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ID PMID Title PublicationDate abstract
1786345 Effect of corticosteroid treatment on hemopoiesis in vivo and in vitro in a patient with F 1991 We have studied the CFU-GM and BFU-E in vitro growth in a neutropenic and anemic patient with Felty's syndrome, either before or one and three months after steroid therapy when neutrophils and erythrocytes returned to normal. Both CFU-GM growth and CSA production were found to be low before therapy, and prednisone was shown to raise them to normal levels. The in vitro growth of BFU-E and the production of BPA by T lymphocytes of the patient were significantly lower than normal when studied before therapy. However, the T lymphocytes incubated in vitro with hydrocortisone regained their ability to stimulate the BFU-E growth. After prednisone therapy both BFU-E growth and BPA production by T lymphocytes returned to normal. Possible pathogenetic mechanisms of impaired granulo- and erythropoiesis in Felty's syndrome are discussed. The in vitro study with hydrocortisone can help to identify steroid-sensitive patients.
3774208 [Angioimmunoblastic lymphoadenopathy with dysproteinemia. A doubtful clinical case]. 1986 Nov 10 After a brief description of the clinical and laboratory picture of angioimmunoblastic lymphadenopathy with Dysproteinaemia (AILD), the case of a 49 year old woman is described. The woman died of haematemesis and with a clinical picture that originated 14 years earlier with Sjogren's syndrome that developed with modest systemic lymphadenopathy, splenohepatomegaly, autoimmune anaemia (Coombs direct positive). Histological examination of the parotid, certain lymph glands, the liver and the spleen suggested a diagnosis of proliferative lymphopathy that might have been either AIDL or low malignity NH lymphoma. The rarity of the syndrome and the clinical difficulties of accurate diagnosis are discussed.
2285265 Normal proprioceptive trigeminal afferents in patients with Sjögren's syndrome and sensor 1990 Dec A pure sensory neuropathy due to neuronal damage in the gasserian and spinal ganglia has been described in patients with Sjögren's syndrome. Conventional electromyographic (EMG) studies can demonstrate the selective involvement of the sensory pathways but they do not provide definite evidence for the site of the lesion. Noting that the trigeminal sensory neurons carrying cutaneous and muscular afferents are differentially located in the gasserian and mesencephalic nuclei, respectively, we carried out an electrophysiological study of the trigeminofacial and trigeminotrigeminal reflexes in 5 patients with Sjögren's syndrome and pure sensory neuropathy, in 10 patients with sensory-motor neuropathies of other causes, and in 10 healthy subjects. Our results show that patients with Sjögren's syndrome and pure sensory neuropathy who exhibited abnormal blink reflexes and an abnormal, cutaneous-induced masseter silent period had normal jaw jerks, whereas patients with sensory-motor neuropathies who exhibited abnormal cutaneous responses had abnormal jaw jerks. These findings suggest that the lesion in pure sensory neuropathy involves damage to the neurons of the gasserian ganglia and not to the trigeminal axons, since an axonal lesion would be expected to involve the large axons from muscle spindle receptors.
2241284 Mast cells in the labial salivary glands of patients with Sjögren's syndrome: a histochem 1990 Sep Mast cells in labial salivary glands obtained from patients with xerostomia with or without focal sialadenitis/Sjögren's syndrome were studied. There was no significant correlation between the intensity of local lymphocyte infiltration and the morphometrically analysed number of mast cells staining positive with toluidine blue. Histamine staining with heterologous 11C antiserum showed significantly fewer positive cells than staining with toluidine blue (mean (SD) 62 (10) v 138 (30)). This suggests heterogeneity of the mast cell population. Furthermore, there was a correlation between the focus score and the number of mast cells containing histamine. This suggests that the proliferation of mast cells containing histamine may be locally regulated by the immune inflammation, possibly through mediators from macrophages and fibroblasts. In contrast, the number of mast cells staining positive with toluidine blue could not be used as a marker of the degree of local inflammation. Although transmission electron microscopic analysis did not disclose signs of 'en masse' mast cell degranulation, mast cells may have a role in the local disease process.
2130456 [Prognostic and therapeutic aspects of lympho-epithelial lesions of the parotid. Apropos o 1990 The so-called benign lympho-epithelial lesion (BLEL) of the parotid is a rare condition. Its diagnosis is based on histology. For a long time there have been problems with its classification and prognosis: classification since certain cases may be classified as Sjögren's syndrome or as a precursor of the latter, prognosis there may be recurrence, contralateral involvement or progression to non-hodgkin's lymphoma. The authors discuss these factors in 8 cases and propose total parotidectomy with facial nerve preservation as the treatment of choice.
2517779 [Lupus anticoagulant and recurrent transient ischemic attacks]. 1989 Jul Lupus anticoagulant (LA) is an immunoglobulin that has been found in association with many diseases and shows an in vitro anticoagulant activity but is clinically associated with thrombosis. We report a patient with a diagnosis of autoimmune disease who developed recurrent transient ischemic attacks; after through investigation of other possible conditions and pathogenetical mechanisms, LA was the only causative factor that was found. We feel that LA should be systematically investigated in cerebrovascular disease, particularly in young patients without other known risk factors, to determine its true incidence and the correct therapeutic approach.
2531242 [Clinical studies of skin manifestations of Sjögren's syndrome]. 1989 May The skin manifestations of the patients with definitive Sjögren's syndrome were clinically studied. Chief complaints at the first consultation included annular erythema, pernio-like erythema, Raynaud's phenomenon, purpura, and malar erythema. Drug eruptions and xerotic eczema were also occasionally observed during the course. However, annular erythema, pernio-like erythema, drug eruptions, purpura, facial pigmented patches, and erythema nodosum were observed much more frequently in patients with primary Sjögren's syndrome. These skin manifestations might be useful clues for the diagnosis of Sjögren's syndrome in dermatological fields.
2539008 Epstein-Barr virus in the sublabial salivary gland in Sjögren's syndrome. 1989 Apr The proposed role of Epstein-Barr virus (EBV) in salivary gland destruction in Sjögren's syndrome (SS) prompted the authors to study the presence of EBV-DNA (hybridohistochemistry) and EBV-encoded proteins (immunohistochemistry) in sublabial salivary glands taken from eight patients with primary and five with secondary SS and from 16 controls. DNA probes and anti-EBV antibodies were controlled for activity by assessment of human blood B-lymphocytes after in vitro infection with EBV. None of the tissues investigated manifested the presence of EBV proteins (nuclear antigen, early antigen R, membrane antigen, or viral capsid antigen). The salivary gland biopsies of four patients with primary SS and two with secondary SS showed EBV-DNA in epithelial cells of acini and ducts but not in other components. The authors data contrast with those of Fox and colleagues (J Immunol 1986;137:3162-3168), who reported that about half of the patients with SS have EBV early antigen D in epithelium of the sublabial salivary gland. The authors conclude that an active EBV infection associated with EBV protein synthesis does not occur in the diseased salivary gland of patients with SS, but the presence of EBV-DNA in the glands does not exclude a possible role of EBV in the disorder.
3165682 Genetic markers in Sjögren's syndrome: the question of its genetic heterogeneity. 1988 Aug Genetic factors may be implicated in the causation of Sjögren's syndrome (SS) as shown by familial clustering of the disease and certain HLA associations. Non-HLA genetic markers in SS have not previously been studied in detail. In this study of 122 unrelated patients with various categories of SS and 104 control subjects, 29 genetic markers were studied (11 blood groups, 5 serum proteins and 13 red-cell enzymes). Almost all systems showed a considerable range of gene frequency among the various subgroups of patients with SS but only a few attained statistical significance (C3 and GPT). Multivariate (kinship) analysis, however, showed clear distinction between the subgroups of SS, suggesting that they are genetically distinct entities.
3700674 Morphometric study of histological changes in sublabial salivary glands due to aging proce 1986 Apr The sublabial salivary glands were studied by morphometric methods in 68 healthy volunteers to establish possible changes related to age in those tissue components that are affected in Sjögren's syndrome and connective tissue diseases (and which might stimulate Sjögren's syndrome). There was an increase in the amount of connective tissue and intralobular ducts with age and a corresponding decrease in acinar tissue. During the aging process changes in the intralobular ducts occurred: the outer and inner diameters of these ducts and the thickness of the epithelium decreased, but the ratio of the outer and inner diameters of the ducts remained constant. The amount of diffuse lymphoplasmacytic infiltrate and the vascularity of the tissue remains constant with age. In 15 of the subjects, however, discrete lymphocytic foci were seen and in six of these more than one focus/4 mm2 of salivary tissue was found, which has been described as suggestive of Sjögren's syndrome. The volume percentage of lymphocytic foci is constant during the aging process. The histological features commonly used to diagnose Sjögren's syndrome may occur in normal people, and false positive diagnoses will occur if these criteria are rigidly adhered to. Morphometry may provide more reliable criteria for distinguishing changes induced by inflammation and related to age which occur in salivary tissue.
1745512 Effects of pilocarpine on salivary flow in patients with Sjögren's syndrome. 1991 Nov Pilocarpine, a muscarinic-cholinergic agonist drug, has been reported to stimulate salivary flow in patients with salivary gland dysfunction. Previous studies involved heterogeneous groups of patients with salivary gland dysfunction and examined the short-term, single-dose, tablet form of pilocarpine. In this single-blind, placebo-controlled study we examined the long-term effects of pilocarpine administration on patients with definitively diagnosed Sjögren's syndrome (SS). Nine subjects with SS who received pilocarpine, and nine age- and sex-matched SS control subjects who received a placebo, participated. Baseline predosing sialometric and clinical data were obtained for all subjects. The study group used 2% pilocarpine as a liquid ophthalmic drop preparation, four drops three times per day, for 6-weeks. Identically appearing placebo solution with the same dosing schedule and duration was used for the control subjects. Sialometric and clinical examinations were performed. The results indicated a significant overall increase in both whole unstimulated salivary flow (0.15 +/- 0.03 ml/min in study subjects vs 0.02 +/- 0.001 ml/min in control subjects; p less than 0.001) and parotid stimulated salivary flow (0.14 +/- 0.04 ml/min in study subjects vs 0.009 +/- 0.002 ml/min in control subjects; p less than 0.001) in the pilocarpine group as compared with the placebo group. The results of this study support the use of pilocarpine to increase salivary flow in patients with SS.
1972576 [Salazosulfapyridine-induced eosinophilic pneumonia with pulmonary and cutaneous epithelio 1990 Apr A 68-year old woman suffering from Sjögren's syndrome for the last 30 years took sulphasalazine (SSP) for severe signs and symptoms at the joints. Soon after the start of this medication she developed progressive cough and shortness of breath. After two years she was referred for evaluation of a hemoptysis and a reddish skin lesion. The chest radiograph showed wide spread interstitial infiltrates in the lower lobes and some fibrotic changes. FVC was slightly reduced, DLCO markedly reduced. There was a high percentage of eosinophils in the bronchoalveolar lavage (55.2%). Transbronchial lung biopsy and skin biopsy demonstrated epitheloid granulomata. SSP was discontinued. After a short period of prednisone treatment the patient's condition improved considerably. After two months of followup neither pulmonary infiltrates nor any skin lesions were found. History and the clinical course after discontinuation of SSP indicate the relation of these infiltrates to SSP treatment. The previously published case reports of SSP-related lung disorders are reviewed. Three of these case reports included bronchoalveolar lavage. Our data suggest that patients with SSP related pulmonary infiltrates may have a marked increase of eosinophils in the bronchoalveolar lavage fluid.
2789649 Neurologic involvement in primary Sjögren's syndrome: a preliminary report. 1989 Aug Forty consecutive patients with primary Sjögren's syndrome (SS) were prospectively evaluated for evidence of neurologic manifestations of the disease. All patients had primary SS diagnosed on the basis of typical sicca symptomatology, objective documentation of keratoconjunctivitis sicca (KCS) and/or xerostomia, and a positive minor labial salivary gland biopsy. None had criteria that would classify him/her as systemic lupus. Evaluation included a detailed neurologic history and physical examination and nerve-conduction studies of all four extremities. Ten patients had a mild sensory or mixed neuropathy of the glove-stocking pattern, and six of them had abnormal nerve conduction studies. Two of those, plus one more, had trigeminal neuropathy. Two more patients had abnormal electrophysiologic studies, one of them with absence of Achilles tendon reflexes as well. None of the above patients volunteered complaints related to these findings. Only one patient with severe primary SS, manifested by cryoglobulinemia, vasculitis and glomerulonephritis, presented with mononeuritis multiplex, that partially responded to intravenous cyclophosphamide and high-dose steroids. Central nervous system (CNS) disease was not detected in any of our patients, suggesting that it is rather rare, whereas peripheral nervous system (PNS) involvement is relatively common and benign in the majority of primary SS individuals.
3316399 Purification of human autoantibodies from cross-linked antigen immunosorbents. 1987 Nov 23 A method is described whereby autoantibodies to the Sjögren's syndrome antigen La (SS-B) can be purified from re-usable immunosorbent columns constructed from covalently linked human autoantibodies to which the antigen is cross-linked. Previous attempts to link the antigen directly to CNBr-Sepharose beads resulted in loss of biological activity and thus each purification of antibody required fresh batches of antigen. The present technique is a significant improvement since the cross-linked immunosorbents prepared from a single batch of antigen can be re-used several times over a 6 month period. Furthermore F(ab')2 fragments of anti-La antibodies can be purified from pepsin-digested serum samples. These antibodies react in ELISA, Western blot and immunofluorescence in an identical way to serum and murine monoclonal anti-La antibodies and show no reaction with the Ro antigen. However, being of human origin the affinity-purified anti-La antibodies have the advantages of bearing the same idiotypes and reacting with the same antigenic epitopes as naturally occurring serum autoantibodies.
2823241 Salivary gland tumors. Diagnostic characteristics of the common types. 1987 Oct The salivary glands can be the site of a wide array of pathologic conditions. Information from the history is very important in diagnosis. During physical examination, the clinician can determine whether a mass is distinct or diffusely enlarged and can check for mobility and skin fixation. The depth of the mass and its relationship to other structures can be assessed and the ductal orifice evaluated for purulence, turbidity, and absence of salivary flow. Studying the patient's face also provides diagnostic clues. Surgery provides the best basis for histologic diagnosis and allows complete removal of the mass. Fortunately, most neoplasms are benign and their treatment is straightforward.
3495873 Symptomatic secondary Sjögren's syndrome in patients with systemic lupus erythematosus (S 1986 The prevalence of symptomatic secondary Sjögren's syndrome in 66 patients with SLE recruited from a defined population in southern Sweden was 59%. In 26 patients (39%) the symptoms were mild and variable, related to disease activity, while in a smaller group (20%) the symptoms appeared to be constant. The latter patients had an increased frequency of thyroid disease and autoantibodies to SS-A. The patients in this group were older, but did not seem to differ in regard to other disease manifestations.
1743786 Acute aortitis and aortic incompetence due to systemic rheumatological disorders. 1991 Nov We report the clinical, laboratory and echocardiographic features of five cases of aortic incompetence associated with ankylosing spondylitis, rheumatoid arthritis and undefined connective tissue diseases. Immunosuppression with steroids and cytotoxic agents was used to suppress aortic root inflammation in four cases; in three the aortic root size stabilized and the patients remain well with no evidence of increasing aortic incompetence. In one case, control of the inflammatory process was never fully achieved for any length of time and the patient died shortly after aortic valve replacement. A fifth case required urgent valve replacement and remains well. A systemic rheumatological disorder should be considered in cases of apparent "lone" aortic incompetence and conversely aortic incompetence should not be overlooked in established systemic rheumatological disease. Immunosuppressive therapy may prevent or delay the need for aortic valve replacement in such cases.
2055269 Role for zinc in a cellular response mediated by protein kinase C in human B lymphocytes. 1991 Jul Recent studies have suggested a role for Zn2+, distinct from that of Ca2+, in the subcellular distribution and activation of protein kinase C (PKC). Here we show that Zn2+ is required for a cellular response mediated by PKC, the rapid loss of expression of a human B cell receptor MER, detected by rosetting with mouse erythrocytes. Zn2+, in the presence of the Zn2+ ionophore pyrithione, caused rapid inhibition of MER rosetting at concentrations which induce the translocation and activation of PKC. This required cellular uptake of Zn2+ and was blocked by 1,10-phenanthroline and TPEN which chelate Zn2+ but not Ca2+. Gold, a metal with similar properties, also induced translocation of PKC and inhibition of MER. By contrast, Ca2+ ionophores A23187 and ionomycin, which induce a different pathway of translocation of PKC, had no effect on MER. Phenanthroline and TPEN also blocked the inhibition of MER induced by the PKC activators phorbol ester and sodium fluoride, suggesting that endogenous cellular Zn2+ is required. We propose that some cellular actions of PKC require a Zn(2+)-dependent event and that these may be a target for gold during chrysotherapy in rheumatoid arthritis.
1896578 [Magnetic resonance in the tunnel carpal syndrome. Possibilities and perspectives of an et 1991 Jul Thirty-four selected patients were evaluated in order to define MRI capabilities in the preoperative evaluation and characterization of the pathogenetic patterns of carpal tunnel syndrome (CTS). MRI examinations were performed by means of a superconductive unit (1.0 T, Magnetom): SE T1 (500/17) and T2 (2000/90) axial images of the carpal region were obtained with a round surface coil. In 8 patients 3D GE (FLASH) pulse sequences were used to obtain 32 images of the hand; 3D reconstruction was also applied. Six patients with rheumatoid arthritis and amyloidosis were also studied after i.v. injection of Gd-DTPA (0.2 mM/kg). MRI findings were compared with both clinico-electrophysiologic and surgical results. High agreement was observed only between MRI and surgical findings. MRI allowed the direct demonstration of carpal tunnel abnormalities in 8 cases, while abnormal findings in the median nerve were observed in 18 patients. The possibility of depicting medial nerve lesions on T2-weighted images when no direct demonstration of the cause of compression is possible, could represent a guideline for the etiopathogenetic investigation of CTS. However, further experience in selected patients is necessary to define all the aspects relative to this very common syndrome.
2067837 [Functional roentgen diagnosis of the upper cervical spine]. 1991 Apr For evaluation of the function of the upper cervical spine, especially assessment of the range of motion, functional X-rays films are useful in addition to the clinical examination. For the diagnosis of segmental instability, passive motion should be induced in order to obtain the full range. If anterior instability of the upper cervical spine is suspected, flexion-extension X-rays in the lateral view are appropriate. If a lesion of the alar ligaments is suspected, then lateral flexion X-rays films should be taken. In the normal situation, the atlas glides in the direction of bending, coupled by forced rotation of the axis. In cases with rotatory instability of the upper cervical spine, functional computed tomography should be performed. Atlantoaxial rotation of more than 52 degrees should be considered pathological as a result of a lesion of the alar ligaments. For examination of the relationship between the spinal cord and bony structures or inflammatory tissue in patients with rheumatoid arthritis, functional MRIs are helpful. Not only the exact diameter of the spinal canal can be measured during flexion and extension, but the degree of basilar impression or cranial migration of the axis can also be assessed. Optimal use of functional X-ray diagnostics, including functional the CT and MRI, is not only helpful for clinical diagnoses, but also for the planning of surgical procedures.