1/38. 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 = labial (Clic here for more details about this article) |
2/38. 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 = 0.5keywords = labial (Clic here for more details about this article) |
3/38. 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.- - - - - - - - - - ranking = 0.5keywords = labial (Clic here for more details about this article) |
4/38. Monozygotic twins with primary sjogren's syndrome.sjogren's syndrome (SS) is a chronic autoimmune disease mainly characterized by dry mouth and dry eyes due to an inflammatory process in the exocrine glands. We describe a pair of Caucasian monozygotic twin sisters and their mother, all having primary SS. The twins had very similar clinical presentation and almost identical serological data, and histological examination of lower labial salivary glands gave a focus score of 3 in both twins. We also present their family medical history, which shows aggregation of immunological disorders among family members, although the twins and their mother were the only individuals with primary SS.- - - - - - - - - - ranking = 0.5keywords = labial (Clic here for more details about this article) |
5/38. Type II diabetes mellitus and primary sjogren's syndrome complicated by pleural effusion.A 73-year-old man was admitted to our hospital because of pleural effusion and nephrotic syndrome. sjogren's syndrome (Sjs) was diagnosed based on a positive test for antibodies to Ro and La, and the result of labial salivary gland biopsy. The pleural effusion showed a high number of lymphocytes and high titers of antibodies to Ro and La. By immunohistochemistry, it was determined that infiltrating CD3 cells predominated over infiltrating CD20 cells in the pleura. nephrotic syndrome was also present, which, as confirmed by renal biopsy was due to advanced diabetic nephropathy. Here, we report a case of Type II diabetes mellitus and primary Sjs complicated by pleural effusion, discuss the available treatment for pleural effusion.- - - - - - - - - - ranking = 0.5keywords = labial (Clic here for more details about this article) |
6/38. Reversibility of histological and immunohistological abnormalities in sublabial salivary gland biopsy specimens following treatment with corticosteroids in sjogren's syndrome.sjogren's syndrome (SS) is a chronic autoimmune disease characterised by specific lesions in exocrine glands, so sublabial minor salivary gland biopsy (SLGB) plays an important part in its diagnosis. The extent and composition of the lymphocytic infiltrate in SLGB specimens can be considered as target organ specific parameters. They are quantified after histological and immunohistological examination by a focus score (describing the extent of the infiltrate) and IgA% score (describing the composition of the infiltrate), respectively. However, little is known about the factors that contribute to the extent and composition of the infiltrate and whether these features are reversible as repeated SLGBs are rarely performed. A patient with SS is described who underwent SLGBs before and after treatment with high dose corticosteroids. After treatment there was not only clinical improvement, but also improvement in the histological and immunohistological parameters. Although these findings need to be confirmed in further studies, this suggests that histopathological changes may be reversible in SS. Furthermore, it shows that the potential effects of corticosteroid use should be taken into account when interpreting SLGB specimens. When clinical changes do parallel histological changes, repeated SLGBs might offer a marker for disease activity in patients with SS.- - - - - - - - - - ranking = 2.5keywords = labial (Clic here for more details about this article) |
7/38. Pulmonary MALT lymphoma with amyloid production in a patient with primary sjogren's syndrome.A 53-year-old woman was admitted to our hospital complaining of cough, low grade fever, chest pain and sicca symptoms. A chest radiograph showed an abnormal shadow and chest computed tomography revealed a tumor in left S6. She was diagnosed as sjogren's syndrome by sialography and histological findings of labial biopsy. The surgically resected tumor specimen showed proliferation of lymphoid cells with lymphoepithelial lesions, which were positive for CD20 and kappa light chain. Kappa light chain-positive amyloid was found within the tumor. The tumor showed rearranged kappa light chain genes. The diagnosis was pulmonary mucosa associated lymphoid tissue lymphoma with amyloid production.- - - - - - - - - - ranking = 0.5keywords = labial (Clic here for more details about this article) |
8/38. Bilateral parotid cysts as presentation of sjogren's syndrome.We present a case of a 40-year woman with bilateral parotid salivary gland enlargement as presentation of primary sjogren's syndrome. Computed tomography (CT) and magnetic resonance imaging (MRI) showed parotid cysts, suggestive of cystic benign lymphoepithelial lesions. A sub-labial biopsy confirmed the syndrome. After 24-month follow-up, the left parotid cysts remain the same, whereas other cysts have appeared in the right parotid gland. Parotid involvement in sjogren's syndrome is discussed.- - - - - - - - - - ranking = 0.5keywords = labial (Clic here for more details about this article) |
9/38. Primary Sjogren syndrome in childhood: report of a case and review of the literature.Sjogren syndrome (SS) in childhood is a rare and possibly underdiagnosed condition. The purpose of this study is to report a case of primary SS (PSS) in a 4-year-old Venezuelan girl and to review the pertinent literature. The patient presented with bilateral recurrent parotid enlargement, predominantly on the right side. She did not complain of dry mouth or eyes; however, decreased stimulated salivary flow rate and positive Schirmer and rose bengal tests were obtained. sialography, sonograms, and a computed tomography scan of the parotid glands revealed pathologic changes consistent with SS. Anti-SS-A and anti-SS-B antibodies were present. Evaluation for antibodies against cytomegalovirus, Epstein-Barr virus, and hiv rendered negative results. Histopathologic examination of incisional biopsies of the right parotid and labial minor salivary glands revealed focal periductal lymphocytic infiltrate and sialoduct ectasia. Taken together, these findings are consistent with the diagnosis of juvenile PSS. The salient features of this rare disease are summarized on the basis of a comprehensive review of the epidemiologic, clinical, and serologic findings of the previously reported cases of PSS in children.- - - - - - - - - - ranking = 0.5keywords = labial (Clic here for more details about this article) |
10/38. Neurosarcoidosis mimicking sjogren's syndrome.PURPOSE: To describe a patient with a long disease history who was finally diagnosed with neurosarcoidosis and to discuss the reasons behind the delayed diagnosis. CASE REPORT: A 58-year-old man with sick sinus syndrome and bradycardia, which was treated with a pacemaker, developed first right and then left facial palsy. Subsequently, multiple cranial nerve palsies developed and later spontaneously resolved. Neurosarcoidosis was suspected at that stage, but excluded because the patient had no typical sarcoid lung changes, his serum and cerebrospinal fluid angiotensin converting enzyme activity levels were normal and a computed tomography scan disclosed no central nervous system changes. During follow-up, the patient developed extremely dry eyes and mouth, suggesting sjogren's syndrome. rheumatology consultation did not reveal any autoimmune or visceral features typical of sjogren's syndrome and autoantibodies were negative. However, both labial salivary gland and conjunctival biopsies revealed non-caseating granulomas, and neurosarcoidosis was diagnosed. CONCLUSIONS: Neurosarcoidosis is a relatively rare disease with a somewhat poor longterm prognosis in one-third of cases, although the neurological manifestations often diminish or disappear in response to glucocorticoid treatment. Diagnosis is often a clinical challenge, especially in the absence of pulmonary changes or other features typical of sarcoidosis. The labial salivary gland and conjunctiva provide helpful biopsy sites for histopathological confirmation of the diagnosis.- - - - - - - - - - ranking = 1keywords = labial (Clic here for more details about this article) |
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