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ID PMID Title PublicationDate abstract
8603842 Cyclosporine therapy suppresses ocular and lacrimal gland disease in MRL/Mp-lpr/lpr mice. 1996 Feb PURPOSE: MRL/Mp-lpr/lpr (MRL/lpr) mice spontaneously develop an autoimmune disease characterized by lymphoproliferation, vasculitis, glomerulonephritis, autoantibody production, and ocular and lacrimal gland inflammation. Lacrimal gland lesions in MRL/lpr mice are a model for the human disorder Sjögren's syndrome. The target organ lesions in MRL/lpr mice, including those in the eye and lacrimal gland, are composed largely of CD4+ T cells, with lesser numbers of CD8+ T cells and B cells. Cyclosporine therapy was evaluated for its effect on the autoimmune disease, particularly in the eye and lacrimal gland. METHODS: MRL/lpr mice were administered cyclosporine intraperitoneally at a dosage of 2 mg daily from age 1 to 5 months. Animals were killed at 5 months and evaluated for the presence of autoimmune disease. Control groups consisted of animals given daily injections with either saline or the cyclosporine diluent. RESULTS: Cyclosporine therapy was effective in reducing the ocular and lacrimal gland disease. Intraocular inflammation was present in 73% of control animals but in only 15% of cyclosporine-treated animals (P < 0.003). Multifocal lacrimal gland inflammatory infiltrates were present in 100% of controls but in only 23% of cyclosporine-treated animals (P < 0.0001). Mean percent area involved by lacrimal gland inflammation was reduced from 19.7% to 4.7% by cyclosporine therapy (P = 0.0003). Systemic autoimmune disease manifestations, including lymphoproliferation, vasculitis, glomerulonephritis, and serologic abnormalities, also were improved. CONCLUSIONS: Chronic cyclosporine therapy, started at an early age, is effective in controlling the autoimmune disease in MRL/lpr mice, including the ocular and lacrimal gland lesions.
7927231 Human combinatorial autoantibodies and mouse monoclonal antibodies to PDC-E2 produce abnor 1994 Oct An increase in the incidence of Sjögren's syndrome in patients with primary biliary cirrhosis has been noted. Indeed, primary biliary cirrhosis has been described as a ductal disease with involvement not only of the biliary tract but of epithelial ductal cells in other organs. We have previously reported the development of a panel of mouse monoclonal antibodies directed at PDC-E2, the major autoantigen of primary biliary cirrhosis. One such antibody, C355.1, but none of the other monoclonal antibodies, reacted not only with mitochondria but also with the apical region of biliary epithelium of patients with primary biliary cirrhosis but not in similar specimens from patients with other liver disease or normal human liver. In addition, we have reported the development of human combinatorial antibodies specific for PDC-E2; these reagents also reacted uniquely with the biliary epithelium of patients with primary biliary cirrhosis. In this paper, we have performed a similar study and have compared the staining of monoclonal antibody C355.1 and a human combinatorial antibody, SP4, with control monoclonal antibodies with respect to their reactivity of salivary glands in 9 patients with primary biliary cirrhosis associated with Sjögren's syndrome, 11 patients with Sjögren's syndrome alone and 7 control patients. Interestingly, the apical region of the salivary gland epithelial cells of approximately 50% of patients with coexisting primary biliary cirrhosis and Sjögren's syndrome had a staining pattern similar to that seen in primary biliary cirrhosis biliary epithelium. In contrast, we did not observe this reactivity in any patient with Sjögren's syndrome alone or in any control patient.(ABSTRACT TRUNCATED AT 250 WORDS)
8910118 A comparison of glandular involvement between chronic graft-versus-host disease and Sjögr 1996 Aug Patients with chronic graft-versus-host disease (cGVHD) occasionally suffer from symptoms of xerostomia and xerophthalmia, which are also features of Sjógren's syndrome (SS). To identify differences in the glandular involvement between cGVHD and SS, we measured the proportions of infiltrating lymphocyte subsets and the expression of HLA-DR antigen and cell adhesion molecules in labial salivary glands (LSG). In cGVHD, more than 90% of the infiltrating lymphocytes were T cells with a slight predominance of CD8+ over CD4+ cells. In SS, CD4+ cells were predominant, and B cells accounted for 10-30% of the infiltrating lymphocytes. Ductal epithelial cell associated with lymphocytic infiltration expressed HLA-DR antigen in both cGVHD and SS. In SS alone, HLA-DR antigen expression also occurred without associated lymphocytic infiltration. The expression of adhesion molecules on ductal epithelial cells, especially vascular cell adhesion molecule 1, was more intense in SS than in cGVHD, while that on endothelial cell was similar in cGVHD and SS. These data suggest that the pathogenesis of glandular involvement of cGVHD is different from that of SS.
9097769 Sicca syndrome in patients infected with the human immunodeficiency virus. 1995 Sep PURPOSE: To investigate an association of the severity of the human immunodeficiency virus (HIV) disease with decreased tear production in a controlled setting. PATIENTS AND METHODS: Seventy-two patients (144 eyes) and 30 age- and sex-matched control subjects (60 eyes) were studied prospectively. Tear production was measured using the Schirmer I test (measured in millimeters after 5 minutes). Patients were classified into three clinical groups: asymptomatic HIV infection, lymphadenopathy syndrome (LAS) or acquired immune deficiency syndrome (AIDS)-related complex (ARC), and AIDS. Additionally, patients were classified according to the CD4+ lymphocyte count. RESULTS: Tear production (mean +/- standard deviation) did not differ (P = 0.32) among eyes of patients with asymptomatic HIV infection (17.1 +/- 10.8; n = 14), LAS or ARC (18.3 +/- 10.3; n = 70), and AIDS (20.7 +/- 10.3; n = 60). In addition, mean tear production was not decreased significantly when comparing each clinical group with the control subjects (17.8 +/- 4.4). However, the relative frequency of eyes with significantly decreased tear production (< 9 mm) was 23.6% in all 144 eyes. In addition, the relative frequency of significantly decreased tear production did not differ (P = 0.52) between eyes of patients with asymptomatic HIV infection (35.7%), LAS or ARC (21.4%), and AIDS (24.1%). In addition, the relative frequency of significantly decreased tear production did not differ (P = 0.30) between eyes of patients with a CD4+ count of more than 400 cells/microliters (23.1%; n = 26), 400 to 200 cells/microliters (31.3%; n = 48), 200 to 50 cells/microliters (14.3%; n = 42), and less than 50 cells/microliters (21.4%; n = 28). CONCLUSION: The authors data show that decreased tear production occurs in approximately 20% to 25% of patients with HIV infection. This increased frequency of decreased tear production is not associated with the CD4+ count, or related to the severity of HIV disease, respectively. The possibility of an autoimmune-like pathogenesis of abnormalities of tear production in patients with HIV infection should be studied intensively.
1458709 Disease activity in systemic lupus erythematosus: report of the Consensus Study Group of t 1992 Sep Using a detailed questionnaire, the cumulative historical and current demographic, clinical and serological data on 704 SLE patients from 29 European centres and 14 countries have been assessed. Ninety-three percent of the patients were Caucasian and the female/male ratio was 10:1. Analysis of the cumulative incidence showed that arthralgia/arthritis (94%), rash (69%), Raynaud's phenomenon (49%), serositis (44%) and renal disease (38%) were the most frequent clinical manifestations. Virtually all the patients (98%) were antinuclear antibody positive, while anti-ds-DNA antibodies (76%), hypocomplementaemia (71%) and anti-Ro(SSA) antibodies (35%) were frequent serological abnormalities. Whilst much of this data is in line with previous reports, it is notable that renal, lung, and central nervous system involvement and the frequency of rheumatoid factor, anti-Sm and anti-RNP antibodies were much lower than in most comparable series in the United States. We assume that ethnic differences and the greater present awareness of lupus could explain this variations. Low dose corticosteroids, non-steroidal anti-inflammatory drugs and anti-malarials were used to treat over half of the patients, 75% of whom were between 15 and 55 years of age. This report offers a useful overview of lupus both clinically and serologically in Europe in the 1990's.
8854150 Etiologies of the sicca syndrome: primary systemic amyloidosis and others. 1996 Aug BACKGROUND: The sicca syndrome has been defined as the occurrence of xerostomia and xerophthalmia. Sjögren's syndrome is the most common cause of the sicca syndrome; however, these two syndromes are not synonymous and there are many potential etiologies of the sicca syndrome. A less known cause of sicca syndrome is amyloidosis that to date has only been reported in the nondermatology literature. OBSERVATIONS: A 79-year-old man with known amyloidosis presented with persistent xerostomia. He had the classic cutaneous findings of periorbital and "pinch" purpura. A labial biopsy showed diffuse deposition of amorphous eosinophilic material surrounding salivary acini. Apple-green birefringence was noted with Congo red staining and the diagnosis was made of amyloidosis in the minor salivary glands causing xerostomia. CONCLUSIONS: The sicca syndrome can be caused by systemic amyloidosis. Because this fact is not in the dermatologic literature, many dermatologists are not aware of this uncommon presentation. The knowledge of the many causes of the sicca syndrome and an understanding of the differences between this and Sjögren's syndromes are important for any dermatologist.
8635291 Nonprecipitating anti-La(SS-B) autoantibodies in primary Sjögren's syndrome. 1996 Jun Anti-La(SS-B) precipitin-negative sera show a restricted epitope recognition and can be easily overlooked in routine laboratory testing. We have therefore determined the prevalence of nonprecipitating anti-La(SS-B) antibodies in patients with primary Sjögren's syndrome and studied their clinical and immunological associations. Clinical details were obtained from 68 patients with primary Sjögren's syndrome, and serum samples were examined by enzyme-linked immunosorbent assay using purified recombinant La, 60-kDa Ro, and 52-kDa Ro proteins and by counterimmunoelectrophoresis. Thirteen patients (19%) were identified with anti-La antibodies which were nonprecipitating. These patients had similar clinical findings to other groups of patients with Sjogren's syndrome, but had significantly lower rheumatoid factor and serum IgG levels than patients with anti-La precipitins. None of the patients with nonprecipitating anti-La antibodies had previously contained anti-La precipitins in their sera. Furthermore, they tended to have lower levels of antibodies directed against denatured 60-kDa Ro (but not 52-kDa Ro) compared with anti-La precipitin-positive patients. Patients with Sjögren's syndrome associated with nonprecipitating anti-La antibodies represent a stable serological and clinical subset in which there appears to be limited diversification of the autoimmune response to the Ro60 and La proteins of the Ro/La ribonucleoprotein.
7677439 Bronchial hyperreactivity in systemic sclerosis patients: influence of associated Sjögren 1995 Aug OBJECTIVE: To determine the frequency and relative risk of bronchial hyperreactivity to methacholine in systemic sclerosis patients with or without associated Sjögren's syndrome. METHODS: A prospective study of 56 patients with systemic sclerosis (42 with the diffuse and 14 with the limited variant; 24 with associated Sjögren's syndrome), 57 with primary Sjögren's syndrome, and 61 healthy controls. RESULTS: Bronchial hyperreactivity (BH) was present in 6.5% of the healthy controls, 25% of the systemic sclerosis patients without associated Sjögren's syndrome, 42.2% of those with primary Sjögren's syndrome, and in 50% of those with systemic sclerosis with associated Sjögren's syndrome. The presence of BH did not correlate with age, disease duration, chest radiograph abnormalities, respiratory, and immunological data. The subgroup of subjects with the limited variant of systemic sclerosis more frequently had associated BH than did those with the diffuse variant of the disease; coexisting Sjögren's syndrome further increased this frequency. CONCLUSIONS: In agreement with previous studies, we have confirmed the high prevalence of bronchial hyperreactivity in primary Sjögren's syndrome; systemic sclerosis likewise appears to be associated with an increased frequency of bronchial hyperreactivity compared with healthy control subjects. There is evidence also that the coexistence of Sjögren's syndrome and systemic sclerosis further increases the frequency and the calculated relative risk of developing bronchial hyperreactivity.
8019045 Fever of unknown origin: a review of 80 patients from the Shin'etsu area of Japan from 198 1994 Feb In this survey involving 10 hospitals, we analyzed data on 80 Japanese patients from the Shin'etsu area (Nagano-ken and Niigata-ken) who were observed for fever of unknown origin (FUO). Our objectives were to identify the underlying causes and the relevant diagnostic methods. Fourteen of the patients died of the underlying illness. The cause of the FUO was infection in 43 patients, allergic or autoimmune disease in 13, neoplasm in 7, miscellaneous causes in 3, and undetermined in 14. FUO was self-limited in 13 patients and persistent in one patient. Methods successfully used to establish the final diagnosis in 66 patients were: evaluation of the clinical course or response to treatment in 16, serologic tests in 12, bacteriologic studies in 10, biopsy in 9, cytologic examination in 6, conventional radiology in 6, necropsy in 3, endoscopy in 2, and biochemical testing in 2.
8164221 Presentation of multicentric Castleman's disease with sicca syndrome, cardiomyopathy, palm 1993 Sep Multicentric Castleman's disease (MCD), or multicentric angiofollicular lymph node hyperplasia, is an uncommon lymphoproliferative disorder which typically present with constitutional symptoms, multicentric lymphadenopathy, hepatosplenomegaly, effusions, and ascites. We describe a patient with several novel manifestations of MCD: sicca syndrome, lacrimal and salivary gland enlargement, cardiomyopathy, and palmar and plantar rash. Treatment of MCD with chlorambucil and prednisone was effective in the short term followup of this patient. MCD merits consideration in patients with lymphadenopathy and multisystem disease, including sicca syndrome, heart failure or rash.
1575588 Damage to the endothelium in Sjögren's syndrome: lack of correlation with antinuclear ant 1992 Mar Serum levels of von Willebrand factor antigen (as an index of damage to the endothelium) and tissue antinuclear antibodies and antibodies to SSA (Ro) (which have been associated with vasculitis) and SSB (La) autoantigens were measured in patients with Sjögren's syndrome. Although high levels of all indices were recorded, there were no intercorrelations. This provides evidence that although tissue autoantibodies may be useful in confirming a diagnosis, they have no place in the pathological course of the disease and so may be an epiphenomenon.
8913660 Acquired hemophilia: a rare complication of Sjögren's syndrome. 1996 Sep Acquired hemophilia is associated with diverse conditions such as post-partum period, autoimmune diseases, cancers, and lymphoproliferative diseases. We report a case of acquired hemophilia occurring in Sjögren's syndrome which was treated with corticoid therapy. A review of the different treatments of acquired hemophilia is presented.
8531347 [Cytokine mRNA expression in the labial glands of patients with Sjögren's syndrome]. 1995 Oct It has been found that the pattern of cytokine mRNA expression was closely associated with the initiation and the progression of the disease process. The cytokine mRNA expression in the labial glands of patients with Sjögren's syndrome was examined by a PCR-based method. Th1 cytokines, such as IL-2 and IFN-gamma, were consistently detected in all patients examined, while Th2 cytokines, such as IL-4 and IL-5, were detected in some cases with strong B cell accumulation in the labial glands. These results suggest that Th1 cytokines are essential in the induction and/or maintenance of the disease, while Th2 cytokines are involved in the progression of the disease process. Other studies were also reviewed and a role of cytokines, produced by T cells and the target organ, was discussed.
7701423 Sjogren's cerebritis complicated by subarachnoid hemorrhage and bilateral superior cerebel 1995 Jan We report a case of chronic Sjogren's cerebritis complicated by bilateral superior cerebellar artery occlusion and diffuse subarachnoid hemorrhage. Although primary Sjogren's syndrome with chronic leptomeningeal involvement has been well documented and a spectrum of central nervous system disease previously outlined, no case of intracranial subarachnoid hemorrhage with major intracranial vessel occlusion has been reported.
8030552 Sjögren's syndrome diagnosis: a comparison of conjunctival and gingival impressions and s 1994 This study reviewed 43 patients with Sjögren's syndrome. All of the patients complained of either dry eye and/or dry mouth. Their clinical records were compared with results of three laboratory diagnostic tests to determine the most sensitive test. Out of 30 patients who complained of both dry eye and dry mouth, 10 had positive conjunctival impressions, 20 had positive gingival impressions and 24 had positive salivary gland biopsies. Of 5 patients who suffered from only dry mouth, 2 had positive conjunctival impressions, 3 had positive gingival impressions and 3 had positive salivary gland biopsies. Of 7 patients with only dry eye complaints, there were 3 positive conjunctival impressions, 5 positive gingival impressions, and 6 positive salivary gland biopsies. Out of 6 healthy controls without complaints of dry eyes/mouth, there were no positive conjunctival impressions, 4 positive gingival impressions, and 5 positive salivary gland biopsies. Gingival impression appears to be nearly as sensitive as salivary gland biopsy in this group of patients suffering from Sjögren's Syndrome.
1343766 Cytokine expression in labial salivary glands from patients with primary Sjögren's syndro 1992 The presence and distribution of 7 cytokines was examined immunohistologically in labial salivary gland (LSG) specimens from patients with primary Sjögren's syndrome (SS) and control subjects. The cytokines interleukin (IL)-1 beta IL-6, tumor necrosis factor (TNF) alpha and interferon (IFN) gamma were identified in defined parts of LSG from patients but not in the corresponding parts of control glands: (a) LSG acinar epithelium expressed IL-1 beta, (b) blood vessels located in both normal LSG stroma and within lymphocytic infiltrates expressed IL-1 beta, IL-6 and IFN gamma, and (c) lymphocytic infiltrates expressed IL-1 beta, IL-6 and TNF alpha. All four cytokines were expressed by salivary ducts within both patient and control specimens, but with generally greater intensity in patients. IL-1 alpha, IL-4 and TNF beta (lymphotoxin) could not be detected in any of the specimens from patients or controls. The locations of cytokines in LSG suggests possible mechanisms of immunologically mediated parenchymal damage in primary SS.
8815861 Human T-cell lymphotropic virus type 1-associated myelopathy, Sjögren syndrome, and lymph 1996 Sep OBJECTIVE: To describe the first case of human T-cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy, Sjögren syndrome, and lymphocytic pneumonitis in a nonendemic area. BACKGROUND: Retroviruses are implicated in the pathogenesis of autoimmune diseases, including Sjögren syndrome. Asymptomatic lymphocytic pneumonitis is prevalent in HTLV-1-associated myelopathy. There are 7 case reports with the combination of HTLV-1-associated myelopathy, Sjögren syndrome, and lymphocytic pneumonitis, all of them in endemic areas for HTLV-1. DESIGN: Case report and literature review. RESULTS: A 40-year-old Creole woman from New Orleans, La, presented with progressive spastic paraparesis and exertional dyspnea. Review of systems revealed chronic complaints consistent with sicca syndrome. She was found to have HTLV-1-associated myelopathy by polymerase chain reaction in the cerebrospinal fluid. Increased levels of SSA, positive results on a Schirmer test, and the findings of biopsy of the minor salivary gland were consistent with Sjögren syndrome. A lung biopsy specimen showed marked lymphocytic infiltration. CONCLUSIONS: The present case raises questions about the role of HTLV-1 in the development of autoimmunity. It also happens to be a unique occurrence in a nonendemic area.
8883092 Evaluation of transparency and barrier function of the cornea by Scheimpflug images. 1996 Two new methods of corneal analysis with Scheimpflug images will be introduced in this paper. The first study was to document the light scattering of the cornea itself using a newly modified Scheimpflug camera with higher magnification. Details of the slit images of the cornea could be evaluated more precisely than those of images taken with an existing camera. The second study was to evaluate the corneal epithelial barrier function by measuring the intensity of the light scattering originating from instilled fluorescein excited through filters. Fluorescein accumulation in the damaged epithelium and diffusion with time into the stromal tissue could be observed in two-dimensional images and the changes could also be documented by measuring the intensity and extent of light scattering. These newly developed methods will be useful for studies of the cornea.
9084292 Salivary gland hypofunction in elderly patients attending a xerostomia clinic. 1995 Dec OBJECTIVES: To investigate the aetiological factors and the prevalence of salivary gland hypofunction (SGH) in patients complaining of xerostomia. DESIGN: Prospective, clinical study. SETTING: Xerostomia clinic in the Department of Oral Medicine at Liverpool University Dental Hospital. SUBJECTS: 100 consecutive patients, aged 60 years or older, referred for investigation of xerostomia. INTERVENTIONS: Patients were asked specific questions concerning their complaint of oral dryness and associated orofacial symptoms. A detailed medical history was recorded and patients underwent a systematic examination of the head, neck and oral structures. All patients underwent haematological, biochemical, immunological investigations, urinalysis and sialometry. Further investigations and referrals to other specialists were undertaken when appropriate. MAIN OUTCOME MEASURES: The causes of xerostomia were established on the basis of clinical and laboratory findings and SGH was defined as an unstimulated whole salivary flow rate of < 0.2ml/min, RESULTS: The causes of xerostomia were identified as: Sjögren's Syndrome (40), iatrogenic (22), psychogenic (14), idiopathic (19), diabetes (1), candidosis (3) and alcohol (1). Sixty five percent of the patients studied had SGH. CONCLUSIONS: This study has shown that 65% of patients whose presenting complaint was xerostomia had objective evidence of SGH. Several aetiological factors were identified, the most common of which was Sjögren's Syndrome. The possibility of associated systemic diseases should be considered when establishing the aetiology of SGH.
7586787 Glomerulonephritis leading to end stage renal disease in a patient with primary Sjögren s 1995 Jul Renal disease as the sole manifestation of extra-glandular involvement is rare in primary Sjögren's syndrome. We report a case of membranous nephritis presenting with proliferative changes including crescents, which led to renal insufficiency in a patient with primary Sjögren's syndrome. The renal involvement was not associated with cryoglobulinemia.