A case of Hughes-Stovin syndrome (incomplete Behçet's disease) with extensive arterial involvement : Unmasking the true face of a rare syndrome.

Hughes-Stovin syndrome (HSS), characterized by the combination of multiple pulmonary artery aneurysms and deep vein thrombosis, is a rare and an under-recognized clinical entity with less than 40 published cases in English medical literature. Vascular venous thrombotic events, as occurring in the course of Behçet's disease (BD), are also described in HSS, e. g., vena cava, intra-cardiac, jugular vein, iliac vein, femoral vein, and dural sinus thrombosis. We describe a 35-year-old man with HSS showing classical features of the syndrome in the form of recurrent thrombophlebitis of the lower limb veins, pulmonary arterial aneurysms, and left lower limb ischemia with extensive arterial tree involvement. The patient presented with critical arterial ischemia in the left lower limb together with aortic and left common iliac artery thrombosis, occlusion of the left superficial femoral artery, and occlusion of both lower limb arteries. Urgent vascular surgeries were carried out for limb salvage. Shortly after, the patient started on pulse corticosteroid/cyclophosphamide therapy, followed by monthly cyclophosphamide for 1 year, with much improvement. We discuss arterial involvement in HSS and similarities of HSS and BD regarding thrombotic events. We summarize the current management options of HSS.

Magnetic resonance imaging features of hip disorders in an Egyptian pediatric population.

Hip disorders in a pediatric population are a diagnostic challenge. The aim of the study is to assess the role of magnetic resonance imaging (MRI) in the evaluation of non-traumatic hip disorders in a series of Egyptian patients and to review the literature on the most common hip conditions. Seventy two consecutive patients [40 males (55.6%) and 32 females (44.4)] with acute onset of hip complaints unrelated to trauma or falls were recruited. All patients underwent an initial full clinical assessment and blood tests as well as contrast enhanced MRI of both hips. The most common diagnosis in this group of Egyptian patients was transient synovitis in 29 (40.3%) cases, followed by seronegative enthesopathy and arthropathy syndrome in 8 (11.1%), septic arthritis in 10 (13.9%), tuberculous arthritis in 4 (5.6%), sickle-cell disease in 7 (9.7%), complicated with septic arthritis in 3 (4.2%), transient bone marrow edema (BME) in 3 (4.2%), osteomyelitis in 2 (2.8%), osteosarcoma in 2 (2.8%), sciatic nerve injury in 1 (1.4%), leukemia with BME in 1 (1.4%), coxa vara of both hips and L5/S1 facet joint ankylosis in 1 (1.4%), and a benign bone cyst in 1 (1.4%). MRI studies showed hip effusion in a total of 51 patients (70.8%), joint space narrowing in 9 (12.5%), and BME in 15(20.8%). MRI is a sensitive tool for assessing hip disorders in a pediatric population and can play an important role in both diagnosis and management of different hip disorders, irrespective of the underlying pathology.

The association of fatigue, comorbidity burden, disease activity, disability and gross domestic product in patients with rheumatoid arthritis. Results from 34 countries participating in the Quest-RA program.

OBJECTIVES: The aim is to assess the prevalence of comorbidities and to further analyse to which degree fatigue can be explained by comorbidity burden, disease activity, disability and gross domestic product (GDP) in patients with rheumatoid arthritis (RA). METHODS: Nine thousands eight hundred seventy-four patients from 34 countries, 16 with high GDP (>24.000 US dollars [USD] per capita) and 18 low-GDP countries (<24.000 USD) participated in the Quantitative Standard monitoring of Patients with RA (QUEST-RA) study. The prevalence of 31 comorbid conditions, fatigue (0-10 cm visual analogue scale [VAS] [10=worst]), disease activity in 28 joints (DAS28), and physical disability (Health Assessment Questionnaire score [HAQ]) were assessed. Univariate and multivariate linear regression analyses were performed to assess the association between fatigue and comorbidities, disease activity, disability and GDP. RESULTS: Overall, patients reported a median of 2 comorbid conditions of which hypertension (31.5%), osteoporosis (17.6%), osteoarthritis (15.5%) and hyperlipidaemia (14.2%) were the most prevalent. The majority of comorbidities were more common in high-GDP countries. The median fatigue score was 4.4 (4.8 in low-GDP countries and 3.8 in high-GDP countries, p<0.001). In low-GDP countries 25.4% of the patients had a high level of fatigue (>6.6) compared with 23.0% in high-GDP countries (p<0.001). In univariate analysis, fatigue increased with increasing number of comorbidities, disease activity and disability in both high- and low-GDP countries. In multivariate analysis of all countries, these 3 variables explained 29.4% of the variability, whereas GDP was not significant. CONCLUSIONS: Fatigue is a widespread problem associated with high comorbidity burden, disease activity and disability regardless of GDP.

Fibromyalgia: Increased reactivity of the muscle membrane and a role of central regulation.

OBJECTIVE: Fibromyalgia (FM) is characterized by widespread muscle pain and central neural deregulation. Previous studies showed increased muscle fiber conduction velocity (CV) in non-painful muscles of FM patients. This study investigates the relationship between central activation and the CV in FM. METHODS: Twenty-two females with primary FM and 21 controls underwent surface electromyography of the non-painful biceps brachii. Mean CVs were calculated from the motor unit potential velocities (CV-MUPs), and the CV-MUPs' statistical distributions were presented as histograms. The amount of muscle activity (average rectified voltage, ARV) was measured. RESULTS: The CV was higher in the FM-group than in the controls (P = 0.021), with CV-MUPs generally shifted to higher values, indicative of increased muscle membrane propagation speeds. The largest increase in the CV of the FM-group occurred when adopting and maintaining a limb position at only 5% of maximum strength (P < 0.001); the CV did not, as normal, increase with greater force. However, the ARV in both groups similarly increased with force. CONCLUSIONS: In fibromyalgia patients, the muscle membrane propagation speed increases independently of the force load or amount of muscle activity produced. When adopting a limb position, the patients show an augmented muscle membrane reaction, suggesting deregulation from higher neural centers. SIGNIFICANCE: These findings contribute to understanding fibromyalgia.

[Cardiovascular and gastrointestinal risks associated with selective and non-selective NSAIDs]. / Cardiovasculaire en gastro-intestinale risico's bij selectieve en niet-selectieve NSAID's.

There has been much discussion regarding the cardiovascular and gastrointestinal safety of traditional and COX-2 selective NSAIDs. The national and international guidelines differ in their recommendations. Selective COX-2 inhibitors seem to have a diminished risk for severe gastrointestinal complications in the short-term, but the long-term benefit has not yet been proven. In various studies, COX-2 selective NSAIDs have been associated with an increased risk of cardiovascular complications. This connection has been clearly demonstrated only for rofecoxib. Celecoxib seems to lead to an increased risk only at high dosages. However, more patients will have to be followed for a longer period to confirm these results. There is insufficient evidence that the COX-2 selective agents lead to more frequent cardiovascular complications than the traditional NSAIDs. In patients with an increased risk of gastrointestinal complications and no cardiovascular risk, there is no preference for either COX-2 selective NSAIDs or the combination of traditional NSAIDs and a proton pump inhibitor. If dyspepsia develops during the use of a traditional NSAID, then it seems more effective to add a proton-pump inhibitor to the traditional NSAID rather than replacing it by a COX-2 selective NSAID.

Successfully living with chronic arthritis. The role of the allied health professionals.

The treatment and care of patients with rheumatoid arthritis (RA) is complex and various health professionals with different areas of expertise may be involved. The objective of this article is to review the treatments and their efficacy as provided by health care professionals in RA care. The requirements for further research in this area are formulated. To achieve better effects of treatment it is necessary to improve the coordination of services as provided by the different specialists. The important roles of the patients themselves in the care and management of the disease are emphasized, as well as the roles of the informal caregivers such as a spouse or other family members and friends and the role of patient societies. The possible role of the International Classification of Functioning, Disability and Health (ICF) to improve the communication and facilitate the coordination among health professionals and between patients and health professionals is mentioned. The topics presented in this article may encourage further discussion and research, particularly concerning the effects of the treatments as provided by allied health professionals. Health professionals play an important role in the life of patients with rheumatic disorders, in all the domains of the ICF: body functions and structure, activities (action by an individual) and participation (involvement in a life situation). Health professionals in rheumatology can make the difference in the lives of RA patients and their families.

Some experience with 57Co-labeled bleomycin as a tumor-seeking agent.

Bleomycin labeled with 57Co was used as a tumor-localizing agent in 132 patients. In patients with pulmonary tumors the primary localization concentrated radioactivity in 52 of the 54 appropriate cases; out of the 22 clinically known metastases, 19 were visible on the scan; 40 unknown metastases especially in hilus and mediastinum were found by the method and subsequently confirmed. In 22 patients with malignant lymphomas, 18 out of 22 known pathologic lymph glands above the diaphragm were visible on the scan; below the diaphragm the results of scanning in lymph glands and spleen were disappointing, probably because of the disturbing concentration of radioactivity in the kidneys, the bladder, the liver, and sometimes the gut. In 25 patients with various other tumors, 16 out of 22 known localizations above the diaphragm were visible; 2 were uncertain and 4 negative. Below the diaphragm the results were usually negative. In 24 patients with benign lesions, uptake of 57Co-bleomycin was visible on the scintigram in 4 patients with cavitating pulmonary tuberculosis, in 2 with pulmonary infections, in 1 with Caplan lesions of rheumatoid arthritis in the lung, and in 1 with sinusitis ethmoidalis. The significance of these results is discussed.

Treating arthritis with locally applied heat or cold.

The scientific for the treatment of arthritis with locally applied heat or cold is reviewed. Experimental studies in vitro, in animals, in healthy subjects, and in patients are considered. Results of investigations of the effects of locally applied heat or cold on the deeper tissues of joints and on joint temperature in patients are not consistent. In general, locally applied heat increases and locally applied cold decreases the temperature of the skin, superficial and deeper tissues, and joint cavity. Most studies dealing with the effects of heat and cold on pain, joint stiffness, grip strength, and joint function in inflamed joints report beneficial effects. In vitro studies show that higher temperatures increase the breakdown of articular cartilage and tissues that contain collagen. Therefore, one goal of physical therapy should be to decrease intraarticular temperature in actively inflamed arthritic joints.

Group education for rheumatoid arthritis patients.

This article reviews the effectiveness of group education programs in improving the knowledge, behavior, and health status of patients with rheumatoid arthritis (RA) and evaluates to what extent various programs fulfill certain criteria for educational self-management programs. Thirty-one studies are reviewed: in 12, patients with various rheumatic diseases including RA were included, and in 19, only RA patients were studied. Group education increased the knowledge of the participants, which was maintained over long intervals. Beneficial behavioral effects were found in mixed populations but less often found in RA patients. Group education often improved physical health status both in mixed and in RA populations, but seldom led to improved psychosocial health status. In general, the beneficial effects of group education were found more often in mixed populations than in strictly RA patients. Further investigations must examine which mechanisms make educational interventions effective and determine the types of interventions or combinations of interventions that are effective. Effects of group education on health status are almost never maintained over long intervals. More research is needed to develop strategies for maintaining and enhancing early gains from group education.

Epidemiological and clinical aspects relating to the variability of rheumatoid arthritis.

OBJECTIVE: To review epidemiological studies dealing with the temporal and geographic variability in the occurrence of rheumatoid arthritis (RA) and clinical studies that address the variability of severity and manifestations among populations. METHODS: An extensive search of the literature, including a Medline search, was completed. Studies addressing the origin, history, and trends in the occurrence of RA were reviewed first. Next, studies of the prevalence and incidence of RA in different populations were reviewed, and occurrence rates compared. Standardization was attempted by tabulating adult prevalence rates of studies using equivalent sets of criteria. Studies comparing RA patients from two populations were sought next. Finally, studies dealing with explanations of the presumed variability were reviewed. RESULTS: Temporal variability is indicated by paleopathological evidence that RA has existed in the New World since 4000 BC, whereas there is no evidence that it occurred in Europe before the 17th century, or in Africa before the 20th century. Epidemiological studies show a possible trend of decreasing incidence of RA in the United States and Western Europe, whereas reports from Africa note a rising incidence. In white populations of Europe and America, prevalence is approximately 1%, and incidence is 0.03%. Significantly higher rates are found in some North American Indians, and significantly lower rates in some Asian and African populations, even when the different population structures are taken into account. In the latter populations, different patterns of occurrence from those observed in whites emerge, such as greater female preponderance and a much younger peak age at onset. Direct standardized comparisons of two diverse populations of RA patients showed some differences in expression, severity, or manifestations of RA between populations. CONCLUSION: The occurrence and manifestations of RA are temporally and geographically variable.

Media coverage of chronic diseases in the Netherlands.

OBJECTIVE: Little is known about the quantity or quality of information on rheumatic diseases provided by the mass media. The aim of this study was to gain insight into the media coverage of rheumatic diseases compared with other chronic diseases in the Netherlands. MATERIALS AND METHODS: Newspaper articles, magazine articles, and medical television programs that appeared or were broadcast during a 1-year period, and contained information on rheumatic diseases, heart disease, cancer, chronic lung disease, or diabetes mellitus, were selected for content analysis. For each article and program, it was determined whether coverage concentrated on treatment, influence of lifestyle, scientific progress, or disease consequences. It was also determined whether professional experts and patients were featured. RESULTS: Nine hundred seventeen newspaper articles, 304 magazine articles, and 163 medical programs were found. Most dealt with cancer (43%) and heart disease (37%). The amount of media attention given to each of the five disease categories was found to correspond with mortality but not with prevalence. The contents of the articles and programs differed significantly according to disease topic. The main focus in rheumatic diseases was on patients' experiences, as well as regular and alternative medications. In heart disease and cancer, the main focus was on professional medical viewpoints, operations, and mortality, whereas in chronic lung disease and diabetes it was on treatments in the context of regular medications, scientific progress, and incurability. The influence of lifestyle on the disease process was mentioned most often in connection with diabetes, rheumatic disease, and chronic lung disease. CONCLUSIONS: The amount of attention a disease category received from the media depended on its fatality rates and not on its prevalence. Heart disease and cancer were portrayed as being more serious than the more lingering diseases. Surprisingly, the proportion of articles and programs that included the influence of lifestyle in their coverage was lowest for cancer and heart disease. More frequent and more accurate coverage of chronic diseases, especially rheumatic diseases, is needed if their image is to be brought into line with their importance for and impact on the community.

Perceptions about perceived functional disabilities and pain of people with rheumatoid arthritis: differences between patients and their spouses and correlates with well-being.

OBJECTIVE: In this study we examined the differences in perceptions of the patient's health status between rheumatoid arthritis (RA) patients and their spouses, and correlates of these differences with patients' and spouses' well-being. METHODS: A sample of 188 couples with one member receiving treatment for RA were selected from the rheumatology clinics in Twente, The Netherlands. The mean age of both RA patients and spouses was 56 years. Respondents completed questionnaires, including estimations of both patients and spouses on the patient's functional disabilities and pain, and scales on affect and marital commitment for patients and spouses. RESULTS: Differences in estimations of patients and spouses were considerable. Both over- and underestimations of the patient's functional disabilities by the spouse were associated with the patient's poorer mental health status. Overestimations of the patient's functional disabilities were associated with poorer mental health among spouses. CONCLUSION: It is essential that any support intended by the spouse is in accordance with the patient's needs. If the patient's condition is misperceived by the spouse, this can lead to ineffective and inappropriate support being given.

Coordinated individual education with an arthritis passport for patients with rheumatoid arthritis.

OBJECTIVE: To evaluate the effects of a program for individual education in combination with the use of an arthritis passport. METHODS: We studied 3 groups of patients with rheumatoid arthritis. The first experimental group received educational materials and followed the program under the guidance of their regular providers of health care whose activities were coordinated through arthritis passports. The second experimental group only received education materials; the control group received only usual care. RESULTS: There were no effects on self-efficacy expectations, knowledge, health status, or behavior in either experimental group. Opinions of general practitioners, physiotherapists, and visiting nurses concerning the arthritis passport were very positive, but rheumatologists' opinions were not. Opinions on coordination of care were more positive among the physiotherapists of the experimental group. CONCLUSIONS: Individual education for patients with rheumatoid arthritis by health care providers during routine consultations, as implemented in this program, had no effect on self-efficacy expectations, knowledge, health status, or health behavior. Likewise, the distribution of educational materials by itself without the involvement of health care providers had no effect. The use of the arthritis passport improved coordination of care, especially for physiotherapists.

Patient education and disease activity: a study among rheumatoid arthritis patients.

OBJECTIVE: To determine whether patients experiencing high disease activity derive more benefit from patient education than those experiencing low disease activity. METHODS: Data from a randomized study on the effects of a program of patient education were analyzed retrospectively. Four subgroups were studied: the high disease activity subgroup of patients who had participated in the educational program, the complementary low disease activity subgroup, the high disease activity subgroup of controls, and its low disease activity complement. Patients with erythrocyte sedimentation rate > 28 mm/first hour were classified as having high disease activity. Effects on frequency of physical exercises, endurance exercises, and relaxation exercises and effects on health status (Modified Health Assessment Questionnaire, Dutch Arthritis Impact Measurement Scales [AIMS]) were measured. RESULTS: There were no significant differences between the adherence parameters of the various pairs of groups. Four months after the educational program began, anxiety and depression scores on the Dutch-AIMS had increased among participating patients who were experiencing high disease activity and decreased among those who were experiencing low disease activity. CONCLUSIONS: Patients experiencing high disease activity did not derive more benefit from patient education than those experiencing low disease activity. On the contrary, an increase of anxiety and depression is found in these patients. Further study is needed to confirm our findings.

Lack of correlation between the mean tender point score and self-reported pain in fibromyalgia.

OBJECTIVES: To study the validity and nature of self-assessed symptoms among patients with fibromyalgia syndrome (FMS) and to compare our data with findings reported in the US. To determine whether tender point scores correlate with self-reported pain and other symptoms and to study the influence of disease duration. METHODS: Tender point scores were assessed in 113 consecutive patients with FMS. All patients completed 2 self-assessment questionnaires (an extended Campbell list, the Enschede Fibromyalgia Questionnaire, and the Dutch Arthritis Impact Measurement Scales). RESULTS: The self-assessed symptoms of the Dutch FMS patients seem to be valid and are comparable with those of American patients. No association between disease duration and number of self-reported symptoms was found. An association between self-reported pain and mean tender point score was lacking for patients with disease of shorter duration and was weak for patients with disease of longer duration. CONCLUSIONS: The use of a self-report questionnaire for patients with FMS is feasible and appears to be valid. Tender point scores and self-reported pain represent very different aspects of pain in FMS.

Patient education for adults with rheumatoid arthritis.

BACKGROUND: Because of the unpredictability people with arthritis face on a daily basis, patient education programmes have become an effective complement to traditional medical treatment giving people with arthritis the strategies and the tools necessary to make daily decisions to cope with the disease. OBJECTIVES: To assess the effectiveness of patient education interventions on health status in patients with rheumatoid arthritis. SEARCH STRATEGY: We searched MEDLINE, EMBASE and PsycINFO and the Cochrane Controlled Trials Register. A selection of review articles (see references) were examined to identify further relevant publications. There was no language restriction. SELECTION CRITERIA: Randomised controlled trials (RCT's) evaluating patient education interventions that included an instructional component and a non-intervention control group; pre- and post-test results available separately for RA, either in the publication or from the studies' authors; and study results presented in full, end-of-study report. MAIN RESULTS: Thirty-one studies with relevant data were included. We found significant effects of patient education at first follow-up for scores on disability, joint counts, patient global assessment, psychological status, and depression. A trend favouring patient education was found for scores on pain. Physician global assessment was not assessed in any of the included studies. The dimensions of anxiety and disease activity showed no significant effects. At final follow up no significant effects of patient education were found, although there was a trend favouring patient education for scores on disability. REVIEWER'S CONCLUSIONS: Patient education as provided in the studies reviewed here had small short-term effects on disability, joint counts, patient global assessment, psychological status and depression. There was no evidence of long-term benefits in adults with rheumatoid arthritis.

Patient education for adults with rheumatoid arthritis.

BACKGROUND: Because of the unpredictability people with arthritis face on a daily basis, patient education programmes have become an effective complement to traditional medical treatment giving people with arthritis the strategies and the tools necessary to make daily decisions to cope with the disease. OBJECTIVES: To assess the effectiveness of patient education interventions on health status in patients with rheumatoid arthritis. SEARCH STRATEGY: We searched MEDLINE, EMBASE and PsycINFO and the Cochrane Controlled Trials Register. A selection of review articles (see references) were examined to identify further relevant publications. There was no language restriction. SELECTION CRITERIA: Randomised controlled trials (RCT's) evaluating patient education interventions that included an instructional component and a non-intervention control group; pre- and post-test results available separately for RA, either in the publication or from the studies' authors; and study results presented in full, end-of-study report. MAIN RESULTS: Twenty-four studies with relevant data were included. We found significant effects of patient education at first follow-up for scores on disability, joint counts, patient global assessment and psychological status. Physician global assessment was not assessed in any of the included studies. The two separate dimensions of psychological status: anxiety and depression showed no significant effects, nor did the dimensions of pain and disease activity. At final follow up no significant effects of patient education were found. REVIEWER'S CONCLUSIONS: Patient education as provided in the studies reviewed here had moderate short-term effects on patient global assessment, and small short-term effects on disability, joint counts and psychological status. There were no long-term benefits.

Patient education for adults with rheumatoid arthritis.

BACKGROUND: Because of the unpredictability people with arthritis face on a daily basis, patient education programmes have become an effective complement to traditional medical treatment giving people with arthritis the strategies and the tools necessary to make daily decisions to cope with the disease. OBJECTIVES: To assess the effectiveness of patient education interventions on health status in patients with rheumatoid arthritis. SEARCH STRATEGY: We searched MEDLINE, EMBASE and PsycINFO and the Cochrane Controlled Trials Register. A selection of review articles (see references) were examined to identify further relevant publications. There was no language restriction. SELECTION CRITERIA: Randomised controlled trials (RCT's) evaluating patient education interventions that included an instructional component and a non-intervention control group; pre- and post-test results available separately for RA, either in the publication or from the studies' authors; and study results presented in full, end-of-study report. MAIN RESULTS: Twenty-four studies with relevant data were included. We found significant effects of patient education at first follow-up for scores on disability, joint counts, patient global assessment and psychological status. Physician global assessment was not assessed in any of the included studies. The two separate dimensions of psychological status: anxiety and depression showed no significant effects, nor did the dimensions of pain and disease activity. At final follow up no significant effects of patient education were found. REVIEWER'S CONCLUSIONS: Patient education as provided in the studies reviewed here had moderate short-term effects on patient global assessment, and small short-term effects on disability, joint counts and psychological status. There were no long-term benefits.