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ID PMID Title PublicationDate abstract
3259356 [Detection of antibodies to extractable nuclear antigens with counterimmunoelectrophoresis 1988 Jan Detection of antibodies against extractable nuclear antigens (ENA) by counter immunoelectrophoresis (CIE) is currently used to screen sera from patients with collagen vascular disease. Although fast and reliable, the method has its limitations with regard to the differentiation of various antigen-antibody systems. In this study, results of the anti-ENA analysis by CIE and by Western-Blot technique are compared. Except for a higher frequency of Sm antibodies by Western-blot, the results of CIE were confirmed. The source of antigen and the higher sensitivity of the Western-Blot technique are responsible for the different results obtained. With respect to the relationship of antibody profile and illness, both methods demonstrated a high frequency of antibodies against U1-sn-RNP in mixed connective tissue disease and of anti-SS-B antibodies in Sjögren's syndrome.
3173983 Tear function parameters in keratoconjunctivitis sicca with and without the association of 1988 In a group of patients with keratoconjunctivitis sicca (KCS) associated with Sjögren's syndrome (SJ) a significant decrease in tear fluid lactoferrin concentration and tear film break-up time was demonstrated in comparison with a matched group in which KCS was not associated with any systemic disease. The Schirmer values were also lower in the SJ of patients and these differences almost reached statistical significance. None of these parameters in itself appeared to be of any clinical value to differentiate between KCS that was or was not associated with SJ. The combination of the tear fluid lactoferrin concentration, the Schirmer I test values and the tear film break-up time, however, provide a basis for differential diagnosis that is clinically of some value.
2976520 [Immunopathology of the labial salivary glands in Sjögren's syndrome and other autoimmune 1988 73 minor salivary gland biopsies (lip biopsy) were performed in patients with primary and secundary Sjögren's syndrome and other collagen diseases without sicca syndrome were studied by means of immunohistochemistry. The results were compared to those obtained in sarcoidosis, sialadenitis and normal glands. In all cases of Sjögren's syndrome and of other sialadenites, we could note predominance of T-lymphocytes with an increase of T-Helpers and a T-suppressor ratio superior to 1. Besides, HLA-DR antigen was present in all the activated cells of lymphocytic infiltrates, in endothelial cells and in some epithelial ductal and acinous cells around the infiltrates. This method cannot permit to differentiate Sjögren's syndrome from the other sialadenites. But, it is very useful to appreciate the evolutivity of the disease (increase in B-lymphocytes in the initial period and in evolutive phases) and to detect its change into pseudolymphoma (polyclonal cells with increase in B-cells) and into malignant lymphoma (usually monoclonal B-lymphocytes proliferation).
3635417 Plasma lactoferrin in patients with neutropenia. 1986 May This study examines the role of plasma lactoferrin in the assessment of neutropenia. In particular, we have studied lactoferrin as an inhibitor of granulopoiesis and as an indicator of the size of the total blood granulocyte pool (TBGP). Plasma lactoferrin concentration was determined in a heterogeneous group of 30 patients with neutropenia. Serial plasma lactoferrin levels in a patient with cyclic neutropenia correlated with the cycles of the neutrophil count. Patients with splenomegaly had a grossly elevated lactoferrin:neutrophil ratio. Most chronic idiopathic neutropenia patients had no real clinical problems and a normal plasma lactoferrin level. The results provide further evidence to support the concept that plasma lactoferrin indicates the size of the TBGP and the lactoferrin: neutrophil ratio indicates the degree of granulocyte margination. There was no evidence to suggest that lactoferrin acting as a feedback inhibitor of granulopoiesis caused neutropenia in these patients.
1990092 Evaluation of ultrasound in the diagnosis of pathologic processes in the parotid gland. 1991 Feb The values of ultrasound in differentiation of pathologic processes in the parotid gland were studied in 61 patients. The ultrasound findings were compared with operative and histologic findings. In 28 patients with chronic inflammatory processes, the ultrasound findings were in complete correlation with the histologic findings, showing enlarged parotid glands, homogenous glandular epithelium, and small, multiple cysts. In 31 patients with a tumor of the parotid gland, ultrasound scanning detected the presence of the tumor. In determining the location of the tumor, the method is highly specific when it is localized in the superficial lobe (100%), and it is less specific when it is localized in the deep lobe of the parotid gland. Also, it is highly specific in determination of tumor size (96%). In two cases with retention cysts, the ultrasound findings also correlated with the histologic and operative findings. The results obtained show that ultrasound is a reliable diagnostic method for detection of pathologic changes in the parotid gland.
2789657 A postmortem study of focal adenitis in salivary and lacrimal glands. 1989 Aug Investigations were carried out on 102 consecutive medicolegal post-mortem subjects to identify signs of focal adenitis in labial, submandibular, and lacrimal glands. There were 19 subjects (18.6%) with focus scores exceeding 1 in one or two of the types of glands. Among the 19, five had had a disease which is generally associated with Sjögren's syndrome or is autoimmune in nature. In another 63 subjects occasional lymphocytic foci were found in one, two, or all three types of gland. Fibrosis, atrophy, and fatty change had occurred most often in the labial salivary glands in those over 50 years of age with or without high focus scores. Fibrosis, atrophy, and fat infiltration had hardly ever occurred in the lacrimal glands, although this is the type of gland in which high focus scores occur most often. The results suggest that there may be a common autoimmune basis for Sjögren's syndrome and other diseases, the mechanisms of which are still incompletely understood. A focus score exceeding 1 may be an indicator of Sjögren's syndrome, but the diagnosis should not be established on the biopsy findings alone.
2422809 Epimyoepithelial' islands in lymphoepithelial lesions. An immunocytochemical study. 1986 A panel of antibodies has been used in an immunoenzyme study in an attempt to characterize the cell types found in the 'epimyoepithelial' islands of lymphoepithelial lesions. Myoepithelial cells, which can be specifically stained with an anti-smooth muscle antibody were not found. The majority of cells stained with anti-prekeratin, suggesting a duct cell differentiation. A subpopulation of duct cells stainable with a monoclonal antikeratin (16a), previously described as occupying a basal location in normal salivary gland ducts, was clearly demonstrated. It is suggested that these cells may be important in the epithelial proliferation in these islands. The hyaline material within and surrounding the epithelial islands was positive for type IV collagen and therefore consists of basement membrane material.
2327738 Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndrome 1990 Mar Thirteen patients, 11 women and 2 men, developed sensory and autonomic neuronopathies in association with features of primary Sjögren's syndrome. In 11, Sjögren's syndrome had not been previously diagnosed at the time of neurological presentation. All had prominent loss of kinesthesia and proprioception. Pain and thermal sensibility were less severely affected. Most had evidence of autonomic insufficiency. In some this was severe, with Adie's pupils, fixed tachycardia, and orthostatic hypotension. The course ranged from an abrupt, devastating onset to indolent progression over years. Stabilization or functional improvement occurred in 6 patients, 2 of whom received no drug therapy. Sensory nerve conduction studies and examination of nerve biopsy specimens demonstrated a wide spectrum in the severity of loss of large myelinated fibers. The cutaneous nerves of 6 patients had perivascular mononuclear infiltrates without necrotizing arteritis. Examination of biopsy specimens of dorsal root ganglia in 3 patients revealed lymphocytic (T-cell) infiltration in the dorsal roots and ganglia, with focal clusters around neurons. In the more mildly affected ganglia, individual sensory neurons were undergoing degeneration. In the most advanced case, very few neurons remained. The possibility of Sjögren's syndrome should be considered in patients, especially women, who develop acute, subacute, or chronic sensory and autonomic neuropathies, with ataxia and kinesthetic loss.
2539280 Persistence of Epstein-Barr virus in salivary gland biopsies from healthy individuals and 1989 Mar Salivary gland biopsies from 12 patients with primary Sjögren's syndrome and 10 controls were examined for Epstein-Barr virus (EBV) DNA by in-situ hybridization and for EBV proteins by immunofluorescence and peroxidase techniques. Viral DNA was found in biopsies from two out of 12 patients with primary Sjögren's syndrome and six out of the 10 controls. The DNA and early antigen were in epithelial cells lining the ducts and acini, early antigen expression being limited to the luminal side of the epithelium. In eight biopsies studied with other antibodies, membrane antigen was identified in both acini and ducts but viral capsid antigen and Epstein-Barr nuclear antigen were not detected. EBV was found in biopsies from five of the controls without inflammation or Class II expression. This suggests that, in health, persistence and replication occur without inducing an immune response, possibly due to the restricted expression of early antigen on the luminal of the epithelium, away from immune surveillance. The inflammation in Sjögren's syndrome could be due to a breakdown of this unusual mechanism for viral persistence leading to a vigorous immune response to the virus. However our study provides no evidence to suggest that EBV infection load is increased in this disease.
3150433 Ultrastructural study of Sjögren's syndrome-like disease in MRL/l mice. 1988 Nov Salivary glands of autoimmune MRL/l mice were examined ultrastructurally and by immunoelectron microscopy to further characterize the Sjögren's syndrome-like disease in these animals. Major salivary glands from 12 female and 7 male MRL/l, two female MRL/n, and one female BALB/c mice were examined by electron microscopy and the glands from 4 female MRL/l mice were subjected to immunoelectron microscopy in order to detect Lyt-1 and Lyt-2 positive lymphoid cells. Mononuclear cell infiltrates were not seen in the salivary gland from the BALB mouse and occurred rarely in glands of MRL/n mice. However, in MRL/l mice, numerous lymphoid cells were present and acinar cells displayed low cytoplasmic density, cytoplasmic vacuolization and cellular lysis. Lymphoid cells were predominantly Lyt-1 positive although some Lyt-2 positive cells were observed. These results suggest that the MRL/l mouse represents a useful model for the study of the pathogenesis of Sjögren's syndrome in man.
3262408 Monocytoid B-cell lymphoma. Clinicopathologic study of 21 cases of a unique type of low-gr 1988 Oct 15 The morphologic and immunologic features of three cases of an unusual and distinct B-cell lymphoma were recently described and termed monocytoid B-cell lymphoma (MBCL) because of the striking resemblance of the neoplastic cells to reactive monocytoid B-lymphocytes. The morphologic spectrum and the clinical behavior of MBCL were investigated in a series of 21 patients. This study indicates that patients with MBCL usually present with lymphadenopathy and Stage I or II disease. MBCL also occurs at extranodal sites including the salivary gland. Because four of the patients with MBCL had Sjögren's syndrome with characteristic laboratory profiles, these results raise the possibility that there may be a relationship between MBCL and Sjögren's syndrome. Eight patients were male and 13 female (M:F = 1:1.6), and MBCL primarily involved the elderly (median age, 66 years). The most striking clinical findings were high percentages of complete remissions and long survival times indicating that MBCL is a low-grade lymphoma. Of 21 patients investigated, 18 were in complete remission at the time of completion of this study. Two patients died with the disease and one was lost to follow-up. Patients with localized MBCL may have a better survival rate than those with generalized disease. Like other low-grade lymphomas, MBCL can progress to a higher grade lymphoma of large cell type. Unlike other low-grade lymphomas, in MBCL splenomegaly, bone marrow involvement, and leukemic conversion are uncommon.
3127416 Factitious elevation of thyrotropin in a new ultrasensitive assay: implications for the us 1988 Mar Three patients who had falsely elevated serum TSH concentrations (initial values, 30.5, 74, and greater than 50 mU/L) in a mouse monoclonal immunoradiometric assay are reported. Two patients were treated for hypothyroidism inappropriately, and one underwent unnecessary diagnostic testing. Immunoaffinity chromatography of serum from one patient indicated that the serum TSH level was truly low. Addition of mouse serum or immunoglobulin G (IgG) or absorption of patient serum with solid phase-bound mouse IgG-1 reduced the TSH content in the serum of the three patients to undetectable levels. Blocking studies revealed that all patients had antibodies directed at mouse IgG-1, the subclass of mouse antibody present in the assay kit. The serum of one patient who had autoimmune disease with elevated serum Igs had much broader species cross-reactivity than that of another patient who had known exposure to rats and mice. We hypothesize that such antimouse antibodies can arise either from endogenous autoimmunity or exogenous animal exposure. Serum TSH elevations also were found when the serum samples were tested in other mouse monoclonal immunoassays, underscoring the fact that antibody interference can potentially affect many assays used in endocrinology and other areas of medicine to make major diagnostic and therapeutic decisions. Clinicians must be aware of such interactions; relatively simple laboratory maneuvers can differentiate true from false results in assays of this type.
1877413 [A case of lacrimal gland malignant lymphoma, associated with Sjögren's syndrome]. 1991 Apr A 64 year-old female complained of left upper lid swelling. Painless mass lesions were found in her bilateral lacrimal gland areas. She also complained of a dry sensation in both eyes and her mouth. The rose bengal test, Schirmer's test and fluorescein dye test were positive. Lip biopsy revealed chronic sialoadenitis and rheumatoid factor titer was elevated. Anti-nuclear and anti-RNP antibodies were positive. Anti-SS-A and anti-SS-B antibodies were both negative. Left pre-and post auricular, cervical and inguinal lymph nodes were palpable. Excisional biopsies of bilateral orbital lesions and an inguinal lymph node revealed the presence of reactive lymphoid hyperplasia in the right orbit, and B cell type, diffuse, large cell lymphoma, in the left orbit and inguinal lymph node. Intensive chemotherapy resulted in complete remission. Lacrimal gland lymphoma must be considered when examining cases of Sjogren's syndrome.
2242064 Association of labial salivary gland histopathology with clinical and serologic features o 1990 Nov Focal sialadenitis is now widely accepted as an objective criterion for the oral component of Sjögren's syndrome (xerostomia). We investigated the association between labial salivary gland histopathologic changes and the clinical and serologic features of 192 patients with suspected connective tissue disorders. A retrospective review of the medical records of all patients was performed, and historical, physical, laboratory, histologic, and roentgenographic data were abstracted. Each patient had undergone labial salivary gland biopsy as part of a rheumatologic evaluation. There were significant associations between positive findings on lip biopsy and the presence of keratoconjunctivitis sicca (P = 0.013), positive antinuclear antibodies (titer greater than or equal to 1:80) (P = 2 x 10(-8)), and positive Ro antibodies (P = 1 x 10(-8)). However, sicca symptoms and glandular enlargement were not statistically associated with positive findings on lip biopsy. Features predictive of a positive lip biopsy included Ro antibodies (P = 0.914), keratoconjunctivitis sicca (P = 0.700), and positive antinuclear antibodies (P = 0.590).
1688881 Analysis of autoantibodies to recombinant La (SS-B) peptides in systemic lupus erythematos 1990 Feb Autoantibodies to a polymerase III transcription factor, La (SS-B), are frequently detected in the serum of patients with Sjogren's syndrome and systemic lupus erythematosus. To define the humoral immune response to this protein, we analyzed the patterns of antibody recognition toward 13 recombinant La peptides by immunoblotting and determined the heterogeneity of antibodies reactive with the immunodominant epitopes. The smallest epitopes that were strongly antigenic and recognized by greater than 70% of sera tested (immunodominant) were encoded by the subclones BgX and XA located in the 5' and 3' halves of the La cDNA, respectively. Conformation of the immunodominant La peptides played a major role in antibody recognition. Although greater diversity in antibody binding to carboxyl-terminal La peptides was observed, the overall pattern of peptide recognition by anti-La antibodies was similar in different diseases. The antibody responses to the immunodominant peptides were strongly correlated (r = 0.68, P less than 0.001). One- and two-dimensional isoelectric focusing of affinity purified IgG anti-La peptide antibodies revealed restricted heterogeneity and oligoclonal bands (kappa light chains). These observations suggest that anti-La antibodies are induced and/or maintained by the self antigen and that their diversity is constrained either by mechanisms related to tolerance or by affinity maturation of the humoral immune response.
3430523 Vasculitis in systemic sclerosis: association with Sjögren's syndrome and the CREST syndr 1987 Oct We describe 7 patients with established systemic sclerosis who developed clinical evidence of vasculitis 1 to 33 (mean 12.7) years after the first symptoms of scleroderma. Six had the CREST variant of systemic sclerosis and also had features of Sjögren's syndrome (SS). Five of 6 patients tested had serum anti-SSA (Ro) antibodies. Vasculitis presented primarily as cutaneous lesions with ulceration and/or mononeuritis multiplex, and 6 patients had severe systemic manifestations. Vasculitis was histopathologically documented in 6 cases in biopsies of skin (4 of 4), muscle (2 of 3) and sural nerve (3 of 3). Patients with systemic sclerosis with CREST syndrome and SS appear to be at increased risk to develop vasculitis.
3033070 Interleukin 1 (IL 1) as a mediator of crystal arthritis. Stimulation of T cell and synovia 1987 May 15 We reported before that monosodium urate (MSU) crystals were potent stimulators of endogenous pyrogen (EP) production from human and rabbit mononuclear phagocytes, and proposed that this property of MSU crystals may be important in the pathogenesis of gout. EP activity is now attributed to interleukin 1 (IL 1) peptides but IL 1 is not the only pyrogenic monocyte-derived cytokine, since both interferon-alpha (alpha-IFN) and tumor necrosis factor (TNF) are also pyrogenic in rabbits. Using a T cell comitogenic assay based on a murine helper T cell clone that does not respond to IFN or TNF, we now report the release of IL 1 activity from human blood monocytes and synovial fluid mononuclear cells (MNC), following stimulation with MSU crystals. MSU-induced supernatants with IL 1 activity were neutralized with rabbit antiserum to human IL 1 and also stimulated the growth ([3H]thymidine incorporation) of long-term fibroblast-like cell lines derived from human synovial rheumatoid exudate. Two other crystals associated with articular inflammation were tested: hydroxyapatite was a much less potent stimulus compared with MSU crystals, and calcium pyrophosphate dihydrate did not stimulate IL 1 release from human monocytes or synovial fluid MNC. As a model for the inflammatory consequences of acute and chronic overproduction of IL 1, gout is the only sterile inflammatory disease where the local and systemic pathology is compatible with such overproduction; raised IL 1 levels have been found at the site of inflammation, and a necessary etiologic agent, crystalline urate, has been shown unequivocally to be a direct activator of mononuclear IL 1 release.
1827160 T cell subpopulations in the labial minor salivary gland histopathologic lesion of Sjögre 1991 Feb The majority of T cells constituting the focal lymphocytic infiltrates in the labial minor salivary glands of patients with Sjögren's syndrome bear the helper phenotype (CD4); activation is expressed by class II (HLA-DR) MHC products. In our immunohistologic study of snap frozen minor salivary gland biopsies from 16 patients with primary Sjögren's syndrome, it was shown that the majority (77%) of the infiltrating T cells exhibit the memory helper/inducer phenotype (UCHL-1) and express LFA-1 molecules. In addition most of the T cells express the alpha beta receptor while only 2.8% express the gamma delta receptor. Our findings suggest that these T cell infiltrates in patients with primary Sjögren's syndrome may explain the intense B lymphocyte hyperreactivity observed in the exocrine glands of patients with Sjögren's syndrome.
2531985 [Cutaneous microcirculation in infectious endocarditis]. 1989 Nailfold capillary microscopy was used to study the microcirculation patterns in 26 adult patients with infective endocarditis. Abnormal patterns were found in 13 patients (50%). Enlargement of capillary loops was never observed. Significant correlations were found between the number of capillary abnormalities and both systemic involvement (cutaneous vasculitis, arthritis, splenomegaly and/or glomerulonephritis) and immunological disturbances (circulating immune complexes, rheumatoid factor and/or hypocomplementemia) (p = 0.02 and 0.003, respectively). Capillary abnormalities were significantly reduced in 14 patients studied 4 to 48 months after endocarditis was cured. However, due to the lack of specificity, nailfold capillary microscopy cannot be regarded as a useful tool for the diagnosis of infective endocarditis. Connective tissue disorders are not the sole diagnosis to be considered in patients with abnormal nailfold capillary microcirculation patterns.
2070429 Rheumatological symptoms due to retinoids. 1991 Apr Isotretinoin and etretinate are synthetic derivatives of vitamin A widely used in the treatment of dermatological diseases, mainly those affecting keratinization. They have numerous side-effects, among which the rheumatic symptoms are not the most common or the most severe. The main skeletal adverse reaction of retinoids is hyperostosis. It mainly occurs with protracted treatments and high dosages, and its incidence may exceed 80% after a few years of administration. Hyperostosis is axial, located in the cervical and thoracic spine, and may be responsible for limitation of movement; in the appendicular bone, enthesopathies occur at the foot, pelvis, hip, and less commonly the shoulder and elbow. They are usually mild and asymptomatic. The radiological appearance is very similar to diffuse idiopathic skeletal hyperostosis. Isotretinoin tends to be responsible for axial involvement, etretinate for peripheral locations. The other skeletal side-effects are uncommon and include periosteal proliferation, calcification of the interosseous membrane of the forearm and diffuse radiological bone hyperlucency. In children, premature epiphyseal closure is very rare. About 20% of patients complain of musculoskeletal pain and arthralgias. A few cases of true arthritis have been reported. Retinoids may be responsible for muscular damage and an abnormality of muscular tone resembling the stiff-man syndrome. Some cases of necrotizing vasculitis and three cases of Wegener's granulomatosis have been observed in patients treated with retinoids. Except for these latter arguable cases, rheumatoid syndromes due to retinoids are rather benign, and should not be an obstacle to the future development of their therapeutic utilization.