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ID PMID Title PublicationDate abstract
16762781 Lipodermatosclerosis in patients with diffuse connective tissue diseases. 2006 Jul Lipodermatosclerosis (LDS) is a clinical condition characterized by the appearance of hardened, painful, and hyperchromic plaques on the legs. We describe three patients with diffuse connective tissue diseases (DCTD) who developed this clinical condition. The first one was a systemic lupus erythematosus patient with secondary antiphospholipid syndrome; the second patient had a superposition of DCTD (rheumatoid arthritis, Sjögren's syndrome, morphea); and the last one had been diagnosed with CREST 10 years earlier but had more recently developed primary biliary cirrhosis. Although its etiopathogenesis is unknown, LDS has been frequently seen in association with venous insufficiency. Its recognition by professionals who deal with DCTD is very relevant since it is characterized by thickening of the skin, similar to scleroderma. Its identification can avoid the inadvertent use of medications such as penicillamine and immunosuppressants, which have potentially serious side effects.
16463225 [The indications for occipito-cervical fixation. A report of three cases]. 2006 Jan Lesions of the cranio-vertebral junction which affect bony structures and ligaments may cause instability and compression of the nervous and vascular structures. The goal of surgery is decompression of these structures and stabilization. The paper presents indications for performing the stabilisation procedure with CCD implementation in three patients suffering respectively from rheumatoid arthritis and neoplastic disease. In one patient spinal instability and spinal cord compression were due to rheumatoid disease and surgery included anterior spinal decompression in connection with posterior stabilisation. In two patients with neoplasms the retromandibular decompression with posterior stabilisation was performed.
18203760 CTLA-4 directly inhibits osteoclast formation. 2008 Nov CTLA-4 is a regulator of co-stimulation and inhibits the activation of T cells through interfering with the interaction of CD80/86 on antigen-presenting cells with CD28 on T cells. CTLA-4 binds to the surface of antigen-presenting cells, such as dendritic cells and monocytes through CD80/86. Monocytes can differentiate in osteoclasts, the primary bone resorbing cells. Herein, we investigated whether the binding of CTLA-4 affects the differentiation of monocytes into osteoclasts in vitro and vivo. We show that CTLA-4 dose-dependently inhibits RANKL- as well as tumour necrosis factor (TNF)-mediated osteoclastogenesis in vitro without the presence of T cells. Furthermore, CTLA-4 was effective in inhibiting TNF-induced osteoclast formation in a non-T cell dependent TNF-induced model of arthritis as well as the formation of inflammatory bone erosion in vivo. These data suggest that CTLA-4 is an anti-osteoclastogenic molecule that directly binds osteoclast precursor cells and inhibits their differentiation. These findings are an attractive explanation for the anti-erosive effect of abatacept, a CTLA-4 immunoglobulin fusion protein used for the treatment of rheumatoid arthritis.
18427722 Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome and adult onset Still's dise 2008 Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP/HUS) is a multisystem disorder characterized by consumptive thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, renal function abnormalities, and fever. Coexistence of TTP/HUS and adult onset Still's disease (ASD) is extremely rare. We report the case of a 46-year-old woman who presented with fever, arthritis, myalgias, petechia on skin and confusion five years after the onset of ASD. Thrombocytopenia, renal failure, marked elevation lactate dehydrogenase, and red cell fragmentation on peripheral blood smear were observed. We made a diagnosis of TTP/HUS associated with ASD, according to physical examination and characteristic laboratory data. She recovered from the TTP/HUS following daily sessions of therapeutic plasma exchange with fresh frozen plasma replacement and glucocorticoid therapy. Awareness of the possible development of TTP/HUS in ASD is important for early diagnosis and treatment.
17181450 Is it safe to use topical NSAIDs for corneal sensitivity in Sjögren's syndrome patients? 2007 Jan Sjögren's syndrome dry eye is an inflammatory disease accompanied by an impairment of the autonomous nervous system of the ocular surface. The therapy for this condition is shifting from the mere tear replacement to a more complex approach including an anti-inflammatory treatment. Clinical trials have evidenced that the use of NSAIDs is followed by a reduction of the ocular discomfort symptoms in dry-eye patients. However, sporadic case reports of corneal melting in dry-eye subjects who underwent surgical procedures has brought attention to the possible effect that NSAIDs may have on corneal sensitivity. Therefore, the effect of NSAID treatment on corneal sensitivity in normal subjects and in patients with dry eye was studied. The results of these trials seem to demonstrate that some NSAIDs, diclofenac in particular, have the effect of reducing corneal sensitivity both in normal subjects and in patients with dry eye. Therefore, NSAIDs should be used with caution in Sjögren's syndrome patients.
16677919 Quantitative analyses of sonographic images of the parotid gland in patients with Sjögren 2006 May The first purpose of this study was to quantify the sonographic images of the salivary gland to differentiate the Sjögren syndrome (SS) group from the non-SS group. We included 132 patients in this study who had been referred to our department because of a suspicion of SS. A total of 91 patients fulfilled the criteria for SS, whereas the remaining 41 patients did not. We placed the regions-of-interest within the lesion. The first purpose was to evaluate which indices obtained by the texture analyses were useful for differentiating the SS group from the non-SS group. The second purpose was to evaluate the relationship between the indices and the degree of severity in the SS group. Out of the several indices evaluated, Hurst coefficients, obtained by fractal analysis, of SS group were found to be significantly lower than those of the non-SS group. Moreover, the Hurst coefficient was associated with the degree of destruction of the parotid gland as assessed by sialography. The Hurst coefficient of a globular stage and an advanced stage were both significantly lower than that of a normal pattern, whereas the Hurst coefficient of a punctate stage was almost similar to that of the normal stage. The Hurst coefficient showed a very weak correlation with the results of either the gum test or serologic tests.
17763472 NF-kappaB and the intestine: friend or foe? 2008 Jan The biological impact of the NF-kappaB transcriptional system in various intestinal biological processes such as cellular proliferation, differentiation and survival, inflammation, and carcinogenesis is a relatively young field of research. Less than a decade ago, reviews addressing NF-kappaB regulation and function in the intestine had to borrow concepts and hypotheses from other bodily systems such as the joints (rheumatoid arthritis), the lungs (asthma), or the cardiovascular system (systemic inflammatory states, sepsis). Since then, important progress has been made in defining the various functional aspects of NF-kappaB signaling in intestinal homeostasis and diseases, and exciting new paradigms have emerged from this research. This review will discuss the function of NF-kappaB in intestinal homeostasis and diseases in relation to injury responses and microbial colonization/infection.
18698187 Update on biologics in juvenile idiopathic arthritis. 2008 Sep PURPOSE OF REVIEW: The purpose of this review is to summarize the recent data on biologic therapies in juvenile rheumatoid arthritis. The armamentarium for treatment of juvenile idiopathic arthritis is expanding at a rapid rate, and improved physical and functional outcomes are anticipated. New data from large prospective randomized trials have demonstrated efficacy of anti-tumor necrosis factor agents and a costimulator signal inhibitor. RECENT FINDINGS: The results of a pivotal trial of infliximab in polyarticular juvenile idiopathic arthritis suggested efficacy, but the primary outcome was not significantly different from placebo. Important information regarding dosing in children was obtained, however. A pivotal trial of adalimumab did prove efficacy, and resulted in U.S. Food and Drug Administration (FDA) approval. The monoclonal antibodies to tumor necrosis factor appear to be more effective in treating chronic uveitis associated with juvenile idiopathic arthritis than etanercept. Anti-IL-1 and anti-IL-6 therapy, particularly for systemic disease patients, looks very promising, as well. The costimulation modifier abatacept was shown to be effective and relatively well tolerated in the short term, also resulting in FDA approval this year. Continued experience with these agents and appropriate systems-based methods such as formal registries, to complement existing FDA procedures for monitoring safety, will improve our ability to identify short-term and long-term toxicities of these new agents. SUMMARY: As experience is gained, and longer-term safety is demonstrated, it is likely that biologics will be introduced as therapy earlier in the course of patients who inadequately respond to conventional disease-modifying antirheumatic drugs.
17159808 Association between tissue gamma-glutamyl-transferase and clinical markers of adjuvant art 2006 Dec OBJECTIVES: To assess glucomannan and pyridoindole derivatives for possible antioxidant therapy of rheumatoid arthritis (RA) by using the model of adjuvant arthritis (AA). We evaluated the association between clinical markers of the adjuvant arthritis model used - increase of hind paw volume (HPV), changes of body mass (CBM), and tissue gamma-glutamyl transferase (GGT) activity assessed in the spleen and the joint. METHODS: AA was induced in Lewis rats by a single intradermal injection of Mycobacterium butyricum. The two independent experiments included healthy animals as reference, arthritic animals without any drug administration and arthritic animals with pyridoindole administration in one dose tested or glucomannan administration in two different doses. The pyridoindoles (PI) studied were stobadine dipalmitate and its derivatives SMe1.2HCl and SMe1EC2.HCl. We monitored CBM and HPV twice a week. Parameter of inflammation - GGT in the spleen and the joint from the hind paw (cartilage and soft tissue without bone) was determined on day 28. The correlation coefficient of GGT activity with CBM and with HPV was calculated. RESULTS: The antioxidants tested were effective in slowing down the progress of adjuvant arhritis. The association between tissue GGT activity and the clinical marker of adjuvant arthritis - CBM was higher in the spleen than in the joint. The other clinical marker assessed - HPV, gave a better association with GGT activity measured in the joint than in the spleen. CONCLUSIONS: It may be concluded that GGT activity in tissues as the spleen and the joint could provide a simple and inexpensive marker for AA and RA development at systemic as well as local level; all the antioxidants studied were effective in slowing down the progress of adjuvant arhritis.
18271807 Steroid-resistant adult-onset Still's disease which showed a quick response to methotrexat 2008 Feb A case of steroid-resistant adult-onset Still's disease is herein reported. The patient consulted us because of night fever, arthralgia and evanescent rashes. She was diagnosed with adult-onset Still's disease, with a C-reactive protein (CRP) value of 29.5 mg/dL and serum ferritin level of 4500 ng/mL. The fever, rashes and arthralgia disappeared after medication of medium-dose oral prednisolone, however, the CRP value persisted at high levels, and the serum ferritin level nevertheless increased by 5200 ng/mL. Following the pulse therapy with corticosteroid, the CRP value decreased once but thereafter returned to a high level again. The serum ferritin level did not respond during that therapy. Finally, 10 days after starting the administration of methotrexate, the CRP value dramatically decreased from 7 mg/dL to 0.16 mg/dL, and thereafter the serum ferritin level started to decline, which thus enabled us to eventually taper the dose of oral prednisolone.
18174671 Macrophage activation syndrome associated with adult-onset Still's disease. 2007 Dec Macrophage activation syndrome (MAS) is a rare and potentially lethal disease, resulting from uncontrolled activation and proliferation of T lymphocytes and macrophages. Adult-onset Still's disease (AOSD) is an inflammatory disease. AOSD resemble reactive MAS in its symptoms and laboratory data. Moreover, AOSD per se induces MAS. It is, therefore, quite difficult to differentiate these syndrome and disease. The immunodeficiency state induced by treatment in AOSD could reactivate latent viruses such as Epstein-Barr virus, which could potentially lead to MAS. The therapeutic agents for AOSD, such as sulfasalazine, also could provoke reactive MAS. Because multiple factors are involved in inducing MAS to a different degree, the main cause should be searched for and targeted for the therapy.
17967752 Multiple chemical sensitivity syndrome in Sjögren's syndrome patients: casual association 2006 Nov Multiple chemical sensitivity (MCS) is defined by multiple symptoms, affecting multiple organs, that wax and wane in response to varying chemical exposures at or below previously tolerated levels. Sjögren's syndrome (SS) is a common autoimmune disease affecting 3% of women aged over 55 years. Except for keratoconjunctivitis sicca (which is associated with SS not MCS), systemic features are common between the 2 diseases, leading to considerable morbidity and, occasionally, mortality. The authors report 3 cases of association between SS and MCS. Three women who were diagnosed with SS showed MCS symptoms and also were diagnosed with MCS. Further studies are needed to understand physiopathogenic mechanisms that eventually may be revealed as common to the 2 syndromes.
19156485 A clinically isolated syndrome: a challenging entity: multiple sclerosis or collagen tissu 2008 Nov Acute isolated neurological syndromes, such as optic neuropathy or transverse myelopathy, may cause diagnostic problems since they can be the first presentations of a number of diseases such as multiple sclerosis (MS) and collageneous tissue disorders. In the present study, particular systemic lupus erythematosus (SLE) and primary Sjogren syndrome (pSS) patients, who were followed up with the initial diagnosis of possible MS with no evidence of collagen tissue disorders for several years, are described. Five patients with the final diagnosis of SLE and five pSS patients are evaluated with their neurologic, systemic and radiologic findings.Over several years, all developed some systemic symptoms like arthritis, arthralgia, headache, dry mouth and eyes unexpected in MS. During the regular and close follow-up laboratory evaluations of vasculitic markers revealed positivity, leading to the final definite diagnosis of SLE or pSS. Patients with atypical neurological presentation of MS, a relapsing remitting clinical profile, or lack of response to the regular MS treatment should be evaluated for the presence of a connective tissue disease. Various laboratory tests, such as cerebrospinal fluid findings, autoantibodies profile, markers, cranial and spinal magnetic resonance imaging, can be helpful for the differential diagnosis. Lack of response to the regular multiple sclerosis treatment, even increasing rate of relapses can force the clinician for the differential diagnosis. In particular cases an accurate diagnosis can only be made after close follow-up.
18163919 Effectiveness of low-dose doxycycline (LDD) on clinical symptoms of Sjögren's syndrome: a 2007 Dec 31 BACKGROUND: Matrix metalloproteinases (MMPs) are proteolytic enzymes that may contribute to tissue destruction in Sjögren's syndrome (SS). Low-dose doxycycline (LDD) inhibits MMPs. We evaluated the efficacy of LDD for the subjective symptoms in primary SS patients. This was a randomized, double blind, placebo controlled cross-over study. 22 patients were randomly assigned to receive either 20 mg LDD or matching placebo twice a day for 10 weeks. The first medication period was followed by 10-week washout period, after which the patient received either LDD or placebo, depending on the first drug received, followed by the second washout period. Stimulated saliva flow rates and pH were measured before and after one and ten weeks of each medication and after washout periods. VAS scale was used to assess the effect of LDD and placebo on following six subjective symptoms: xerostomia; xerophtalmia; difficulty of swallowing; myalgia; arthralgia; and fatigue. The effect was evaluated for each medication and washout period separately. RESULTS: Overall, the effects of medications on subjective symptoms were minor. Wilcoxon test demonstrated increased fatigue with LDD during medication (p < 0.05). The differences may, however, reflect normal fluctuation of symptoms in SS patients. CONCLUSION: LDD may not be useful in reducing the primary SS symptoms.
17631733 Risk factors for Sjögren's syndrome: a case-control study. 2007 May OBJECTIVE: The aim of this study was to investigate potential risk factors for Sjögren's syndrome (SS) by means of a multi-centre case-control study, focusing in particular on familial and environmental risk factors. 140 female SS patients and 109 female controls with orthopaedic problems were consecutively enrolled in seven university hospitals in Italy. METHODS: Information regarding the patient's lifestyle, her medical, menstrual and pregnancy history, and any family history of autoimmune diseases (AD) was obtained through a detailed structured questionnaire. The odds ratio (OR) and 95% confidence interval (95%CI) were calculated using unconditional logistic regression, adjusting for age and family size. The probability of first-degree relatives developing an autoimmune disease was also investigated. RESULTS: A positive family history of AD was significantly associated with SS. Subjects with a first-degree relative (FDR) with AD showed a seven-fold increase in the risk for SS compared to controls (OR=7.4, 95%CI 2.8-20.1); the strength of this association increased with the number of relatives affected. Similarly, the FDR of SS patients had a higher risk of AD in comparison to subjects without FDR affected by SS. Women with one or more pregnancies had an increased risk of SS (OR=2.1, 95%CI 1.0-4.3). CONCLUSION: This study suggests that a family history of AD is associated with SS.
17424713 [Salivary gland biopsy : experience of La Rabta Hospital's pathology departement]. 2007 Jan BACKGROUND: The minor salivary glands biopsy is a very common diagnostic procedure in oral medicine rather its efficiency has not been statistically proved. AIM: Assessment of Rabta pathologic department experience METHODS: 297 biopsies have been studied with special attention to the suspected diagnosis before biopsy and the final histologic result. RESULTS: The minor salivary gland biopsy confirmed the initial diagnosis in 78 cases. Although if the minor salivary gland biopsy is in most cases not contributively, it is a very simple procedure which gives the diagnosis of Gougerot-Sjogren disease, amylosis and sarcoidosis.
17091912 Methotrexate should not be used for patients with end-stage kidney disease. 2006 Jul Methotrexate is a widely used disease-modifying anti-rheumatic drug. Its effectiveness has been proven in placebo-controlled trials and in comparison with other disease-modifying anti-rheumatic drugs. The pharmacokinetics of methotrexate are highly variable and unpredictable. In patients with normal renal function, the recommended dose in rheumatoid arthritis ranges between 7.5 and 15 mg/week, but in recent years, even dosages up to 25 mg weekly are used. Toxicity includes myelosuppression, gastrointestinal adverse effects, hepatotoxicity and pneumonitis. Renal impairment and age are considered major risk factors for developing methotrexate toxicity, but studies show conflicting results. Whether methotrexate can be administered to patients with end-stage kidney disease has not been formally tested. The present case illustrates the severe side effects of low-dose methotrexate treatment in a patient with end-stage kidney disease. Seven other cases have reported similar and even more severe and irreversible consequences after low-dose regimen. In view of these side effects we strongly recommend to monitor toxicity rigorously in patients with stage 3 or stage 4 kidney disease and not to use methotrexate in patients with stage 5 kidney disease.
19100010 [Cardiac manifestations in Sjogren syndrome]. 2008 Apr OBJECTIVE: To analyze the cardiac manifestations of patients with primary and secondary Sjogren syndrome. METHODS: Clinical data (clinical manifestations, serologic measurements, echocardiogram) of 396 patients with Sjogren syndrome who admitted to our hospital from 2004--2007 were retrospectively analyzed. Patients with congenital, rheumatic and coronary heart diseases, hypertension and diabetes (n = 221) and patients with incomplete clinic data (n = 51) were excluded. RESULTS: A total of 124 cases were included in this analysis (mean age 47.4 years old; 5 males; average disease duration 85.5 months). Cardiac involvement in Sjogren syndrome is usually asymptomatic. Pericardial effusion (PE) were evidenced in 20.2%, left ventricular diastolic dysfunction (LVDD) in 13.7%, pulmonary artery hypertension (PAH) in 12.9%, left atrium enlargement/in 7.3%, mitral insufficiency in 4.8%, aortic dilation in 5.6%, tricuspid insufficiency in 3.2%, left ventricular enlargement in 2.4% and left ventricular systolic dysfunction in 0.8% patients by echocardiography examinations. Patients with PE had significantly lower CH50, C3, C4 levels and significantly higher C reactive protein level (CRP) and SSA positive rate than patients without PE (all P < 0.05). The serum level of CRP was significantly associated with PE (OR 0.976, 95% CI 0.956 - 0.997, P < 0.05). Age is positively correlated to LVDD (OR 0.884, 95% CI 0.811 - 0.964, P < 0.005). The gammaglobulin level is significantly higher in the PAH group than that in the non-PAH group (P < 0.05). CONCLUSIONS: Cardiac involvement is not rare in patients with Sjogren syndrome. PE, LVDD and PAH are usual cardiac manifestations in these patients. The serum level of CRP is positively related to PE in these patients with Sjogren syndrome.
17644897 [Adult onset Still's disease: about 11 cases]. 2007 Jun BACKGROUND: Adult onset Still's disease (AOS) is an inflammatory disorder which associates variable articular and systemic manifestations. Despite a better knowledge of its biological and clinical particularities; the pathogeny of this disease remains unknown. The aim of this study is to analyze the epidemiological, clinical, biological and, outcome characteristics of AOS. METHODS: It is a retrospective study about 11 cases of AOS hospitalized over a 24-year-period (1982-2005) at The Rheumatology Department of Charles Nicolle's Hospital of Tunis. All patients responded to the Yamaguchi criteria. RESULTS: Mean age was 35.4 years [20y-70y] with a sex-ratio of 0.57. Fever and articular involvement were the most frequent signs. Cutaneous symptoms were present in 6 patients. Three of our patients developed destructive arthritis. Renal amyloidosis, a rare complication of AOS, has been noted in one case. CONCLUSION: AOS is an unfrequent disorder characterized by its diagnosis and treatment difficulties. Recent advances in immunotherapy may better the management of AOS.
17486027 Tear lipid layer thickness and ocular comfort with a novel device in dry eye patients with 2007 Apr BACKGROUND: To measure changes in tear-film lipid-layer thickness (LLT) and symptoms in patients with dry eye symptoms with and without Sjögren's syndrome after using a novel device. The device is designed to promote release of meibomian sebum into the tear film by delivering latent heat to the eyelids. STUDY DESIGN: Two prospective, controlled, randomised, observer-masked, single-intervention studies. METHODS: Two independent studies were conducted in a major university hospital in the South West of England. The first study involved 24 patients with dry eye symptoms without Sjögren's [the PDE study] and the second study involved 31 patients with dry eye symptoms and Sjögren's syndrome (the SS study). The PDE study was randomised into two groups. Group I (12 patients) underwent 10 min of treatment with the activated device and Group II (12 patients) had no treatment. The SS study was similarly randomised into Group I (17 patients) and Group II (14 patients). The LLT and subjective alterations in ocular comfort of each subject were assessed prior and immediately after 5 and 30 min subsequent to the 10-min period. In the SS study, a further assessment was carried out at 60 min. RESULTS: In the PDE study, treated patients exhibited a bilateral increase of LLT at 5 min (right eyes, 1.2 levels, p<0.0005; left eyes, 1.0 levels, p<0.0005, Mann-Whitney) and at 30 min (right eyes, 0.7 levels, p<0.005; left eyes, 0.6 levels, p<0.005). Mean symptom scores improved in the treated group compared with the control group at 5 min (treatment group, +2.0; control group, +0.2; p<0.05) and 30 min (treatment group, +2.8; control group, +0.4; p<0.015). In the SS study, treated patients exhibited a bilateral increase of LLT, 5 min (right eyes, 0.5 levels, p<0.009; left eyes, 0.5 levels, p<0.005, Monte Carlo 2-tailed), 30 min (right eyes, 0.5 levels, p<0.007; left eyes 0.5 levels, p<0.002) and 60 min (right eyes, 0.3 levels, p<0.1; left eyes, 0.3 levels, p<0.05). There was no change in any of the control patients in any of the assessments. With regard to symptom scores, the mean change at 5 min measured +0.8 in the treatment group and remained relatively unchanged at +0.1 in the control group (p<0.1). At 30 min, this change measured +1.3 in the treatment group and +0.1 in the control group (p<0.03) and at 60 min, the change measured +1.5 in the treatment group and remained at +0.1 in the control group (p<0.02). CONCLUSION: Meibomian therapy with this novel device increases LLT and ocular comfort in patients with dry eye symptoms with and without Sjögren's syndrome.