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ID PMID Title PublicationDate abstract
20444860 Central nervous system involvement in Sjögren's syndrome: unusual, but not unremarkable-- 2010 Aug OBJECTIVES: To perform an observational retrospective cross-sectional case-control study to evaluate prevalence, clinical patterns and outcomes of CNS involvement in a large cohort of primary SS (pSS) patients. METHODS: A total of 424 pSS patients, diagnosed according to the 2002 criteria proposed by the American-European Consensus Group, were checked for CNS involvement after exclusion of secondary causes. Demographic, clinical, seroimmunological data were compared between patients with and without CNS involvement. Neuroimaging data were also analysed. RESULTS: CNS involvement was detected in 25 (5.8%) patients (24 females and 1 male) both at disease onset (52%) and later (48%) with a mean latency after diagnosis of 7 years. Diffuse (40%), focal/multifocal (36%), multiple sclerosis (MS)-like disease (20%) and isolated optic neuritis (4%) were the most common CNS clinical pictures. Disease duration, lung involvement and decreased C(4) were associated with CNS involvement, while articular manifestations were more frequently observed in patients without neurological complications. Most cases had an acute, often recurrent course with spontaneous remission or only mild neurological impairment. CONCLUSIONS: CNS involvement represents a rare but not negligible complication of pSS, which may occur with a bimodal temporal pattern, both at onset and later, prompting attention in the differential diagnosis of apparently isolated neurological syndromes. Lung involvement emerged as the strongest risk factor for CNS involvement with a relative risk of 7.9, along with disease duration and decreased C(4).
20123591 Adult-onset Still's disease complicated with hemophagocytic syndrome. 2010 Jan Hemophagocytic syndrome (HS) that occurs in the course of adult-onset Stills disease (AOSD) has been reported only rarely in the literature. HS and AOSD share overlapping clinical and laboratory features, therefore, it is difficult to recognize HS as a complication of AOSD. Here, we report the case of a 46-year old woman with classical features of AOSD. Severe pancytopenia and jaundice associated with extreme hyperferritinemia occurred during high-dose steroid treatment. Bone marrow biopsy showed typical pathological features of hemophagocytosis, which confirmed the coexistence of HS with AOSD. The patient was treated with methylprednisolone pulse therapy of 500 mg/day for 3 days, as recommended in cases of HS complicating AOSD, and her condition improved gradually. During the disease course, extensive studies could not identify any viral infection or other known underlying etiology for the reactive hemophagocytosis. Currently, the patient is in remission on low-dose prednisolone and azathioprine.
18829369 Diagnostic evaluation and classification criteria in Sjögren's Syndrome. 2009 Jan OBJECTIVES: Our objective is to carry out a clinical study of the performance of the preliminary European classification criteria for Sjögren Syndrome and that of the criteria proposed by the American European Consensus Group. METHODS: Eighty-eight patients who had undergone a biopsy of the salivary gland on suspicion that they were suffering from Sjögren Syndrome were studied by two independent rheumatologists. Two pathologists independently revised the biopsies without knowing the diagnosis. With all of this information, the clinicians established, or did not establish, a diagnosis of primary Sjögren Syndrome or secondary Sjögren Syndrome. Finally, it was analysed whether or not the patients met the American European Consensus Group classification criteria and the preliminary European criteria for primary Sjögren Syndrome and secondary Sjögren Syndrome, and their sensitivity and specificity with respect to the clinical diagnosis were determined. RESULTS: Clinicians estimated that 35 cases (39.8%) had primary Sjögren Syndrome (kappa 0.718) and 17 cases (19.3%) had secondary Sjögren Syndrome (kappa 0.761). The specificity and sensitivity of American European Consensus Group criteria, with regard to the clinical diagnosis, for primary Sjögren Syndrome were 97.2% and 48.6%, respectively. For secondary Sjögren Syndrome, the specificity was 97.2% and the sensitivity 64.7%. The preliminary European criteria for primary Sjögren Syndrome demonstrated a lesser specificity (75%), but a higher sensitivity (65.7%). In secondary Sjögren Syndrome the specificity reached 97.2% with sensitivity at 70.6%. CONCLUSIONS: These results underline the difficulty in applying the Sjögren Syndrome classification criteria from the American European Consensus Group and the preliminary European criteria, in the diagnosis of individual patients.
19752179 Impact of primary Sjogren's syndrome on smell and taste: effect on quality of life. 2009 Dec OBJECTIVES: To assess the prevalence of abnormal smell and taste perception in people with primary SS and the effect on quality of life (QoL). METHODS: Twenty-eight participants with SS and 37 controls were enrolled in a cohort-matched, prospective, cross-sectional study. Smell and taste thresholds were measured using standardized, validated tests. QoL was assessed by the Short Form 12 (SF-12). RESULTS: Smell threshold was reduced by 1 point (P = 0.002; 95% CI 0.35, 1.54) and taste threshold was reduced by 3.5 points (P < 0.001; 95% CI 1.80, 5.22) in the SS group compared with controls. The physical and mental components of SF-12 were reduced by 14.2 points (P < 0.001; 95% CI 9.47, 19.02) and 7.5 points (P = 0.002; 95% CI 2.97, 12.02), respectively, in the SS group compared with controls. Taste threshold was significantly correlated with both the physical (r = 0.48; P < 0.001) and the mental (r = 0.30; P = 0.015) components of SF-12. Smell threshold correlated with the physical (r = 0.457; P < 0.001), but not the mental component (r = 0.154; P = 0.222) of SF-12. CONCLUSIONS: Clinically important impairment of chemosensory perception occurred in the SS group compared with age- and gender-matched controls. Assessment using SF-12 suggests that this impairment contributed to the reduced health-related QoL that characterized these individuals.
20299380 Articular manifestations in primary Sjögren's syndrome: clinical significance and prognos 2010 Jun OBJECTIVES: Articular manifestations (AMs) occurred in approximately 30-60% of patients with primary SS (pSS). We conducted the current study to describe clinical presentation, specific treatment and to report clinical outcome of pSS patients with AM in a large bicentric French cohort. METHODS: Clinical, biological and immunological features of 419 consecutive patients with pSS were recorded in order to describe the clinical and immunological course of pSS AM and to point out the impact of those rheumatological features on pSS evolution. RESULTS: A total of 188 patients with pSS (172 women, 16 men) exhibited AM. They preceded sicca symptoms in 32, were simultaneous to pSS diagnosis in 98 and followed diagnosis in 59 patients. Clinical presentation was polyarticular and concerned mostly peripheral joints (synovitis, n = 66). Symptoms responded readily to symptomatic treatment in 45 cases (24%). DMARDs or immunosuppressive treatments were introduced in 133 patients: HCQ (n = 111), corticosteroid (n = 53), MTX (n = 12), SSZ (n = 6), AZA (n = 3), LEF (n = 1), etanercept (n = 1) and allochrysine (n = 1). Only one case of RA was diagnosed during the evolution. Statistical analysis identified clinical and biological factors associated with AM (P < or = 0.05): RP, muscular manifestations, renal involvement, peripheral neuropathy, cutaneous vasculitis, and positivity of RF, anti-SSB antibodies and cryoglobulinaemia. Patients with AM at diagnosis were characterized by a multisystemic involvement at the end of the follow-up period (P < 0.001). CONCLUSION: Although AMs are frequent and usually mild in pSS, these manifestations are associated with a pluri-systemic involvement of pSS.
19452244 Sjögren's syndrome patients presenting with hypergammaglobulinemia are relatively unrespo 2009 Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by sicca symptoms, including dry eyes and dry mouth. Cevimeline is used for the treatment of dry mouth in patients with SS. Here we prospectively tested the clinical effectiveness of cevimeline at increasing saliva secretion in patients with SS, and the results were compared with the clinical parameters of the patients. Saliva secretion was increased >160% in 17 of 30 (56.7%) patients (P < 0.005). When the clinical parameters were compared between the patients who responded to cevimeline treatment and those who did not respond to the treatment, the frequency of patients presenting with hypergammaglobulinemia was significantly higher in the nonresponder group (P < 0.05). It thus appears that cevimeline is effective in SS patients with milder disease activity.
20692541 Pulmonary involvement in Sjögren syndrome. 2010 Sep Sjögren syndrome is a slowly progressing autoimmune disease. Pulmonary manifestations are frequent in primary Sjögren syndrome but often not clinically significant; the most common are xerotrachea, interstitial lung diseases, and small airway obstruction. Pulmonary manifestations in Sjögren syndrome have a slow progression and favorable prognosis, with the exception of primary pulmonary lymphoma and pulmonary hypertension.
20147445 Primary Sjogren's syndrome: too dry and too tired. 2010 May Chronic fatigue is one of the most prevalent and debilitating symptoms in primary SS (pSS). Approximately 70% of pSS patients suffer from disabling fatigue, which is associated with reduced health-related quality of life. In this article, we review the instruments used for evaluating pSS-related fatigue, our current understanding of the underlying psychosocial and physiological mechanisms of fatigue in pSS and the therapeutic strategies that have been studied in the management of fatigue in pSS.
19220446 Cerebral white matter hyperintensities are not increased in patients with primary Sjögren 2009 May BACKGROUND AND PURPOSE: It is frequently thought that cerebral white matter hyperintensities (WMHs) on T-2 weighted MRI scans are increased in patients with autoimmune diseases. An increased frequency of WHMs has been described in primary Sjögren's syndrome (PSS), but no controlled studies exist. The aim of this study was therefore to compare WMHs in PSS patients and healthy subjects applying the new European-American criteria for PSS. METHODS: Cross-sectional controlled study of 68 unselected PSS patients and 68 healthy subjects was carried out. WMHs were rated using Scheltens method. RESULTS: There were no differences in total or any regional WMH scores between PSS patients and healthy subjects. CONCLUSIONS: Patients with PSS do not have increased WMH load or distribution when compared with healthy subjects.
20704609 A case of refractory adult-onset Still's disease treated with anakinra. 2010 Aug Adult-onset Still's disease (AOSD) often presents both a diagnostic and a therapeutic challenge. We report a 40-year-old Chinese woman, in whom multiple adjustments of drug combinations were required before successful control of the patient's disabling symptoms. The patient failed multiple therapies including non-steroidal anti-inflammatory drugs, glucocorticoid, methotrexate (MTX), cyclosporine, leflunomide and infliximab. Treatment was complicated by hyperglycemia, glucocorticoid-induced osteoporosis, worsening hypertension and vaginal candidiasis. She suffered recurrent hospitalisation for active disease, developed carpal joint erosions and lost her employment over the course of 1 year. In view of refractoriness to multiple conventional therapies, anakinra was initiated in combination with MTX with a rapid and sustained improvement in clinical and laboratory parameters over 12 months. However, radiographic damage ensued despite aggressive therapies.
20338890 A single centre retrospective analysis of AECG classification criteria for primary Sjogren 2010 Jul OBJECTIVE: To assess the usefulness and performance of the American European Consensus Group (AECG) criteria based on minor salivary gland biopsy (MSGB) in Japanese patients with primary SS. METHODS: Among 208 MSGB cases, we retrospectively selected 112 subjects who satisfied the complete set of AECG classification criteria. Of the 112 subjects studied, 63 primary SS patients and 49 non-SS group subjects were classified according to the AECG criteria. The contribution of subjective and objective components was statistically analysed. RESULTS: Sex, dry eye, Saxon test, Schirmer's test, anti-SSA/Ro antibody, MSGB grading and sialography statistically contributed to the diagnosis. Multiple logistic regression analysis showed that positive MSGB [odds ratio (OR) 105; 95% CI 13, 849), positive anti-SSA/Ro antibody (OR 96; 95% CI 10, 923), a positive Saxon test (OR 46; 95% CI, 6, 340) and the existence of dry eye (OR 8, 95% CI 2, 43) were associated with the diagnosis of primary SS. Among the components of the AECG criteria, MSGB and anti-SSA/Ro antibody were very strong contributors. Furthermore, the abnormal-finding positive rate in sialography significantly correlated with MSGB grading (P-value for trend = 0.0006), although other subjective and objective components were not associated with MSGB grading. CONCLUSION: The usefulness of the AECG criteria for Japanese primary SS patients was confirmed.
19217338 Neurological manifestations revealing primitive Gougerot-Sjogren syndrome: 9 cases. 2009 Mar INTRODUCTION: Neurological manifestations in Gougerot-Sjogren syndrome (GSS) are valued differently. This is essentially the achievement of the peripheral nervous system. METHODS: We report 9 cases of neurological manifestation revealing primitive Gougerot-Sjogren syndrome collected over a period of 8 years (1997-2004). GSS diagnosis was retained according to Americano-European group criteria consensus revised on 2002. RESULTS: All our patients were female with an average age of 43 years. Peripheral nervous system manifestation occurred in 78% (Truncal Neuropathy in 44%, anterior horn involvement in 2 cases). Central nervous system involvement was observed in 55.6% (chronic myelopathy and aseptic meningoencephalitis). DISCUSSION AND CONCLUSIONS: The analysis of neurological manifestations in GSS encounters three difficulties: the lack in homogeneity of diagnostic criteria (which makes it difficult to compare the frequency of neurological complications in different series), the limited number of large series, and the cases with neurological manifestations revealing this syndrome.
19890748 Thrombotic microangiopathy in adult-onset Still's disease: case report and review of the l 2009 Coexistence of thrombotic microangiopathy and adult-onset Still's disease is extremely rare. There is increasing evidence that this association could be more than just coincidental. We report on the case of a 34-year-old male diagnosed with adult-onset Still's disease and successfully treated with intravenous glucocorticoids. Nine months after onset the patient exhibited the presence of asymptomatic thrombocytopenia during treatment with chloroquine. The physical status was unremarkable except for pallor of the skin and mucosa. Laboratory evaluation revealed profound thrombocytopenia and hemolytic anemia. Coombs' tests were negative; renal function tests were all normal. The peripheral blood smear showed frequent schistocytes. Based on the presence of thrombocytopenia and microangiopathic hemolytic anemia, and with the exclusion of other known causes, the patient was diagnosed with thrombotic microangiopathy and successfully treated with plasma exchange and intravenous glucocorticoids. We also review the literature on the association between adult-onset Still's disease and thrombotic microangiopathy; our case is the 15(th) report on such an association. The mean age at onset of adult Still's disease in these cases was 31.60 years and the interval between the diagnosis of Still's disease and the onset of thrombotic microangiopathy ranged from 3 days to 17 years, with a female/male ratio of 2 : 1. In more than half the patients thrombotic microangiopathy occurred within the first 6 months after the diagnosis of the Still's disease. Eleven of the 15 (73%) patients were treated with plasmapheresis in addition to glucocorticoid therapy: eight of 11 (73%) had complete remission, the other three had permanent visual impairment and/or digital ischemia. Of the four patients who were not treated with plasmapheresis, two died, one developed end-stage renal disease and one had complete remission. Awareness of the possible development of thrombotic microangiopathy in patients with adult-onset Still's disease is critical, so that treatment can be initiated early and the complications and recurrence of thrombotic microangiopathy prevented. Patients with adult-onset Still's disease should be closely monitored for signs and symptoms of thrombotic microangiopathy during the first six months after diagnosis of the Still's disease.
19579028 Myocarditis as a form of relapse in two patients with adult Still's disease. 2010 Jun Still's disease is a subset of juvenile idiopathic arthritis (JIA) that usually presents with intermittent fever, rash, and arthritis. Extra-articular flares can occur several years after disease onset. We report two cases of adult Still's disease with myocarditis after several years of being in remission. A 34-year-old Caucasian man with history of systemic juvenile arthritis in remission since age 13 was admitted in hospital with 10 days history of fever, odynophagia, and arthralgias. Chest X-ray and cardiac ultrasound showed cardiac enlargement. An endomyocardial biopsy revealed acute myocarditis. He was treated with methylprednisolone and intravenous gammaglobulin, with improvement of his general condition and cardiac parameters. A 16-year-old Caucasian male patient with history of systemic JIA in remission for the last 7 years was admitted with 7 days history of fever, odynophagia, arthralgias, and myalgias. Two days after admission, he developed chest pain and pericardial rubbing was found on examination. Cardiac ultrasound showed left ventricular dilatation with impaired systolic function, and posterior, inferior and apical-septal wall hypokinesia. Blood test showed elevated creatine phosphokinase levels. He was treated with IV methylprednisolone with normal follow-up cardiac ultrasound. Cardiac involvement in patients with systemic JIA can be the first symptom of disease reactivation, even after many years of disease remission.
19417984 [The expression of interferon-alpha in peripheral blood of patients with primary Sjogren's 2009 Apr PURPOSE: To investigate the interferon-alpha level in peripheral blood of patients with primary Sjogren's syndrome (pSS), and explore preliminarily the correlation between pSS and interferon-alpha. METHODS: ELISA and real-time PCR were used to evaluate the interferon-alpha level in peripheral blood in 37 patients with pSS and 24 patients with non-pSS. Statistical analysis was performed by Student's t test or Fisher's exact test using SAS6.12 software package. RESULTS: The results demonstrated that 43.24% (16/37) patients with pSS had detectable IFN-alpha concentration in plasma (>or=12.5 pg/mL), the other 21 patients (56.76%) with pSS had undetectable IFN-alpha concentration(<12.5 pg/mL). All 24 controls had undetectable IFN-alpha expression.There was significant difference between the two groups(P=7.02 x 10(-5)). Furthermore, the mRNA expression levels of IFN-alpha gene in peripheral blood cells were over-expressed in patients with pSS(P=0.0124). CONCLUSIONS: The study reveals that interferon-alpha level in peripheral blood with pSS patients is overexpressed.
19579736 Adult onset Stills disease in a tertiary care hospital of Pakistan. 2009 Jul OBJECTIVE: To study the clinical characteristics of Stills disease in a tertiary care hospital of Pakistan and compare it with similar published studies. MATERIALS AND METHODS: (Retrospective descriptive study) Thirteen patients with Adult onset stills disease were identified by chart review of last ten years from 1995 to 2005 at Aga Khan University Hospital (AKU), a tertiary care private medical university in Karachi Pakistan. Demographic and other specific information was recorded on standardized data sheet and analyzed by SPSS 11.5 software. RESULTS: Mean age of patients was 26.54 +/- 10.34 years, of which 8 (61.5%) were males. The most common presenting features were fever (100%), arthralgias and myalgias (100%) with large joint involvement (91.7%), significant weight loss (30.8%), sore throat (53.8%). None of the patients had skin rashes with fever, chest and abdominal pains. One patient had cervical lymph node swelling and 4 (33.3%) patients had splenomegally. The common laboratory findings were: leukocytosis and anaemia (100%), elevated ESR and C reactive protein (100%). Thrombocytosis (56.2%) and elevated Liver function tests (62%). Seven patients had serum ferritin checked which was in the range of 1872 to 16652 iu/l. None of the patients had positive ANA, Anti-DNA or RA factor. Twelve patients had a chest x-ray done, among them 11 were normal, one had pleural effusion. The clinical course of the patients who were followed for three months, was monocyclic (53.8%), polycyclic (30.8%) and persistent (15.4%). CONCLUSION: Clinical characteristics of Stills disease in our country are mostly similar to those seen in other regions, suggesting that same pathogenetic factors may be implicated in patients from different genetic back grounds and geographic locations.
20827446 Combined methotrexate and coenzyme Q₁₀ therapy in adjuvant-induced arthritis evaluated 2010 Rheumatoid arthritis is a common severe joint disease that affects all age groups, it is thus of great importance to develop new strategies for its treatment. The aim of the present study was to examine the combined effect of coenzyme Q₁₀ (CoQ₁₀) and methotrexate (MTX) on the progression of adjuvant-induced arthritis in rats. Adjuvant arthritis (AA) was induced by a single intradermal injection of heat-inactivated Mycobacterium butyricum in incomplete Freund's adjuvant. The experiments included healthy animals, arthritic animals not treated, arthritic animals treated with CoQ₁₀, with methotrexate, and with a combination of CoQ₁₀ and methotrexate. The two latter groups received a daily oral dose of 20 mg/kg b.w. of CoQ₁₀, either alone or with methotrexate in an oral dose of 0.3 mg/kg b.w. twice a week. We found that CoQ₁₀ potentiated both the antiarthritic (decrease of hind paw volume) and the antioxidant effect of methotrexate on the level of oxidation of proteins (suppression of protein carbonyl level in plasma) as well as lipoperoxidation (suppression of levels of HNE-adducts and MDA-adducts to plasma proteins). The same effect was observed for plasmatic levels of CoQ₉ and IL-1α, and partially also for γ-glutamyltransferase activity assessed in joints and spleen. Moreover, the combination therapy improved the functionality of peripheral blood neutrophils in AA, with a balancing effect on the immunosuppression caused by MTX monotherapy. In summary, combined administration of CoQ₁₀ and methotrexate suppressed arthritic progression in rats more effectively than did MTX alone. This finding may help improve treatment of rheumatoid arthritis.
20077403 Treatment of collagen-induced arthritis by Natura-alpha via regulation of Th-1/Th-17 respo 2010 Feb Cytokines and CD4(+) Th cells play a crucial role in the pathogenesis of rheumatoid arthritis. Among the Th populations, Th-1 and Th-17 have been described as pathogenic in collagen-induced arthritis (CIA) whereas Th-2 and Treg were found to have protective effects. The objective of this study was to examine the affect of Natura-alpha, a newly developed cytokine regulator, on CIA and on Th cell development. Natura-alpha treatment was administered before or during arthritis induction. Anti-type II collagen antibodies and cytokine expression were evaluated by ELISA. Emergence of CD4(+)CD25(+)Foxp3(+) T cells was assessed by flow cytometry. Th-17 differentiation of naive CD4 T cells was assessed in cultures with anti-CD3 and anti-CD28. We showed that Natura-alpha both prevented and treated CIA. We further demonstrated that in vivo treatment with Natura-alpha inhibited IL-17 production and anti-type II collagen IgG development. We showed in vitro, using an APC-free system, that Natura-alpha acted directly on differentiating T cells and inhibiting the formation of Th-1 and Th-17 cells but did not affect Th-2 cells. Since Natura-alpha inhibits a large spectrum of important pathogenic factors in CIA, it may provide a new and powerful approach to the treatment of rheumatoid arthritis and other inflammatory diseases.
20858143 Hereditary isolated metatarsophalangeal arthritis. 2011 Jan OBJECTIVE: To describe a family with 13 members in four generations affected by early-onset isolated painful arthritis limited to the first metatarsophalangeal (MTP) joint but without evidence of generalized joint disease at follow-up. METHODS: A complete family pedigree was constructed and radiographs from the affected family members and their offspring were taken. Laboratory tests including serum measurements of C-reactive protein (CRP), urate, and rheumatoid factor (RF) were performed to exclude gout and rheumatoid arthritis from the diagnosis. RESULTS: The age at onset of first MTP joint symptoms varied from 12 to 51 years. Both females and males were affected in the four successive generations, including male-to-male transmission as well as maternal inheritance. The affected patients were often treated surgically with good pain-relieving results. CONCLUSION: To our knowledge, this is the first report of early-onset isolated foot metatarsal arthritis with apparent autosomal dominant inheritance.
22493652 Septic arthritis in rheumatology: review of five cases. 2009 Aug This report illustrates five cases of patients admitted to medical ward in HUKM, diagnosed and treated as septic arthritis over the course of two months. Their age ranged from 32 to 67 years old with one patient had history of monoarticular pain and the other four had polyarticular pain. Two of these patients had pre-existing joint disease, namely gouty arthritis and rheumatoid arthritis, and another patient with background history of mixed connective tissue disease on long term steroid therapy. The diagnosis of septic arthritis was made mainly from clinical assessment, supported by synovial fluid assessment and blood investigations. All patients received minimum of two weeks intravenous antibiotic followed by one month course of antibiotic. All of them had arthrocentesis for diagnostic and therapeutic purposes and two had laparoscopic arthroscopy with wash out done. KEYWORDS: Septic arthritis; Arthrocentesis; Arthroscopy.