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Ulbricht Kai U - - 2003
Alpha-fodrin is a part of the membrane skeleton and expressed in the majority of mammalian cells. It is cleaved in apoptosis by caspase 3. One of the cleavage products, a 120-kDa protein, represents a neoantigen. Antibodies against that cleavage product of alpha-fodrin have originally been described in a murine model ...
Plaza G - - 2003
We present a case of a 40-year woman with bilateral parotid salivary gland enlargement as presentation of primary Sjögren's syndrome. Computed tomography (CT) and magnetic resonance imaging (MRI) showed parotid cysts, suggestive of cystic benign lymphoepithelial lesions. A sub-labial biopsy confirmed the syndrome. After 24-month follow-up, the left parotid cysts ...
Mihas Anastasios A - - 2003
There is much confusion in the literature regarding the differences between Mikulicz disease and Mikulicz syndrome. This may be because there seems to be a connection between the disease and disease processes associated with the syndrome. This article provides historical data discussing the emergence of Mikulicz disease, confusions regarding its ...
Ago┼čton-Coldea Lucica - - 2002
Sjögren's Syndrome is a very frequent autoimmune disease, characterised by exocrine gland involvement. Immunologic disorders are also responsive for extraglandular manifestations of the disease, mostly for the digestive involvement. We report a case of primary Sjögren's syndrome with multiple extraglandular manifestations: vasculitis, cryoglobulinaemia, hepatic involvement and presumably neurologic involvement. The ...
Winer Shawn - - 2002
BACKGROUND: Sjögren's syndrome is a common (about 1% of the population) autoimmune disease of salivary and lacrimal glands. Its cause and pathogenesis are poorly understood, and treatments are mostly for symptoms of the disease. ICA69 is a self-antigen expressed in brain, pancreas, salivary, and lacrimal glands. NOD-strain mice are an ...
Mahlstedt Kathrin - - 2002
OBJECTIVE: Minor salivary gland biopsy is suggested for diagnosing Sjögren's syndrome in patients with clinical signs of sicca syndrome. This biopsy is mainly examined on the basis of the semiquantitative morphologic criteria without taking into account the detection of characteristic myoepithelial sialadenitis (MESA). DESIGN: The role of minor salivary gland ...
Folli F - - 2002
Sjögren's syndrome is a chronic autoimmune disease affecting exocrine glands, resulting in xerostomia and xerophthalmia. Lymphocytic infiltration and fibrosis of exocrine glands as well as the presence of autoantibodies against organ-specific and non-organ-specific antigens are the hallmarks of the disease. We investigated whether some patients affected by Sjögren's syndrome might ...
Shimoyama Masaki - - 2002
Autonomic failure is rare in patients with Sjögren syndrome (SS). We report the case of a 46-year-old woman with severe autonomic cardiovascular failure, manifested by incapacitating postural hypotension, as the first symptom of primary SS. Treatment with glucocorticoid resulted in a dramatic improvement of her symptoms and objective findings of ...
Akahane Chiho - - 2002
A 55-year-old man suffered from nasal obstruction, swelling of the salivary glands, and diplopia caused by markedly enlarged lacrimal glands. The diagnosis of primary Sjögren's syndrome was made by a positive Schirmer's test and nasal mucosal biopsy with severe lymphocyte infiltration. He was also found to have swelling of the ...
Campanati Anna - - 2002
An unusual case of widespread acne unresponsive to treatment with early onset in a child with Apert's syndrome is presented. The patient eventually responded to oral isotretinoin therapy. The morphological profile of the sebaceous glands and the expression of proliferative markers and androgen receptors were evaluated in seboblasts and sebocytes ...
Malatskey Shelton - - 2002
Frey syndrome is a disorder characterized by unilateral sweating and flushing of the facial skin in the area of the parotid gland occurring during meals. The syndrome is a sequela of parotidectomy and may follow other surgical, traumatic, and inflammatory injuries of the parotid and submandibular glands and the cervical ...
Petrek M - - 2002
OBJECTIVE: Interleukin-1 receptor antagonist (IL-1Ra) and also mononuclear cell-attractant chemokines CCL3, CCL4 and CCL5 have been implicated in the immunopathogenesis of primary Sjögren syndrome (pSS). Both the gene coding for receptor CCR5 binding the aforementioned CCL ligands and the gene for IL-1Ra are polymorphic. We have therefore in a case ...
Khanna Dinesh - - 2002
Patients with Sjögren's syndrome (SS) have an increased risk of developing lymphoproliferative disorders, mainly non-Hodgkin's lymphoma and pseudolymphoma. We describe a young Caucasian woman with a 7 year history of SS, who presented with meningeal signs and symptoms and was found to have non-Hodgkin's lymphoma of the central nervous system.
Tsubota K - - 2002
The objective of our study was to investigate the possibility of Fas ligand protein abnormalities in certain types of Sjögren's syndrome patients with enlarged exocrine glands. Fas ligand expression by lymphocytes infiltrating the lacrimal glands and by peripheral blood monocytes in Sjögren's syndrome patients with enlarged exocrine glands was assessed ...
Vitali C - - 2002
Classification criteria for Sjögren's syndrome (SS) were developed and validated between 1989 and 1996 by the European Study Group on Classification Criteria for SS, and broadly accepted. These have been re-examined by consensus group members, who have introduced some modifications, more clearly defined the rules for classifying patients with primary ...
Jonsson Roland - - 2002
Sjögren's syndrome is a chronic autoimmune and rheumatic disorder with prominent sicca complaints from the mucous membranes because of lack of proper exocrine secretions. There is no straightforward and simple diagnostic test for Sjögren's syndrome, although several classification criteria have been designed including several oral diagnostic tests. A new set ...
Reinker Kent - - 2002
Two patients with Marinesco-Sjögren syndrome had striking orthopaedic abnormalities that seemed to arise from multiple areas of physeal growth arrest. Major involvement was seen in the distal femora, where bilateral hypoplasia of the lateral condyles resulted in progressive valgus, patellar dislocation, and quadriceps dysfunction. Marinesco-Sjögren syndrome seems to arise from ...
Azuma Takanori - - 2002
Sjögren's syndrome is a chronic autoimmune disease characterized by focal lymphocytic infiltration of lacrimal and salivary glands, but the precise mechanism of this syndrome is poorly understood. To clarify the mechanism of onset and progression of Sjögren's syndrome, it is necessary to identify Sjögren's syndrome-related genes. For this purpose, we ...
Taura Shin-ichi - - 2002
PURPOSE: The authors assessed the uptake of Tc-99m pertechnetate in the thyroid using salivary gland scintigraphy in patients with Sjögren syndrome and in healthy controls. MATERIALS AND METHODS: Salivary gland scintigraphy and a labial biopsy were performed in 73 patients with Sjögren syndrome. Based on the labial biopsy findings, 32 ...
Mochizuki Hitoshi - - 2002
Most of the peripheral neuropathies in Sjögren's syndrome (SS) are sensory- or autonomic-dominant. In this report, we present two cases of a rare type of neuropathy, motor dominant neuropathy, in SS. One showed signs similar to those of Guillain-Barré syndrome, and the other showed signs characteristic of chronic inflammatory demyelinating ...
Merlini L - - 2002
OBJECTIVE AND BACKGROUND: To describe three Gypsy families with Marinesco-Sjögren syndrome (MSS), demyelinating neuropathy, and recurrent episodes of myoglobinuria in five of the six affected subjects. Because these families originated from the same genetically isolated founder population as did patients with congenital cataracts facial dysmorphism neuropathy (CCFDN) syndrome, and because ...
Herrero-González J E - - 2002
We report the case of a woman with a primary Sjögren's syndrome who developed asymptomatic anetoderma lesions with no other pathology responsible. This dermatosis has been associated with many autoimmune disorders, in particular lupus erythematosus and lupus-like syndromes. Our literature review found only one previous description of primary anetoderma associated ...
Gran Jan Tore - - 2002
During the last years, several sets of criteria for the diagnosis and classification of primary Sjögren's syndrome (SS) have been promulgated. So far none has achieved universal acceptance. It is conceivable that the lack of pathognomonic features of SS and the frequent coexistence of SS with other connective tissue di-seases ...
Ostuni Pierantonio - - 2002
OBJECTIVE: To assess the prevalence of fibromyalgia in primary Sjögren's syndrome and to evaluate the clinical differences between patients affected with both primary fibromyalgia and primary Sjögren's syndrome and those affected only with primary fibromyalgia. METHODS: Clinical features of fibromyalgia were evaluated in 100 consecutive outpatients with primary Sjögren's syndrome ...
Simón J Abraham - - 2002
Hyperviscosity syndrome is a disorder first described in patients with Waldenström's macroglobulinemia and is not commonly seen in rheumatic diseases. Its association with Sjögren's syndrome is very rare and it has been reported in very few patients. We report the case of a patient with primary Sjögren's syndrome presenting as ...
Karanikas Georgios - - 2002
OBJECTIVE: To evaluate the usefulness of Tc-99m-HIG scintigraphy in patients with Sjögren's syndrome. METHODS: Twelve consecutive patients with verified secondary Sjögren's syndrome were included in this prospective study. The control group consisted of seven patients with Lupus erythematosus; none of them showed clinical signs of Sjögren's syndrome. Planar and SPECT ...
Johansson G - - 2001
OBJECTIVES: To study the effect of mouthrinses with salivary replacement substances on oral conditions in patients with primary Sjögren's syndrome. DESIGN: Cross-over, double-blind study. SETTING: Facilities at the Centre for Oral Health Sciences, Malmö University and at Malmö University Hospital, Malmö, Sweden. SUBJECTS: Twenty-two patients with Sjogren's syndrome. INTERVENTION: Linseed ...
Ochoa E R - - 2001
We report a case of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type of the salivary gland arising in a background of chronic sclerosing sialadenitis. Chronic sclerosing sialadenitis is a common fibrosing chronic inflammatory lesion of the submandibular gland, which is thought to be the result of ...
Beroukas D - - 2001
Secretions from salivary and lacrimal glands are reduced in patients with primary Sjögren's syndrome (PSS). Since aquaporin 5 is involved in transport of water and is present in salivary and lacrimal glands, this protein was thought to have a major role in the pathogenesis of PSS. We used indirect immunofluorescence ...
Koyama M - - 2001
The purpose of this study was to describe the high-resolution computed tomography (HRCT) findings of pulmonary involvement in primary Sjögren's syndrome. The study included 60 patients who met the diagnostic criteria for primary Sjögren's syndrome. The authors retrospectively reviewed the presence, extent, and distribution of various HRCT findings. Results showed ...
al-Hashimi I - - 2001
Sjögren's syndrome is a multi-system chronic inflammatory disease of the exocrine glands. Inflammation of the salivary glands leads to reduction in salivary output, which imposes a significant impact on oral health. Dentists and dental hygienists are the primary healthcare providers to identify early signs and symptoms of Sjögren's syndrome. Early ...
Kondo K - - 2001
A 61-year-old woman, who had been diagnosed as having Sjögren's syndrome, developed an anterior mediastinal mass. She was diagnosed with Sjögren's syndrome with thymoma, preoperatively. Extended thymectomy was performed. Macroscopically, the mediastinal mass showed thick-walled multiloculated cavities filled with turbid yellow fluid. Microscopically, the cyst lining was continuous with thymic ...
Voulgarelis M - - 2001
Sjogren's syndrome is a chronic inflammatory process involving primarily the exocrine glands. Its association with lymphoma is well documented. A low grade marginal-zone lymphoma related to mucosa-associated lymphoid tissue is the commonest lymphoid neoplasia in Sjogren's syndrome. We review the literature and comment on the molecular, clinical, histopathologic and therapeutic ...
Gottfried J A - - 2001
We describe a case of pediatric Sjögren's syndrome with progressive neurologic involvement. At age 4 years, she had been diagnosed with Melkersson-Rosenthal syndrome. After being stable with facial diplegia and swelling for 5 years, she acutely presented with diplopia, vertigo, and ataxia. Cranial magnetic resonance imaging (MRI) showed a left ...
Reddy Ashwin - - 2001
This is a case report of Rosai-Dorfman syndrome in a 36-year-old Caucasian male, involving the lacrimal gland, cervical lymph nodes, nasal and sinusal mucosa. It was successfully treated with appropriate immunosuppression. He had initially presented to the ENT surgeon with nasal and sinusal mucosal thickening and bleeding. Cervical lymph node ...
Al-Hashimi I - - 2001
The purpose of this study was to examine the reproducibility of biopsy grades at various tissue depths in Sjögren's syndrome. The biopsy grades of 38 minor salivary gland biopsies were examined at 6 microm, 50 microm, 100 microm, 150 microm, 200 microm, and 250 microm tissue depths. Tissue sections were ...
Young W - - 2001
Four cases-of congenital dysfunction of the major salivary glands as well as of Prader-Willi, congenital rubella, and Sjögren's syndromes-were identified in a series of 500 patients referred for excessive tooth wear. Although there was evidence of consumption of highly acidic drinks, some occlusal parafunction, and unacceptable toothbrushing habits, salivary dysfunction ...
De Vita S - - 2001
A classification of nonmalignant lymphoproliferation in Sjögren's syndrome is presented, based on the results of international meetings regarding Sjögren's syndrome-associated lymphomagenesis and on our results of a clinico-pathologic and molecular study and long-term follow-up in well-characterized patients. Sjögren's syndrome pathobiology has similarities to hepatitis C virus-related B-cell lymphoproliferation. Antigen stimulation ...
Delèvaux I - - 2001
A 48 year old woman was referred to hospital for buccal discomfort. Physical examination showed a macroglossia and features of xerostomia. She was diagnosed as having primary Sjögren's syndrome according to the criteria proposed by the European Community study group in 1993. Furthermore, a lower lip salivary gland biopsy showed ...
Streckfus C - - 2001
Sjögren's syndrome is an autoimmune disorder which causes diminished salivary flow due to autoimmune sialoadenitis. This decrease in saliva flow is the result of inflammation and atrophy of the salivary glands. Most treatment regimens are palliative in nature, but treatment with interferon (IFN) holds promise for Sjögren's syndrome sufferers. Several ...
Urban P P - - 2001
Isolated cranial nerve involvement in primary Sjögren's syndrome (primary SS) has rarely been described. We report the case of a patient with sensory neuropathy of the trigeminal and also the glossopharyngeal and vagal nerves, which has not been identified previously. The electrophysiological findings in our patient with primary SS confirmed ...
Wada M - - 2001
Sjögren's syndrome occurs as an occasional complication of autoimmune hepatitis, and purpura or thrombocytopenia develops in some patients with this syndrome. This report describes a 62-year-old woman with a 6-year history of autoimmune hepatitis who concurrently had hypergammaglobulinemic purpura, immune thrombocytopenia and Sjögren's syndrome. Treatment with prednisolone resulted in marked ...
Tsubota K - - 2001
Dry eyes and dry mouth are clinical hallmarks of Sjögren's syndrome. We assessed the distribution of aquaporin-5 (AQP5) in lacrimal gland biopsy samples. Healthy controls and patients with Mikulicz's disease or non-Sjögren's syndrome dry eye had the expected apical distribution of AQP5 in lacrimal acinar cells. By contrast, cytoplasmic AQP5 ...
Patel S - - 2001
The signs and symptoms of diffuse infiltrative CD8 lymphocytosis syndrome (DILS), a subset of HIV, include parotid swelling, cervical lymphadenopathy and a serologic CD8 elevation. A case report is used to illustrate the condition. Patients with the syndrome will be seen in the dental office. Recognition and appropriate referral are ...
Flaitz C M - - 2001
Parotid swelling may be associated with a variety of glandular disorders in children. This case report describes the characteristic features of juvenile Sjögren's syndrome in an adolescent girl who presented with recurrent and bilateral parotid gland enlargement. Special emphasis is placed on an age-specific differential diagnosis for major salivary gland ...
Tonami H - - 2001
PURPOSE: The purpose of this work was to compare the diagnostic accuracy of MR sialography with that of salivary gland scintigraphy in Sjögren syndrome. METHOD: One hundred thirty patients clinically suspected of having Sjögren syndrome were examined by MR sialography and salivary gland scintigraphy. A labial gland biopsy was performed ...
Kalk, Wouter Warner Iwe
This thesis comprehends seven clinical studies related to the oral component of Sjögren’s syndrom (SS), aiming to improve current diganotics and to obtain clinical outcome parameters.
Manthorpe R - - 2001
Primary Sjögren's syndrome seems to be the most common of the chronic systemic inflammatory connective tissue diseases, according to epidemiological investigations. Basic and clinical research, including treatment within this group, is increasing while simultaneously there is increasing confusion and uncertainty about the classification criteria for Sjögren's syndrome. Within the last ...
Fujimoto T - - 2001
We report a case of hypokalaemic, flaccid quadriparesis with sudden respiratory arrest in a 27-year-old Japanese woman in whom the discovery of distal renal tubular acidosis led to the diagnosis of primary Sjögren's syndrome (SS). Hypokalaemic periodic paralysis as the initial manifestation of primary SS is rare, but when it ...
Hamburger J - - 2001
This article provides a brief overview of the various orofacial presentations of Sjögren's syndrome. The paper summarises the clinical features of patients with xerostomia, and alludes to differential diagnosis, investigative procedures and therapeutic modalities for the oral component of Sjögren's syndrome.
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