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ID PMID Title PublicationDate abstract
9539328 A case of Farber lipogranulomatosis. 1998 Feb A 35 month old girl had suffered from painful joint contractures of the whole body since a few months after birth, and she gradually developed numerous periarticular and subcutaneous nodules, hoarseness, swallowing difficulty with recurrent respiratory infections, nystagmus, and mental and developmental retardation. She was misdiagnosed as having juvenile rheumatoid arthritis at several university hospitals. Serologic studies for rheumatoid arthritis were all negative. Radiologic findings of the whole body showed osteoporosis and bony erosions; on brain CT the brain was diffusely atrophied. On cine-esophagography barium refluxed into the nasopharynx. Light microscopically, the reticular dermis and subcutis were markedly thickened with hyalinized sclerotic collagen bundles. There were interstitial and perivascular aggregates of foamy histiocytes which were positive for CD-68 immunostaining. On electron microscopy, foamy histiocytes were packed with numerous membrane-bound inclusions having C-shaped or worm-like profiles in addition to many myelin figures, occasional lipid droplets and rare banana-like bodies.
10048358 Polyethylene wear and calcar osteolysis. 1999 Jan We have examined retrospectively the correlation between linear wear of the polyethylene socket and calcar height loss (osteolysis) in 266 patients with 410 total hip arthroplasties who were followed up for a minimum of 10 years. A positive correlation existed between wear and osteolysis. The correlation was stronger in patients with osteoarthrosis. Simultaneous bilateral hip arthroplasty showed no difference in the relationship between polyethylene wear and calcar height loss when comparing the right and left hip. There was poor correlation between calcar height loss and wear in patients with rheumatoid arthritis. Our findings support the theory that femoral osteolysis in the total hip arthroplasty is related to the amount of liberated particles generated by the wear of components. The poor correlation found in cases with rheumatoid arthritis could be related to suppression of the immune response in these patients.
24383631 Prevalence of cervical lesions in rheumatoid arthritis: cross-sectional study on 263 patie 2000 Dec Abstract Spinal lesions in upper and sublaxilar cervical vertebrae were studied radiologically in 263 patients (25 men and 238 women) with rheumatoid arthritis (RA). Their average age was 58.9 years, and their disease duration was ranged from 6 months to 24 years (mean 13 years). Functional lateral views of the cervical spine were made. Atlantaxial subluxation (AAS) and vertical subluxation (VS) were evaluated as upper cervical lesions. Subaxilar subluxation (SAS) and endplate erosion were evaluated as subaxilar cervical lesions. One hundred and seventy-eight (67.7%) of the patients had a cervical lesion. Upper and subaxilar cervical abnormalities were recognized in 136 (51.7%) and 113 (43.0%) patients, respectively. There was no linkage between upper and subaxilar cervical lesions. While the prevalence of these lesions increased with time, the frequency was found to be over 50% within only 5 years from onset in patients with mutilating deformity. This prevalence tended to be associated with disease activity.
10910631 X-linked agammaglobulinemia presenting as juvenile chronic arthritis: report of one case. 1999 Jul Bruton agammaglobulinemia (X-linked agammaglobulinemia, XLA), transmitted by X-linked recessive inheritance, affects only males. Twenty percent of patients with XLA may have arthritis. Septic arthritis may occur, but there is also a form of arthritis that is similar to rheumatoid arthritis or juvenile chronic arthritis. Here we report one case of XLA in a boy with non-erosive chronic right knee arthritis. There was no evidence of septic arthritis. Regular intravenous gammaglobulin replacement therapy and oral naproxen resulted in dramatic improvement in the arthritis. This case illustrates that XLA should be considered as a possible underlying cause of juvenile chronic arthritis in males.
10717011 Etanercept in children with polyarticular juvenile rheumatoid arthritis. Pediatric Rheumat 2000 Mar 16 BACKGROUND: We evaluated the safety and efficacy of etanercept, a soluble tumor necrosis factor receptor (p75):Fc fusion protein, in children with polyarticular juvenile rheumatoid arthritis who did not tolerate or had an inadequate response to methotrexate. METHODS: Patients 4 to 17 years old received 0.4 mg of etanercept per kilogram of body weight subcutaneously twice weekly for up to three months in the initial, open-label part of a multicenter trial. Those who responded to treatment then entered a double-blind study and were randomly assigned to receive either placebo or etanercept for four months or until a flare of the disease occurred. A response was defined as an improvement of 30 percent or more in at least three of six indicators of disease activity, with no more than one indicator worsening by more than 30 percent. RESULTS: At the end of the open-label study, 51 of the 69 patients (74 percent) had had responses to etanercept treatment. In the double-blind study, 21 of the 26 patients who received placebo (81 percent) withdrew because of disease flare, as compared with 7 of the 25 patients who received etanercept (28 percent) (P=0.003). The median time to disease flare with placebo was 28 days, as compared with more than 116 days with etanercept (P<0.001). In the double-blind study, there were no significant differences between the two treatment groups in the frequency of adverse events. CONCLUSIONS: Treatment with etanercept leads to significant improvement in patients with active polyarticular juvenile rheumatoid arthritis. Etanercept is well tolerated by pediatric patients.
11469516 New criteria for diagnosing Sjögren's syndrome: a step forward?--or.. 2001 Primary Sjögren's syndrome seems to be the most common of the chronic systemic inflammatory connective tissue diseases, according to epidemiological investigations. Basic and clinical research, including treatment within this group, is increasing while simultaneously there is increasing confusion and uncertainty about the classification criteria for Sjögren's syndrome. Within the last three decades nine different classification criteria sets have been introduced including the recent US-European classification criteria set. The diagnostic criteria used in daily practice seem to differ even more and many forget that the function of both the lachrymal glands and the salivary glands should be diminished. Consequently many rheumatologists prefer to diagnose patients according to classification criteria. The advantages and disadvantages of the various classification criteria for primary Sjögren's syndrome are dealt with but it is stressed that the majority of these will exclude former/present smokers (from the diagnoses)--more than half of the population!
11433768 [A case of primary Sjögren's syndrome with acute transverse myelopathy and polyneuropathy 2001 Jan We report a case of primary Sjögren's syndrome with acute transverse myelopathy and polyneuropathy as the initial manifestations. A 72-year-old man developed acute right hemiparesis and his symptoms deteriorated to quadriparesis in four days. A cervical spinal MRI showed an extensive intraparenchymal lesion with high T2-weighted signal intensity, gadolinium enhancement, and cord swelling. An electromyographic study and a sural nerve biopsy showed severe axonal degeneration. The patient complained of thirst and a salivary gland biopsy revealed inflammatory changes, while salivary gland scintigraphy showed diminished secretion. These findings led to a diagnosis of Sjögren's syndrome. Only one case of Sjögren's syndrome with acute transverse myelopathy as the initial manifestation has been reported. In our case, however, polyneuropathy was observed in addition to transverse myelopathy as the initial manifestation. Even if prominent sicca symptoms are absent, all patients with acute transverse myelopathy and polyneuropathy should be carefully examined with considerations of possible presence of Sjögren's syndrome.
10855361 [Gougerot-Sjögren syndrome disclosed by MALT lymphoma of the salivary glands. Report of 3 2000 Mar Sjögren's syndrome is characterized by an increased risk of developing non-Hodgkin's lymphoma. The lymphoma is most frequently extra-nodal, preferentially affecting the salivary gland: low-grade MALT lymphoma. Conversely, underlying Sjögren's syndrome has been recently identified by some authors in patients with non-Hodgkin's lymphoma. In the present report, we present three cases of Sjögren's syndrome disclosed by low-grade salivary gland MALT lymphoma. The patients were all women aged 33, 38 and 52 years. Extension work-up revealed nodal and bone marrow involvement in one case and no evidence of disseminated disease in the two others. Using the European criteria, all of our patients had certain Sjögren's syndrome. Labial salivary gland biopsy and immunopathological studies in newly diagnosed low-grade MALT lymphoma would be helpful in identifying the real frequency of this association.
11277183 Intense immunosuppression followed by purified blood CD34+ cell autografting in a patient 2001 Feb A 15-year-old boy with refractory juvenile rheumatoid arthritis (JRA) underwent intense immunosuppressive therapy followed by purified blood CD34+ cell autografting. He had been taking prednisolone (PDN) daily or every other day combined with methotrexate once a week to control the disease for 7 years. He suffered from psychological complications and a very short stature due to the adverse effects of these drugs. CD34+ cells were purified in bulk from G-CSF-mobilized PBSC using an Isolex 300. After the administration of cyclophosphamide (200 mg/kg) and anti-lymphocyte globulin (45 mg/kg), 3.6 x 10(6)/kg purified CD34+ cells were infused. His post-transplant course was uneventful except for herpes-zoster infection. He is now more than 1 year post transplant and has not taken any immunosuppressive medication. His rate of growth has increased (>10 cm/year) due to the effects of the cessation of PDN and the administration of recombinant human growth hormone (rGH), in contrast to the gain of 2 cm in the preceding 3 years with rGH treatment. Although the durability of this remission is unknown, intense immunosuppressive therapy followed by purified blood CD34+ cell autografting might be acceptable for adolescent patients with refractory JRA to achieve a drug-free period for physical and psychological maturation.
10566567 Pharmacokinetics of etodolac in patients with stable juvenile rheumatoid arthritis. 1999 Oct This was a single-center, open-label, single-dose pharmacokinetic study of etodolac in pediatric and adolescent patients with stable juvenile rheumatoid arthritis (JRA). Eleven male and female patients with JRA (8.1 to 14.8 years of age, weighing 26.4 to 59.5 kg) received a single oral dose of etodolac (200, 300, or 400 mg based on body weight). Clinical laboratory measurements, measurement of vital signs, and physical examinations were performed to monitor safety. Concentrations of etodolac were determined in plasma using high-performance liquid chromatography with ultraviolet detection with a limit of quantitation of 0.2 mg/L and were analyzed using a noncompartmental pharmacokinetic method. Pharmacokinetic parameters observed were consistent in magnitude and degree of variability with data from healthy adult subjects receiving a single 400- or 600-mg dose of etodolac. Although the mean fraction of unbound drug in patients with JRA was higher than in healthy adults, the oral clearance was independent of age. No serious adverse events occurred during this study. Etodolac yielded consistent pharmacokinetic values among stratified dose subgroups. Single doses of all etodolac treatments were well tolerated in both pediatric and adolescent patients.
9086476 The presence of secretoneurin in human synovium and synovial fluid. 1997 Mar 14 Secretoneurin is a neuropeptide formed from the proprotein secretogranin II. It is found in afferent nerve fibres and has chemotactic activity for monocytes, neutrophils and fibroblasts. We investigated the presence of secretoneurin in synovial fluid and synovium from patients with osteoarthritis and rheumatoid arthritis. The secretoneurin immunoreactive material found in synovial fluid was identified by high performance liquid chromatography as the free peptide secretoneurin. Its level in hip joints was 15.6, in knee joints of osteoarthritis patients 17.3 and in rheumatoid patients significantly lower (8.6 fmol/ml). Immunocytochemistry provided evidence for the presence of sub-intimal secretoneurin-immunoreactive nerve fibres in knee synovium in osteoarthritic patients. In rheumatoid synovium, only very few immunoreactive fibres were found these being mostly localised in deep stroma. The results show that secretoneurin is present in osteoarthritic joint and suggest that secretoneurin levels are down-regulated in rheumatoid joint. Therefore, secretoneurin may participate in acute or mild phases of inflammation but is unlikely to have a major role when more severe inflammation is present such as that seen in rheumatoid joint.
11469517 Monitoring the disease activity. 2001 Primary Sjögren's syndrome is a chronic systemic rheumatic disease characterized as an autoimmune exocrinopathy or autoimmune epithelitis thereby suggesting a pathogenesis leading to tissue specific autoimmune lesion. The development of internationally approved criteria for the classification and diagnosis of Sjögren's syndrome has been a major scientific task for nearly two decades with consensus now approaching. In contrast, an international dialogue on how to develop additional common criteria for the assessment of disease activity, organ damage and outcome in Sjögren's syndrome has just recently been initiated. Such assessment criteria would provide useful measures for patient management and are mandatory for comparing efficacy between different clinical trials. The lack of common assessment criteria may be explained by missing uniform diagnostic criteria, by the multispeciality and systemic nature of the disease and the difficulties in separating out what is activity and what is damage in Sjögren's syndrome. Attempts are now made to overcome these problems. The purpose of this paper is to give a brief introduction to the concepts of disease activity, damage and outcome in Sjogren's syndrome with reference to the results obtained from a recent workshop on assessment tools in Sjögren's syndrome held in Oxford, England in March 2000.
11126657 [Evaluation of the histologic criteria for the diagnosis of Sjögren's syndrome]. 1999 Apr OBJECTIVE: The most important diagnostic criterion in Sjögren's syndrome (SS) is considered to be the histologic focus score of the labial salivary glands. The focus score is defined as the number of lymphocytic foci per 4 mm2 of the salivary gland according to the criterion of Chisholm & Mason. On the other hand, in the criteria of the Sjögren's Disease Research Committee of the Ministry of Health and Welfare in Japan it is defined as the number of lymphocytic foci per a lobule of the salivary gland. By setting the limited criteria for SS on the basis of objective signs of both dry eyes and dry mouth, we compared the usefulness of these two diagnostic criteria for the diagnosis of SS in terms of the sensitivity, the specificity and laboratory data. METHODS: The biopsy of labial salivary glands was performed in 245 patients (230 females and 15 males, with a mean age of 54.9 years) who were suspected of SS in our hospital during the time between 1975 and 1996. Labial salivary glands were histologically assessed and the focus score was calculated according to the criterion of Chisholm & Mason and to that of the Sjögren's Disease Research Committee, respectively. RESULTS: The average area per a lobule of the salivary gland was 0.70 mm2. According to the limited criteria for SS, the Japanese histologic diagnostic criteria showed a higher specificity (93.3%) and a lower sensitivity (23.5%). The sensitivity of the criterion of Chisholm & Mason was 72.1%, and the specificity was 80.0%. The margin of the lobule was sometimes difficult to be identified because of the fatty change and fibrosis in some salivary glands. CONCLUSIONS: By comparing the two different histologic criteria using our limited criteria, it was better to use the histologic criterion of Chisholm & Mason as a criterion for the diagnosis of SS than that of the Sjogren's Disease Research Committee of the Ministry of Health and Welfare in Japan in terms of the sensitivity, the specificity and laboratory data.
9141270 The clinical features of Sjögren's syndrome in Japanese children. 1997 Apr Sjögren's syndrome (SS) is thought to be uncommon in children. An epidemiological study to describe the clinical features distinguishing SS in Japanese children was performed by sending questionnaires to hospitals. A total of 61 cases of SS were reported from 1290 hospitals. The diagnosis of SS was based on histopathological changes and/or sialographic changes in the salivary glands. Forty-two cases had primary SS and 19 were secondary SS with other autoimmune disorders. Fourteen cases (65%) of secondary SS were associated with systemic lupus erythematosus. In primary SS, the initial symptoms were systemic manifestations (fever, exanthema, arthralgia, etc) except for sicca symptoms. In laboratory studies, antinuclear antibodies, elevated serum IgG, rheumatoid factor, anti-Ro/SS-B antibodies were frequently observed.
10453557 [The value of salivary beta 2 microglobulin concentration for diagnosis of Sjögren's synd 1997 Feb In this paper we measured salivary beta 2 microglobulin (beta 2 m) concentration in 40 patients with Sjögren's syndrome, including 20 patients with systemic lupus erythematosus, 12 patients with recurrent aphthous ulceration and 16 patients with single xerostomia, and in 40 normal controls. The results showed that salivary beta 2 m concentration was more significantly elevated in Sjögren's syndrome patients, compared with that in both normals and other patients. The measurement of beta 2 m concentration in saliva may offer a simple and valuable method for diagnosis of Sjögren's syndrome.
11084952 Neuroendocrine manifestations in Sjögren's syndrome. 2000 Nov Molecular biology has had a major impact on our concepts of the immune system and its relation to neuroendocrine axes, in particular, the adrenal, gonadal, and thyroid axes. It is now well established that not only are the biosynthetic and catabolic pathways of glucocorticoids and sex hormones (estrogen, progesterone, and testosterone) closely related but that the receptors for these hormones are part of a supergene family of receptors which include (in addition to these hormone receptors) the mineralocorticoid receptor, thyroid hormone receptor, retinoic acid receptors, and vitamin D receptors. This suggests a complex network of steroid hormones and receptors for the control and integration of a multitude of physiologic functions at a systemic level. The immune system seems to be tightly integrated into this homeostatic neuroendocrine regulatory network. The neurophysiologic and biochemical events that promote successful adaptation during stressful situations are now identified for illnesses that seem to occur as a result of or are associated with dysregulation of the stress response. One difficulty in interpreting the mechanisms of HPA axis dysfunction in autoimmune-inflammatory syndromes arises from the plasticity of the hormonal systems involved. Levels of hormones produced and receptors reset rapidly with changes in the hormonal milieu (deficiency or excess) and have likely changed during the course of the chronic immune disorder. This, in turn, is further confounded by the pleomorphic natural history of most autoimmune-inflammatory diseases such as SS. The levels of sex hormones and their receptors are tightly linked to HPA axis function. It may be that significant changes in the estrogen-to-androgen ratio or the ratio of their receptors alter the activity of steroid-sensitive cells such as the individual immune cells or epithelial cells, thus providing a means for endocrine regulation of the immune response in SS. Studies in the closely related disorder RA support this hypothesis. Taken together, adrenal and gonadal steroid hormone deficiency plus elevated PRL levels probably greatly facilitate cellular immunity in SS patients. This hypothesis in SS is supported by a growing body of data indicating that RA develops as a consequence of a deficiency in adrenal and gonadal steroid hormone production. It is noteworthy that the findings in female SS patients indicated a central deficiency in all three neuroendocrine axes: adrenal, gonadal, and thyroid. At present, it is not clear if any one system plays a primary role in the expression of the disease. Rather, it is likely that the net effect involves the synergistic and antagonistic effects of multiple hormones, making the specific effects of individual hormones difficult to discern.
9782808 Disease and family contributors to adaptation in juvenile rheumatoid arthritis and juvenil 1998 Jun OBJECTIVE: Research in the areas of pediatric rheumatology and pediatric chronic illness has emphasized comprehensive models of adaptation involving risk and resistance factors. This study examined adaptation, within this framework, among a large sample of children with chronic illness and children without chronic illness. METHODS: A comprehensive battery of adaptation measures was administered to a sample of 107 children with juvenile rheumatoid arthritis, 114 children with insulin-dependent diabetes mellitus, and 88 healthy controls. RESULTS: Medical diagnosis was associated with mothers' depression and a composite measure of parental (mother and father) distress and passive coping. Children's emotional and behavioral functioning was not related to medical diagnosis, but mothers' depression and parental distress were associated with child behavior problems. CONCLUSION: Because parental distress was associated with child functioning, interventions to ameliorate parental distress may have beneficial effects on the children's behavior and on parents' reactions to their children.
10470519 Recognition and treatment of arthritis in children. 1999 Jun Characteristics and differential diagnosis of juvenile rheumatoid arthritis (JRA) and diagnostic challenge of distinguishing it from juvenile spondyloarthropathy are reviewed. Although most children with limb pain do not have JRA, a reasonable approach is offered for those afflicted.
9632085 Sleep fragmentation in children with juvenile rheumatoid arthritis. 1998 Jun OBJECTIVE: To characterize sleep patterns of patients with juvenile rheumatoid arthritis (JRA). METHODS: Sixteen patients with JRA aged 12+/-4 years and 9 controls aged 11+/-3 years underwent a comprehensive evaluation by self-report questionnaire and formal all night polysomnographic recordings. Multiple sleep latency test was performed in 7 patients. RESULTS: Patients had 90% more arousals and awakenings (p<0.01) and the median length of occurrences of uninterrupted sleep in stages 2 and 3 and rapid eye movement (REM) sleep was 60% shorter than in controls (p<0.01). The overall amount of sleep stage shift from deeper to lighter sleep was 23.5+/-10.8 events in patients compared to 14.9+/-4.0 in controls (p<0.05). In 15 of 16 patients 15% of non-REM sleep consisted of alpha-delta (alpha-rating) sleep, compared with less than 1% in controls (p<0.001). Multiple sleep latency test for patients was 10.3+/-2.6 min. There were no differences between JRA and controls in self-reported questions. However, patients reported longer afternoon naps, 1.8+/-1.3 h compared to 0.3+/-0.8 h in controls (p<0.05). CONCLUSION: Objective polysomnographic evidence of abnormal sleep has been confirmed in patients with JRA. Sleep disturbance was associated with daytime sleepiness as evidenced by abnormal multiple sleep latency test and longer afternoon naptime.
11511763 Changes in the incidence of juvenile rheumatoid arthritis in Finland. 2001 Aug OBJECTIVE: To study trends in the incidence of juvenile rheumatoid arthritis (JRA). METHODS: The study covered subjects who were entitled under the nation-wide sickness insurance scheme to receive specially reimbursed medication for juvenile rheumatic diseases in 11 of 21 central hospital districts in Finland (the base population comprised about 445,000 children <16 yr of age) in 1995. Data from the years 1980, 1985 and 1990 were compared with data from 1995 concerning the central part of the area, which had been included in a previous study by us. RESULTS: A total of 87 incident cases (58 girls and 29 boys) satisfied criteria for JRA in 1995 in the study area. The incidence of JRA was 19.5 per 100 000 [95% confidence interval (CI) 15.6-24.1] of the population <16 yr of age for the whole area. It was 22.7 per 100,000 (95% CI 17.3-29.2) for the area that had been covered by the earlier study (five districts) and 14.9 per 100,000 (95% CI 9.8-21.7) for the new area (six additional districts). The incidence of JRA was significantly higher than in the earlier years (1980, 1985 and 1990) in the same district (trend, P=0.024). The highest incidence, 60.3 per 100,000 (95% CI 35.8-95.4), was noted in 1995 among girls in the age group 10-15 yr in the southernmost part of the study area. CONCLUSIONS: There was both temporal and regional variation in the incidence of JRA. Results of the present study suggest that environmental factors may influence the frequency of JRA.