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Rigante Donato - - 2008
The aberrant induction of salivary/lacrimal proteins is considered to be crucial in the pathogenesis of sicca-symptoms related to primary Sjögren syndrome (SS). We report the case of an 11-year-old boy who was admitted to hospital due to recurrent bilateral parotid gland enlargement and keratoconjunctivitis, which were diagnosed as primary SS ...
Sahlin Sven - - 2008
The injection of botulinum toxin into the medial lower eyelid causes a local paralysis of the orbicularis oculi muscle. The paralysis leads to a decreased action of the lacrimal pump and an improved lubrication of the ocular surface. The injection reduces the discomfort in eyes of patients with Sjögren's syndrome.
Lima Isabella - - 2008
Sjögren's syndrome (SS) is a chronic autoimmune disease that is characterized by lymphocytic infiltration of the exocrine glands, mainly the salivary and lachrymal glands, usually manifesting with xerostomia and xerophthalmia. Around 50% of patients with primary SS develop systemic complications, lymphoma being the most feared of these. The majority of ...
Kobayashi Hideo - - 2008
A 53-year-old woman reported having a persistent cough and bloody sputum. She did not smoke but had received a diagnosis of Sjögren's syndrome. Chest CT revealed middle lobe syndrome, bronchiectasis and diffuse centrilobular nodular lesions. Bronchoscopy displayed multiple whitish polypoid lesions protruding from the cartilage rings and tracheobronchopathia osteochondroplastica was ...
Yaguchi Hiroaki - - 2008
OBJECTIVE: This study was to clarify the neuropathological findings of acute encephalomyelopathy with Sjögren's syndrome. METHODS: We examined an autopsied case of acute encephalomyelopathy with Sjögren's syndrome. CASE REPORT: A 40-year-old woman developed acute myelopathy and brainstem dysfunction. Magnetic resonance imaging (MRI) revealed high-intensity lesions on T2-weighted axial images (T2WI) ...
Weber Juliane - - 2008
Cevimeline is an orally administered muscarinic receptor agonist that is indicated for the treatment of symptoms of dry mouth in patients with Sjogren's syndrome. Several well designed placebo-controlled trials demonstrated that 4-12 weeks' therapy with cevimeline 30 mg three times daily improved symptoms of dry mouth in patients with Sjogren's ...
Teshigawara Katsunobu - - 2008
Sjogren's syndrome can cause many organic changes, but is rarely accompanied by pleuritis. We report a 65-year-old patient with primary Sjogren's syndrome who developed bilateral pleuritis with moderately large effusions. He was diagnosed as having Sjogren's syndrome, based on xerophthalmia, xerostomia, positive results for anti-Sjogren's syndrome (anti-SS-A/SS-B) antibodies, the Schirmer ...
Kinoshita Naoe - - 2008
A 68-year-old Japanese man with Sjögren's syndrome was pointed out to have a nodular shadow in the anterior mediastinum. The tumor was resected under video-assisted thoracoscopy and it was pathologically diagnosed to be thymic mucosa-associated lymphoid tissue (MALT) lymphoma. There has been no recurrence for 17 months after surgery. Mucosa-associated ...
Rashtak Shadi - - 2008
Autoimmune diseases present with varied and broad-ranging cutaneous manifestations. Connective tissue disorders have a plethora of skin manifestations such as rheumatoid nodules in rheumatoid arthritis, psoriatic plaques in psoriatic arthritis, acne and pustulosis in SAPHO syndrome, livedo reticularis and ulceration in antiphospholipid antibody syndrome and xerosis in Sjögren syndrome. Cutaneous ...
Assimakopoulos Stelios F - - 2007
Patients with primary Sjögren syndrome frequently present hematologic abnormalities, consisting mainly of immune cytopenias. Pure red cell aplasia is a very rare complication of primary Sjögren syndrome. This is the first report in the literature describing the development of pure red cell aplasia combined with autoimmune hemolytic anemia in a ...
Giusti Laura - - 2007
In the last few years, a growing interest has arisen in the application of proteomic analysis to rheumatic disease. Sjögren's syndrome is a systemic disease that affects exocrine glands directly, and is therefore expected to influence the composition of the whole human saliva and lachrymal fluid. Therefore, a rising number ...
Fernandes Merle - - 2007
A 21-year-old febrile lady presented to the emergency service with severe lid oedema, conjunctivitis, dry mouth and abdominal skin rash. Over 5 days, she developed silvery scales and pustules on the lids, and generalized pustules on an erythematous base. Multiple focal sterile corneal infiltrates were seen. Haematological investigations and a ...
Migita Kiyoshi - - 2008
Familial Mediterranean fever (FMF) is a hereditary syndrome characterized by recurrent episodes of fever and serositis. In this report, we describe a Japanese patient with FMF and Sjögren's syndrome, in whom acute elevations of transaminase occurred. The histological findings from the liver biopsy specimens demonstrated a nonspecific hepatitis, with liver ...
Laroche Michel - - 2007
OBJECTIVE: Since the results of the ATAC study, women who have undergone surgery for breast cancer and who require adjuvant hormone therapy are often treated with aromatase inhibitors. With these treatments, joint pain is frequent (30% to 40%) and quite often disabling (5% to 10%). Our objective was to investigate ...
Hyon Joon Young - - 2007
PURPOSE: To evaluate the clinical efficacy of anti-inflammatory therapy in the management of primary Sjögren syndrome. METHODS: Thirty-eight patients with primary Sjögren syndrome were included in this study. The diagnosis of Sjögren syndrome was made on the basis of the classification criteria of the American-European Consensus Group. Fluorescein staining score, ...
Coaccioli S - - 2007
The Sjogren's syndrome (SS) is an chronic inflammatory autoimmune disease of the exocrine glands as well as of internal apparatus. The therapy of exocrinopathy is represented by parasympathomimetic drugs such as pilocarpine and cevimeline. The therapy of systemic manifestations, actually is represented by the inhibitors of TNF alfa, as well ...
Coha Bozena - - 2007
Relapsing polychondritis is an autoimmune disease that primarily manifests as cartilaginous tissue destruction. However, the immune impairment may also involve noncartilaginous tissues such as kidneys, blood vessels, etc. The disease may occur as a primary disorder or in association with other diseases. The case of a female patient with concurrent ...
Soyfoo M S - - 2007
Sjögren's syndrome (SS) is an autoimmune disorder characterized by ocular and oral dryness as well as systemic manifestations. The immunopathogenesis of SS is complex with different intricate factors. Because of the delay in the appearance of symptoms and due to ethical issues it is very difficult to study the wide ...
Srinivasan Sathish - - 2007
Sjögren syndrome is a chronic autoimmune disorder of the exocrine glands with associated lymphocytic infiltration of the affected glands. The exocrinopathy can be encountered alone (primary Sjögren syndrome or in association with other autoimmune disorders, the three most common ones being rheumatoid arthritis, systemic lupus erythematosus, and progressive systemic sclerosis ...
Natsuga Ken - - 2007
Amicrobial pustulosis is a rare clinical entity characterized by a relapsing pustular eruption, primarily involving the skin folds. We describe a case of amicrobial pustulosis associated with autoimmune diseases (APAD). The patient suffered from IgA nephropathy and Sjögren's syndrome. Skin symptoms were alleviated dramatically after corticosteroid pulse therapy and tonsillectomy.
Jonsson Roland - - 2007
Primary Sjögren's syndrome is a complex autoimmune disorder, considered to represent an ideal disease with which to study the mechanisms underlying autoimmunity because its manifestations are both organ specific and systemic in nature. The characteristic histologic finding in target organs is a progressive focal infiltration of mononuclear lymphoid cells, replacing ...
Porola Pauliina - - 2007
Sjögren's syndrome is an autoimmune disease affecting the exocrine glands, most typically salivary and lacrimal glands. In Sjögren's syndrome, the acinar cells of these glands are damaged and destroyed, leading to diminished secretion of saliva and tear fluid. Accordingly, the current American-European criteria of Sjögren's syndrome include xerostomia (dry mouth) ...
Williams Pamela H - - 2007
Sjögren's syndrome (SS) is a complex polygenic autoimmune disorder. A few major genetic effects have been identified. Historically, HLA and non-HLA genetic associations have been reported. Recently, the HLA region continued to reveal association findings. A new susceptibility region has been suggested by a study of a D6S349 microsatellite marker. ...
Adam Alexander C - - 2007
Jo-1 syndrome is an autoimmune disease with autoantibodies against the histidyl tRNA synthetase. Characteristic clinical findings include inflammatory myopathy and interstitial lung disease. We present the first case of a patient with Jo-1 syndrome (positive Jo-1 autoantibodies, myositis, interstitial alveolitis) who developed Hodgkin's lymphoma of nodular-sclerosing type. Thus, patients with ...
Civilibal Mahmut - - 2007
Primary Sjögren syndrome (pSS) is an uncommon disease in childhood. Childhood pSS might have different clinical manifestations than adult pSS. We describe a 13-year-old girl with multiple episodes of bilateral parotid swelling lasting 2 years. Her history included severe arthralgia, local edema, and purpura episodes since 9 years of age. ...
Pournaras J-A C - - 2007
BACKGROUND: Neuro-ophthalmic findings are uncommon in the setting of Sjögren syndrome. We report the case of a patient with bilateral, sequential optic neuropathy as the initial manifestation of Sjögren syndrome. HISTORY AND SIGNS: A 38-year-old male presented with sudden painless visual loss in his left eye in May 2005. Fundus ...
Yurtseven Nuran - - 2007
OBJECTIVE: To compare oral sugar clearance, salivary flow rate, buffer capacity, salivary counts of mutans streptococci and lactobacilli in whole saliva, and root caries in subjects with secondary Sjögren syndrome with those of healthy subjects. METHOD AND MATERIALS: Twenty subjects with secondary Sjögren syndrome and 20 healthy subjects (age 30 ...
Wilson C - - 2007
We report the first case of Sjögren's syndrome causing gross lacrimal gland swelling and secondary hypoglobus and uncharacteristically absent sicca syndrome. Histopathological diagnosis of the excision biopsy of the orbital lobe of the gland confirmed a mixed T and B cell infiltrate of the gland with no lymphomatous transformation. The ...
Fox Philip C - - 2007
Autoimmune diseases include a diverse group of over 80 conditions. Sjögren's syndrome is the second most common autoimmune rheumatic disease, with an estimated prevalence in the United States of 2-4 million persons. There are prominent and consistent oral and dental findings in Sjögren's syndrome related to the autoimmune-mediated loss of ...
Daridon Capucine - - 2007
Analysis of salivary glands of patients with primary Sjögren's syndrome has yielded conflicting results with respect to T helper (Th)1/Th2 polarization. This balance might parallel the progress of the local lesions. B-cells are now taking center stage in this disease. They can also be primed to differentiate into two cytokine-production ...
Konstantinidis I - - 2007
The presence of multiple calculi in the major salivary glands is an uncommon finding. Sjögren's syndrome is a chronic autoimmune disease characterized by lymphocyte-mediated destruction of the exocrine glands. The case is presented of a 49-year-old female with Sjogren's syndrome found to have bilateral multiple sialolithiasis in the parenchyma of ...
Miyachi Kiyomitsu - - 2007
IP(3)R2 and IP(3)R3 double knock-out mice present with exocrine dysfunctions such as secretion deficits of saliva and pancreatic juice. Therefore, we investigated whether the presence of antibodies to IP(3)Rs could be found in patients with Sjögren's syndrome, and the location of the epitopes. Subjects included 35 primary Sjögren's syndrome, 39 ...
Gökçay Figen - - 2007
This report presents a patient with Devics neuromyelitis optica associated with primary Sjögrens syndrome. Her first attack was right-sided optic neuritis at age 10 years. Attacks involving both optic nerves and medulla spinalis were recorded during the ensuing years. The diagnosis of Sjögrens syndrome could not be made until the ...
Iwanaga Nozomi - - 2007
The most common renal manifestation of Sjögren's syndrome is tubulointerstitial nephritis, and glomerular disease is rare (3). A 62-year-old woman with primary Sjögren's syndrome developed nephrotic syndrome. Kidney biopsy was consistent with membraneous glomerulonephritis. Steroid pulse therapy was not effective. Three months later she was diagnosed with non-Hodgkin's lymphoma of ...
Tamme Tiia - - 2007
We report the case of a 22-year-old woman who is suspected of having primary Sjögren s syndrome. She complaining of bilateral swelling of eyelids and the parotid glands of three weeks duration. Physical examination revealed a bilateral enlargement of both parotid glands, which were solid and painful. Sjögren s syndrome ...
Sunada Koichi - - 2007
Mucosa-associated lymphoid tissue lymphoma (MALToma) has been reported in several organs. Among MALTomas, thymic and pulmonary MALTomas are rare. The present report describes a patient with Sjögren's syndrome who presented thymic and pulmonary MALTomas. Although the exact pathogenetic relationship between these two tumours is uncertain, it is likely that the ...
Fujita Yoshimasa - - 2007
We report a patient with Sjögren's syndrome and chronic natural killer lymphocytosis, who developed severe neutropenia, autoimmune hemolytic anemia, and immune thrombocytopenia. High-dose prednisolone therapy improved the hemolytic anemia and thrombocytopenia, but not the CD16(+) CD56(-) NK lymphocytosis completely. Interestingly, indomethacin farnesil (a prodrug of indomethacin) was effective for myalgia ...
Panchovska M - - 2007
The aim of the present study was to define more accurately the necessary health care in patients with the primary Sjogren's syndrome and to compare it to those necessary for patients with another autoimmune disease--rheumatoid arthritis without a concomitant Sjogren's syndrome (Tab. 1, Ref. 5). Full Text (Free, PDF) www.bmj.sk.
Onat Ahmet Mesut - - 2007
Triple A syndrome is a rare autosomal recessive disorder characterized by alacrima, achalasia, and adrenal insufficiency. Sjögren's syndrome (SS) is a chronic inflammatory disorder manifested primarily by diminished lacrimal and salivary gland secretions, resulting in symptoms of dry eyes and dry mouth, the so-called "sicca complex". However, a variety of ...
Kocijančič, Ksenija
A rare case of a young nonsmoker woman with Sjögren’s syndrome and salivary gland non-Hodgkin’s lymphoma, diagnosed one year later, is presented. Three years after treatment of the lymphoma, asymptomatic progression of the Sjögren’s syndrome was observed with pulmonary involvement – predominantly bullous or cystic lung disease. To our knowledge, ...
Kocijancic Ksenija - - 2006
A rare case of a young nonsmoker woman with Sjigren's syndrome and salivary gland non-Hodgkin's lymphoma, diagnosed one year later, is presented. Three years after treatment of the lymphoma, asymptomatic progression of the Sjögren 's syndrome was observed with pulmonary involvement--predominantly bullous or cystic lung disease. To our knowledge, this ...
Sughimoto Koichi - - 2006
Sjögren's syndrome is one of the major autoimmune diseases, however infective endocarditis associated with Sjögren's syndrome has not previously been reported. A patient with Sjögren's syndrome associated with aortic valve regurgitation due to infective endocarditis, underwent successful aortic valve replacement. Patients with Sjögren's syndrome tend to be at a higher ...
Pirgon Ozgur - - 2006
Sjögren-Larsson syndrome is a rare hereditary metabolic disorder characterized by congenital ichthyosis, mental retardation, and spastic diplegia or tetraplegia. This genetic disease is caused by fatty acid aldehyde dehydrogenase deficiency, leading to an accumulation of long-chain alcohols. The role of enzyme in the degradation of leukotrienes paved the way to ...
Yang Yi-Sun - - 2007
Sjögren's syndrome is an autoimmune disorder involving exocrine glands that occurs alone or in association with various autoimmune and connective tissue diseases. The severity of Sjögren's syndrome ranges from isolated sicca syndrome to severe complications such as vasculitis, lung and renal involvement. Overt or latent renal tubular acidosis caused by ...
García-Carrasco Mario - - 2006
The term Sjögren's syndrome refers to keratoconjunctivitis sicca and xerostomia due to lymphocytic infiltrates of lachrymal and salivary glands. The current used criteria for diagnosis of primary Sjögren's syndrome is the American-European consensus. Primary Sjögren's syndrome is an autoimmune disorder characterized by lymphocytic infiltrates and destruction of the salivary and ...
Migliore Alberto - - 2006
Multiple chemical sensitivity (MCS) is defined by multiple symptoms, affecting multiple organs, that wax and wane in response to varying chemical exposures at or below previously tolerated levels. Sjögren's syndrome (SS) is a common autoimmune disease affecting 3% of women aged over 55 years. Except for keratoconjunctivitis sicca (which is ...
Bachrach G - - 2006
Antimicrobial peptides play an important role in the innate immune response. Deficiency in salivary LL-37 antimicrobial peptide has been implicated in periodontitis in patients with morbus Kostman syndrome. Down syndrome is associated with periodontitis, diminished salivary flow, and salivary immunoglobulin deficiency. In the present study, levels of LL-37 and its ...
Scofield R Hal - - 2007
Autoimmune thyroid disease is common in systemic lupus erythematosus (SLE). About 20% of patients with SLE have secondary Sjögren's syndrome. Families with more than one patient with SLE were identified. All patients met the revised classification criteria, although SLE-unaffected relatives were confirmed not to satisfy these criteria. Diagnosis of autoimmune ...
Lodde B M - - 2006
Sjögren's syndrome is an autoimmune exocrinopathy, mainly affecting the lacrimal and salivary glands, and resulting in ocular and oral dryness (keratoconjunctivitis sicca and xerostomia). The aetiology and pathogenesis are largely unknown, and only palliative treatment is currently available. Data obtained from experimental animal and human studies using biological agents or ...
Alió Alessandra B - - 2006
Sjögren-Larsson syndrome (SLS) is an autosomal recessive neurocutaneous disorder most commonly seen in the Scandinavian population and characterized by congenital ichthyosis, mental retardation, and spastic diplegia or quadriplegia. We report a case of SLS in an 11-month-old girl of Lebanese and Mexican-Syrian ancestry who presented with ichthyosis, developmental delay, and ...
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