Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0043352 (xerostomia)
 4,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary Sjogren's syndrome is an autoimmune disorder characterized by lymphocytic infiltration of the salivary and lacrimal glands, producing associated dry eyes (keratoconjunctivitis sicca), dry mouth, and intermittently swollen salivary glands. A high proportion of the infiltrating B lymphocytes express surface and cytoplasmic Ig bearing a kappa-L chain-associated CRI defined by reactivity with the murine mAb, 17.109. To determine the structural basis for CRI expression in this disease, we generated CRI+ lymphoblastoid cell lines and a cDNA library from lymphocytes extracted from Sjogren's syndrome patients' salivary gland biopsy specimens. Nucleic acid sequence analyses of the mRNA of one such 17.109-CRI+ lymphoblastoid cell line (NOV) reveals the expressed kappa light chain variable region gene (V kappa gene) to be homologous to Humkv325, a conserved V kappa gene used at relatively high frequency in certain B cell malignancies. In addition, synthetic oligonucleotides, corresponding to the first and third frameworks and the second complementarity determining region of the Humkv325 gene, were used to identify and isolate clones from a cDNA library generated from SS salivary gland lymphocytes. Clones annealing specifically with one or more of these oligonucleotide probes contained kappa light chain cDNA. The sequences corresponding to the variable region of two clones (Taykv320 and Taykv306) were homologous to Humkv325. The V kappa genes of four other cDNA clones (Taykv322, Taykv310, Taykv308, and Taykv312) most likely were generated somatically from the rearranged Humkv325 gene through a limited number of nucleic acid base substitutions. Our results suggest that the high frequency of 17.109-CRI expression in Sjogren's syndrome patients results from a multiclonal expansion of B cells using Humkv325, and that the expressed Humkv325 may undergo somatic diversification in an apparent Ag-driven response.
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PMID:Molecular characterization of a major autoantibody-associated cross-reactive idiotype in Sjogren's syndrome. 254 6

Primary Sjogren's syndrome (pSS) is a common autoimmune connective tissue disease in China yet without a universally accepted diagnostic criteria. In this study a new criteria was proposed and compared with other six sets of criteria. Fifty-five items in 112 pSS and 185 controls were evaluated. Results show the criteria we proposed contained one major and nine minor items. For the purpose of identifying patients in clinical studies, a major with at least three of the nine minor items or at least five of the minor items should be presented. The major item is anti-SSA/SSB(+) and the minors are, (1) dry eyes or dry mouth (> 3 months, persistently), (2) swollen salivary glands (recurrently or persistently), (3) rampant dental caries, (4) Schirmer test (< 5 mm in 5 min.) or corneal staining(+), (5) unstimulated salivary flow (< 0.03 ml/min) or abornal parotid sialography, (6) minor salivary gland biopsy (> or = 1 focus), (7) renal tubular acidosis, (8) hypergammaglobuminemia (gamma globulin > or = 30%) or hypergammaglobuminemic purpura, (9) RF > 1 : 20 or ANA > 1 : 20. Other connective tissure diseases, pre-existing lymphoma, AIDS, sarcoidosis, graft vs host disease must be excluded. The criteria we proposed had a high specificity of 98.2% and sensitivity of 94.1%.
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PMID:[Preliminary diagnostic criteria for primary Sjogren's syndrome in China]. 927 31

Primary Sjogren's syndrome (pSS) is a chronic autoimmune disorder of the exocrine glands. The diagnosis is largely based on keratoconjunctivitis sicca and xerostomia in the presence of anti-SS-A and/or SS-B antibodies. Anticentromere antibodies (ACA) have occasionally been reported in patients with pSS. We describe two patients with pSS associated with ACA, initially diagnosed as limited systemic sclerosis. Symptoms at the time of initial presentation were dry eyes and mouth, arthralgias, and Raynaud's phenomenon. Both patients developed small vessel cutaneous vasculitis, parotid enlargement, low C4 complement levels, positive rheumatoid factor, and lymphoma. These findings suggest that patients with pSS who have ACA may be a subgroup of patients at increased risk of extraglandular systemic manifestations and lymphoma.
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PMID:Primary Sjogren's syndrome with anticentromere antibodies--a clinically distinct subset. 2008 42

Primary Sjogren's syndrome (pSS) can have a myriad of presentations, ranging from mild xerostomia to more diffuse systemic involvement. It is well established that pSS is associated with a variety of pulmonary pathologies, and it is also known that pSS patients are at higher risk for lymphoma development. Here, we present an unusual case of a woman with primary Sjogren's syndrome who had both diffuse cystic lung disease as well as extranodal MALT lymphoma, successfully treated for both conditions with the CD-20 monoclonal antibody rituximab.
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PMID:A patient with primary Sjogren's syndrome, cystic lung disease, and MALT lymphoma treated successfully with rituximab: a case-based review. 3186 12

Primary Sjogren's syndrome (pSS) is an autoimmune disorder in which lymphocytic infiltration leads to lacrimal and salivary glands dysfunction, which results in symptoms of dryness (xerophthalmia and xerostomia). Extraglandular features are common and may affect several organs. Renal involvement has long been known as one of the systemic complications of pSS. The most classical lesion observed in pSS is tubulointerstitial nephritis (TIN) and less frequently membranoproliferative glomerulonephritis (MPGN), which is related to cryoglobulinemia. In some cases, renal biopsy is necessary for the definitive diagnosis of kidney involvement. Patients may present with proximal renal tubular acidosis, distal renal tubular acidosis and chronic kidney disease. Response to treatment is usually favorable. However, occasionally severe and rarely lethal outcomes have been described. Recently, several case series and cross-sectional studies have been published which investigated the factors associated with renal involvement in pSS and the most accurate screening tests for early detection. The presence of xerophthalmia, anti-SSA and rheumatoid factor positivity, low C3 levels and other features have all shown either positive or inverse associations with the development of renal complications. Serum creatinine, alpha-1-microglobulin, cystatin-C have been evaluated as early detection biomarkers with variable accuracy. More advanced techniques may be necessary to confirm proximal and distal renal tubular acidosis, along with nephrogenic diabetes insipidus. The aim of the current paper is to summarize and critically examine these findings in order to provide updated guidance on serum biomarkers and further testing for kidney involvement in pSS.
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PMID:Biomarkers and Diagnostic Testing for Renal Disease in Sjogren's Syndrome. 3304 42