1/235. Analysis of V(H)-D-J(H) gene transcripts in B cells infiltrating the salivary glands and lymph node tissues of patients with sjogren's syndrome.OBJECTIVE: In patients with sjogren's syndrome (SS), B lymphocytes have been found to infiltrate salivary glands, resulting in sialadenitis and keratoconjunctivitis. The disease is frequently associated with benign and neoplastic lymphoproliferation. The present study was undertaken to investigate whether clonal B cell expansion takes place in lymphocytic infiltrations of salivary glands under (auto- [?]) antigen stimulation, by analyzing in more detail the variable part (V(H)-D-J(H)) of the immunoglobulin heavy chain genes expressed in these B cells. methods: Biopsies of the labial salivary glands and lymph nodes were performed on 2 female patients with SS. The Ig gene rearrangements in these tissues were amplified by reverse transcriptase-polymerase chain reaction using specific primers. RESULTS: A total of 94 V(H)-D-J(H) transcripts were cloned and sequenced. Our data suggest a polyclonal origin of the B cell infiltrates. In 92 of the transcripts, V(H) genes were modified by somatic mutation. Further analysis showed counterselection for replacement mutations within the framework regions, suggesting that those B cells were stimulated and selected for functional expression of a surface Ig. In labial salivary glands from both patients, clonally related B cells became evident. Members of 1 particular clone were found in both the lip and lymph node material. CONCLUSION: These data provide evidence, on the nucleotide sequence level, that an antigen-triggered clonal B cell expansion takes place in the salivary glands of patients with SS who do not have histologic evidence of developing lymphoma. It may be speculated that those B cell clones expand during disease progression, resulting in lymphomagenesis.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/235. Anaplastic large cell lymphoma associated with sjogren's syndrome.We report a case of a 20-year-old Japanese female with recurrent anaplastic large cell lymphoma (ALCL) associated with sjogren's syndrome (SjS). She was first diagnosed to have ALCL presenting with axillary lymphadenopathy, which within a month underwent spontaneous remission, at the age of 12 years. Eight years later she developed left inguinal lymphadenopathy with clinical overt sicca symptoms associated with elevated serum IgG, interleukin (IL)-1beta and IL-6 levels. Lymph node biopsy was now diagnostic of ALCL characterized by large pleomorphic CD30 blast cells with the specific chromosomal abnormality, t(2;5)(p23;q35). In contrast to this the salivary gland and renal biopsy revealed infiltration of small lymphocytes, morphologically and cytogenetically distinct from the ALCL cells. Interestingly, SjS symptomatology correlated with disease activity of ALCL and based on an association with elevated IgG and IL-6 levels, suggesting that the concurrence of these two diseases could be more than coincidental. To the best of our knowledge, this is the first reported case of ALCL presenting concurrently with SjS.- - - - - - - - - - ranking = 0.11111111111111keywords = gland (Clic here for more details about this article) |
3/235. Identification of a novel PTEN mutation (L139X) in a patient with Cowden disease and sjogren's syndrome.Cowden disease is an autosomal dominant disorder associated with an increased risk of breast, thyroid, and skin cancer in which germline mutations in a candidate tumour suppressor gene (PTEN) have been identified previously. sjogren's syndrome is a chronic inflammatory and autoimmune disorder of exocrine glands for which the genetic basis is unknown. This report describes a novel PTEN mutation (L139X) in a patient with Cowden disease and sjogren's syndrome. This observation raises the possibility of a link between mutations in the PTEN gene and sjogren's syndrome.- - - - - - - - - - ranking = 0.11111111111111keywords = gland (Clic here for more details about this article) |
4/235. Bilateral and multicystic major salivary gland disease: a rare presentation of primary sjogren's syndrome.We present a case of a 15-year-old girl with bilateral parotid and sub-mandibular salivary gland enlargement as the sole presentation of primary sjogren's syndrome. The clinical, radiological, immunological and pathological features have been discussed. The relevant literature has been reviewed. To our knowledge this is the only reported case of sjogren's syndrome presenting as multicystic disease with bilateral major salivary gland involvement.- - - - - - - - - - ranking = 0.66666666666667keywords = gland (Clic here for more details about this article) |
5/235. Increased serum CA 19-9 antibodies in sjogren's syndrome.A 67-year-old woman with a history of thyroiditis presented with recent intermittent epigastric pain and nausea. Hyperamylasaemia, oedema of the pancreas, and high serum levels of lipase and CA 19-9 were found. xerostomia and dry eyes developed later, accompanied by an abnormal Schirmer's test. The diagnosis of sjogren's syndrome was confirmed by increased anti-Ro and anti-La antibodies and the histological findings of parotid gland biopsy. Two additional cases of sjogren's syndrome with elevated serum CA 19-9 are also described. These observations of elevated serum lipase and serum CA 19-9 in sjogren's syndrome without evidence of malignancy may reflect pancreatic involvement in this disorder.- - - - - - - - - - ranking = 0.11111111111111keywords = gland (Clic here for more details about this article) |
6/235. The relationship between facial annular erythema and anti-SS-A/Ro antibodies in three East Asian women.A distinct annular erythema developed on the cheeks of three East Asian women who had anti-SS-A/Ro (SSA) antibodies. The erythema was characterized by a wide, elevated border and central pallor. Histologically, there was a coat-sleeve-like infiltration of lymphocytes around the blood vessels, appendages, and secretory gland cells in the dermis. Immunohistological analysis clarified that the majority of infiltrating lymphocytes were CD4-positive T cells. Abnormal expression of hla-dr antigens in the perivascular, appendage, and secretory gland cells in the dermis was also observed. The differential diagnosis of the three patients lay between Sjogren syndrome (SjS), Sjogren/systemic lupus erythematosus overlap syndrome and an asymptomatic clinical state. These results are consistent with recent findings of major histocompatibility complex class II expression on target organs in various autoimmune diseases. Based on these findings, erythema appears to represent a broad cutaneous manifestation of these diseases. Furthermore, the presence of SSA antibodies, aberrant HLA-DR expression, and sun exposure may be responsible for the development of erythema.- - - - - - - - - - ranking = 0.22222222222222keywords = gland (Clic here for more details about this article) |
7/235. Primary sialoangiectasia--a diagnostic pitfall in sjogren's syndrome: case report.A case of primary sialoangiectasia, which in this case was initially misdiagnosed as sjogren's syndrome, is described. Other diseases, including hiv infection, psoriatic arthritis, and acute parotitis, may cause glandular changes similar to the changes found in the syndrome. Therefore, sialography must be combined with other methods of assessment of the oral cavity when suspicion is high for sjogren's syndrome. Properly applied, sialography provides essential information regarding the severity of glandular damage and the progression of the disease.- - - - - - - - - - ranking = 0.22222222222222keywords = gland (Clic here for more details about this article) |
8/235. lung adenocarcinoma in lymphocytic interstitial pneumonitis associated with primary sjogren's syndrome.We experienced a rare case of lung adenocarcinoma associated with lymphocytic interstitial pneumonitis caused by primary sjogren's syndrome. A 78-year-old woman was referred to our hospital because of progressive sicca syndrome and nodular opacities in the right lower lobe on chest radiograph. This patient was diagnosed as primary sjogren's syndrome by a labial gland biopsy and classical clinical features including xerophthalmia, xerostomia and immunoserological findings. Pathological findings including immunohistochemical studies in a surgically resected lung revealed adenocarcinoma in lymphocytic interstitial pneumonitis associated with primary sjogren's syndrome. There was no evidence of malignant lymphoma in the lymph nodes or resected lung tissue. Pulmonary involvement of sjogren's syndrome is now regarded both clinically and histopathologically as a wide spectrum of lymphoproliferative disorders ranging from benign to malignant. However, lung cancer associated with primary Sjogren's syndrome, as in our case, has apparently not been reported previously.- - - - - - - - - - ranking = 0.11111111111111keywords = gland (Clic here for more details about this article) |
9/235. Simultaneous phenotypically distinct but clonally identical mucosa-associated lymphoid tissue and follicular lymphoma in a patient with sjogren's syndrome.A 44-year-old woman with a 12-year history of sjogren's syndrome (SS) developed a low-grade mucosa-associated lymphoid tissue (MALT) lymphoma in the parotid gland. Two years later, she presented with generalized lymphadenopathy and hepatosplenomegaly and a follicular lymphoma was diagnosed. To investigate the relationship of the two histologically distinct lymphomas, we re-examined their histology and immunophenotype and studied the lymphomatous tissue from the parotid, cervical lymph node, and spleen using molecular genetic methods. Histologic and immunophenotypic studies confirmed the previous diagnoses and also identified a previously unnoticed focus of follicular lymphoma in the second parotid gland biopsy. polymerase chain reaction (PCR) amplification of the rearranged Ig heavy-chain gene showed the same sized dominant product in the MALT lymphoma and the follicular lymphoma. Similarly, PCR analysis of the t(14:18) translocation yielded an identical sized band from both MALT and follicular lymphoma. Cloning and sequencing of the Ig PCR products showed an identical CDR3 sequence from each lesion, indicating a common clonal lineage. The follicular lymphoma of the parotid gland lymph node and the follicular lymphoma of the spleen showed an identical mutation signature to that of the salivary gland MALT lymphoma. We propose that follicular lymphoma in the parotid gland lymph node may have resulted from colonization of lymphoid follicles by MALT lymphoma cells, following which the tumor cells were induced to express a follicular lymphoma phenotype, due to Bcl-2 overexpression caused by t(14;18), leading to a change in clinical behavior resulting in rapid widespread dissemination of disease. These observations suggest that the distinct phenotypes of low-grade B-cell lymphomas may be the consequence of interplay between genetic and local microenvironmental factors.- - - - - - - - - - ranking = 0.55555555555556keywords = gland (Clic here for more details about this article) |
10/235. A combination of autoimmune hepatitis, sensory-dominant peripheral neuropathy, and primary sjogren's syndrome in the same patient: a rare association.Although autoimmune hepatitis and sensory-dominant neuropathy have been known to independently accompany primary sjogren's syndrome, the combination of all these conditions in the same patient has not been described. We report the case of a woman who initially suffered from autoimmune hepatitis and later was diagnosed with primary sjogren's syndrome upon the development of sensory-dominant neuropathy. In this patient, autoimmune hepatitis preceded neuropathy by one year. All of the diagnoses were confirmed by histological examinations of the liver, sural nerve, and minor salivary gland. Her autoimmune hepatitis was relieved with conservative treatment, and her sensory-dominant neuropathy was alleviated by prednisolone therapy. Our case indicated that the multiple organ involvement, especially that in the liver and peripheral nerves, should be taken into account in the course of primary sjogren's syndrome.- - - - - - - - - - ranking = 0.11111111111111keywords = gland (Clic here for more details about this article) |
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