Sjogren's syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)
3113925 Epilepsia partialis continua in Sjögren's syndrome. 1987 Jul Epilepsia partialis continua with Sjögren's syndrome is reported. The patient had extensive involvement of the nervous system with left middle cerebral artery occlusive stroke, mononeuritis multiplex, right partial sensory motor seizures and epilepsia partialis continua involving the right big toe and foot. The EEG showed nonspecific bilateral theta slowing. The epilepsia partialis continua did not respond to antiepileptic drugs.
3959477 [Sjögren syndrome in childhood]. 1986 Jan The authors report on the case of a 7-year-old girl with keratoconjunctivitis sicca. The diagnostic difficulties are discussed and the literary data on Sjögren's syndrome in childhood are summarized.
3296156 Salivary histopathology in diagnosis of Sjögren's syndrome. 1986 Although the etiology of Sjögren's syndrome (SS) remains unknown, the histopathologic examination of affected organs provides tissue diagnosis, a way to assess severity, and clues to pathogenesis. A common feature in organs affected by SS is lymphocytic infiltration, which presumably causes the functional changes and the diverse clinical features associated with this disease. So far, the salivary glands are the best studied organs in SS. Biopsy of major salivary glands is not a clinically convenient nor a very effective way to diagnose SS and can have serious sequelae. For these reasons, the minor salivary glands in SS have been studied for the last 20 years and used in diagnosing the salivary component of SS for more than 10 years. However, minor gland specimens can present different patterns of inflammation, and in diagnosing SS it is critical to distinguish between these patterns. Furthermore, a minor gland biopsy specimen must be taken from clinically normal-appearing mucosa and must contain sufficient glandular tissue for assessment.
1681789 Meningitis associated with sulphasalazine in a patient with Sjögren's syndrome and polyar 1991 Sep A case of meningitis occurring in a 37 year old woman with Sjögren's syndrome, seropositive arthritis, and a possible overlap syndrome soon after starting sulphasalazine and after subsequent rechallenge is reported.