Cases reported "Sjogren's Syndrome"

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11/235. gastric mucosa as an additional extrahepatic localization of hepatitis c virus: viral detection in gastric low-grade lymphoma associated with autoimmune disease and in chronic gastritis.

    The hepatitis c virus (HCV) has been linked to B-cell lymphoproliferation and autoimmunity, and has been localized in several tissues. The clinical observation of an HCV-infected patient with sjogren's syndrome (SS) and helicobacter pylori (HP) positive gastric low-grade B-cell non-Hodgkin's lymphoma (NHL), which did not regress after HP eradication, led us to investigate the possible localization of HVC in the gastric microenvironment. HCV genome and antigens were searched in gastric biopsy specimens from the previously mentioned case, as well as from 9 additional HCV-infected patients (8 with chronic gastritis and 1 with gastric low-grade B-cell NHL). HCV-specific polymerase chain reaction (PCR) and immunohistochemistry procedures were used. The gastric B-cell NHL from the patient with SS was characterized by molecular analyses of B-cell clonality. HCV rna was detected in both the gastric low-grade B-cell NHL and in 3 out of 6 gastric samples from the remaining cases. HCV antigens were detected in the residual glandular cells within the gastric B-cell NHL lesions, in glandular cells from 2 of the 3 additional gastric lesions that were HCV positive by PCR, and in 1 additional chronic gastritis sample in which HCV-rna studies could not be performed. By molecular analyses, of immunoglobulin genes, the B-cell NHL from the patient with SS was confirmed to be a primary gastric lymphoma, subjected to ongoing antigenic stimulation and showing a significant similarity with rheumatoid factor (RF) and anti-HCV- antibody sequences. Our results show that HCV can localize in the gastric mucosa.
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12/235. Cholinergic-drug induced sicca syndrome in Parkinson's disease: a case report and a review of the literature.

    A 67-year-old woman developed severe sicca manifestations after initial treatment of Parkinson's disease with an anti-cholinergic drug, which prompted us to look for the presence of sjogren's syndrome. The results of sialography, labial salivary gland biopsy, Rose-Bengal test as well as the presence of antinuclear antibody were consistent with the diagnosis of sjogren's syndrome. The sicca symptoms diminished by cessation of the anti-cholinergic drug, and the parkinsonian features were controlled by levodopa. We suggest that sjogren's syndrome should be considered, if patients with Parkinson's disease complain severe xerostomia.
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13/235. Recognition and management of sjogren's syndrome: strategies for the advanced practice nurse.

    sjogren's syndrome is an autoimmune disorder characterized by lymphocytic infiltration of salivary and lacrimal glands. The systemic production of autoantibodies leads to dry eyes, dry mouth, and other symptoms related to decreased salivary and lacrimal gland function in patients with sjogren's syndrome. This article discusses origins, epidemiology, contributing factors, symptoms, assessment, diagnostic studies, management, expected outcomes, and research concerning sjogren's syndrome.
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14/235. sjogren's syndrome: comparison of assessments with quantitative salivary gland scintigraphy and contrast sialography.

    This study compared the quantitative parameters of salivary gland scintigraphy and the sialographic stages in patients with sjogren's syndrome. methods: One hundred sixteen patients suspected of having sjogren's syndrome were examined with salivary gland scintigraphy and contrast sialography. When contrast sialography was used as the gold standard, sjogren's syndrome was diagnosed in 50 of these 116 patients; sjogren's syndrome was not seen in the other 66 patients. After injection of 370 MBq 99mTc-sodium pertechnetate, dynamic salivary gland scintigraphy with lemon juice stimulation was performed for 50 min. Functional parameters for the parotid and submandibular glands were calculated, and scintigraphic and sialographic results were compared. RESULTS: With the progression of sialographic stages from 0 to 4, the quantity of tracer accumulation decreased in the submandibular gland (P < 0.0001), and the quantity of tracer secretion decreased in the parotid gland (P < 0.0001). The sialographic stage in patients with sjogren's syndrome was correlated with these scintigraphic parameters (P < 0.0001): sialographic stage = 3.243 - 0.337 x (submandibular gland uptake ratio) - 0.026 x (parotid gland maximum secretion). CONCLUSION: The decreased accumulation in the submandibular gland and the decreased secretion in the parotid gland were highly sensitive indicators of salivary gland disease in sjogren's syndrome. The sialographic stage was correlated with these scintigraphic parameters.
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keywords = submandibular gland, gland, submandibular
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15/235. Oral swelling in sjogren's syndrome.

    A case is reported of a 69-year-old female with sjogren's syndrome who presented with a problem of interest to the oral surgeon. The clinical features and cryosurgical management of a rare cause of palatal swelling are described. The confusion that exists in the literature concerning the nomenclature of chronic inflammatory salivary gland lesions is discussed.
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16/235. Primary sjogren's syndrome manifested as multiple sclerosis and cutaneous erythematous lesions: a case report.

    sjogren's syndrome is a chronic autoimmune disorder characterized by lymphocytic infiltration of the lacrimal and salivary glands, leading to dryness of eyes (kerato-conjunctivitis sicca) and mouth (xerostomia). The skin lesions in sjogren's syndrome are usually manifested as xeroderma, but sometimes appear as annular erythema or vasculitis. central nervous system symptoms may be presented as one of extraglandular manifestations, though rare in incidence, and need differential diagnosis from multiple sclerosis. We report a case of a 45-year-old woman diagnosed as multiple sclerosis at first but later as neurologic manifestation of primary sjogren's syndrome, showing signs of multiple sclerosis and cutaneous erythematous lesions.
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17/235. Salivary gland lymphomas in patients with sjogren's syndrome may frequently develop from rheumatoid factor B cells.

    OBJECTIVE: patients with sjogren's syndrome (SS) have an increased risk of developing monoclonal B cell non-Hodgkin's lymphomas (MNHL), which frequently occur in the salivary glands (SG). The transition from the benign lymphocyte infiltrate of the gland that characterizes SS to MNHL is not well understood. Previous sequence analyses of the expressed variable (V) region genes have supported the theory that the surface Ig (sIg) plays an important role in the initial expansion of nonmalignant B cell clones and in lymphomagenesis. However, the antigenic specificities of these B cells were unknown. We describe the specificities of the Ig expressed by 2 cases of MNHL that developed in the SG of 2 patients with SS. methods: The expressed V genes were amplified by polymerase chain reaction from biopsy specimens, sequenced, and subcloned into eukaryotic expression vectors. The constructs were transfected into P3X63-Ag8.653 cells to obtain 2 monoclonal cell lines, each secreting 1 of the sIg expressed by the MNHL. These IgM were tested by enzyme-linked immunosorbent assay and immunofluorescence against a panel of antigens potentially implicated in SS. RESULTS: Our main finding was that the Ig products of the neoplastic B cells were rheumatoid factors (RF). Contrary to expectations, they did not react with nuclear or cytoplasmic antigens, double-stranded dna, self antigens commonly bound by natural autoantibodies, or SG tissue. CONCLUSION: Previous analyses of V gene use have provided indirect evidence that SG MNHL may frequently express RF. We demonstrate that this hypothesis is true in the 2 patients we studied. Large-scale studies will be needed to establish the exact frequency of RF specificity among SS-associated MNHL.
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18/235. sjogren's syndrome with primary biliary cirrhosis, complicated by transverse myelitis and malignant lymphoma.

    A 53-year-old woman with sjogren's syndrome (SS) and primary biliary cirrhosis (PBC) complicated by transverse myelitis (TM) and malignant lymphoma (ML) is reported. TM has been described only in seven cases of primary SS, including three with PBC and four without PBC. The features of SS associated with PBC and complicated by TM were less typical compared with those seen in SS without PBC complicated by TM. This case is the first report of a case with SS, PBC, TM and ML. SS in association with PBC is, in general, overlooked, but such cases must be investigated with great caution for extraglandular complications.
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19/235. Annular erythema of sjogren's syndrome in a white woman.

    Annular erythema of sjogren's syndrome (AE-SS) is believed to be the Asian counterpart of subacute cutaneous lupus erythematosus (SCLE) in white persons. We report the second case of a white person with AE-SS, diagnosed by clinical and serologic findings, as well as the absence of histologic criteria for SCLE. The diagnosis of AE-SS was established by symptoms of xerophthalmia and xerostomia, as well as by examination of skin and salivary gland biopsy specimens. Evaluation showed the presence of anti-Ro(SS-A) and anti-La(SS-B) autoantibody, with the presence of anti-Ro(SS-A) antibody against the 60-kd, but not the 52-kd, epitope, a pattern frequently seen in both the Asians with AE-SS and in white patients with SCLE. Both skin and sicca symptoms were alleviated with combination antimalarial therapy, which included hydroxychloroquine and quinacrine. This case demonstrates that AE-SS can occur in white patients and that the autoantibody profile is similar to that described in Asians with this disease.
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20/235. sjogren's syndrome with acute transverse myelopathy as the initial manifestation.

    This is the first report of a patient with acute transverse myelopathy as the initial manifestation of primary sjogren's syndrome (SS). The patient developed tetraparesis and sensory disturbance within 6 days of onset. Spinal MRI showed an extensive intraparenchymal lesion with high T2-weighted signal intensity, gadolinium enhancement, and cord swelling. Although the patient did not show clinical sicca symptoms, primary SS was diagnosed on the basis of clinical tests on lacrimal and salivary glands which showed high levels of autoantibodies. Treatment with prednisone improved motor and sensory symptoms and resulted in improvement of MRI findings. The present case suggests that acute transverse myelopathy can occur as an initial symptom of SS.
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