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ID PMID Title PublicationDate abstract
11915495 [Primary Gougerot-Sjögren syndrome in a 13-year-old girl]. 2002 Feb Sjögren's syndrome is uncommon in children, and occurs most often in association with autoimmune diseases (secondary Sjögren's syndrome). We describe the clinical and biological features of a 13-year-old girl with primary Sjögren's syndrome, revealed by recurrent parotitis. CASE REPORT: This adolescent girl was referred for investigation of multiple episodes of bilateral parotid swelling since age nine, without systemic symptoms. Examination was unremarkable except for enlarged and painless parotid glands. Laboratory investigations, measurement of saliva production, parotid sialography, labial salivary gland biopsy, revealed Sjögren's syndrome without associated disease. Hydroxychloroquine was prescribed with clinical improvement. CONCLUSION: Recurrent parotitis in children is an uncommon condition. The onset of parotid swelling at five years or over deserves screening for dysimmune disorders, sarcoidosis or Sjögren's syndrome. Diagnosis of Sjögren's syndrome is based on laboratory evidence of autoimmune disorders and minor salivary gland biopsy.
15039497 Adult onset Still's disease and collapsing glomerulopathy: successful treatment with intra 2004 Jun In this Grand Round we present a 32-yr-old African man who became severely ill after a 5-month history of weight loss, pyrexia, arthralgia, sweats and rash. He went on to develop pericarditis, pericardial effusion with tamponade, hepatomegaly with abnormal liver function tests, lymphadenopathy, massive proteinuria and required ventilatory, circulatory and renal support. The differential diagnosis was adult onset Still's disease, systemic lupus erythematosus (SLE), infection and lymphoma. Primary infection and lymphoma were excluded and he was treated, with dramatic success, with intravenous immunoglobulins (i.v.IG). Subsequent renal biopsy excluded SLE but confirmed collapsing glomerulopathy. The proteinuria improved dramatically following treatment with mycophenolate mofetil. We discuss some of the difficult diagnostic and management issues raised by this patient and the different uses and mechanisms of action of i.v.IG.
12486386 [Sjögren's syndrome in the elderly]. 2002 Oct Sjögren's syndrome is an immune exocrinopathy. This paper will deal with the primary Sjogren's syndrome non associated with another rheumatologic disease (polyarthritis or systemic lupus erythematosus). Its prevalence is claimed to increase with age. The main clinical manifestation is salivary and/or ocular dryness due to a lymphocytic infiltration of the salivary or lacrimal glands or both. However, sicca symptoms are very frequent in the elderly, most often iatrogenic. The problem, particularly in this population, is the diagnosis of Sjögren's syndrome. There are yet no consensual criteria, the most used in the literature are the European criteria, published in 1993 and revised in 1996. Some tests, such as the Schirmer's test and non stimulated salivary flow, are not specific over 60 years. The results of other tests are discussed as the focus score on the lip salivary glands biopsy. The diagnosis of Sjögren's syndrome should be envisaged in the elderly, when systemic manifestations are associated with sicca symptoms.
14565525 Extradural spinal cord compression by rheumatoid nodule. 2003 Jun Rheumatoid disease is a systemic disorder affecting multiple organs. It is known to affect the nervous system in a variety of ways, but its presentation with spinal cord compression by a rheumatoid nodule is rare. We report two cases presenting with cord compression by a rheumatoid nodule who underwent surgical decompression with good recovery.
12730284 Brief report: child-rearing practices of caregivers with and without a child with juvenile 2003 Jun OBJECTIVE: To evaluate predictions from professionals in pediatric rheumatology regarding the child-rearing practices of caregivers of children with juvenile rheumatoid arthritis (JRA) and healthy classmates. METHODS: Sixteen professionals identified items from the Child-Rearing Practices Report (CRPR) that were expected to differentiate between caregivers of children with JRA (64 mothers, 45 fathers) and caregivers of healthy classmates (64 mothers, 40 fathers). Families were interviewed, and physician ratings of disease severity were obtained. RESULTS: Experts predicted difficulties in protectiveness, discipline, and worry. Ratings from parents of children with JRA showed modest agreement with the professionals, surprising similarity to controls, and a limited association with disease factors. CONCLUSIONS: Contrary to expert opinion, JRA has only a modest influence on some child-rearing practices. Educating health care providers may minimize misperceptions about caring for children with JRA, and screening parents of children with more severe disease may assist in allocating education and services for families.
14613264 Prognostic factors in a large cohort of patients with early undifferentiated inflammatory 2003 Nov OBJECTIVE: Inflammatory arthritis of the hands is a frequent clinical presentation with a variable outcome. Patients not satisfying the classification criteria for recognized arthritides are described as having undifferentiated inflammatory arthritis, for which there are no accepted therapeutic algorithms. This study assessed the clinical outcome of patients with undifferentiated arthritis of the hands after use of a treatment algorithm, and evaluated the prognostic features in these patients. METHODS: One hundred consecutive patients with undifferentiated arthritis of the hands were assessed following use of a pragmatic treatment algorithm that was based on clinical presentation and response to treatment. The following standard step-up treatment protocol was used: 1) nonsteroidal antiinflammatory drugs (NSAIDs), 2) a single dose of corticosteroid administered by either intramuscular or intraarticular injection, and 3) disease-modifying antirheumatic drugs (DMARDs). Patients with specific rheumatologic diagnoses were excluded. The primary outcome was persistence of synovitis at 12 months. RESULTS: Seventy-eight percent of patients received NSAIDs, 72% received corticosteroids, and 30% received DMARD therapy. Among patients who had synovitis at 12 months, the prevalence of rheumatoid factor (RF) seropositivity, swollen joints, and synovitis at baseline was greater than in those without persistent synovitis. Logistic regression analysis showed baseline investigations to be poor predictors of subsequent DMARD use, with the best predictor being persistence of synovitis at 12 weeks. Rheumatoid arthritis (RA) developed in 14 patients. Logistic regression analysis showed that significant predictors of RA were RF seropositivity and the painful joint count at baseline. No patient who experienced resolution of synovitis by 12 weeks had persistent synovitis that subsequently required DMARD therapy. Only 13% of patients entered remission. Early resolution of synovitis was associated with an excellent prognosis. CONCLUSION: Undifferentiated arthritis of the hands is not a benign condition, with 30% of patients receiving DMARD therapy by 12 months and low remission rates. Results of the clinical assessment at 12 weeks is the single best predictor of future therapy. This study provides background data for use in determining future therapeutic interventions.
12747288 Occurrence of chronic inflammatory rheumatic diseases among parents of multiple offspring 2003 Mar OBJECTIVE: The rarity of reports on extended multiplex families points out that the genetic component in juvenile idiopathic arthritis (JIA) might not be particularly strong. Our objective was to determine the frequency of chronic inflammatory rheumatic diseases among the parents who had two or more offspring affected by JIA. METHODS: During the last 17 years patients with JIA treated at the Rheumatism Foundation Hospital in Heinola and their parents have been systematically asked about the familial occurrence of rheumatic diseases. A total of 45 families with more than one sibling affected by JIA were found among about 2,300 JIA cases. In these "multicase families", 9 parents from 8 families also had a diagnosis of chronic inflammatory rheumatic disease. Their case histories were studied. RESULTS: Four of the parents had had JIA (one subsequently developed ankylosing spondylitis), and 4 had rheumatoid factor-negative chronic arthritis (one had also had chronic iritis since the age of 10, resembling that seen in JIA). Three of them had features of JIA and only one met the classification criteria for rheumatoid arthritis. One had ankylosing spondylitis. CONCLUSIONS: Since the expected number of JIA cases among the 90 parents was about 0.2, there was drastic increase in JIA frequency among the parents in families with multiple offspring also affected by JIA. These results suggest that JIA susceptibility genes may likely be clustered in these families.
17041983 The role of social comparison in coping with rheumatoid arthritis: an interview study. 2004 OBJECTIVE: This study aimed to explore the effects of participants' perceptions of support, ways of coping and feelings of control, on their accounts of well-being. METHODS: Semi-structured interviews were conducted with 10 patients with rheumatoid arthritis (RA) and explored the dynamics of participants' accounts of coping with their everyday lives. RESULTS: Four categories of coping mechanisms were evident in all participants' accounts: support during the medical consultation, nurse support, social support, and comparative strategies. This paper focuses on just one of the themes that emerged from our analysis. Further insight into coping mechanisms was provided by the prevalence of comparative coping strategies. A comparison with previous disease states was used by every participant. This comparison enhanced their sense of control and fostered a sense of well-being. Social comparison was used by seven participants to show themselves how fortunate they were in relation to others with RA. CONCLUSION: Comparative coping has been identified as an important strategy adopted by these participants that could be explored in further intervention-based studies of coping.
17041967 An exploration of patients' experiences of anti-TNF therapy. 2004 OBJECTIVE: To explore the experience of rheumatoid arthritis patients receiving anti-tumour necrosis factor (anti-TNF) therapy on physical, psychological and social well-being. METHOD: Seven patients with rheumatoid arthritis (RA) who received six months of infliximab were interviewed in depth by one researcher to identify their experiences of this treatment. The interviews were tape-recorded and analysed using Colaizzi's procedural steps. RESULTS: Four main categories were identified from the interview relating to the patient's experience of anti-TNF therapy. These included: The impact of living with RA on physical, psychological and social function. The influence of anti-TNF therapy on physical, psychological and social function. The experience of attending for treatment. The impact of discontinuing anti-TNF treatment. CONCLUSION: All seven patients experienced benefits from receiving anti-TNF therapy. These benefits were not maintained once the treatment period was completed.
17041466 Macrophage activation syndrome in a patient with systemic onset rheumatoid arthritis: resc 2003 Aug We report the case of a 7-year-old boy with systemic onset juvenile rheumatoid arthritis (JRA) who developed macrophage activation syndrome during a period of relative remission. He presented with high-grade fever, enlarged lymph nodes, acute hepatitis, severe pancytopenia, and evidence of disseminated intravascular coagulopathy. He became severely ill, with persistent mucosal bleeding, hypovolemia, and vascular instability, and he was admitted to the intensive care unit. No response was observed to treatment with high-dose intravenous corticosteroids and blood product replacements, but the patient fully recovered after the completion of two pulses of intravenous immunoglobulin. This treatment modality may be an alternative to anti-TNF-alpha and cyclosporine A for a prompt response in JRA patients with this potentially fatal condition.
19807346 Usefulness of resource utilization estimates from piggyback studies in rheumatoid arthriti 2003 Dec Several methods to derive estimates of resource utilization for pharmacoeconomic evaluations exist. A common method is to collect this information alongside a clinical trial. A common criticism of this data collection strategy is that it may yield inaccurate results as the clinical trial protocol places many restrictions on the study design, patient enrolment and frequency of data collection, which may lead to an artificial picture of resource consumption and costs. In this special report, this problem is examined in rheumatoid arthritis patients. Data were collected from two sources: a Phase III clinical trial with piggyback resource utilization and a cross-sectional database utilizing retrospective recall of resource use. The patients from each database were matched in terms of age, gender and Health Assessment Questionnaire Disability Index and major measures of resource use were compared. The results of this study suggest that there were limitations in the use of patient-reported data to estimate resource utilization in this clinical trial.
17043441 Association of primary biliary cirrhosis and rheumatoid arthritis. 2003 Dec Primary biliary cirrhosis (PBC) and rheumatoid arthritis (RA) are chronic medical conditions in which, although the etiology is uncertain, autoimmune features predominate. These two conditions are, in fact, multisystemic diseases, with a multitude of manifestations that are not limited to their target end organ. It has been well established that patients with PBC may have musculoskeletal complaints and patients with RA may have evidence of hepatic dysfunction. Both of these conditions are fairly rare, with RA being found in approximately 1% of the population and PBC being found in approximately 20/100,000 women and 2/100,000 men.The likelihood of these uncommon conditions occurring in the same patient is very rare and unusual. We have identified a unique group of 25 patients who have met disease-defining criteria for both RA and PBC. Awareness of this combination should prompt rheumatologists to assess for PBC with antimitochondrial antibody testing in patients with abnormal liver tests and also to raise awareness that abnormal liver tests in RA patients are not always due to drugs.
12191678 Bronchiolitis obliterans organizing pneumonia in a patient with primary biliary cirrhosis 2002 Aug A 62-year-old woman with primary biliary cirrhosis (PBC) and rheumatoid arthritis (RA) was examined at our hospital for a 2-week history of non-resolving fever, cough and malaise. Her chest radiograph revealed left lower lobe opacity. Various kinds of antibiotics were not effective and transbronchial biopsy revealed non-specific inflammatory alveolar lesions. Chest radiograph at 14 days after admission revealed migration of the pulmonary opacity, suggesting bronchiolitis obliterans organizing pneumonia (BOOP), which responded well clinically and radiologically to oral corticosteroid therapy. BOOP may be one of the possible non-hepatic complications of PBC especially in patients associated with other connective tissue diseases.
24383895 Chemokines in synovial inflammation in rheumatoid arthritis: basic and clinical aspects. 2002 Jun Abstract  Rheumatoid arthritis (RA) is a chronic, multisystem autoimmune disease characterized by persistent synovitis. Since chemotactic cytokines (chemokines) may play critical roles in the recruitment of leukocytes in RA, analyses of chemokines and their receptors should provide insight into events in synovial inflammation in RA. The production of chemokines is regulated by cytokines such as tumor necrosis factor (TNF)-α produced in the inflamed joint, suggesting that the efficacy of anti-TNF-α therapy is mediated at least partly by the reduction of chemokine production. Chemokines have a role in joint inflammation not only by inducing leukocyte chemotaxis, but also by activating immune cells and angiogenesis. The pathogenesis of RA has been shown to be mediated by Th1-type T cells, because Th1-related chemokine receptors are preferentially expressed on cells in synovial fluid and synovial tissue. Accordingly, antichemokine therapy may be important as a possible new approach to therapeutic intervention in RA.
12529771 [Results of C1-C2 transarticular screw fixation in a series of 20 patients]. 2002 Dec OBJECTIVE: C1-C2 transarticular fixation is an increasingly used surgical method of treating atlantoaxial instability. When properly performed, it can safely provide fusion rates near 100%, but techniques of fixation in this region allow only a small margin of error. We here report the results of C1-C2 transarticular fixation in a series of 20 patients in which different procedures were selected according to the presenting disorder in each case. METHODS: The study group included 9 men and 11 women with a mean age of 48 years (range 17 to 68 years). The causes of instabilities were rheumatoid arthritis in nine patients, type II and III Hangman's fracture of traumatic origin in nine (in association with other lesions in three cases), pseudoarthrosis after type II odontoid fracture in one, and type III complex C1 fracture in a patient with ankylosing spondylitis. Preoperative assessment included flexion and extension plain radiographs and computed tomography (CT) and magnetic resonance imaging (MRI) scans. Operations included transarticular screw fixation in all cases; in patients with rheumatoid arthritis it was associated with sublaminar fixation and bone grafting following Sonntag's technique in all but two cases. Postoperative results were evaluated in relation to the biomechanical stability and fusion was studied by flexion and extension radiographs and CT scans. Pain relief in patients with rheumatoid arthritis patients was assessed using a 0-10 visual analogic scale (V.A.S.). RESULTS: In the traumatic group, a consolidation of the fracture and radiologic stability was achieved in all cases. In patients with rheumatoid arthritis, pain improved in all but not the neurological deficit, and in all cases a C1-C2 biomechanical stability was reached despite interlaminar graft resorption in two (25%) cases. With respect to complications, a lesion of the vertebral artery occurred in one case, deviation of the screw in two cases, and breakage of the screw without clinical repercussion in two other cases. CONCLUSION: C1-C2 transarticular screw fixation was a useful technique to achieve satisfacory biomechanical stability in patients with atlatoaxial instability of both inflammatory and traumatic origin with a low rate of complications.
17043499 Biologic therapies on the horizon for rheumatoid arthritis. 2004 Jun Recent research on rheumatoid arthritis (RA) has broadened our understanding of this disease, and mediators of the disease process have been identified as potential therapeutic targets. A series of novel agents have been developed specifically targeting these mediators and are collectively referred to as biologic response modifiers or biologic agents. A number of these agents have been introduced into clinical practice or are currently in clinical trials. Agents that have been approved include 2 monoclonal antibodies and a receptor fusion protein, all of which target tumor necrosis factor (TNF), as well as an inhibitor of interleukin-1 (IL-1). Other areas that are currently being investigated are new methods of blocking TNF and other cytokines, including IL-1 and IL-6. Emerging data also suggest that both T and B cells have critical roles in the pathogenesis of RA and could provide rational targets for therapy. Additionally, the role of adhesion molecules in RA continues to expand, and several agents have been proposed as potential therapies. These rapid advances in our understanding of RA and their implications for rationally targeted therapies are reviewed.
15632991 [Reduction of periodontoid masses following posterior arthrodesis: review of two new cases 2004 Dec Inflamatory periodontoid pannus is quite common in patients with rheumatoid arthritis. However, the occurrence of a pannus-like periodontoid mass that is unassociated with rheumatic inflammation is less frequent. Transoral surgery associated with a posterior stabilization has long been considered one of the most efficient methods to resolve the problem of instability in patients presenting neurological deficits secondary to the pannus. We present two cases of non rheumatic etiology, in which an occipito-cervical arthrodesis was used to resolve the proliferative lesion around the odontoid apophysis. Two women (67 and 60 years old respectively) presented symptoms of pain and neurological deterioration with an antecedent of possible past odontoid fracture in one case, and a previous large anterior cervical arthrodesis to correct spondylarthrosis in the other case. Both patient's neurological condition improved after placement of an arthrodesis through a posterior approach.
12476753 Overlap syndromes. 2002 Many connective tissue diseases share common signs and symptoms, which frequently makes the diagnosis of a specific rheumatic disease difficult. Rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, polymyositis, dermatomyositis (DM), mixed connective tissue disease, and Sjögren's syndrome can present with similar clinical features, particularly during the first 12 months of symptoms. Overall, a rheumatic disease can appears in conjunction with features of one or more other connective tissue diseases, for example, patients can have a combination of rheumatoid arthritis and systemic lupus erythematosus ("rhupus"), or systemic sclerosis and polymyositis, defining an "overlap syndrome", where the diseases comply with the diagnosis criterias. Finally, when a person has symptoms of various connective tissue diseases without meeting the full criteria for any one of them, it is often called Undifferentiated Connective Tissue Disease.
12403205 Ocular diseases in patients with rheumatic diseases. 2002 Aug To study the distribution of ocular involvement among persons with rheumatic disease, a cross-sectional survey was performed in 224 patients attending the Division of Rheumatology, Department of Medicine, Maharaj Nakorn Chiang Mai Hospital. Of these patients, 102 presented with rheumatoid arthritis, 74 systemic lupus erythematosus, 39 systemic sclerosis, 6 mixed connective tissue disease, 2 polymyositis and 1 juvenile rheumatoid arthritis. It was found that the ocular involvement probably related to diseases including dry eye (19.9%) and uveitis (0.4%). The ocular involvement was presumably related to treatment including retinopathy (7.6%), cataract (6.3%), and glaucoma (0.9%). Rapid recognition of these complications would lead to early and appropriate management, which would prevent their sequelae.
20704944 Drug targets in immunological diseases: Focus on rheumatoid arthritis. 2004 Dec Extract: There are a large number of diseases involving inappropriate activation of the immune system. These are often classified as immune-mediated diseases and include autoimmune diseases such as rheumatoid arthritis (RA), multiple sclerosis, and type I diabetes mellitus, allergic diseases such as asthma, and immune-mediated graft/organ rejection. RA is a chronic systemic inflammatory disease involving the destruction of articular cartilage and bone in joints. Despite the advances in our understanding of RA, the cause remains elusive. It is now recognized that there are two arms to the disease, namely an inflammatory arm and a destructive arm. The inflammation causes many of the symptoms of pain and swelling whereas the joint destruction is the major cause of long term disability associated with this disease. It is clear that the joint destruction occurs early in the course of the disease and mediators, such as cytokines (non-antibody proteins that act as intracellular mediators), are important in this process.