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ID PMID Title PublicationDate abstract
8295197 Immunoblastic malignant lymphoma in adult onset Still's disease. 1993 Oct Lymphadenopathy commonly occurs in patients with adult onset Still's disease (AOSD). Besides the more common reactive hyperplasia, the histological pattern may occasionally be confused with that of malignant lymphoma. A case of AOSD that developed an immunoblastic malignant lymphoma is described. Immunohistochemistry showed the B cell nature of the proliferating cells. As a result, AOSD should be added to the list of systemic rheumatic diseases, the course of which may be complicated by lymphoproliferative malignancies. Our case underlines the need for a careful examination and of close followup to detect this eventuality earlier.
8531342 [Investigations of various animal models for Sjögren's syndrome]. 1995 Oct Several animal models for studying immune-mediated sialadenitis include autoimmune-prone mice which develop lesions spontaneously, and non-autoimmune-prone mice in which the lesions can be induced by various experimental manipulations. Autoimmune disease-prone mice such as NZB, NZB/WF1, MRL/lpr, SL/Ni, and NOD (non-obese diabetic) have been used as animal models for the investigation of Sjögren's syndrome in humans. We have previously demonstrated murine experimental systems in which autoimmune sialadenitis was induced in several strains of thymectomized mice, and developed spontaneously in certain strains of aged mice with H-2 restriction. We have recently established an animal model for primary Sjögren's syndrome in NFS/sld mutant mice bearing an autosomal recessive gene with sublingual gland differentiation arrest. A significantly higher incidence of autoimmune lesions in the salivary and lacrimal gland was found in female mice, and the anti-salivary duct autoantibodies were detected in sera from mice with autoimmune lesions. A preferential use of TCRV beta gene was detected in autoimmune lesions from the onset of disease, suggesting that TCR-based immunotherapy is possible.
7582709 Destructive lymphadenopathy and T-lymphocyte activation in adult-onset Still's disease. 1995 Oct Recurrent arthritis, fever and lymphadenopathy are symptoms of adult onset of Still's disease (AOSD). Differential diagnosis requires the exclusion of infections or malignant lymphomas. We report on a case of AOSD showing destructive lymphadenopathy, immunophenotyping of peripheral blood leucocytes revealed strong activation of T-lymphocytes. Bone marrow biopsy also showed an increase of lymphopoietic cells to 23%. Analysis of peripheral blood lymphocytes (PBL) after remission showed no remaining signs of activation. Analysis of lymphocyte activation by flow cytometry correlated with disease activity.
7653944 Sjögren's syndrome and virus. 1995 Though there have been claims for a key-role for a thus far unknown virus in the pathogenesis of Sjögren's syndrome (SS), conflicting results are to be discussed in this review. One candidate is Epstein-Barr virus which has been found within epithelial cells of labial salivary glands (LSG), using in situ hybridization and polymerase chain reaction. Hepatitis virus C has also been suspected, but an issue has to be made on serological false-positive results. In the light of the detection of the tax gene of human T lymphotropic virus type 1 in LSG, retroviruses have also been incriminated.
7926870 Collection and concentration of tear proteins studied by SDS gel electrophoresis. Presenta 1994 Jul An obvious obstacle in the analysis of tear film samples from patients with dry eyes is the drastically reduced tear volume. To overcome this problem we have developed a method by which the surface of the eye is flushed with saline, diluting the sparse tear fluid, followed by a concentration of the wash fluid. We compared undiluted tears, diluted tears, tear fluid collected by flushing, reconcentrated diluted tears and reconcentrated flush fluid. Gel electrophoresis of the tear samples obtained showed a representative collection of tear proteins, with bands ranging in molecular weight from about 10 kDa to about 90 kDa. The method may therefore provide a useful alternative to the analysis of undiluted tear fluid in such patients. After storage at -80 degrees C for 1 month, electrophoresis often showed some weakening of band intensities. However, no specific loss of bands was found, and by ultracentrifugation a band profile similar to that of fresh, undiluted tears was obtained. For most purposes, therefore, samples may be kept by this means for later analysis.
8082485 Phenotypic and functional abnormalities in the peripheral blood T-cells of patients with p 1994 Mar 15 Changes in regulatory T-cell subset (including the recently described CD4 helper inducers or suppressor inducers) balance in the peripheral blood may play a role in the pathogenesis of primary Sjogren's syndrome (SS). Direct immunofluorescence and flow cytometry were used to quantitate and analyse peripheral blood lymphocytes in 15 patients with primary SS and 15 control subjects. A reduction in the percentage of circulating CD4 lymphocytes was observed in patients with SS. There was no quantitative abnormality in the percentage of circulating CD4+ 2H4+ (suppressor inducer), CD4+ 4B4+ (helper inducer), CD2, CD3, CD8, CD8+ 2H4+, CD8+ 4B4+, CD25 (IL-2R), CD19, CD16, CD57 lymphocytes in the patients. Circulating CD8 lymphocytes expressing the activation marker HLA-DR were increased in the patients. The functional status of peripheral blood lymphocytes was assessed by PHA (phytohaemagglutinin) stimulation followed by monitoring their proliferative response by radiolabelled thymidine uptake and expression of CD25 (Interleukin-2 receptor). A reduction in the proliferative response of total, CD4-depleted, and CD8-depleted lymphocytes suspensions to PHA was demonstrated. The level of expression of CD25 (IL-2 receptor) was similar in patients and controls before and after 24 h stimulation with PHA. We conclude that there is a disturbance in the functional properties of peripheral blood T cells that can contribute to the immunopathogenesis of SS. Meanwhile, the quantitative reduction of suppressor/inducer lymphocytes as defined by the CD4 2H4 phenotype can be precluded from a role in the development of such an autoimmune condition.
8170852 Pseudorheumatoid nodule (deep granuloma annulare) of childhood: clinicopathologic features 1994 Mar Pseudorheumatoid nodule of childhood is seen uncommonly by pathologists and is thought to be a variant of granuloma annulare. We report a series of 20 patients (13 M, 7 F) age 1 to 17 years. Fourteen lesions arose on the front of the lower limb, and 14 children had numerous lesions at diagnosis. Seven developed some form of recurrence, except for one case occurring at the site of the original lesion. Only one patient had a weakly positive rheumatoid factor, and was the patient with remote recurrence. As a significant number of lesions arose around the foot when the child was beginning to walk or starting to wear shoes, it is suggested that trauma plays a role in the development of some of these lesions.
7984249 [Crescentic glomerulonephritis and primary Gougerot-Sjögren syndrome]. 1994 Glomerulonephritis complicating primary Gougerot-Sjögren's syndrome is extremely rare. We report the case of a 72-year old woman with primary Gougerot-Sjögren syndrome complicated by progressive renal failure. Kidney biopsy revealed a crescentic glomerulonephritis. A rapid improvement occurred with corticosteroids. This observation is, at our knowledge, the first case of crescentic glomerulonephritis described during this disease. A review of the literature concerning glomerulonephritis complicating primary Gougerot-Sjögren syndrome is reported.
8352376 Necrobiotic palisading suture granulomas simulating rheumatoid nodule. 1993 Sep Five patients without clinical evidence of rheumatic disease developed postoperative periarticular suture reactions featuring necrobiotic granulomas histologically similar to rheumatoid nodules. Suture granulomas are to be included in the differential diagnosis of palisading granulomas simulating rheumatoid nodule.
1504432 Hypokalemic paralysis associated with distal renal tubular acidosis. 1992 May A 68-year-old man had hydronephrosis due to ureteral stones for two months earlier and then increasing muscle weakness developed. A 30-year-old woman had rapidly progressive quadriparesis. In both cases, severe hypokalemia with metabolic acidosis was observed and the diagnosis of distal renal tubular acidosis was made. The former was considered to be an idiopathic incomplete form and the latter was a secondary complete form associated with Sjögren syndrome. Hypokalemic paralysis may occur as a complication of distal renal tubular acidosis.
9046856 [A patient with Sjögren syndrome with central pontine myelinolysis and hypokalemic myopat 1996 Nov We report a 42-year-old woman who presented with intermittent episodes of muscular weakness lasting approximate 7 to 10 days, about once a year, since 1985. During these episodes of weakness, hypokalemia and elevation of serum creatine kinase were noted. On admission, xerostomia, keratoconjunctivitis sicca (positive Schirmer test), abnormal sialographic findings (apple tree-like appearance), and positive serum autoantibodies against ss-A and nucleus were noted. These findings were compatible with Sjögren syndrome. She also had hyperchloremic metabolic acidosis with normal anion gap, hypokalemia and renal calcifications. Therefore, she had renal tubular acidosis (type 1) in addition to Sjögren syndrome. Brain magnetic resonance imaging (MRI) demonstrated a symmetrical circumscribed lesion in the ventral part of the central pons. We considered this lesion to be central pontine myelinolysis (CPM), because of its typical location and characteristic MRI appearance. This CPM was clinically silent. Pathogenesis and etiology of CPM are obscure, and CPM associated with hypokalemia without hyponatremia has rarely been reported in the literature. However, this is the first report of CPM associated with Sjögren syndrome and hypokalemic myopathy complicated with renal tubular acidosis.
8804997 [Detection of Helicobacter pylori in dermatoses. Clinical incidental finding or pathogenet 1996 Aug Recently, several reports have been describing a possible relation between Helicobacter pylori infection of the gastric mucosa and dermatological diseases. Associations have been reported for urticaria, roascea, Sjögren's syndrome and Schönlein-Henoch purpura. In this paper we review the current knowledge on Helicobacter pylori infection and its relevance for skin diseases, especially its clinical and pathophysiological aspects.
9387537 [Sialographic follow-up study of patients with Sjögren's syndrome]. 1996 Mar Sialographic follow-up studies of 36 cases of Sjögren's syndrome (SS) were performed with the duration of 5-72 months (mean 25 months). Three groups were divided according to the intenational criteria: secondary group, 14 cases (xerophthalmia, or/and xerostomia, and connective tissue diseases), primary group, 14 cases (xerostomia and xerophthalmia), and possible group, 8 cases (xerophthalmia or xerostomia only). Sialographic findings at follow-up examination included (1) delayed evacuation, which may occur as early as 5 months later; (2) increased punctate sialectasis; (3) decreased punctate sialectasis with globular increase; (4) Punctate, globular, and cavity sialectasis with radiolucent sialolith; and (5) centripetal atrophy. The cases with secondary and possible SS without xerostomia demonstrated little involvement of the parotid gland, without sialectasis. But sialodochitis was shown when radiolucent sialolith formed. The stimulated whole salivary flow rate (SWSFR) was closely related to sialographic appearance, i.e., the cases with lower value of SWSFR showed more sialectasis sialographically, but with higher SWSFR value, and less sialectasis.
8838880 Ultrasound features of Sjögren's syndrome. 1996 Feb The aim of this study is to demonstrate the different ultrasound appearances of the salivary glands in Sjögren's syndrome, and discuss the differential diagnosis. A reticulated appearance of the salivary and lacrimal glands in Sjögren's syndrome was also demonstrated which, to our knowledge, has not been previously described.
8531370 [Atrophic gastritis in Sjögren's syndrome]. 1995 Oct In patients with Sjögren's syndrome (SJS), it has been found that chronic atrophic gastritis is often associated with low HCl secretion or achlorhydria. However, the role of autoimmune injuring mechanism in the pathogenesis of the atrophic gastritis in SJS is unclear. In recent studies, Helicobacter pylori (HP) infection is accepted as the major cause of chronic gastritis. In our studies, HP infection was present in the half of SJS patients with atrophic gastritis. We also determined HP specific IgG antibody levels by enzyme-linked immunosorbent assay (ELISA). In SJS patients with gastric inflammatory cell infiltration, the IgG levels were high. We determined gastric parietal cell antibody (PCA) levels by ELISA, using a recombinant fusion protein of the alpha subunit of gastric H+, K(+)-ATPase. Our observation suggests that atrophic gastritis in SJS is caused by HP infection associated with gastric mucosal injury due to PCA.
7863283 Lacrimo-auricolo-dento-digital syndrome mimicking primary juvenile Sjögren's syndrome. 1995 We describe a 24 year old female patient affected with symptoms of severe xerostomia and keratoconjunctivitis sicca since her childhood. She also had several malformations involving face and digits as well as cup shaped ears, enamel dysplasia and absence of lacrimal puncta. Hence, the diagnosis of Lacrimo-Auricolo-Dento-Digital (LADD) syndrome was made. The main differences between this rare, inherited disease and primary juvenile Sjögren's syndrome are pointed out.
8162641 Endogenous avidin-binding activity in epithelial cells of the ducts of human salivary glan 1994 Jan Avidin-biotin (AB) systems are commonly employed to investigate salivary gland sections either in immunohistochemistry or in immunofluorescence techniques. We noted non-specific staining in the ductal epithelium of minor salivary gland (SG) sections from 5 Sjögren's syndrome (SS), 5 chronic sialoadenitis (CS), and one normal parotid gland (NP) when incubated with AB peroxidase complex. Inhibition of endogenous peroxidase did not prevent the staining while saturation of supposed biotin-like molecules in the tissue with added avidin led to the loss of this non-specific staining. These results demonstrate the presence of endogenous avidin binding activity (EABA) in the epithelial cells of salivary gland ducts. We suggest that the avidin and streptavidin systems should not be used for immunohistological examination of the ductal epithelium of salivary glands.
8151574 Sjögren's syndrome with amyloid A presenting as multiple pulmonary nodules. 1994 Jan A 29-year-old woman presented with diffuse pulmonary nodular amyloidosis and was subsequently diagnosed as having Sjögren's syndrome. We believe that this is the first case of Sjögren's syndrome presenting with secondary amyloidosis of the lung.
8352240 Cell-mediated immune-pancytopenia complicating primary Sjögren's syndrome. 1993 Jul A 64-year-old woman with mild bilateral parotid gland swelling and bilateral lower extremity purpura was admitted for evaluation of xerostomia and pancytopenia. The patient had an increased erythrocyte sedimentation rate, pancytopenia, and positive tests for antibodies to nuclear antigen, SS-A, and SS-B. Impaired cell-mediated immunity was also present. Bone marrow aspiration showed a hypoplastic marrow with an increased percentage of lymphocytes. A positive Schirmer's test and keratoconjunctivitis were also noted. A diagnosis of primary Sjögren's syndrome was made by sialography and histological salivary gland findings. Therapy with prednisolone improved the pancytopenia. Addition of the patient's peripheral blood mononuclear cells to cultures of bone marrow mononuclear cells derived from a healthy volunteer dose dependently inhibited colony formation, including mixed hemopoietic colonies. On the other hand, addition of the patient's serum failed to inhibit colony formation by normal bone marrow mononuclear cells. These results suggested that one of the causes of pancytopenia in primary Sjögren's syndrome might be mediated by the inhibition of mononuclear cells to the hemopoietic progenitors.
1437923 Systemic lupus erythematosus in 50 year olds. 1992 Jun We compared the clinical and serological characteristics of 15 patients with onset of systemic lupus erythematosus after the age of 50 with those of 232 younger patients. The sex distribution was similar in both groups. All 15 patients were Caucasian. Autoimmune thyroiditis was found in 20% of the elderly patients. Initial manifestations, which presented more frequently in the older group, included thrombocytopenia (P < 0.05), sicca syndrome (P < 0.01) and cardiomyopathy (P < 0.005), whereas butterfly rash (P < 0.05) presented more frequently in the younger group. Analysis of cumulative clinical symptoms showed that butterfly rash (P < 0.05) and livedo reticularis (P < 0.05) were less frequent in the elderly. However, this group presented a significantly increased incidence of sicca syndrome (P < 0.005) and cardiomyopathy (P < 0.005). Antibodies to double-stranded DNA tended to occur less frequently in older patients (P < 0.05).