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Results for “SJOGREN'S SYNDROME”

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At least 19 recordsLinked to original sources
PubMed1980

Granulocytopenia with marked lymphocytosis manifesting Sjogren syndrome.

Sjogren syndrome is a multi-system disease leading to diverse organ involvement during its course [1, 2]. Hematologic abnormalities described in Sjogren syndrome include anemia, mild leukopenia [3, 4], eosinophilia, elevated erythrocyte sedimentation rate, hypergammaglobulinemia, mixed cryoglobulinemia, and a variety of autoantibodies [5]. Marked lymphocytosis with granulocytopenia is distinctly unusual and has not been previously reported. We report a case of Sjogren syndrome who presented with constellation of the latter problems without prominent sicca manifestations.

Agranulocytosis↗
PubMed1984

Primary Sjogren syndrome: clinical and immunopathologic features.

Primary Sjogren syndrome is an autoimmune condition in which dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) result from lymphocytic infiltration of lacrimal and salivary glands. Clinical and laboratory features of 60 primary Sjogren syndrome patients seen at our clinic during the past three years are presented. These patients illustrate the wide spectrum of extraglandular features that may occur as a result of lymphoid infiltration of lung, kidney, skin, stomach, liver, and muscle. They further emphasize the difficulty in classifying a patient as primary or secondary Sjogren syndrome (ie, sicca symptoms associated with systemic lupus erythematosus, rheumatoid arthritis, or scleroderma), particularly early in the disease course. As an initial step in understanding the pathogenesis, the lymphocytes that infiltrate the salivary glands and lymph nodes were characterized by using monoclonal antibodies that recognize distinct lymphocyte subsets and by using in vitro functional assays. These studies have demonstrated that affected tissues have infiltrates of T cells with helper/inducer activity and with a high frequency of "activation antigens." The immunohistologic techniques are useful in differentiating "benign" and "pseudolymphoma" lesions (both due predominantly to T cells) from non-Hodgkin lymphoma (usually due to B-cell infiltrates). Although there is no "cure" for primary Sjogren syndrome patient's symptoms may be significantly improved by measures aimed at prevention of ocular and dental complications and by the recognition of extraglandular features that may be amenable to specific treatment.

Adolescent↗
PubMed2005

[Observation on therapeutic effect of acupuncture and moxibustion in 60 cases of Sjogren syndrome].

OBJECTIVE: To observe clinical therapeutic effect of acupuncture on Sjogren syndrome. METHODS: Treatment group (n=60) were treated with acupuncture of clearing away dryness and toxic substance and removing obstruction in collaterals at Quze (PC 3), Taichong (LR 3), Xuehai (SP 10), Sanyinjiao (SP 6) and Taixi (KI 3), and the control group (n=60) with prednisone. Their therapeutic effects were compared. RESULTS: The total effective rate was 73.3% in the treatment group and 56.7% in the control group with a significant difference between the two groups (P < 0.05); there was no reverse effect in the treatment group. CONCLUSION: The needling method for clearing dryness and removing obstruction in collaterals is effective and safe for treatment of Sjogren syndrome.

Acupuncture↗
PubMed1989

Sjogren syndrome in childhood: report of two cases.

A 12-year-old boy who developed primary Sjogren syndrome and a girl, whose diagnosis of secondary Sjogren syndrome was established at the age of 3, are reported. The importance of some unusual manifestations at disease onset and possible differences in expression between children and adults are briefly discussed.

Age Factors↗
PubMed2003

Sjogrens syndrome as failed local immunohomeostasis: prospects for cell-based therapy.

Sjogrens syndrome has been estimated to affect between 0.2% and 2% or more of the population. It is an autoimmune disease with the hallmark histopathology of focal, periductal, and perivascular CD4(+) cell infiltration of the lacrimal and salivary glands. The immunohistopathology is typically associated with severe lacrimal and salivary dysfunctions, which contribute to debilitating ocular surface and oral symptoms. The quality of life of patients with Sjogrens syndrome often is degraded further by serious, multisystemic manifestations, and they are subject to a forty-fold increased risk of developing B cell lymphomas. In normal lacrimal glands, secretory epithelial cells, autoimmune effector lymphocytes, and regulatory lymphocytes can be seen as collaborating to maintain a local immunohomeostasis. The epithelium contributes by secreting immunomodulatory paracrine factors and also by continuously exposing autoantigens, which thereby become available for uptake by professional antigen presenting cells (APCs). Local or systemic perturbations may initiate autoimmune pathophysiology by impairing the replacement of normally-turning-over regulatory cells, by altering epithelial production of immunomodulatory paracrine factors, by inducing intact epithelial cells to begin secreting previously cryptic epitopes (epitopes that previously were not available to bind to major histocompatibility complex (MHC) molecules and so could not be recognized by T cell antigen receptors), and by inducing epithelial cells to begin expressing MHC Class II molecules and presenting formerly cryptic epitopes directly to CD4(+) cells. This process has been modeled ex vivo with mixed cell reactions comprised of isolated epithelial cells and autologous lymphocytes. This development has occurred as studies of anterior chamber-associated immune deviation (ACAID) and other immunoregulatory phenomena have elucidated the origins and functions of several different kinds of regulatory lymphocytes and shown that regulatory lymphocytes can be generated ex vivo. It now is possible to envision strategies for exploiting each possible mode of epithelial autoantigen exposure to produce therapeutic regulatory cells that might be capable of re-establishing normal immunohomeostasis. Consideration of the hypothetical therapies identifies a number of basic questions that warrant investigation.

Journal Article↗
PubMed1992

[The manifestations of the nervous system in primary Sjogren syndrome].

We reported 9 cases of primary Sjogren syndrome (SS) who were complicated with nervous system involvement. All were women. Age between 24 to 58 years old. Their clinical symptoms of the nervous system varied widely, 7 of them manifested with the involvement of central nervous system, the main features were in case 1 bouts of seizures, case 2 multi-level damage of the brain and spinal cord which was similar to multiple sclerosis, case 3 recurrent hemiplegia caused by cerebral thrombosis then fatal vascular hemorrhage, case 4 sudden hemiplegia, case 5 persistent psychological disturbance, case 6 cranial neuropathy of V and VII, case 9 multi-focal symptoms of ataxia, myelopathy and transient blindness. 5 of the 9, case 3, 6-9 all appeared with the symptoms of peripheral neuropathy, case 7 also complicated with carpal tunnel syndrome. 4 of the 9 cases also complicated with renal tubular acidosis and/or chronic active hepatitis and/or fibrosing alveolitis and/or thrombocytopenic purpura and/or myositis. No differences of the positivity of autoantibodies was observed between those with or without nervous system involvement.

Adult↗
PubMed1979

Sjogren syndrome presenting as pulmonary pseudolymphoma: report of a case.

A case of pulmonary pseudolymphoma and Sjogren syndrome is presented. Unusually, the lung involvement preceded the salivary disease by more than two years. The initial gammopathy and abnormal serologies are more consistent with uncomplicated Sjogren syndrome, but were present when the pseudolymphoma was solely apparent.

Adult↗
PubMed1992

Sjogren syndrome: comparison of sialography and ultrasonography.

Ultrasonography and sialography of parotid glands were prospectively performed on 30 patients with Sjogren syndrome and follow-up ultrasonography was done on 12 patients. Ultrasonography revealed multiple hypoechoic areas (MHA) 2 mm to 5 mm in diameter within the glands in 43% of the patients with intermediate severity of the disease, either homogeneous or slightly nonhomogeneous parotid glands in patients with early stage of the disease, and marked nonhomogeneous glands in patients with advanced diseases. Follow-up ultrasonography showed MHA in one patient, reduction in nonhomogeneity in another three, and no interval changes in the remaining cases. Pathologic studies suggested that MHA represent enlarged parotid lobules replaced by lymphocytic infiltration. We suggest that MHA are characteristic of Sjogren syndrome and that ultrasonography is useful in following the course of the disease and response to treatment.

Female↗
PubMed2004

Central nervous system disease in primary Sjogrens syndrome: the role of magnetic resonance imaging.

OBJECTIVES: To examine the frequency of central nervous system (CNS) disease in primary Sjogrens syndrome (pSS) and indicate ways in which cerebral magnetic resonance imaging (MRI) may help determine the significance of CNS involvement. METHODS: The current review was based on a Medline (Pubmed) literature search through May 2003, focused on Sjogrens syndrome, other vasculitides, multiple sclerosis (MS), specific MRI techniques, and MRI findings with regard to the above-mentioned diseases. Additional literature was identified in the reference sections of articles listed in Medline. RESULTS: Severe CNS manifestations reminiscent of MS have been described in pSS patients. Moreover, the prevalence of nonfocal neuropsychological abnormalities has been found to be elevated in some pSS patient populations. MRI studies suggest discrete cerebral tissue damage even in neurologically asymptomatic patients. However, small white matter lesions are nonspecific and may be related to age or cerebrovascular risk factors such as hypertension. A large controlled study, complementing established T2-weighted MRI with fluid-attenuated inversion recovery (FLAIR) to achieve high sensitivity in lesion detection, could indicate the disease specificity of white matter lesions in pSS. Newer MR techniques, such as spectroscopy and magnetization transfer imaging, applied, for example, in MS and systemic lupus erythematosus (SLE) to evaluate CNS tissue injury, could help determine the extent and mechanisms of macroscopic and microscopic CNS lesions in pSS. CONCLUSIONS: Future controlled studies will be necessary to more precisely estimate the prevalence of CNS lesions in pSS, specifically of discrete white matter abnormalities. Newer MRI techniques have the potential to provide information on the severity and pathophysiological mechanisms of CNS tissue damage.

Brain↗
PubMed1992

Lymphoma in Sjogren's syndrome.

Sjogren's syndrome is an autoimmune disease with a known predisposition for lymphoma development. Eight of 120 patients with primary Sjogren's syndrome followed at the University of Ioannina over the past 7 years developed non-Hodgkin's lymphoma diagnosed according to the Kiel classification. The lymphomas differed by location and grading. Six were called low grade (immunocytoma) and two intermediate grade non-Hodgkin's lymphomas. Five of the immunocytomas involved the minor salivary or lacrimal glands. Immunoperoxidase staining for light chains revealed monoclonal populations. Two patients showed spontaneous regression not previously reported in Sjogren's syndrome. Thus, in Sjogren's syndrome, low grade non-Hodgkin's lymphomas and especially immunocytomas are the most common lymphomas. These lymphomas tend to evolve very slowly and may regress spontaneously. Given these facts, a conservative approach to treatment is indicated in those patients with only localized disease.

Adult↗
PubMed1987

Myopathy in Marinesco-Sjogren syndrome.

Progressive muscular weakness, hypotonia and atrophy are among the cardinal signs of the Marinesco-Sjogren syndrome but have not been extensively investigated. Our study focused on 6 related patients who are members of an inbred population. Muscle biopsies revealed myopathic alterations with variation of fiber size, rounding, degeneration and regeneration of fibers, internalization of nuclei and endomysial fat and fibrosis. Most patients had elevated serum creatine kinase levels. One patient revealed endstage neuromuscular disease and had normal serum creatine kinase levels. Of particular interest was the finding of conspicuous myopathy in 2 young children. Thus far, it has not been appreciated that myopathy represents an early sign of the Marinesco-Sjogren syndrome.

Adult↗
PubMed1983

High incidence of free monoclonal lambda light chains in the sera of patients with Sjogren's syndrome.

Sjogren's syndrome presents a wide spectrum of disease manifestations ranging from benign to malignant lymphoproliferation. Sera from 21 patients with primary Sjogren's syndrome, 63 patients with other autoimmune rheumatic diseases, and 140 normal controls were studied by using high-resolution gel electrophoresis combined with immunofixation and specific absorption studies. Two-thirds of the sera from Sjogren's syndrome patients contained free monoclonal lambda light chains. In addition, one patient with Sjogren's syndrome and pseudolymphoma had a circulating monoclonal IgM-lambda immunoglobulin. In contrast, only 16% of the patients with other autoimmune diseases and 5% of normals had monoclonal bands; none of the other patients studied exhibited monoclonal-free lambda light chains in their serum. Our findings suggest that the monoclonal process in Sjogren's syndrome starts early in the disease process in a subset of B cells.

Antibodies, Monoclonal↗