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ID PMID Title PublicationDate abstract
9038756 Immunohistological analysis of tumour growth factor beta 1 expression in normal and inflam 1996 Sep AIM: To determine whether transforming growth factor-beta 1 (TGF-beta 1) has a pathogenetic role in disease of the salivary glands. METHODS: An indirect immunohistochemical technique was used to analyse TGF-beta 1 expression in six specimens of normal salivary gland and 23 surgical specimens. RESULTS: TGF-beta 1 was strongly expressed in the ductal epithelial cells of normal salivary gland tissues (six of six cases) and in inflammatory conditions (eight of 11 cases). In contrast, TGF-beta 1 was not detectable in ductal epithelial cells expressing HLA-DR around infiltrating CD4+ CD45RO+ activated T cells, in the salivary gland tissue of patients with Sjögren's syndrome. CONCLUSION: Because TGF-beta 1 has an essential role in the mucosal immunity of salivary glands, abnormal expression of this cytokine must be regarded as a candidate in the pathogenesis of Sjögren's syndrome.
8701383 Systemic lupus erythematosus diagnosed during interferon alfa therapy. 1996 Aug We describe a patient who had clinical manifestations of several autoimmune disorders: Sjögren's syndrome, benign hypergammaglobulinemic purpura of Waldenström, and systemic lupus erythematosus (SLE). The SLE was diagnosed during therapy with interferon alfa. Testing for anti-Ro and anti-La antibodies was negative until the serum was diluted to eliminate a possible prozone phenomenon of antibody excess.
8908435 The lactoferrin tear test in the diagnosis of Sjögren's syndrome. 1996 Jul PURPOSE: To assess the sensitivity and specificity of the lactoferrin tear test (LTT) in the diagnosis of keratoconjunctivitis sicca due to Sjögren's syndrome (SS), comparing it with the other lacrimal tests and with immunological tests. METHODS: 25 patients suffering from SS (24 women and 1 man, median age 51.5 years, s.d. 16.3); control group: 20 patients with various kinds of conjunctivitis without dry eye. Tests in both groups: Schirmer I (ST), BUT, ferning test, lactoferrin immunoassay, fluorescein and Rose Bengal staining. Immunological tests: serum titers of anti-nuclear (ANA), anti-DNA, anti-ENA (SS-A, SS-B, RNP, FR) antibodies. In the patients with SS, labial salivary gland biopsy was also performed. RESULTS: LTT had a specificity of 95% and a sensitivity of 72%, compared to 85% and 64% for the Schirmer I test. The ferning test has the highest sensitivity (92%), and none of the cases positive to BUT was negative to the ferning test. The combination of LTT and ferning test gave a value of 78% compared to 70% with ST. The correlations between positive LTT and positive ANA, SS-A and labial biopsy were respectively 83%, 67% and 80%, as against 67%, 50% and 65% for the Schirmer I test. CONCLUSIONS: In our study, LTT showed very high specificity, good sensitivity particularly when combined with qualitative tear tests, and a good correlation with the immunological and bioptic tests for SS. Since it is easy to perform, in our opinion LTT can be included in the diagnostic routine for keratoconjunctivitis sicca in SS.
7889697 Corticosteroid-responsive parkinsonism associated with primary Sjögren's syndrome. 1994 Nov A 74-year-old woman with primary Sjögren's syndrome confirmed by salivary gland biopsy presented with parkinsonism. Magnetic resonance imaging (MRI) of the brain revealed multiple small high intensity lesions in the deep white matter, basal ganglia and pons on T2-weighted images. Treatment with L-dopa failed to improve the parkinsonian features. After the initiation of prednisolone 30 mg/day, the parkinsonian signs and symptoms significantly improved. Some lesions on MRI were decreased in size after corticosteroid therapy. These findings suggest that parkinsonism associated with primary Sjögren's syndrome is at least in part attributable to small vessel vasculopathy such as focal inflammation or edema.
1346409 Lymphocytic sialadenitis of Sjögren's syndrome associated with chronic hepatitis C virus 1992 Feb 8 Viral infection has often been suggested as a possible cause of Sjögren's syndrome or chronic lymphocytic sialadenitis, and Epstein-Barr virus has been found in the salivary glands of patients with this condition. After we had noted Sjögren's syndrome in several patients infected with hepatitis C virus (HCV), a virus also excreted in saliva, we set up a prospective study to investigate the association of chronic lymphocytic sialadenitis, with or without symptoms, to chronic HCV liver disease. The histological appearances of labial salivary glands in patients with proven HCV hepatitis or cirrhosis were compared with those in dead controls. Histological changes characteristic of Sjögren's syndrome were significantly more common in HCV-infected patients (16 of 28, 57%) compared with controls (1 of 20, 5%). Focal lymphocytic sialadenitis characteristic of Sjögren's syndrome (though only 10 patients had xerostomia and none complained of xerophthalmia) appears to be common in patients with chronic HCV liver disease; if this association is confirmed, identification of the underlying mechanism may improve our understanding of both disorders.
9000806 SS-A/Ro antibodies in Chinese patients with systemic lupus erythematosus and Sjögren's sy 1996 Dec Serum from 53 patients with systemic lupus erythematosus (SLE) and 23 patients with primary Sjögren's syndrome (SS) were studied for anti-52-kDa SS-A/Ro, anti-60-kDa SS-A/Ro, and anti-SS-B/La antibodies by immunoblotting and enzyme-linked immunosorbent assay (ELISA). By immunoblotting, anti-SS-A/Ro was detected in 16 (30%) patients with SLE and 17 (74%) patients with SS. Anti-SS-B/La was detected in 22 (41%) patients with SLE and 15 (65%) patients with SS. Serum from 14 of the 16 SLE patients with anti-SS-A/Ro reacted with the 60-kDa protein and 15 serum samples from these patients recognized the 52-kDa protein. Serum with anti-60-kDa SS-A/Ro alone was not found. Serum from all of the 17 SS patients with anti-SS-A/Ro reacted with the 52-kDa protein, whereas serum from only two of these patients recognized the 60-kDa protein. By ELISA, the frequency of anti-SS-A/Ro (antibodies to the 60-kDa and/or 52-kDa of SS-A/Ro proteins) in patients with SLE and SS was 43/53 (81%) and 15/23 (65%), respectively. Anti-48-kDa SS-B/La was found in 28% and 48% of SLE and SS patients, respectively. Serum from 77% of SLE patients and 48% of SS patients reacted with the 60-kDa SS-A/Ro protein. Serum from 45% of SLE patients and 52% of SS patients reacted with the 52-kDa SS-A/Ro protein. Patients with SLE had significantly higher titers of antibodies to 60-kDa SS-A/Ro compared with patients with SS. Anti-SS-A/Ro and anti-SS-B/La are common in both SLE and SS. The different reactivities of anti-52-kDa and anti-60-kDa antibodies in serum from patients with SLE and SS may represent differences in conformation-dependent epitopes of SS-A/Ro autoantigens.
8942017 Sjögren's syndrome with membranous glomerulonephritis detected by urine screening of scho 1996 Oct A case of Sjögren's syndrome with glomerulonephritis is presented. The patient was a 13 year old male with hematuria and proteinuria discovered by urine screening of school children. Evaluation showed no evidence of any associated connective tissue disease. Kidney biopsy was consistent with membranous glomerulonephritis. Sjögren's syndrome with membranous glomerulonephritis is rare and the patient was the youngest case in the literature.
7876863 Chronic dacryosialadenitis in HTLV I associated myelopathy. 1995 Feb A prospective study was carried out on 48 patients with HTLV I associated myelopathy/tropical spastic paraparesis (HAM/TSP) to assess the association between this entity and Sjögren's syndrome. Fourteen patients (29.1%) had chronic dacryosialadenitis confirmed by a positive Schirmer's test and salivary gland biopsy. None of these patients had evidence of collagen disease and tests for Ro, La, and rheumatoid factor were negative except in one case. Therefore, the dacryosialadenitis could not be classified as either primary or secondary Sjögren's syndrome. Ten of the 14 patients (71.4%) had other systems (haematological, articular, dermatological, or respiratory) involved apart from the neurological and exocrine gland pathology. The findings suggest that the dacryosialadenitis associated with HTLV I is a disease of viral origin distinct from Sjögren's syndrome.
8050188 Possible involvement of Epstein-Barr virus in the pathogenesis of Sjogren's syndrome. 1994 Aug We examined the possible involvement of Epstein-Barr virus (EBV) in the pathogenesis of Sjogren's syndrome (SS) using B cell lines (BCLs) spontaneously established from the peripheral blood. These BCLs were positive for EBNA and produced a large amount of infecting EBV in culture, a feature unique to SS-BCLs. Polymerase chain reaction analysis revealed that EBV with a B95-8-like U2 region was dominant in SS-BCLs. The nucleotide sequences of the U2 region of SS-EBV obtained so far have shown high homology to those of B95-8 EBV. However, there was a substantial amount of deletion and substitution of nucleotides within the U2 region of SS-EBV when compared with B95-8 EBV. This might enable SS-BCLs to escape immune recognition by cytotoxic T cells specific to EBNA-2. In addition, SS-EBV contains the sequence that spans the B95-8-deleted region. Furthermore, transfer of SS-BCLs to SCID mice induced monoclonal lymphoproliferative disorders that resembled those arising in SS. These data further support the motion that reactivation of EBV is deeply involved in polyclonal B cell activation and the development of B cell malignancies in SS.
7949267 Ulcerative colitis and Sjogren's syndrome in the same patient: report of two cases and a r 1994 Jul Two cases of coexisting ulcerative colitis and Sjogren's syndrome are presented. Both patients were women and in both ulcerative colitis preceded the diagnosis of Sjogren's syndrome by several years. The course of ulcerative colitis before the onset of Sjogren's syndrome was quite severe. On the basis of clinical signs and serological and immunogenetic patterns, both patients were classified as suffering from secondary Sjogren's syndrome. Although the combination of the two diseases in the same patient seems to be the result of chance, we think that this combination offers an opportunity for future studies related to the pathogenesis of ulcerative colitis.
8371221 Neonatal lupus erythematosus syndrome: late detection of isolated heart block. 1993 Jul Heart block in neonatal lupus erythematosus is typically complete and detected in utero or in the neonatal period. We describe a child diagnosed with incomplete heart block at 9 years of age whose mother was diagnosed with Sjögren's syndrome and anti-Ro(SSA) 2 years after diagnosis of heart block in her child. This is the first case of late detection of incomplete heart block in a child felt to be causally related to the presence of anti-Ro(SSA) in the mother.
1457582 Treatment of neutropenia in Felty's syndrome with granulocyte-macrophage colony-stimulatin 1992 Nov We report on a 67-year-old man with Felty's syndrome (FS) complicated by recurrent pneumonia and an infected wound, which was not healing in spite of maximal antibiotic and local therapy. Encouraged by previous experience, we treated him with granulocyte-macrophage colony-stimulating factor (GM-CSF). His total leukocyte count rose, but the patient's pneumonia deteriorated. In addition, a previously known chronic obstructive lung disease (COLD) was exacerbated acutely. These complications finally led to his death. Postmortem examination revealed widespread pneumonia with invasive aspergillosis and a peripheral adenocarcinoma in his left lung.
1419498 The molecular genetics of systemic lupus erythematosus and Sjögren's syndrome. 1992 Oct Refinements in molecular genetic technology as well as in the organization of the major histocompatibility complex and the genes contained therein continue to lead the way to elucidation of the immunogenetics of systemic lupus erythematosus and Sjögren's syndrome. In this article, recent advances in these areas are reviewed, and major histocompatibility complex associations with systemic lupus erythematosus and Sjögren's syndrome will be explored with particular emphasis on autoantibody subsets of these diseases. Data thus far support the hypothesis that systemic lupus erythematosus and Sjögren's syndrome are clinically and serologically heterogeneous disorders of major histocompatibility complex class II allele-associated autoantibody subsets, to which other major histocompatibility complex genes (C4 null alleles) and non-major histocompatibility complex genes (such as T-cell receptor genes) may contribute in susceptibility.
1341448 Possible viral implication in the pathogenesis of Sjögren's syndrome. 1992 Jul How then could the observed pathological changes in the exocrine glands of patients with pSS be related to the various in vitro and in vivo laboratory findings? To begin with, the fact of inappropriate HLA-D/DR expression in epithelial cells in the absence of any infiltrating T cells or residual IFN-gamma must argue for an exogenous agent such as a virus that modulates the gene expression of epithelial cells. Such inappropriate expression is probably accompanied by the expression of other molecules that promote adhesion of lymphocytes in the proximal endothelium and infiltration into the exocrine gland. The autoimmune response takes place in these very exocrine glands and probably is initiated by the presentation of antigen(s) to the CD4+ T cells by the HLA-D/DR+ epithelial cells. The HLA-D/DR association shown in patients with pSS probably has to do with the antigenic fragments of the autoantigens that these molecules can preferentially bind to. The activated CD4+ T cells can then deliver help to specific B lymphocytes leading them to autoantibody-secreting plasma cells. In addition the extent of polyclonal activation of B lymphocytes and their potential for cytokine secretion and proliferation in vitro, is consistent with the view of virally-induced activation of these cells. The cross-reactivity to retroviral antigens observed in the sera of pSS patients, and the retroviral sequences detected in the salivary glands argue for some type of a retroviral infection of pSS patients in the course of the disease.(ABSTRACT TRUNCATED AT 250 WORDS)
7987902 Sjogren's syndrome: pathology, oral presentation, and dental management. 1994 Sep As one of the major autoimmune conditions involving the oral, head, and neck regions, Sjögren's syndrome has a significant level of dentally related pathology. As a result of salivary dysfunction, the teeth and mucosa may develop a wide array of changes. If these changes are not managed properly, major oral dysfunction can occur. An evaluation of salivary function, the management of xerostomia and its effects, dietary counseling, and an overall appreciation of the extraoral components of Sjögren's syndrome are presented. The dental component of comprehensive patient management is one of the most important aspects of this condition, with the dental practitioner being an essential part of the health-care team.
1380277 Whipple's disease, familial Mediterranean fever, adult-onset Still's disease, and enteropa 1992 Aug Whipple's disease is a rare multisystem disorder of infectious etiology. Efforts to culture the responsible organism have been unsuccessful. Nucleotide sequencing and amplification of bacterial 16S ribosomal DNA revealed the organism to be most similar to bacteria of the Rhodococcus, Streptomyces, and Arthrobacter genera. Several clinical studies of the long-term use of colchicine for the treatment of familial Mediterranean fever demonstrate its utility for symptom control and prevention of complications by amyloidosis in both adults and children. Normal growth, development, and subsequent fertility were seen in children treated with colchicine. Adult-onset Still's disease has previously been thought to have a generally good outcome, although some patients develop chronic arthritis and disability. No markers have been available for prognosis. A study of 62 patients revealed the presence of polyarthritis, root joint involvement, and rash at initial presentation to be associated with a poorer outcome. Enteropathic arthritis may be seen as a complication of both Crohn's disease and ulcerative colitis. The onset of peripheral arthritis coincides with or follows the onset of bowel symptoms in most cases, whereas spondylitis may precede the onset of inflammatory bowel disease by years. HLA-B27 is present in 50% to 75% of cases of spondylitis. No HLA association with inflammatory bowel disease or peripheral arthritis has been consistently found.
7492227 Comparison of clinical and self reported diagnosis for rheumatology outpatients. 1995 Oct OBJECTIVE: To examine the sensitivity of patient self reported diagnoses compared with physician diagnoses in a rheumatology outpatient population. METHODS: A mailed survey to 472 rheumatology outpatients (81% response rate) asked about joint symptoms, disabilities, and underlying rheumatic conditions. The self-reported diagnoses were linked with physician diagnoses in the rheumatology clinic computer based diagnostic registry. RESULT: Overall there was an 87% sensitivity for self reported compared with physician diagnoses when the matching criteria included compatible yet different diagnoses such as rheumatoid arthritis (RA) and osteoarthritis (OA). The sensitivity for exact match was 65%, and it varied with the underlying clinical diagnosis, and was greatest for RA (90%) and ankylosing spondylitis (AS) (100%), and intermediate for OA (52%) and psoriatic arthritis (50%). The sensitivity of self report was primarily related to the type of diagnosis (RA or AS v other rheumatic conditions; odds ratio = 16.3, 95% confidence interval (CI) 9.0 to 29.5), and also to difficulty in activities of daily living (odds ratio = 2.3, 95% CI 1.1 to 4.6) but not age, gender, duration of disease, or clinic attendance, as shown by multivariate analysis. CONCLUSIONS: This study in a rheumatology outpatient population indicated that most patients report a diagnosis which is compatible with the clinical diagnosis. These findings give an upper limit to the sensitivity of self reported diagnoses, though further research is needed to assess the extent to which our results may be generalised to other settings.
8240438 Inflammatory polyarthritis in mice transgenic for human T cell leukemia virus type I. 1993 Nov OBJECTIVE: We have recently reported that arthropathy develops in high incidence among transgenic mice carrying the pX region of human T cell leukemia virus type I (HTLV-I). In the present study, the histopathologic features of the joints in these mice were examined in order to compare the animal disease with rheumatoid arthritis (RA) in humans. METHODS: Paraffin sections of limbs (right and left fingers, wrists, elbows, shoulders, toes, knees, and ankles) were stained with hematoxylin and eosin, periodic acid-Schiff, azan-Mallory, or phosphotungstic acid hematoxylin, and examined by light microscopy. RESULTS: Abnormalities of the limbs began to occur as early as 3 weeks of age, and the incidence gradually increased until the mice were 12 months old. The incidence of arthropathy was 22% (48 of 217) at 3 months of age and 28% (18 of 64) at 6 months. The severity of the histopathologic changes in the joints of the transgenic mice ranged from grade I to grade IV. CONCLUSION: The major histopathologic features in the joints of HTLV-I transgenic mice are similar to those in humans with RA. Thus, these mice may represent a useful model for the study of the disease in humans.
8911652 Neuroendocrine changes in systemic lupus erythematosus and Sjögren's syndrome. 1996 May It has become clear that the neuroendocrine and immune systems are closely linked and interdependent. The exact mechanisms of this interaction are only beginning to be unravelled. The complexity of these connections may partly explain why the aetiopathogenesis of autoimmune diseases remains obscure and why genetic, hormonal, microbial, environmental, as well as a host of other factors, have all been put forward as explanations. What has become clear is that a number of neuroendocrine and hormonal factors have important immunomodulatory roles in health and disease.
8052931 [A case of aseptic meningoencephalitis in a patient with secondary Sjögren syndrome with 1994 Jun A 26-year-old woman, who had been treated with prednisolone 30 mg/day for secondary Sjögren syndrome (SjS) with systemic lupus erythematosus (SLE), was complicated with aseptic meningoencephalitis (AME). Cerebrospinal fluid (CSF) cell counts were 26/3, total protein 126 mg/dl, glucose 41 mg/dl. The CSF, blood, nasopharyngeal swab were cultured for bacteria, mycobacteria, viruses and fungi, but no evidence for an infectious etiology was obtained in this patient. The pair sera study for viruses titer were performed, but no significant titer elevation was observed. Head magnetic resonance imaging showed remarkable atrophy of bilateral occipital lobe and dilatation of posterior horn of bilateral ventricles. The high 2-5AS enzyme activity in the CSF suggested indirectly that the cause of this meningoencephalitis was virus infection. In this case microbiological studies were negative, so central nervous system SLE and/or central nervous system SjS were not completely neglected.