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ID PMID Title PublicationDate abstract
17219411 Amelioration of inflammation, angiogenesis and CTGF expression in an arthritis model by a 2007 May OBJECTIVE: To evaluate the effect of a thrombospondin 1 (TSP1)-derived peptide on inflammation and angiogenesis in an animal model of erosive arthritis and to assess the relationship between TSP1 and connective tissue growth factor (CTGF) in the pathophysiology of rheumatoid arthritis. METHODS: Erosive arthritis in Lewis rats was induced by peptidoglycan-polysaccharide (PG-PS). Animals were divided into four groups: (1) negative control and groups receiving, (2) no treatment, (3) treatment with a TSP1-derived peptide, and (4) treatment with a scrambled peptide. Samples obtained from ankle joint, spleen and liver were studied using histology, histomorphometry, immunohistochemistry and RT-PCR. RESULTS: Histological data indicated that the TSP1-derived peptide treatment decreased neovascularization, leukocyte infiltration and thickening of the synovial lining of the joint, and reduced granuloma formation in the spleen and liver when compared to control groups. Higher concentrations of CTGF and TSP1 proteins were observed in the affected areas of animals which did not receive TSP1-derived peptide treatment. Also, immunofluorescence and RT-PCR analyses showed an increase in CTGF protein expression and regulation, respectively, in the tissues of untreated animals when compared to the TSP1-derived peptide treated animals. By immunofluorescence, TSP1 expression was decreased in the TSP1-derived peptide treated animals. Moreover, macrophage/monocyte-specific staining revealed a decrease in cell infiltration in the articular tissue of the TSP1-derived peptide treated animals. CONCLUSION: Both inflammation and angiogenesis were decreased after TSP1-derived peptide treatment indicating a potential pathway by which TSP1 interaction with neutrophils induces CTGF in RA affected tissues.
17713674 The use of stem cells for the treatment of autoimmune diseases. 2007 Dec Autoimmune diseases constitute a heterogeneous group of conditions commonly treated with anti-inflammatory, immunosuppressant and immunomodulating drugs, with satisfactory results in most cases. Nevertheless, some patients become resistant to conventional therapy. The use of high doses of drugs in such cases results in the need for bone marrow reconstitution, a situation which has stimulated research into the use of hematopoietic stem cells in autoimmune disease therapy. Stem cell transplantation in such diseases aims to destroy the self-reacting immune cells and produce a new functional immune system, as well as substitute cells for tissue damaged in the course of the disease. Significant results, such as the reestablishment of tolerance and a decrease in the recurrence of autoimmune disease, have been reported following stem cell transplantation in patients with autoimmune disease in Brazil and throughout the world. These results suggest that stem cell transplantation has the potential to become an important therapeutic approach to the treatment of various autoimmune diseases including rheumatoid arthritis, juvenile idiopathic arthritis, systemic lupus erythematosus, multiple sclerosis, systemic sclerosis, Crohn's disease, autoimmune blood cytopenias, and type I diabetes mellitus.
24019592 Interaction between the immune system and bone metabolism: an emerging field of osteoimmun 2007 Jun The interaction between the immune and bone systems has long been appreciated, but recent research into arthritis as well as various bone phenotypes found in immune-related knockout mice has highlighted the importance of the interplay and the interdisciplinary field called osteoimmunology. In rheumatoid arthritis, IL-17-producing helper T cells (TH17) induces receptor activator of NF-κB ligand (RANKL), which stimulates osteoclast differentiation through nuclear factor of activated T cells (NFAT)c1. Accumulating evidence suggests that the immune and skeletal systems share cytokines, signaling molecules, transcription factors and membrane receptors. In addition, the immune cells are maintained in the bone marrow, which provides a space for mutual interaction. Thus, bone turns out to be a dynamic tissue that is constantly renewed, where the immune system participates to a hitherto unexpected extent. This emerging field of osteoimmunology will be of great importance not only to the better understanding of the two systems but also to the development of new treatment for rheumatic diseases.
16542122 [Tumor necrosis factor blocking agents: a review. Part I: Clinical efficacy evaluation]. 2006 Jan Blockade of tumor necrosis factor with monoclonal antibodies, has emerged as one of the most promising therapies in some autoimmune conditions as rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis and Crohn s disease. They have shown effectiveness on reducing symptoms and modifying the progression of the disease. However, they disrupt the balance of inflammatory and immune responses and some risks associated with TNF-blockers have become apparent. The purpose of this article is to review the evidence about benefits and risk associated with the use of TNF-blockers in approved indications and to provide practical recommendations for its use in the management of these conditions.
17994192 Tumor necrosis factor-a antagonist-induced psoriasis: yet another paradox in medicine. 2008 Mar The therapeutic use of tumor necrosis factor a (TNFa) antagonists has added a highly effective treatment in the field of inflammatory musculoskeletal, skin, and bowel diseases. Most of the side effects of these very potential agents, like infections or skin reactions, were predictable; the development of psoriatic lesions was not, as they are very successfully used to treat psoriasis and psoriatic arthritis, too. There is a number of cases of anti-TNFa-induced psoriatic lesions in the literature, some of them developing with the use of two agents in the same patient, clearly suggesting a class effect. We report an additional series of 12 cases from a total of 300 patients (>800 patient years) and hypothesize on several mechanisms for the explanation of this paradoxical phenomenon namely, local action of TNF, dysregulation of regulatory T cells, or, finally, imbalance between TNF and interferon-a locally. Further studies are needed to elucidate the exact pathogenesis of these manifestations, so that the use of these agents will not only have changed the course of diseases like rheumatoid arthritis or ankylosing spondylitis but may also aid our in depth understanding of the underlying process of disease.
17921182 Inflammasomes and rheumatic diseases: evolving concepts. 2008 Oct The realisation that the production of inflammatory cytokines in inflammatory rheumatic diseases may be induced by non-infectious endogenous signals has encouraged researchers to explore mechanisms of innate immunity and their contribution to the pathogenesis of these diseases. The nucleotide-binding and oligomerisation domain (NOD)-like receptors (NLRs) sense pathogens, products of damaged cells or endogenous metabolites and could potentially be involved in the initiation, amplification and progression of the inflammatory response in rheumatic diseases. NLRs are involved in the regulation of innate immune responses with some of them promoting the activation of inflammatory caspases within multiprotein complexes, called inflammasomes. A typical inflammasome consists of a sensor, an NLR protein, an adaptor protein such as ASC (for apoptosis-associated speck-like protein containing a caspase recruitment domain (CARD)) and an effector protein that is a caspase that activates pro-inflammatory cytokines such as interleukin (IL)1beta and IL18. Recent data suggest a role of the inflammasome in the pathogenesis of autoinflammatory as well as inflammatory rheumatic diseases such as juvenile chronic arthritis, adult onset Still disease, rheumatoid arthritis and gout. Modulation of these pathways may be a potential therapeutic target for inflammatory rheumatic diseases.
17083755 Complexities in defining remission in rheumatic diseases. 2006 Nov The rheumatology community has devoted increasing attention to the subject of remission over the past 2 decades, on the basis of greater appreciation of the long-term severity of inflammatory rheumatic diseases and availability of new therapies and approaches to improve outcomes. Nonetheless, description of remission in rheumatic diseases is complex, compared to many nonrheumatic diseases. Recognition of remission requires a set of measures or an index rather than a single "gold standard." Spontaneous remission is not infrequent in people with early inflammatory arthritis, including some who may meet criteria for rheumatoid arthritis (RA) over less than a few months, and may be confused with a drug-induced remission. Remission may be transient in many patients over short periods, and the length of time required to maintain remission status varies in different reports. Maintenance of a state of remission in autoimmune diseases that result from dysregulatory processes, rather than invasion of foreign cells or toxins, generally requires ongoing therapy indefinitely. Patients who have organ damage or functional disability may be described as "in remission," although they are free of disease activity only, but not necessarily free of disease consequences. A status of "low disease activity" or "near remission" with 70% to 90% of the features of an ideal remission may be adequate for many people with rheumatic diseases to avoid risks that may be required to reach 100% remission status. Thus, the subject of remission remains under active discussion in the rheumatology community.
16715039 [Prolactin in connective tissue diseases]. 2006 This paper presents interactions between prolactin (PRL) and the immune system. We describe the role of PRL in the pathogenesis of rheumatic diseases, particularly connective tissue diseases, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), primary Sjögren's syndrome, systemic sclerosis, polymyalgia rheumatica, and seronegative arthritis. We present current opinion on the mechanisms responsible for hyperprolactinemia in SLE patients and the association between hyperprolactinemia and SLE activity and organ involvement. The role of dopamine receptor agonists in the treatment of connective tissue diseases is discussed.
16582691 Role of adhesion molecules in synovial inflammation. 2006 May PURPOSE OF REVIEW: The ability of cells to adhere to other cells and extracellular matrix (ECM) through cellular adhesion molecules (CAMs) is central to tissue remodeling and inflammation. This review discusses the potential role of CAMs in development of synovial inflammation through regulating the recruitment of inflammatory cells via endothelial-leukocyte interactions, the organization and activation of leukocytes in the synovial sublining, and the formation and behavior of the hyperplastic synovial lining. RECENT FINDINGS: Over the past several years valuable insight has been gained into the role of cell-cell and cell-ECM adhesive interactions in synovial organization and inflammation. Recently, cadherin-11 was identified on fibroblast-like synoviocytes and has been demonstrated to play a central role in synovial lining organization. Furthermore, studies using animal models of inflammatory arthritis have demonstrated critical roles for E- and P-selectins, CD11a/CD18 [lymphocyte function-associated antigen (LFA)-1], alpha4beta1 integrin, and cadherin-11 in the development of synovial inflammation. SUMMARY: Cell-cell and cell-ECM interactions through CAMs play an important role in synovial inflammation. Future studies of CAMs are needed to define more thoroughly their role in synovial inflammation and their potential as therapeutic targets in the treatment of rheumatoid arthritis and related inflammatory arthritic conditions.
18668606 Increased angiogenesis and cellular proliferation as hallmarks of the synovium in chronic 2008 Aug 15 OBJECTIVE: To characterize histologic alterations and inflammatory infiltrates in the synovium of patients with chronic septic arthritis (SeA). METHODS: Synovial membranes from patients with SeA (9 specimens; disease duration >4 weeks) were compared with specimens from patients with septic joint prosthesis loosening (septic total arthroplasty [SeTA]; 9 specimens), rheumatoid arthritis (RA; 25 specimens), osteoarthritis (25 specimens), and normal histology (10 specimens). Sections were stained with hematoxylin and eosin, tissue gram stain, and immunostains for von Willebrand factor (vWF; blood vessels), Ki-67 (dividing cells), CD15 (neutrophils), CD3 (T cells), CD20 (B cells), CD38 (plasma cells), and CD68 (macrophages). RESULTS: Gram stains were positive in all SeA and SeTA specimens. Mixed polymorphonuclear and mononuclear infiltrates predominated in SeA and SeTA. SeA could be differentiated from RA by higher densities of CD15+ cells (SeA:RA ratio 6.5:1; P < 0.001) or Ki-67+ cells (ratio 2.1:1; P = 0.012). The inflammatory infiltrate of SeTA was similar to SeA but contained fewer CD3+ cells (SeTA versus SeA 0.26:1; P = 0.009) and a tendency toward fewer CD20+ cells. Mean vascular density was strikingly increased in SeA (SeA:normal ratio 3.0:1; P < 0.001) and, to a lesser extent, in the vascularized areas of the SeTA specimens (SeTA:normal ratio 1.9:1). Ki-67/CD31 double immunostains demonstrated proliferating endothelial cells in small subintimal blood vessels, suggesting angiogenesis. Receiver operating characteristic curve analysis identified higher densities of CD15+ and Ki-67+ cells and vWF-positive vessels as histologic markers that differentiated SeA from RA. CONCLUSION: This first analysis of the synovium in patients with chronic pyogenic arthritis identified dramatic neovascularization and cell proliferation, accompanied by persistent bacterial colonization and heterogeneous inflammatory infiltrates rich in CD15+ neutrophils, as histopathologic hallmarks.
16646024 Intraarticular corticosteroids decrease synovial RANKL expression in inflammatory arthriti 2006 May OBJECTIVE: Intraarticular corticosteroids are frequently used as successful adjuvant therapy for inflammatory arthritides, but little is known about their effects on molecules that regulate bone biology. We undertook this study to investigate the effect of intraarticular corticosteroids on the synovial expression of RANKL and osteoprotegerin (OPG). METHODS: We evaluated RANKL, OPG, and surface marker expression by immunohistochemical methods in synovial knee biopsy samples obtained from 13 patients with inflammatory arthritis before and 2 weeks following intraarticular injection of triamcinolone hexacetonide. We further investigated the effect of dexamethasone (DEX) on RANKL expression by lymphocytes from rheumatoid arthritis synovial fluids (RA SF), using flow cytometric analysis. Finally, we evaluated the in vitro effect of DEX on RANKL and OPG expression in osteoblast-like cells, by Western blotting. RESULTS: Intraarticular corticosteroids induced a decrease in the number of synovial T cells without influencing the number of macrophages, evaluated as both CD68+ and CD163+ cells. This change was paralleled by a decrease of synovial RANKL expression with a concomitant reduction of the RANKL:OPG ratio. DEX down-regulated RANKL expression on lymphocytes derived from RA SF. Moreover, in vitro pretreatment of osteoblast-like cells with tumor necrosis factor favored an antiresorptive effect of DEX treatment through a similar down-regulation of RANKL expression. CONCLUSION: The decrease in inflammation attributed to intraarticular corticosteroids is accompanied by down-modulation of bone destruction markers. These findings offer a rationale for the beneficial effect of corticosteroids on joint erosion in arthritis.
16941192 Enhanced MR imaging of tenosynovitis of hand and wrist in inflammatory arthritis. 2006 Nov OBJECTIVE: The purpose of this study is to describe the appearance of tenosynovitis in various tendon groups in the wrist and hand and to compare MR enhanced and non-enhanced imaging evaluation of tenosynovitis of hand and wrist in inflammatory arthritis. DESIGN AND PATIENTS: We reviewed 72 MRI studies of hands and wrists, including coronal, axial and sagittal images in 30 consecutive patients with inflammatory arthritis and tenosynovitis. We compared the degree of synovitis on T2-weighted vs contrast-enhanced T1-weighted images, using a predetermined scale. We also measured the extent of tenosynovitis in three dimensions. The tendons were assigned to volar, dorsal, ulnar and radial groups in the wrist and to extensor, flexor and thumb groups in the hand. Degree of tenosynovitis (graded 0-3), cross-sectional area and volume of the inflamed synovium in various tendon groups were then compared by statistical analysis. RESULTS: Review of the medical records revealed the following diagnoses in our patient population: rheumatoid arthritis (n=16), unspecified inflammatory polyarthritis (n=9), psoriatic arthritis (n=2), CREST syndrome (n=1), systemic lupus erythematosus (n=1), paraneoplastic syndrome with arthritis (n=1). The average T2 brightness scores and post-gadolinium enhancement scores were 1.0 and 1.7, respectively (P<0.001) in the wrist studies. The average T2 brightness scores and post-gadolinium enhancement scores were 0.7 and 1.4, respectively (P<0.001) in the hand studies. The average sensitivity of T2-weighted imaging for detection of tenosynovitis was 40% in the hand and 67% in the wrist tendons, when contrast-enhanced images were used as a reference. Carpal tunnel flexor tendons were the most frequently affected tendons of the wrist. The most frequently affected tendons of the hand were second and third flexor tendons. The hand flexors demonstrated higher degrees of enhancement and larger volumes of the inflamed tenosynovium than did the hand extensors and tendons of the thumb. CONCLUSION: Enhanced MR imaging of the hand and wrist is a superior technique for detection of tenosynovitis. We observed carpal tunnel flexor tendons to be the most frequently affected tendons of the wrist. The flexor tendons of the second and third digits were the most frequently affected tendons of the hands. Higher contrast-enhancement scores and inflammation were noted in the hand flexor than in the extensor tendons.
16855167 DHEA metabolism in arthritis: a role for the p450 enzyme Cyp7b at the immune-endocrine cro 2006 Jun For dehydroepiandrosterone (DHEA) both immunosuppressive and immuno-stimulating properties have been described. The immunosuppressive effects may be explained by the conversion of DHEA into androgens and/or estrogens. The described immuno-stimulating effects of DHEA may be due to the conversion of DHEA into 7alpha-hydroxy-DHEA (7alpha-OH-DHEA) by the activity of the p450 enzyme, Cyp7b. 7alpha-OH-DHEA is thought to have anti-glucocoticoid activity preventing the anti-inflammatory action of endogenous glucocorticoids. To investigate a putative role of Cyp7b in the arthritic process, tissues from both the murine collagen-induce arthritis (CIA) model and from patients with rheumatoid arthritis (RA) were studied. We determined the Cyp7b expression levels in synovial tissue and the level of 7alpha-OH-DHEA in both serum and arthritic joints of mice with CIA. Our studies showed that the severity of arthritis correlates with increased Cyp7b activity. Next, we investigated Cyp7b expression and activity in RA patients where the proinflammatory cytokines tumor necrosis factor-alpha (TNF-alpha) and interleukin-1beta (IL-1beta) are known to control the disease process. Fibroblast-like synoviocytes (FLS), isolated from RA synovial biopsies were found to express Cyp7b mRNA. In addition, Cyp7b enzymatic activity was detected in these cells. We also investigated whether Cyp7b activity is regulated by cytokines. Proinflammatory (e.g., TNF-alpha and IL-1beta) cytokines were found to stimulate Cyp7b activity and the anti-inflammatory cytokine transforming growth factor-beta (TGF-beta) was found to suppress Cyp7b activity in FLS. Next, we studied which signal transduction pathway is involved in the TNF-alpha-mediated induction of Cyp7b activity in human FLS. The results show a role for nuclear factor kappa B (NFkappaB) and activator protein-1 (AP-1) in the regulation of Cyp7b expression. Finally, we established that the effects of DHEA or 7alpha-OH-DHEA on the immune system can not be explained by glucocorticoid receptor (GR) engagement. The role of the p450 enzyme Cyp7b in DHEA metabolism and its relevance in the arthritic process will be discussed.
16736518 Surface-bound anti-type II collagen-containing immune complexes induce production of tumor 2006 Jun OBJECTIVE: Type II collagen (CII) is a major component of hyaline cartilage, and antibodies against CII are found in a subgroup of patients with rheumatoid arthritis. We undertook this study to investigate whether and how antibodies directed against CII can form solid-phase immune complexes (ICs) with cytokine-inducing properties in a model theoretically resembling the situation in the inflamed joint, in which CII is exposed for interaction with anti-CII antibodies during periods of inflammation. METHODS: Sixty-five arthritis patients with varying levels of anti-native CII antibodies and 10 healthy controls were evaluated concerning anti-CII and cytokines induced in a solid-phase IC model. Monocytes were either depleted or enriched to define responder cells. Antibodies blocking Fc gamma receptors (Fc gammaR) were used to define the responsible T cell surface receptors. RESULTS: ICs containing anti-CII from arthritis patients induced the production of tumor necrosis factor alpha (TNFalpha), interleukin-1beta (IL-1beta), and IL-8. We found a close correlation between enzyme-linked immunosorbent assay optical density values and induction of TNFalpha (r = 0.862, P < 0.0001), IL-1beta (r = 0.839, P < 0.0001), and IL-8 (r = 0.547, P < 0.0001). The anti-CII-containing IC density threshold needed for cytokine induction differed among peripheral blood mononuclear cell donors. Anti-CII-containing IC-induced cytokine production was almost totally abolished (>99%) after monocyte depletion, and receptor blocking studies showed significant decreases in the production of TNFalpha, IL-1beta, and IL-8 after blocking Fc gammaRIIa, but not after blocking Fc gammaRIII. CONCLUSION: These findings represent a possible mechanism for perpetuation of joint inflammation in the subgroup of arthritis patients with high levels of anti-CII. Blockade of Fc gammaRIIa and suppression of synovial macrophages are conceivable treatment options in such patients.
16762145 Diagnostic value of procalcitonin measurement in febrile patients with systemic autoimmune 2006 Mar OBJECTIVE: To determine the usefulness of plasma procalcitonin (PCT) measurement to suspect infectious etiology in febrile patients with systemic autoimmune disease. METHODS: PCT, C-Reactive protein (CRP), erythrocyte sedimentation rate (ESR) and white blood cell count (WBC) were measured in 44 consecutive inpatients with a diagnosis of systemic autoimmune disease and fever >38 masculine C. After careful microbiologic screening no obvious infection was demonstrated in 24 patients (Group A) while an infectious bacterial complication was diagnosed in 20 cases (Group B). RESULTS: Median PCT levels were significantly higher in the group B (1.11 vs 0.24 ng/ml; p = 0.0007), whereas the differences for CRP, WBC and ESR did not reach statistical significance. PCT also exhibited a good sensitivity and specificity (75%) in differentiating patients with infection from those with disease flare. With respect to positive and negative predictive values (71.4% and 78.2%), PCT markedly exceeded the other variables. By analyzing PCT values by disease we identified a false positive subgroup of patients suffering from adult onset Still's disease (AOSD), showing markedly elevated PCT levels in absence of infection. By excluding these patients, PCT showed a very good sensitivity and specificity (73.6% and 89.4%) and the area under receiver operating characteristics (ROC) curve rose from 0.801 to 0.904. CONCLUSION: Our data indicate that elevated PCT concentrations offer good sensitivity and specificity for the diagnosis of systemic bacterial infection in febrile patients with systemic autoimmune diseases. However, in fever associated with AOSD PCT may be elevated even in the absence of infectious complication.
16522680 Identification of parotid salivary biomarkers in Sjogren's syndrome by surface-enhanced la 2006 Sep OBJECTIVES: To identify the most significant salivary biomarkers in Sjögren's syndrome (SS) using proteomic methods. METHODS: Parotid saliva from 20 non-SS subjects and 41 primary SS patients was analysed. Protein expression profiles for each sample were generated by surface-enhanced laser desorption/ionization time-of-flight-mass spectrometry (SELDI-TOF-MS). Mean peak intensities of SS patients and non-SS subjects were compared by univariate analyses. Samples pooled by diagnosis (SS and non-SS) and labelled with different Cy dyes were compared by two-dimensional difference gel electrophoresis (2D-DIGE). Two protein levels that were most significantly different by SELDI-TOF-MS and 2D-DIGE were validated by enzyme-linked immunosorbent assay in individual samples. RESULTS: SELDI-TOF-MS of 10-200 kDa peaks revealed eight peaks with >2-fold changes in the SS group that differed from non-SS at P < 0.005. Peaks of 11.8, 12.0, 14.3, 80.6 and 83.7 kDa were increased, while 17.3, 25.4, and 35.4 kDa peaks were decreased in SS samples. 2D-DIGE identified significant increases of beta-2-microglobulin, lactoferrin, immunoglobulin (Ig) kappa light chain, polymeric Ig receptor, lysozyme C and cystatin C in all stages of SS. Two presumed proline-rich proteins, amylase and carbonic anhydrase VI, were reduced in the patient group. Three of these ten biomarkers have not been associated previously with SS. CONCLUSIONS: The salivary proteomic profile of SS is a mixture of increased inflammatory proteins and decreased acinar proteins when compared with non-SS. Future studies will test the ability of these biomarker levels, alone and in combination, to diagnose the salivary component of SS.
16234994 A case of cutaneous polyarteritis nodosa manifested by spiking high fever, arthralgia and 2006 May A 24-year-old Japanese woman was admitted for investigation of recurrent spiking high fever associated with a macular eruption of the upper extremities associated with fever and polyarthralgia. These symptoms were self-limiting but recurrent and seemed to be consistent with a diagnosis of adult-onset Still's disease (AOSD). However, livedo reticularis was detected on the lower extremities, suggesting the presence of vasculitic disease rather than AOSD. Investigation did not reveal any evidence of visceral involvement. Skin biopsy of the affected lower extremity demonstrated a necrotizing vasculitis of small muscular artery and confirmed a diagnosis of cutaneous polyarteritis nodosa (PAN) rather than AOSD. Treatment with 30 mg of prednisolone daily improved the skin lesions and the recurrent spiking high fever and the arthralgia were resolved. PAN and AOSD are clinically similar, and discrimination may be sometimes difficult. The presence of livedo reticularis and the finding of a characteristic skin biopsy appearance may be diagnostically useful to distinguish PAN from AOSD. Indeed, the clinical features of cutaneous PAN may be more similar to AOSD than systemic PAN, and a skin biopsy may be necessary to distinguish cutaneous PAN from AOSD in some cases.
17156671 [Clinical features of undifferentiated connective tissue diseases: analysis of 145 patient 2006 Sep 19 OBJECTIVE: To investigate the clinical features and prognosis of undifferentiated connective tissue disease. (UCTD) METHODS: 1105 connective tissue disease (CTD) patients, including 751 cases of systemic lupus erythrematosus (SLE), 63 cases of systemic sclerosis (SSc), 103 cases of polymyositis/dermatomyositis (PM/DM), 159 cases of primary Sjögren's syndrome (pSS), and 29 cases of overlap syndrome (29), were randomly selected. The clinical data of these patients were analyzed to identify those who displayed the manifestations of UCTD as the onset manifestations so as to summarize the clinical manifestation, immunological parameters, and long term development of UCTD. RESULTS: These 145 patients with UCTD developed SLE, SSc, SS, PM/DM, or overlap syndrome within two to five years. The patients with arthritis and arthralgia often developed into SLE. Raynaud's syndrome was often related to SSc. The patients with rash or face edema were more likely turned out to be PM/DM patients. The patients with dry eyes or dry mouth often developed into pSS patients. CONCLUSION: UCTD can develop into various autoimmune diseases, such as SLE, SSc, pSS or PM/DM. Some clinical features of onset are related with the outcome.
15988601 Polymyalgia rheumatica in primary Sjögren's syndrome. 2006 Mar The aim of the study was to describe the clinical and laboratory aspects of primary Sjögren's syndrome (pSS) associated with polymyalgia rheumatica (PMR). The retrospective study compares the clinical and laboratory aspects of patients with pSS associated with PMR on a relatively large cohort of patients (n=16) and pSS patients without PMR (n=531). The prevalence of PMR among pSS patients was 3%, while in the average population, the prevalence of PMR is only 0.75%. PMR developed 8.7 years after the diagnosis of pSS in the older female pSS population (over 50 years of age), and in those with only glandular features. Interestingly the pSS/PMR patients had hypo gammaglobuline levels, while in the pSS patient group hypergammaglobulinaemia presented. Furthermore, positive ANA serology was more frequent among pSS/PMR patients. Since the clinical management of pSS/PMR is different from pSS, a better understanding of this clinical entity is essential.
18710591 Altered fractalkine cleavage results in an organ-specific 17 kDa fractalkine fragment in s 2008 Sjögren's syndrome is a rheumatic disease in which the salivary and lacrimal glands are the principal targets of a pathological autoimmune reaction. Previous studies in mice indicated that delayed organogenesis and aberrant cell physiology followed by an increase in acinar cell apoptosis precede chronic focal inflammation in the salivary glands and the manifestation of impaired exocrine gland secretion. In a recent study by Wildenberg and colleagues, the authors report aberrant proteolytic activity in the salivary glands of non-obese diabetic mice and the generation of a unique organ-specific 17 kDa fragment of the chemokine and adhesion molecule fractalkine.