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.

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.

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.

Prompt remission of severe SLE with only three doses of rituximab infusion and low dose steroid: the first case report from Indonesia.

A 31-year-old Chinese lady presented with severe SLE with nephrotic syndrome, anemia, leucopenia and thrombocytopenia, skin lesions, and joint inflammation after failing previous standard therapy. After treatment with three infusions of rituximab she showed immediate improvements regarding clinical and laboratory parameters. She received no cytotoxic drugs and remained well for at least 7 months, despite stopping prednisolone.

Cost-effectiveness of Spa treatment for fibromyalgia: general health improvement is not for free.

OBJECTIVES: To estimate the cost-effectiveness of an adjuvant treatment course of spa treatment compared with usual care only in patients with fibromyalgia syndrome (FM). METHODS: 134 patients with FM, selected from a rheumatology outpatient department and from members of the Dutch FM patient association were randomly assigned to a 2(1/2) week spa treatment course in Tunisia or to usual care only. Results are expressed as quality-adjusted life years (QALYs) for a 6-month as well as a 12-month time horizon. Utilities were derived form the Short Form 6D (SF-6D) scores and the visual analogue scale (VAS) rating general health. Costs were reported from societal perspective. Mean incremental cost per patient and the incremental cost utility ratio (ICER) were calculated; 95% confidence intervals (CIs) were estimated using double-sided bootstrapping. RESULTS: The data of 128 (55 spa and 73 controls) of the 134 patients (96%) could be used for analysis. Improvement in general health was found in the spa group until 6 months of follow-up by both the SF-6D (AUC 0.32 vs 0.30, P < 0.05) and the VAS (AUC 0.23 vs 0.19, P < 0.01). After 1yr no significant between-group differences were found. Mean incremental cost of spa treatment was 1311 Euro per patient (95% CI 369-2439), equalling the cost of the intervention (thalassotherapy including airfare and lodging), or 885 Euro per patient based on a more realistic cost estimate. CONCLUSIONS: The temporary improvement in quality of life due to an adjuvant treatment course of spa therapy for patients with FM is associated with limited incremental costs per patient.

[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.

Validation of a Dutch translation of the fibromyalgia impact questionnaire.

OBJECTIVES: To validate a Dutch translation of the fibromyalgia impact questionnaire (FIQ). MATERIALS AND METHODS: Data were taken from two randomized clinical trials on Spa treatment and venlafaxine in fibromyalgia (FM). Participants completed the Dutch FIQ and a set of validated questionnaires for general health (RAND-36), depression (Beck depression inventory, BDI), pain (McGill pain questionnaire, MPQ) and fatigue (checklist individual strength, CIS). Internal consistency within the FIQ item 'physical functioning' was studied using Cronbach's alpha. Test-retest reliability was studied with intra-class-correlation (ICC) in a subsample of 76 control subjects over a 3 month period without specific intervention. Construct validity was evaluated by correlating the FIQ to other questionnaires. Sensitivity to change was studied using standardized response means (SRM). RESULTS: The study sample consisted of 213 women and 11 men (mean age 47 yrs, mean disease duration 11 yrs). Cronbach's alpha for the item 'physical functioning' was 0.91, indicating high internal consistency. Test-retest reliability was acceptable, with ICC ranging from 0.45 for 'morning tiredness' to 0.71 for 'physical function'. FIQ correlated significantly with the RAND-36, with Spearman's rho ranging from -0.60 to -0.70 for items measuring the same concept. Similar patterns of correlation were seen with MPQ, BDI and CIS. Sensitivity to change was sufficient, with SRM after Spa treatment ranging from 0.3 for 'work days missed' to 0.9 for 'days felt good'. Similar SRM were found in the venlafaxine trial for patients reporting general improvement. CONCLUSION: The Dutch FIQ is a valid instrument for measuring health status in FM, showing sufficient reliability, construct validity and responsiveness.

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.

Spa treatment for primary fibromyalgia syndrome: a combination of thalassotherapy, exercise and patient education improves symptoms and quality of life.

OBJECTIVES: To study the effect of a combination of thalassotherapy, exercise and patient education in people with fibromyalgia. METHODS: Patients with fibromyalgia, selected from a rheumatology out-patient department and from members of the Dutch fibromyalgia patient association, were pre-randomized to receive either 2(1/2) weeks of treatment in a Tunisian spa resort, including thalassotherapy, supervised exercise and group education (active treatment) or treatment as usual (control treatment). Primary outcome measure was health-related quality of life, measured with the RAND-36 questionnaire. Secondary measures included the Fibromyalgia Impact Questionnaire, the McGill Pain Questionnaire, the Beck Depression Inventory, tender point score and a 6-min treadmill walk test. RESULTS: Fifty-eight participants receiving the active treatment reported significant improvement on RAND-36 physical and mental component summary scales. For physical health, differences from the 76 controls were statistically significant after 3 months, but not after 6 and 12 months. A similar pattern of temporary improvement was seen in the self-reported secondary measures. Tender point scores and treadmill walk tests improved more after active treatment, but did not reach significant between-group differences, except for walk tests after 12 months. CONCLUSIONS: A combination of thalassotherapy, exercise and patient education may temporarily improve fibromyalgia symptoms and health-related quality of life.

Sensitivity to change of AIMS2 and AIMS2-SF components in comparison to M-HAQ and VAS-pain.

OBJECTIVE: To examine sensitivity to change of Dutch versions of AIMS2 (arthritis impact measurement scales-2) and AIMS2-SF (short form) components, in comparison with M-HAQ (modified health assessment questionnaire) and the 100 mm visual analogue scale for pain (VAS-pain) in patients with rheumatoid arthritis. METHODS: 218 patients participated in a study on patient education. Participants completed the Dutch AIMS2, M-HAQ, and VAS-pain at baseline and after one year; 165 completed both assessments. The education programme did not have any effect on health status. Patients were classified according to change over one year in their responses to the AIMS2 question about general health perception: improved health (n = 32), no change (n = 101), and poorer health (n = 32). Changes in scores over one year were tested with paired t tests, and standardised response means were calculated for AIMS2 and AIMS2-SF components, M-HAQ total score, and VAS-pain in the three classifications of change in health perception. RESULTS: AIMS2 and AIMS2-SF physical, symptom, and affect components showed similar sensitivity to change. The physical and symptom components performed better than M-HAQ and VAS-pain. AIMS2 and AIMS2-SF social interaction and role components were not sensitive to changes in general health perception. The role component was only applicable in 63 patients, because the others were unemployed, disabled, or retired. CONCLUSIONS: AIMS2-SF is a good alternative to the AIMS2 long form for the assessment of health status in rheumatoid arthritis, and is preferable to M-HAQ and VAS-pain. Use of the AIMS2-SF makes it easier and less costly to collect data and reduces the burden on patients.

A system of networks and continuing education for physical therapists in rheumatology: a feasibility study.

PURPOSE: To evaluate the feasibility of regional physical therapy networks including continuing education in rheumatology. The aim of these networks was to improve care provided by primary care physical therapists by improving specific knowledge, technical and communicative skills and the collaboration with rheumatologists. METHODS: In two regions in The Netherlands continuing education (CE) programmes, consisting of a 5-day postgraduate training course followed by bimonthly workshops and teaching practices, were organised simultaneously. Network activities included consultations, newsletters and the development of a communication guideline. Endpoint measures included the participation rate, compliance, quality of the CE programme, teaching practices, knowledge, network activities, communication, number of patients treated and patient satisfaction. RESULTS: Sixty-three physical therapists out of 193 practices (33%) participated in the project. They all completed the education programmes and were formally registered. All evaluations of the education programmes showed positive scores. Knowledge scores increased significantly directly after the training course and at 18 months. A draft guideline on communication between physical therapists and rheumatologists was developed, and 4 newsletters were distributed. A substantial proportion of physical therapists and rheumatologists reported improved communication at 18 months. The mean number of patients treated by physical therapists participating in the networks increased significantly. Patients' satisfaction scores within the networks were significantly higher than those from outside the networks at 18 months. CONCLUSIONS: Setting up a system of networks for continuing education for physical therapists regarding the treatment of patients with rheumatic diseases is feasible. Further research will focus on the effectiveness of the system and its implementation on a larger scale.

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.

Fibromyalgia and the therapeutic domain. A philosophical study on the origins of fibromyalgia in a specific social setting.

OBJECTIVES: Fibromyalgia has always attracted controversy. Wolfe states that fibromyalgia will always exist regardless of the name given to the syndrome. Hadler describes fibromyalgia as a form of illness behaviour escalated by labelling. However, we believe that fibromyalgia, as other functional somatic syndromes, is not waiting below the surface until it becomes manifest by labelling. METHODS: We developed our hypothesis on the relationship between a specific social setting (called the therapeutic domain) and fibromyalgia using empirical philosophical arguments based on Foucault and Hacking. A therapeutic domain is a real and heterogeneous medical domain in which people, their thoughts and practices, and medical technology in any form coexist and communicate. In this domain blood is aspirated, radiographs are taken and classification criteria are made and applied. It is a domain where patient and therapist have initiated a relationship, which is influenced by the media and political pressure. This results in a looping effect where classification criteria and images give structure to perceptions and form the description for human behaviour; the person thus diagnosed (!) constantly has to grow into the conformity of these classification criteria, which also have to be constantly revised. The fibromyalgia concept becomes manifest in an individual as non-specific aches and pains along with other features. RESULTS: In other times and settings this resulted in analogue syndromes like railway spine, telegraph wrists, neurocirculatory asthenia or perhaps repetitive strain injury. In the application of American College of Rheumatology fibromyalgia classification criteria, labels and medical technology it is possible that invisible experiences manifest themselves in a therapeutic domain. It is not only a phenotype induced by the physician, but in this domain a certain power creates reality making the 'disease' become manifest. CONCLUSION: The only certainty in fibromyalgia is that it is still being diagnosed. For prevention and treatment of fibromyalgia, doctors as well as politicians and media have to start by fundamentally changing the therapeutic domain. In such a renewed setting, fibromyalgia cannot become manifest in an individual and thus fibromyalgia syndrome can no longer exist. A firm public message that symptoms can be psychological in origin to prevent their spread, as Wessely recently stated in the comparable case of mass psychogenic illness, is only a part of the answer.

Psychometric properties of a Dutch short form of the Arthritis Impact Measurement Scales 2 (Dutch-AIMS2-SF).

OBJECTIVE: To evaluate the reliability and validity of a Dutch version of the Arthritis Impact Measurement Scales 2 short form (AIMS2-SF) and examine the agreement between the AIMS2 and AIMS2-SF in rheumatoid arthritis (RA) patients. METHODS: Data were collected from 587 RA patients from three studies. Patients completed the Dutch-AIMS2, Modified Health Assessment Questionnaire (M-HAQ), and Visual Analogue Scale for pain (VAS-pain), and clinical data were collected to calculate the Disease Activity Score 28 (DAS28). Short-form component scores were calculated from the AIMS2 long-form data. In addition, a Modified Symptom component score was calculated by replacing item 42 with item 38 as was suggested by Haavardsholm et al. [7] for the Norwegian version. RESULTS: The internal consistency of the Physical, Symptom and Affect components was good (Cronbach's alpha= 0.75-0.87), moderate for the Role component (alpha=0.62) but rather low for the Social Interaction (0.51) component. Replacing item 33 with item 31 of the long-form AIMS2 increased internal consistency for the Social Interaction component to 0.63. Test-retest reliability of the AIMS2-SF components was high (intraclass correlation coefficients >0.70). Mean scores of the AIMS2-SF were generally close to those from the AIMS2, but the limits of agreement were rather wide. Both the Modified Symptom and Modified Social Interaction components showed better agreement than the original short-form components. Plots of differences between AIMS2 and AIMS2-SF against the mean of the two scores for the five components showed that the differences varied over the range of the measurements. Factor analysis confirmed the three-factor structure, with a physical, psychological and social dimension that has been found for the Dutch-AIMS2 long form. Correlations of the AIMS2-SF components with M-HAQ total score, functional class, VAS-pain and DAS28 were very similar to the correlations for the original AIMS2. CONCLUSION: The Dutch-AIMS2-SF, with Modified Symptom and Social Interaction components has good psychometric properties, similar to those of the Dutch-AIMS2 long form.