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ID PMID Title PublicationDate abstract
1603202 Primary Sjögren's syndrome: clinical spectrum and mode of presentation based on an analys 1992 Apr In this study we report on the clinical characteristics at the time of referral and during follow-up of 50 patients with primary Sjögren's syndrome (SS) in a rheumatological practice. The mean age of the patients at the time of diagnosis was 51.5 years. Four of them were men and 46 women. During a mean follow-up period of 9.1 yr (1-21 yr) from symptom onset the most common signs and symptoms were arthralgia (94%), complaints of dryness of the eyes (90%), mouth (82%) and skin (56%), weariness (64%), exanthema (48%), Raynaud's phenomenon (42%) and arthritis (42%). An elevated erythrocyte sedimentation rate (72%), elevated serum gammaglobulin (49%) and leucocytopenia (42%) were the most frequently encountered abnormalities of routine laboratory tests. After the initial hospital evaluation the diagnosis primary SS was made in only 10 (20%) of the patients. The diagnostic delay was on average 3.1 yr. Knowledge of the considerable heterogeneity of the disease may prevent extensive evaluations and provide better clinical care for patients with primary SS.
8934915 Sjogren's syndrome: clinical spectrum and current diagnostic controversies. 1996 Apr Although we have had a useful and internationally agreed-upon definition of Sjögren's syndrome (SS) for more than 30 years, we have not yet agreed upon criteria for diagnosing it. Understanding the clinical spectrum of SS and the various tests used to diagnose its components provides a basis for discussing controversies about diagnostic criteria. A review of the clinical features of SS and their diagnostic tests is followed by assessments of seven sets of diagnostic criteria and a proposal for future criteria. The various existing criteria use different combinations of clinical features and tests, which results in populations of different sizes and homogeneities being given the diagnosis of SS. Some criteria propose alternative tests in diagnosing components of SS, while others do not. Internationally accepted diagnostic criteria for SS that are as disease-specific as possible are needed for us to learn the epidemiology, pathogenesis, treatment, and prognosis of this clinically and scientifically important disease.
8002340 [Still's syndrome in the adult (adult-onset Still's disease). 2 case reports and review of 1994 Oct Adult-onset Still's disease (AOSD) is a rare syndrome of unknown origin consisting of recurrent fever, urticarial rash, arthralgias or arthritides, lymphadenopathia, splenomegalia and nonspecific sore throat. It is always linked with leucocytosis and negative blood cultures; rheumatic and antinuclear factors are mostly negative. Very high ferritin levels are frequent and could be a marker of the disease. We report on two cases and give a review of the literature.
7681711 GM-CSF and G-CSF in Felty's syndrome. 1993 Feb The risk of infection is increased in patients with Felty's syndrome, neutropenia being one of the main reasons for the susceptibility to infection. We report the case of a 56-year-old patient with Felty's syndrome in whom successive therapy with GM-CSF, splenectomy, and G-CSF was tried because of recurrent severe infections. Therapy with GM-CSF and G-CSF resulted in improvement of neutropenia and in successful treatment of cutaneous and pulmonary infections.
1496162 Salivary and oral components of Sjögren's syndrome. 1992 Aug The intraoral symptoms and signs of SS are not specific to SS, being shared with other conditions in which salivary function is diminished. The decrease in saliva causes chronic oral discomfort and functional problems and predisposes patients to dental caries and oral candidiasis. Many methods have been used to assess SGs in SS objectively, but at present a labial salivary gland biopsy specimen showing focal lymphocytic sialadenitis provides the best diagnostic criterion for the salivary component of SS, in terms of its disease specificity, convenience, availability, and low risk. The treatment of xerostomia in patients with SS consists of (1) preventing new and recurrent dental caries by frequent and regular application of topical fluoride, careful dental supervision, and avoidance of sucrose and other metabolizable carbohydrates between meals; (2) reducing oral symptoms by diagnosing and treating oral candidiasis (repeatedly if necessary); and (3) attempting to replace lost saliva by stimulating salivary secretion with physiologic sialogogues or pilocarpine, or if adequate amounts of saliva cannot be stimulated, using some form of saliva substitute, especially for patients wearing complete dentures.
8835240 Sjögren's syndrome: terminology. 1995 Nov In spite of our continuously improved pathobiological understanding, there is still no consensus on terminology and disease criteria in Sjögren's syndrome (SS). This survey points out discrepencies in the current description of the syndrome, and argues for a new classification model. We suggest that the present nomenclatures for the global disease (Sjögren's disease) and disease subsets (primary and secondary SS) be retained until additional pathobiological insights give rise to new and descriptive terms. We do find evidence, however, to support a new terminology and classification of the main immunoinflammatory manifestations of primary SS. Accordingly, three "exocrine" and four "non-exocrine" subgroups of disease manifestations are here defined. The usefulness of the proposed model should be evaluated in clinical studies and in a debate engaging all of the medical specialities involved.
1486743 Adult onset Still's disease: clinical features and course. 1992 Dec Fifteen patients with adult onset Still's disease are described, all diagnosed according to recognized criteria. Mean delay in reaching a firm diagnosis was 16 months. Besides the typical clinical picture, there was a high frequency of pruriginous rash, one instance of overlapping polymyositis and recurrent systemic manifestations in most cases. Chronic polyarticular involvement predominated, with radiological progression particularly in wrist, proximal interphalangeal and hip joints. However, functional prognosis at the end of a mean 4.8-year course was satisfactory, as also the response to treatment mainly with steroid drugs and, on occasion, with remitting agents to alleviate arthritis.
8687310 Feet and systemic disease. 1996 Jun Diabetes mellitus and rheumatoid arthritis often affect the foot. Indeed in either of these, the foot is commonly the initial site of symptoms. Early diagnosis appropriate treatment can prevent or at least slow the progression of the disease in the foot. Extra depth shoes and moulded inserts are the keystone of non operative treatment. Surgery is indicated when the deformity is severe. Surgery does have good results in rheumatoid arthritis but in diabetes mellitus it is usually reserved as a salvage procedure.
24441428 Rheumatoid arthritis and household work limitations in women. 1996 The purpose of this article is to examine the impact of rheumatoid arthritis (RA) on a woman's capacity to perform household work. Household work involves both instrumental functions (e.g., cooking, cleaning, shopping) and nurturant functions (e.g., caring for children, listening and comforting). This article includes a review of the relevant literature, an interview with a rheumatologist, and case studies of two women with RA. The literature suggests that household work limitations exist in women with RA, especially those women with more severe RA; and that the patient may benefit from appropriate social support and ongoing treatment and education.
1464876 Preliminary report of higher dose methotrexate treatment in juvenile rheumatoid arthritis. 1992 Oct Methotrexate (MTX) is widely used to treat juvenile rheumatoid arthritis (JRA). Although most patients respond to lower doses (0.15-0.5 mg/kg/wk), some patients have required higher doses of MTX to control their arthritis. Thirteen children were treated with MTX 0.82-1.1 mg/kg/wk for 2-26 months. Although all children initially responded (> 50% improvement in joint index, erythrocyte sedimentation rate, morning stiffness and global evaluation), 4 patients discontinued treatment because of side effects or lack of prolonged efficacy. Five patients have continued taking higher dose MTX for 4-26 months, while 4 other patients have been able to decrease their MTX dose and maintain improvement. Twenty-four hour MTX levels were done on all patients at initiation of higher dose MTX and all cleared MTX well. Our report suggests that MTX in doses of 0.82-1.1 mg/kg/wk can successfully treat active synovitis in some children with severe JRA with few short term toxicities. Longterm use at these doses has not been studied and thus its safety is not known.
24283638 Co-existence of Lambert-Eaton myasthenic syndrome and subacute cerebellar degeneration in 1995 Jul A 38 year old woman with rheumatoid arthritis had a rare co-existence of Lambert-Eaton myasthenic syndrome and subacute cerebellar degeneration. She had mild but transient improvement in muscle power following plasmapheresis, which correlated with the degree of increment on high rate repetitive nerve stimulation (RNS). Her cerebellar signs did not improve, however. This differential therapeutic response may be due to different mechanisms of injury and tissue susceptibility.
8333924 Autoimmune recognition of cartilage collagens. 1993 Jun Recent advances in basic research on the immune system and molecular biology of cartilage components have greatly increased our understanding of the role of autoimmunity in inflammatory diseases affecting joints, particularly rheumatoid arthritis. Many of these diseases are common and their complex pathogenesis probably involves a large number of genes polymorphic in the population as well as environmental factors. Characteristic features of inflammatory arthritis include expansion of the synovial tissue into a pannus containing lymphocytes and macrophages, autoimmune reactions against cartilage antigens, and erosion of cartilage. Since hyaline cartilage of the articular surfaces is the only structure within the joint known to contain joint-specific antigens this tissue is the prime suspect as the target of the autoimmune This review will first present the capacity of the immune system to discriminate between self and non-self structures, and then summarize our current understanding of the structures of cartilage collagens. Subsequently we will discuss how the immune system normally interacts with cartilage and how such interactions can lead to arthritis. We propose that collagen-induced arthritis (CIA) is valuable for understanding the autoimmune recognition of cartilage collagen which precedes the outbreak of arthritis and may perpetuate its chronicity, and serves as an animal model of rheumatoid arthritis.
7727553 An educational needs assessment of children with juvenile rheumatoid arthritis. 1994 Sep OBJECTIVE: Our objective is to describe the use of the PRECEDE model (predisposing, reinforcing, and enabling causes in educational diagnosis and evaluation) to organize needs assessment data in order to define self-management behaviors and plan an educational intervention for children with juvenile rheumatoid arthritis (JRA) and their families. METHODS: Analysis was done of needs assessment data collected from several sources: 1) literature review, 2) survey of parents of 51 children with JRA, 3) group interview of seven parents of children with JRA, 4) results of pilot programs, and 5) clinical experience of an interdisciplinary pediatric rheumatology team. RESULTS: Two sets of interrelated behavioral factors were identified through the needs assessment: 1) those related to managing the school environment to facilitate optimal participation and to minimize school-related disability, and 2) those related to treating pain and stiffness, intervening in the disease process, and preserving joint function. CONCLUSION: Both of these sets of behavioral factors may be related to the optimization of children's mobility, joint function, and autonomy of activities of daily living and should be targets of an educational intervention.
1642753 Slow bacterial infections or autoimmunity? 1992 May In this article, Graham Rook and John Stanford propose that a group of idiopathic diseases that are often associated with a degree of autoimmunity and arthritis, including rheumatoid arthritis, inflammatory bowel disease, sarcoidosis and psoriasis, are caused by extremely slow-growing bacteria. They suggest that these diseases are one end of a continuous spectrum caused by related slow-growing genera, which ranges from rheumatoid arthritis, through Takayasu's arteritis and Whipple's disease, to reach the conventional mycobacterioses such as tuberculosis and leprosy.
1390970 Physical fitness levels in children with polyarticular juvenile rheumatoid arthritis. 1992 Jun Children with juvenile rheumatoid arthritis (JRA) often exhibit fatigue and prolonged exercise recovery. Improved fitness through physical conditioning has not been a goal of standard medical or physical treatment regimens for JRA, and fitness levels of children with JRA have rarely been studied. We compared physical fitness in 20 6 to 11-year-old patients with polyarticular JRA with sex-, age-, and size-matched controls, using the Health Related Physical Fitness Test (HRPFT), a national, standardized, norm-referenced test. We correlated fitness scores with summary joint counts, and with an articular severity index (sum of joint swelling, tenderness, pain, and limited range for each child). The results showed that children with polyarticular JRA were less physically fit than normally active (noncompetitively athletic) children of the same sex, age, and size. There was no statistically significant relationship between increased joint counts, and/or disease severity scores, and reduced fitness scores. This suggests that physical fitness levels are less related to degree of "disease activity" than is often thought. We conclude that (1) a readily available, nationally standardized fitness test can be used to assess children with JRA: and (2) fitness levels and measures of disease activity do not correlate. We believe that multiple factors, perhaps including family, physician, and school concerns about potential disease exacerbation following exercise, may account for the low fitness levels observed in children with JRA.
8499740 [Immunomodulating therapy of systemic juvenile rheumatoid arthritis: administration of hig 1993 Apr Two children of 9 and 10 years suffering from severe systemic juvenile rheumatoid arthritis were treated intravenously with high-dose human immunoglobulin. Treatment was performed every 4 weeks for 7 and 18 months, respectively. Improvement of arthritic symptoms was demonstrable by significant decreases of Ritchie index and number of swollen joints and the disappearance of heated joints in one patient. The other patient was free of arthritic symptoms since the introduction of immunoglobulin therapy. Clinical symptoms of systemic illness were markedly improved and no relapse was seen. Laboratory parameters also improved, including erythrocyte sedimentation rate, C-reactive protein, hemoglobin, and serum iron levels. Parallel investigations of immunological parameters revealed a decrease of serum Il-1 beta and Il-6 levels and a diminished in vitro production of Il-1 beta, Il-6, and tumor necrosis factor-alpha. Therefore, we suggest a decreased activation status of the monocyte-macrophage system as one possible mode of action.
8557804 Viral arthritis: the lessons of parvovirus B19. 1995 Nov 15 Viruses are among the most common and least recognized causes of joint symptoms--including syndromes misclassified as seronegative rheumatoid arthritis. Parvovirus B19 is notably underappreciated in this role. Many cases resolve spontaneously. The challenge is to exclude nonviral causes of joint disease.
8129769 Enhanced synthesis of cysteinyl leukotrienes in juvenile rheumatoid arthritis. 1994 Jan OBJECTIVE: Endogenous synthesis of cysteinyl leukotrienes in juvenile rheumatoid arthritis (JRA) was investigated. METHODS: Cysteinyl leukotriene synthesis was assessed by measuring the excretion of leukotriene E4 (LTE4) in urine by radioimmunoassay. The identity of urinary LTE4 was investigated by gas chromatography-mass spectrometry (GC-MS), and 2,3-dinor-thromboxane B2 was measured with GC-MS. RESULTS: Excretion of LTE4 into urine was significantly (P < 0.05) enhanced in children with JRA compared with that in healthy children (n = 10). Aspirin, in a dosage of 2.5 gm/day, had no effect on urinary LTE4 levels, but it reduced urinary 2,3-dinorthromboxane B2 levels by more than 85% in healthy adults. There was a positive correlation between LTE4 excretion and the number of affected joints. CONCLUSION: This study demonstrates a markedly enhanced cysteinyl leukotriene synthesis and a positive correlation between LTE4 excretion and the number of affected joints in children with JRA.
8656140 A new model for classification of disease manifestations in primary Sjögren's syndrome: e 1996 Jun OBJECTIVES: The clinical features of 80 patients with primary Sjögren's syndrome (PSS) were revised in order to evaluate the descriptive and analytical facilities of a newly proposed model for classification of the exocrine and nonexocrine disease manifestations in PSS. DESIGN: Retrospective, long-term (median 7.5 years follow-up) observational, clinical study. SETTING: Patients were recruited from our Department, which is a tertiary referral centre for PSS patients. SUBJECTS: Eighty patients fulfilling the Copenhagen criteria for keratoconjunctivitis sicca and/or xerostomia and followed between 1972 and 1991 were studied. RESULTS: All patients had 'surface exocrine disease' and in 31% this was the only disease manifestation. 'Internal organ exocrine disease' was found in 25% of the patients, whilst 2.5% developed 'monoclonal B lymphocyte disease' (non-Hodgkin's lymphoma). 28% displayed 'inflammatory vascular disease', 25% 'noninflammatory vascular disease', 41% "mediator-induced disease' and 2.5% 'autoimmune endocrine disease' (thyroiditis). In patients with 'internal organ exocrine disease' the frequencies of "mediator-induced disease' (70%; P < 0.01) and 'inflammatory vascular disease, (50%; P < 0.03) were significantly higher than expected by chance. The level of immunoinflammatory activity (assessed by plasma IgG, serum ANA and focus scoring of minor labial salivary gland biopsies) correlated with the extent of clinical disease as assessed by the model. CONCLUSIONS: We conclude that this theoretically based model for classification of disease manifestations in PSS contains descriptive and analytic powers which may assist the clinical handling of these patients.
1556680 The iridocyclitis of early onset pauciarticular juvenile rheumatoid arthritis: outcome in 1992 Jan In a cohort of 72 patients with iridocyclitis (iritis) and early onset pauciarticular juvenile rheumatoid arthritis (EOPA-JRA) the course of the eye disease was matched with ocular outcome. Chronicity of inflammation (greater than 6 months/episode) was correlated with complications of eye disease that caused impairment of vision. HLA antigens in these patients were compared with the HLA antigens in a cohort of 77 patients with EOPA-JRA in whom iridocyclitis had failed to develop over a followup of 5 years or longer. HLA-DR5 (11) was correlated with the presence of eye disease, and HLA-DR1 with its absence; HLA-DRw8, which strongly predisposes to EOPA-JRA, was neutral with respect to eye disease.