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ID PMID Title PublicationDate abstract
10955331 Clinical course of primary Sjögren's syndrome: salivary, oral, and serologic aspects. 2000 Aug OBJECTIVE: To assess changes in symptoms and signs, salivary function, serologic activity, and disease progression in primary Sjogren's syndrome (SS). METHODS: Treatment records on 80 patients seen in clinic and diagnosed with primary SS by defined criteria were reviewed. Forty-nine patients were evaluated at least twice a minimum of 5 years (mean 7 years) apart. Salivary flow rates from each of the major salivary glands and laboratory values were obtained. A structured interview with questions pertaining to signs and symptoms of primary SS was given and a physical examination was performed. An additional 26 patients completed a followup questionnaire by mail and their current medical records were obtained for review. For this group, the followup period was a mean of 10 years after their initial evaluation. Five patients were deceased. RESULTS: The patients seen twice showed relative stability in their salivary measurements and in their serologic values. The subjective sicca symptoms of oral and ocular dryness among the 75 surviving patients remained prominent. Very few individuals developed another connective tissue disease, therefore evolving into secondary SS. Among the 80 patients, 6 cases of B cell lymphoma were recognized during the followup period. CONCLUSION: Although it is not a benign condition, primary SS is a very slowly progressing disease without rapid deterioration in salivary function, systemic markers of disease activity, or dramatic changes in symptoms, with the exception of a high incidence of lymphoma.
10381045 The prevalence of hyperprolactinemia in patients with primary Sjögren's syndrome. 1999 Jun OBJECTIVE: To assess the prevalence of hyperprolactinemia in 55 patients with primary Sjögren's syndrome (SS), and its clinical significance. METHODS: Concentrations of serum prolactin (PRL) were determined in 55 consecutive patients with primary SS and 110 controls by a fluoroimmunometric assay in a prospective case-control design. RESULTS: The 55 patients with primary SS had higher serum PRL than 110 matching controls (271.5 vs 205.9 mIU/l; p < 0.02), and this difference was most evident in patients diagnosed before the age of 45 years (374.8 vs 245.5 mIU/l; p < 0.05), a patient population characterized by active immunological disease. Serum PRL did not correlate to disease duration, serum immunoglobulin, autoantibodies, or focus score in biopsies from minor salivary glands, but did correlate to score for internal organ disease (r = 0.33, p < 0.05). Two patients were diagnosed as having primary SS 12 years after hyperprolactinemia was first detected, and both patients had aggressive primary SS as indicated by extraglandular manifestations. One of the patients developed primary SS after being treated with bromocriptine, an inhibitor of PRL synthesis, for 12 years. CONCLUSION: Patients with primary SS have moderately increased levels of serum PRL, especially evident in patients diagnosed at a young age with active immunological disease. Serum PRL is correlated to index for internal organ disease, and primary SS may be preceded by hyperprolactinemia for many years.
9776714 Abnormal DNA damage-inducible protein in cells from Sjögren's syndrome patients. 1998 Aug Antinuclear antibodies are commonly found in patients with Sjögren's syndrome. It has been suggested that the development of antinuclear antibodies depends on the activation of the spliceosome and other transcription-related subcellular particles, some of which have recently been shown also to function in DNA-modifying processes, such as DNA repair and V(D)J recombination. These observations add weight to a previously proposed model for the aetiology of Sjögren's syndrome. This includes the abnormal processing of the T-cell receptor and immunoglobulin genes. To test this hypothesis further, the present study on DNA-modifying proteins in Sjögren's syndrome was initiated. Gel-shift experiments using protein extracted from UV-treated Sjögren cells provided evidence of high molecular weight DNA-binding protein in six out of 12 Sjögren patients studied (but not among seven healthy controls). Some Sjögren sera displayed antibodies to protein extracts from cells treated with psoralen plus UVA radiation. These results indicate an abnormal DNA damage-inducible response in Sjögren's syndrome. It may therefore be concluded that alterations in nuclear protein may play a role in the aetiology of Sjögren's syndrome.
9667626 Prevalence of Sjögren's-like syndrome in a cohort of HIV-1-positive patients: descriptive 1998 Jun The aims of the study were (a) to investigate the prevalence of Sjögren's-like syndrome (SLS) in an unselected population of HIV-1-positive patients and (b) to describe the pathology and immunopathology of the labial minor salivary gland biopsy. Seventy-seven HIV-1-positive patients were asked to answer the validated questionnaire of the European preliminary criteria for the classification of Sjögren's syndrome on oral and ocular sicca symptoms. Twenty-six patients gave one positive answer to both ocular and oral symptoms, and of these 14 (hepatitis C virus negative) consented to participate in the study (patients group). Ten age- and sex-matched HIV-1-positive patients with a negative questionnaire constituted the control group. Patients and controls had: (a) Schirmer's test and slit-lamp examination after Rose Bengal staining; (b) parotid gland scanning with technetium; (c) detection of autoantibodies in sera to Ro/SSA and La/SSB; (d) labial salivary gland biopsy (patients group only). The control group gave negative parotid gland scanning and only one gave a positive Rose Bengal staining test. In the patients group, parotid gland enlargement was manifested by three patients and only one gave positive Rose Bengal staining test. Six out of the 14 patients had biopsies identical with Sjögren's syndrome and five of these gave positive parotid gland scanning. In the biopsies of four other patients, mucoid degeneration of the stroma was found. Immunopathology revealed that the predominant cells were T cells with the CD8 phenotype. None of the patient and control sera had autoantibodies to Ro/SSA and La/SSB, whereas all patients had hypergammaglobulinaemia. The overall prevalence of possible SLS in a mixed population of HIV(+) patients (88.3% men and 11.7% women) was 7.79% which is >2.5 times higher than that observed in normal Greek adult females.
11268412 Perturbations of arginine vasopressin secretion during inflammatory stress. Pathophysiolog 2000 Pro-inflammatory cytokines, such as interleukin-1 beta (IL-1 beta), interleukin-6 (IL-6), and tumor necrosis factor-alpha (TNF alpha), released from inflammatory foci, can activate the hypothalamus to produce corticotrophin-releasing hormone (CRH) and arginine vasopressin (AVP). These hypothalamic peptides in synergy increase ACTH production by the pituitary gland and hence corticosteroid (CS) secretion by the adrenal cortices. CS dampens inflammation. The pituitary also produces prolactin (PRL), which is pro-inflammatory, and macrophage inhibitory factor (MIF), which by counteracting the anti-inflammatory and immunosuppressive effects of CS, is pro-inflammatory. Lewis rats develop a variety of induced-autoimmune inflammatory conditions, such as streptococcal cell wall arthritis, whereas the histocompatible F344 Fisher rats are resistant to this condition. Lewis rats have a defective hypothalamic-pituitary adrenal (HPA) response to a variety of hypothalamic stimuli, but have augmented systemic secretion of AVP. Patients with rheumatoid arthritis (RA) have deficient CS with exaggerated PRL responses to inflammatory stimuli. Within inflammatory foci, CRH is pro-inflammatory. AVP, which augments autologous mixed lymphocyte reactions, can replace the IL-2 requirement for gamma IFN production by T cells via V1a receptors, and potentiates primary antibody responses, is also pro-inflammatory. Lewis rats have significantly high plasma levels, hypothalamic content, and in vitro release of AVP in comparison to the inflammatory disease-resistant Fischer rats. Immunoneutralization of AVP attenuates inflammatory responses. In Sprague-Dawley rats, AVP potentiates PRL secretion. Preliminary studies in patients with RA have shown that the circulating levels of AVP are significantly increased, which might be a compensatory response to low CS levels or a result of elevated levels of IL-6 in these patients but could nevertheless contribute to rheumatoid inflammation. A similar observation has been made in patients with ankylosing spondylitis.
11729668 [A case of chronic erosive polyarthritis which developed 14 years after an intestinal bypa 2001 Oct A 38-year-old female patient developed the symptoms and signs of arthritis in the right tarsal joint for the first time after 14 asymptomatic years following an ileo-colic intestinal bypass operation which had been performed as an emergency procedure for acute ileus due to intestinal adhesions caused by the previous abdominal surgery. Her arthritis took a progressively severe course thereafter, primarily involving the joints of both lower extremities, and 13 years after the onset of symptoms she still continued to have active polyarthritis. However, no concomitant skin lesions of any form had been recognized throughout the course of the arthritis. On the articular radiographs erosive changes were evident in the right tarsal joints and also in the MTP joints of both big toes. Laboratory examinations of the serum revealed negative results for rheumatoid factor, circulating immune-complexes, anti-nuclear antibodies, and anti-DNA antibodies, while the serum level of CRP as well as the erythrocyte sedimentation rate were elevated. Other routine laboratory tests were all unremarkable, and neither HLA-B 27 nor HLA-DR 4 were positive. Therapeutic drug regimens consisting of NSAIDs, oral as well as intraarticular steroids, DMARDs, methotrexate, and combinations of these drugs were unsuccessful in controlling the severe symptoms of the arthritis. In view of this, a revision operation of intestinal bypass was performed 13 years after the onset of the arthritis. After the revision the severe pain of the arthritis began to subside gradually, and 1 year and 6 months later the patient achieved complete remission of the arthritis, and her CRP and ESR values returned to normal.
10615976 Long-term results of the GSB III elbow arthroplasty. 1999 Nov Between 1978 and 1986, 59 patients received a GSB III elbow prosthesis, six of them in both elbows. Rheumatoid arthritis (RA) was the underlying cause in 51 of the patients and post-traumatic osteoarthritis (PTOA) in eight. Of these, 24 patients (28 prostheses) have since died; two, both operated on bilaterally, had had their implants for more than ten years and had already been assessed for inclusion in the long-term follow-up. Two patients, each with one elbow prosthesis, have been lost to follow-up and three males who are still living (two with PTOA, one with juvenile RA) had their prosthesis removed before ten years had elapsed. The remaining 32 patients (28 RA, 4 PTOA) with 36 GSB III elbows were examined clinically and radiologically after a mean period of 13.5 years. Pain was considerably reduced in 91.6%. Mobility was increased by 37 degrees in those with RA and by 67 degrees in those with PTOA. There were three cases of aseptic loosening and three of deep infection. The main complication was disassembly of the prosthetic component in nine elbows (13.8%). This last group included two patients with postoperative fractures unrelated to the operative technique and one with neuropathic arthritis. Ulnar neuritis occurred in two patients. Since 87.7% of all the GSB III prostheses implanted in this period remained in situ, our results are comparable with those for hip and knee arthroplasty.
10395698 Paradoxical effects of adenovirus-mediated blockade of TNF activity in murine collagen-ind 1999 Jul 15 Collagen-induced arthritis (CIA) is an experimental model of arthritis widely used to dissect the pathogenesis of human rheumatoid arthritis and to identify potential therapeutic targets. Among these, TNF-alpha has been recognized to play an important role. Here we investigate the feasibility and therapeutic efficacy of prolonged blockade of TNF-alpha activity through the adenovirus-mediated gene delivery of a dimeric chimeric human p55 TNFR-IgG fusion protein and compare it to protein therapy in established CIA. A single i.v. administration of the replication-deficient adenovirus yielded microgram serum levels of the chimeric fusion protein and ameliorated CIA for 10 days. Subsequently, benefit was lost and a rebound to greater inflammatory activity was observed despite the continual presence of bioactive TNFR fusion protein. A similar trend was also observed in mice injected directly with comparable amounts of a human TNFR-IgG fusion protein, whereas the administration of a control adenovirus-encoding beta-galactosidase or of a control human IgG1 protein did not significantly affect the disease course. The mechanisms of the rebound of CIA were investigated, and augmented Ab response to collagen type II and TNFR were identified as potential causes. Our results confirm the feasibility of adenovirus-mediated gene delivery of cytokine inhibitors in animal models of autoimmune diseases for investigational purposes and highlight the importance of prolonged studies. Further investigations are needed to optimize ways of exploiting the potential of adenoviral gene therapy in RA.
9817866 Rubella virus and chronic joint disease: is there an association? 1998 Dec Synovial fluid samples and/or biopsies from 79 patients with various chronic inflammatory joint diseases or traumatic joint injury were tested for rubella virus (RV) in order to confirm or refute results from other studies that suggested RV as a cause of chronic inflammatory joint disease. Sixty-eight of the 72 patients tested had RV antibodies. RV RNA was detected by reverse transcription-PCR in the synovial fluid cells from two patients. RV was also isolated by cell culture from the synovial fluid of one of these two patients. This patient was a 42-year-old female with common variable immune deficiency and Mycoplasma hominis arthritis, while the other was a 68-year-old female with rheumatoid arthritis. While these results fail to confirm that RV is associated with chronic inflammatory joint disease, they suggest that RV may persist within a joint and be reactivated when cell-mediated immunity is suppressed.
11346130 The clinical potential of matrix metalloproteinase inhibitors in the rheumatic disorders. 2001 Rheumatoid arthritis (RA) and osteoarthritis are chronic diseases that result in cartilage degradation and loss of joint function. Currently available drugs are predominantly directed towards the control of pain and/or the inflammation associated with joint synovitis but they do little to reduce joint destruction. In the future, it will be important to have drugs that prevent the structural damage caused by bone and cartilage breakdown. In this review, we will outline the structure and function of cartilage and the key features of matrix metalloproteinases (MMPs), enzymes involved in joint destruction. We will present evidence for the role of MMPs in RA and osteoarthritis, and describe the potential of synthetic inhibitors to control MMP activity and so prevent joint destruction. MMPs are able to cleave all components of the cartilage matrix. Regulation of MMPs is aberrant in osteoarthritis and RA, and MMPs have been implicated in the collagen breakdown that contributes to joint destruction in these diseases. Synthetic MMP inhibitors have been developed. In animal models of osteoarthritis and/or RA, these agents have shown chondroprotective effects. However, results from clinical trials in RA have been equivocal, with some studies being terminated because of lack of efficacy or safety concerns. Nevertheless, this approach remains promising. Increased understanding of the structure, regulation and function of individual MMPs may lead to more effective strategies, and approaches aimed at multiple steps of the pathogenesis of arthritis may be needed to break the chronic cycle of joint destruction.
10628590 Pharmacology and efficacy of cyclooxygenase (COX) inhibitors. 1999 Dec 13 Discussion of the pharmacology and efficacy of cyclooxygenase (COX) inhibition requires careful attention to the methodology used for making those comparisons. In vitro analysis of COX-2/COX-1 ratios requires consideration of the target tissues being examined, the details of incubation periods, outcome measures, and other parameters. Application of those data to the in vivo situation requires careful cognizance of pharmacokinetic factors such as clearance and serum half-life, protein binding, lipophilicity, and pKa. Proceeding from in vitro studies to efficacy studies also requires a careful consideration of methodology. Studies must be well controlled, of sufficient duration (> or = 12 weeks for rheumatoid arthritis), and, importantly, require equipotent dosing regimens. Equipotence may be hard to prove because in vitro and animal studies cannot predict equipotence in humans. Clinical studies are needed to prove equipotence. The obverse of efficacy is toxicity, and only with the knowledge of equipotent doses can toxicity be appropriately compared. Based on approximate equipotence, highly selective COX-2 inhibitors may cause fewer peptic ulcers and bleeds than drugs that are COX-1 preferential. COX-2 preferential (not highly selective) drugs may occupy a place between the two.
9523387 Third-degree atrioventricular block in a patient under chloroquine therapy. 1998 Jan The first case of chronic cardiac toxicity due to an antimalarial agent was reported in 1971 and since then several cases of heart failure, restrictive cardiomyopathy or atrioventricular block have been ascribed to this family of drugs. We report the case of a 43-year-old woman who developed juvenile chronic arthritis at the age of ten, followed in adulthood by sero-positive rheumatoid arthritis. In 1980 she was put under chloroquine sulfate (hydroxychloroquine was not available) in a dose of 200 mg/d (152.66 mg of chloroquine), with 10 mg/day of prednisone. She developed myalgia and increased skin pigmentation, but disregarded recommendations that these symptoms required discontinuation of chloroquine therapy. She was lost to follow-up, but continued the chloroquine therapy of her own accord. In December 1993, she developed a third-degree atrioventricular block with syncopes requiring implantation of a pacemaker. The rare but well-documented myopathy induced by antimalarial agents can produce early severe lesions of the cardiac muscle, which may have a predilection for the interventricular septum, explaining the risk of atrioventricular block. Although histologic studies were not performed in our patient, the clinical evidence of toxicity, absence of underlying heart disease and fairly young age of the patient pointed to chloroquine toxicity. Periodic cardiac investigations including electrocardiography may be warranted in patients under antimalarial therapy.
9125260 Primary juvenile fibromyalgia: psychological adjustment, family functioning, coping, and f 1997 Apr OBJECTIVES: 1) To determine the importance of psychological adjustment and family functioning in primary juvenile fibromyalgia by assessing these factors in children with fibromyalgia and in their parents, compared with children with juvenile rheumatoid arthritis (JRA) and with pain-free control children and their parents. 2) To examine which of these factors predict functional disability. METHODS: Fifteen children in each of the 3 study groups, and their parents, completed self-report questionnaires and pain diaries. A medical evaluation of each child was performed, including assessment of tender points by palpation and by dolorimetry. RESULTS: All children in the fibromyalgia group met the Yunus and Masi criteria for fibromyalgia, and 11 met the American College of Rheumatology criteria. There were almost no significant group differences in the children's or parents' psychological adjustment, ratings of family functioning, or coping strategies. Significant group differences in functional disability, pain, fatigue, tender point threshold, and control point tolerance were found. A number of the psychological adjustment, pain, fatigue, and coping variables were significantly associated with functional disability. CONCLUSION: The notion that fibromyalgia is a psychogenic condition is not supported by these results. Fibromyalgia is associated with disability of a magnitude comparable to that of other chronic pain conditions. Disability among children with fibromyalgia or JRA is a function of the children's psychological adjustment and physical state, and of the parents' physical state and method of coping with pain.
10648038 FK506 attenuates developing and established joint inflammation and suppresses interleukin 2000 Jan OBJECTIVE: To determine the efficacy of therapeutic administration of FK506 (Tacrolimus) in suppressing developing and established joint inflammation, proinflammatory cytokine expression, and nitric oxide (NO) production in peptidoglycan-polysaccharide (PG/PS) induced experimental polyarthritis in rats. METHODS: Chronic joint inflammation was induced by intraperitoneal injection of PG/PS, and joint inflammation was quantified using arthritis index and paw volume. Serum and joint levels of interleukin 6 (IL-6) were measured by bioassay and Western blot analysis respectively, and serum levels of NO production were determined by the Griess procedure and the expression of the inducible isoform of nitric oxide synthase (i-NOS) in the joints was determined by Western blot analysis. RESULTS: Arthritis induced by PG/PS is biphasic, progressing through an initial acute phase and a remission phase, which is followed by a persistent chronic phase. Daily administration of FK506 initiated during the remission phase significantly attenuated the onset and development of chronic joint inflammation. We observed a significant reduction in joint inflammation and swelling, an apparent suppression of pannus development, and minimal erosive damage to the articular cartilage and subchondral bone. Fully established chronic joint inflammation was also ameliorated by daily administration of FK506. Joint swelling and inflammation was significantly reduced by 5 days post-treatment with FK506 and the erosive activity associated with the pannus appeared diminished. The elevated expression of IL-6 and NO characteristic of chronic joint inflammation in the serum and in joint tissue was significantly reduced by FK506 treatment. CONCLUSION: Therapeutic administration of FK506 has a profound antiinflammatory effect on the development of the chronic, erosive arthritis induced by PG/PS. This attenuation in joint inflammation was associated with suppression of IL-6 and NO production systemically and locally in the joints. Our data suggest that FK506 may be effective in the treatment of chronic joint inflammation associated with rheumatoid arthritis.
11490518 Immunogenetics of Sjögren's syndrome. 1998 Feb The etiology for SS remains unknown where multifactorial influences contribute to the pathogenesis of subsequent development of the disease. The genetic influence is also multifactorial and the data suggests a familial component indicative of autosomal-dominant genes as well as genetic contributions associated with class-I and class-II HLA alleles. Various auto-antibodies found with increased frequency in SS (anti-Ro and anti-La) were shown to be associated with HLA class-II alleles at the DQA1 and DQB1 loci that were also found to have in common specific amino acid residues (10). Ethnic groups have also been studied and show varying HLA associations with primary SS. Multiple ethnic groups, however, share a DQ allele supporting the idea that the majority of SS patients carry a common allele which may predispose to primary SS. In some cases, the HLA-DR antigen may be induced and cause to appear on epithelial cells where they present antigen to CD 4+ T-cells, which then go on to aid in the destruction of salivary gland epithelial cells specifically. The further elucidation of disease associations as well as possible immunogenetic pathogenic mechanisms may help to explain the causes for the development of various autoimmune diseases such as Sjögren's Syndrome. This may eventually result in the ability to immunomodulate these abnormal immune responses. In so doing, an approach to treatment by genetic engineering may also be possible once a further understanding of the genetic influences and mechanisms in the causation of autoimmune disease is further elucidated.
11157873 The role of Fas-Fas ligand-mediated apoptosis in autoimmune lacrimal gland disease in MRL/ 2001 Feb PURPOSE: MRL/MpJ mice spontaneously develop lacrimal gland inflammation and are a model for the human disorder Sjögren's syndrome. MRL/MpJ-lpr/lpr (MRL/lpr) and MRL/Mp-+/+ (MRL/+) mice are congenic substrains, which differ only by a single autosomal recessive gene, the lpr mutation. This mutation results in defective Fas protein, defective lymphocytic apoptosis, and accelerated autoimmune lacrimal gland disease in MRL/lpr mice. We evaluated apoptosis in the lacrimal glands of MRL/lpr and MRL/+ mice. METHODS: Inflammatory cells in the lacrimal glands of MRL/lpr and MRL/+ mice were evaluated for apoptosis with TUNEL staining and Fas and Fas ligand expression with immunohistochemistry. RESULTS: MRL/lpr mice had a greater percentage of the lacrimal gland replaced by inflammatory infiltrate (30.3% +/- 7.0%) than did MRL/+ mice (13.0% +/- 3.0%, P = 0.02). However, similar amounts of lymphocytic apoptosis were present in the lacrimal glands of MRL/lpr and MRL/+ mice. The mean number of apoptotic cells per unit area of inflammation was 23.8 +/- 2.4 in MRL/lpr mice and 24.6 +/- 6.0 in MRL/+ mice (P = 0.91). Fas expression was absent on lymphocytes in MRL/lpr mice but was present on lymphocytes in MRL/+ mice. Fas ligand expression was present on epithelial structures in both substrains. CONCLUSIONS: The accelerated lacrimal gland disease inflammation in MRL/lpr mice does not appear to be due to decreased apoptosis in the microenvironment of the lacrimal gland of MRL/lpr mice. It appears that in MRL/lpr mice there is defective extrathymic lymphoid apoptosis, permitting a relatively greater expansion of autoreactive T cells, which subsequently invade the lacrimal gland.
10515640 Zidovudine in primary Sjögren's syndrome. 1999 Sep OBJECTIVE: To evaluate the efficacy of the administration of zidovudine (AZT), an antiretroviral drug, in patients with primary Sjögren's syndrome (SS). METHODS: Seven female patients (age 57 +/- 8.6 yr) with primary SS were enrolled in an open, uncontrolled trial of AZT (250 mg b.i.d.) for the treatment of primary SS. The efficacy variables were oral and ocular dryness symptoms, fatigue, tender points, physician's and patient's global assessments (GA), ocular function tests (fluorescein tear break-up time, Schirmer's test, Rose Bengal staining) and laboratory parameters [erythrocyte sedimentation rate (ESR), serum IgG, IgA and IgM]. RESULTS: A significant improvement was observed in all subjective manifestations, as well as the objective parameters of ocular dryness. The treatment was well tolerated, except for mild and transitory gastrointestinal disturbances in 6/7 patients. Laboratory parameters did not change significantly. The clinical benefit persisted in 5/7 patients 1 month after the end of therapy. CONCLUSION: AZT seems to be effective and well tolerated in patients with primary SS.
10451983 [A study of immunopathology on salivary gland of Sjögren's syndrome]. 1997 Sep OBJECTIVE: To understand the mechanism and pathogenesis of Sjögrens syndrome (SS) and to provide theoretical basis for the treatment of SS. METHOD: Salivary gland lymphocytes (SGL) from 20 patients with SS were analyzed by using immunohistochemical avidin-biotin-peroxidase complex (ABC) method. RESULT: A predominance of T cells was found in SGL with the majority belonging to the CD4+(TH), and the CD8+(TS) subset was obviously decreased, while the CD4+/CD8+ ratio was considerably increased. Most lymphocytes expressed HLA-DR antigen in SS. The deficiency of Ts and the overactivity of TH would result in autoantibody production, that would contribute to the reaction of autoimmune. CONCLUSION: The immune hyperfunction at exocrine glands of SS is the direct cause of the pathogenesis of SS. SS is further proved to be an autoimmune disease.
10381047 Cerebral white matter lesions in primary Sjögren's syndrome: a controlled study. 1999 Jun OBJECTIVE: To determine the prevalence of neurological and magnetic resonance imaging (MRI) abnormalities in a well defined population of unselected patients with primary Sjögren's syndrome (SS) and age and sex matched healthy patients. METHODS: Thirty patients with SS and 29 age and sex matched controls were examined by a neurologist and subsequently underwent MRI scanning with a 1.0 Tesla Siemens Impact MR scanner. Scans were graded by a neuroradiologist blinded to the clinical status of each subject. The number and location of white matter lesions > 3 mm in long axis (to exclude non-specific perivascular changes) were recorded for each subject. RESULTS: There was a significant increase in lesions detected by MRI in SS patients versus controls (p = 0.02) including deep white matter lesions (p = 0.03) and subcortical white matter lesions (p = 0.02). The presence of white matter lesions did not correlate with serum IgG or rheumatoid factor levels, or with presence of anticardiolipin antibodies. No subjects had symptoms or signs of serious neurological disease including multiple sclerosis, and corpus callosal lesions commonly seen in multiple sclerosis were notably absent in this study. CONCLUSION: Cerebral white matter lesions detected by MRI are more frequent in patients with primary SS than control subjects, yet do not appear to be associated with significant clinical manifestations. Although the pathological nature of these lesions is yet to be defined, their presence should not be over-interpreted.
9647028 Autoimmune conditions associated with primary biliary cirrhosis: response to ursodeoxychol 1998 Jun OBJECTIVES: A variety of autoimmune conditions occur in association with primary biliary cirrhosis. Among these conditions are sicca syndrome, Raynaud's phenomenon, arthritis, and Hashimoto's thyroiditis. Information is sparse regarding the prevalence and natural history of these conditions when associated with primary biliary cirrhosis and their response to ursodeoxycholic acid treatment. We evaluated the prevalence, natural history, and response to ursodeoxycholic acid therapy of these conditions coassociated with primary biliary cirrhosis. METHODS: One hundred-eighty patients with primary biliary cirrhosis, enrolled in a prospective randomized controlled trial of ursodeoxycholic acid (13-15 mg/ kg/day), were included. Patients were assessed at study entry and annually. RESULTS: At entry, 77/180 patients (43%) had one of the four conditions, and 18/180 patients (10%) had two or more conditions. Sicca syndrome was the most common, occurring in 58/180 patients (32%). After 2 yr, there was no difference between the treatment groups with regard to resolution or spontaneous onset of these autoimmune features. Sicca syndrome was the most common spontaneously developing condition (9% per yr). Sicca syndrome was the most common associated autoimmune condition, present in one-third of our patients. The associated conditions tended to improve over time, with a low rate of spontaneously developing these conditions. Although ursodeoxycholic acid therapy leads to improvement in the underlying liver disease, it did not appear to influence either the development or resolution of these autoimmune features. CONCLUSIONS: Although ursodeoxycholic acid is beneficial in the treatment of primary biliary cirrhosis, it had no measurable effect on the autoimmune conditions coassociated with the disease.